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A 36-year-old man presents to his physician complaining of right scrotal swelling.

He states that the swelling has been present for 1 week. He


initially noticed the swelling shortly after moving furniture for his new living room.
He denies any nausea, vomiting, change in bowel habits,
abdominal pain, or urinary tract symptoms. He has no other significant medical or
surgical history. On examination, he has an enlarged right
hemi-scrotum with a mass that appears to be originating at the level of the
external inguinal ring. With the patient completely relaxed, the
physician is able to reduce the mass by pushing it back through the external
inguinal ring. With the mass reduced, the physician instructs the
patient to perform a Valsalva maneuver, upon which a protrusion is felt at the
external inguinal ring. Once the mass is reduced, the testicle
appears normal in size and consistency.
Question 1 of 4
Which of the following is the most likely diagnosis?
/ A. Hydrocele
/ B. Femoral hernia
/ C. Inguinal hernia
/ D. Testicular cancer
/ E. Varicocele

Explanation - Q: 1.1 Close

The correct answer is C. This patient has a reducible inguinal hernia. A
hernia is a protrusion of a structure, usually intestine, through tissue that
normally contains it. Inguinal hernias are either direct or indirect. Indirect
inguinal hernias occur through the internal inguinal ring in a protrusion of
peritoneum along the spermatic cord in the internal spermatic fascia. Direct
inguinal hernias occur through the floor of the inguinal canal, separate from
the spermatic cord as a result of breakdown of the transversus abdominis
aponeurosis and transversalis fascia. If the mass, i.e., hernia, is easily
returned back to its normal position, then it is called reducible. If the mass is
not reducible, then this is called incarcerated. And, if the mass becomes
incarcerated and develops compromised blood supply, it is termed
strangulated. Strangulated hernias require emergent repair because the
intestinal contents will necrose and cause the patient to become sick. As
long as the mass is reducible, surgical repair can be performed on an
outpatient basis.
A hydrocele (choice A) is a fluid collection contained within the tunica
vaginalis that surrounds the testicle. It presents as a painless swelling of the
scrotum, which transilluminates light when it is placed against the mass. A
hydrocele cannot be reduced.
Femoral hernias (choice B) are more common in women, presenting as
swellings in the upper part of the thigh. The neck of the hernia sac lies at the
femoral ring, below and lateral to the pubic tubercle, distinguishing these
from inguinal hernias, which are above and medial to the tubercle.
A mass caused by testicular cancer (choice D) cannot be reduced either.
Patients will complain of a dull, heavy, aching feeling within the testicle. The
testicle itself is usually enlarged, hard, irregular, and nontender.
A varicocele(choice E) is caused by dilatation of the pampiniform venous
plexus of the spermatic cord. It is typically described as feeling like "a bag of
worms" on physical examination. The dilatation is more easily appreciated
with the patient standing or during the Valsalva maneuver.

A 36-year-old man presents to his physician complaining of right scrotal swelling.
He states that the swelling has been present for 1 week. He
initially noticed the swelling shortly after moving furniture for his new living room.
He denies any nausea, vomiting, change in bowel habits,
abdominal pain, or urinary tract symptoms. He has no other significant medical or
surgical history. On examination, he has an enlarged right
hemi-scrotum with a mass that appears to be originating at the level of the
external inguinal ring. With the patient completely relaxed, the
physician is able to reduce the mass by pushing it back through the external
inguinal ring. With the mass reduced, the physician instructs the
patient to perform a Valsalva maneuver, upon which a protrusion is felt at the
external inguinal ring. Once the mass is reduced, the testicle
appears normal in size and consistency.
Question 2 of 4
Which of the following nerves travels along the spermatic cord within the inguinal
canal and may be damaged during a surgical procedure to
correct this patient's condition?
/ A. IIiohypogastric
/ B. IIioinguinal
/ C. Lateral femoral cutaneous
/ D. Obturator
/ E. Pudendal

Explanation - Q: 1.2 Close

The correct answer is B. Although it is now possible to perform inguinal
hernia repairs laparoscopically, the traditional approach is through an
incision over the inguinal canal. At the time of surgery, the inguinal canal is
opened via sharp dissection through the external oblique aponeurosis (the
anterior wall of the inguinal canal). The ilioinguinal nerve is then encountered
as it runs on the anterior aspect of the spermatic cord. If not properly
identified at time of surgery it is possible to transect the ilioinguinal nerve or
to "trap" it during closure. If the ilioinguinal nerve is transected or entrapped
in closure, the patient will complain of numbness over the nerve's
distribution, i.e., the upper medial aspect of the thigh and the anterior portion
of the scrotum on the affected side.
The iliohypogastric, lateral femoral cutaneous, obturator, and pudendal
nerves are not encountered during traditional inguinal hernia repair.
The iliohypogastric nerve(choice A) is derived from L1 (as is the ilioinguinal
nerve) and runs with the ilioinguinal nerve as they both pierce the
transversus abdominis muscle near the anterior superior iliac spine. They
then pass through the internal and external oblique muscles to supply the
skin of the suprapubic and inguinal regions and the abdominal musculature.
The iliohypogastric sends a lateral branch to the skin of the gluteal region
and then continues on, to pass through the superficial inguinal ring.
The lateral femoral cutaneous nerve (choice C) originates from L2 and L3
and is a direct branch of the lumbar plexus. It enters the thigh deep to the
lateral end of the inguinal ligament near the anterior superior iliac spine and
supplies the skin on the anterior and lateral aspects of the thigh.
The obturator nerve(choice D) is the nerve of the adductor muscles of the
thigh. It arises from the lumbar plexus (L2, L3, L4), enters the pelvis minor,
and then leaves the pelvis via the obturator foramen. The obturator nerve
also sends a small cutaneous branch to the medial aspect of the mid thigh.
The pudendal nerve (choice E) arises from the sacral plexus (S2, S3, S4),
accompanies the internal pudendal artery, and leaves the pelvis between the
piriformis and coccygeus muscles. The nerve hooks around the
sacrospinous ligament to enter the perineum through the lesser sciatic
foramen to supply the muscles of the perineum, including the external anal
sphincter, and then ends as the dorsal nerve of the penis or clitoris. It also
supplies some sensation to the external genitalia
A 36-year-old man presents to his physician complaining of right scrotal swelling.
He states that the swelling has been present for 1 week. He
initially noticed the swelling shortly after moving furniture for his new living room.
He denies any nausea, vomiting, change in bowel habits,
abdominal pain, or urinary tract symptoms. He has no other significant medical or
surgical history. On examination, he has an enlarged right
hemi-scrotum with a mass that appears to be originating at the level of the
external inguinal ring. With the patient completely relaxed, the
physician is able to reduce the mass by pushing it back through the external
inguinal ring. With the mass reduced, the physician instructs the
patient to perform a Valsalva maneuver, upon which a protrusion is felt at the
external inguinal ring. Once the mass is reduced, the testicle
appears normal in size and consistency.
Question 3 of 4
If a segment of terminal ileum becomes strangulated as a consequence of his
condition, it may become infarcted and necrotic due to
occlusion of a branch of which of the following vessels?
/ A. Celiac trunk
/ B. Inferior mesenteric artery
/ C. Middle colic artery
/ D. Right colic artery
/ E. Superior mesenteric artery
Explanation - Q: 1.3 Close

The correct answer is E. The small bowel is a derivative of the midgut and
therefore receives its blood supply from the superior mesenteric artery. This
artery emerges from the aorta 1 cm below the celiac trunk and passes
ventral to the left renal vein to give off 12 to 15 jejunal and ileal arteries. As
these arteries divide, they join with an adjacent branch to form arches. These
arches may then communicate to form an arcade. Straight arteries also
emerge from these arches to supply the bowel. Although there are variants,
the ileal artery is usually a branch of the superior mesenteric artery, which
supplies a branch to the terminal ileum.
The celiac trunk (choice A) provides branches to supply the stomach, liver,
pancreas, and duodenum. It is not responsible for supplying blood to the
terminal ileum.
The inferior mesenteric artery (choice B) provides branches that supply a
limited part of the transverse colon near the splenic flexure and the
descending and sigmoid colon.
The middle colic artery (choice C) is a branch of the superior mesenteric
artery. There are two branches, right and left. The right branch supplies the
right half of the transverse colon and the left branch supplies the left half of
the transverse colon.
The right colic artery (choice D) is also a branch of the superior mesenteric
artery. It is responsible for supplying the hepatic flexure as well as that part
of the ascending colon not supplied by the ileocolic artery.
A 36-year-old man presents to his physician complaining of right scrotal swelling.
He states that the swelling has been present for 1 week. He
initially noticed the swelling shortly after moving furniture for his new living room.
He denies any nausea, vomiting, change in bowel habits,
abdominal pain, or urinary tract symptoms. He has no other significant medical or
surgical history. On examination, he has an enlarged right
hemi-scrotum with a mass that appears to be originating at the level of the
external inguinal ring. With the patient completely relaxed, the
physician is able to reduce the mass by pushing it back through the external
inguinal ring. With the mass reduced, the physician instructs the
patient to perform a Valsalva maneuver, upon which a protrusion is felt at the
external inguinal ring. Once the mass is reduced, the testicle
appears normal in size and consistency.
Question 4 of 4
Which of the following pathological processes might cause the patient's
underlying condition to occur in an infant?
/ A. Defect in the floor of the inguinal canal
/ B. Defect in the internal inguinal ring
/ C. Defect in the linea semilunaris
/ D. Patent processus vaginalis
/ E. Persistent lumen of the tunica vaginalis
Explanation - Q: 1.4 Close

The correct answer is D. The pathologic process that causes hernias is
different in neonates and infants than in an adult. Embryologically, in the
seventh week, the testes begin descending from their location at the 10th
thoracic level into the scrotum. After the eighth week, a peritoneal
evagination called the processus vaginalis forms just anterior to the
gubernaculum. The gubernaculum is a condensation of peritoneum that
attaches superiorly to the gonad and inferiorly to the fascia that is developing
between the external and internal oblique muscles in the region of the
labioscrotal swellings. The processus vaginalis pushes out as a "sock-like"
extension into the transversalis fascia, the internal oblique muscle, and the
external oblique muscle, thus forming the inguinal canal. After the processus
vaginalis has evaginated into the scrotum, the gubernacula shorten and
simply pull the gonads through the canal. Within the first year after birth, the
superior portion of the processus vaginalis is usually obliterated, leaving only
a distal remnant sac, the tunica vaginalis, which lies anterior to the testis.
During infancy, this sac wraps around most of the testis. Its lumen is normally
collapsed, but under pathologic conditions it may fill with serous secretions,
forming a testicular hydrocele (choice E). If the processus vaginalis remains
patent, a connection between the abdominal cavity and scrotal sac will occur.
Loops of intestine may herniate into this processus, resulting in an indirect
inguinal hernia.
In adults, hernias are caused by a protrusion of a structure, usually intestine,
through a tissue that normally contains it. Direct inguinal hernias occur
through the floor of the inguinal canal separate from the spermatic cord
(choice A). Direct inguinal hernias occur because of a breakdown of the
transversus abdominis aponeurosis and transversalis fascia.
An indirect inguinal hernia occurs through the internal inguinal ring in a
protrusion of peritoneum along the spermatic cord in the internal spermatic
fascia. Therefore, a large indirect inguinal hernia will descend into the
scrotum along the spermatic cord (choice B).
Spigelian hernias are rare and occur due to a weakness in the linea
semilunaris (choice C), which is located at the lateral margin of the rectus
sheath.
A 2-year-old child is seen for a welI-child visit in a pediatric clinic. Abdominal
examination demonstrates a palpable, non-tender mass on the
Ieft side of the abdomen. The mother had no idea the mass was present and the
pediatrician did not note the presence of the mass at the
child's 18-month welI-child visit. Physical examination is otherwise unremarkable.
Question 1 of 5
CT examination demonstrates an 8-cm diameter, roughly round, mass involving
the lower pole of the kidney. Which of the following is the
most likely cause of this mass?
/ A. Ewing sarcoma
/ B. Neuroblastoma
/ C. Renal cell carcinoma
/ D. Transitional cell carcinoma
/ E. Wilms tumor

Explanation - Q: 2.1 Close

The correct answer is E. Wilms tumor is an embryonal malignancy of the
kidney that most commonly affects children from birth to age 4, but can affect
children up to about age 15. This tumor is the second most common
extracranial solid tumor in children and tends to form large, round, solitary
masses of the kidney, although bilaterality and multicentricity may also occur.
Wilms tumors usually present with a palpable abdominal mass, with less
common presentations including abdominal pain, hematuria (indicating
invasion of the collecting system), hypertension, fever, nausea, and vomiting.
Ewing sarcoma (choice A) most commonly involves the long bones of older
children and young adolescents.
Neuroblastoma (choice B) may also effect very young children, and typically
arises in the adrenal gland. Extensions into the kidney can occur, but usually
involve the upper pole first.
Renal cell carcinoma (choice C) and transitional cell carcinoma (choice D)
would be much more likely to involve the kidney of an adult.
A 2-year-old child is seen for a welI-child visit in a pediatric clinic. Abdominal
examination demonstrates a palpable, non-tender mass on the
Ieft side of the abdomen. The mother had no idea the mass was present and the
pediatrician did not note the presence of the mass at the
child's 18-month welI-child visit. Physical examination is otherwise unremarkable.
Question 2 of 5
If a CT guided biopsy of the mass were performed, which of the following
histological patterns would be most suggestive of the likely
diagnosis?
/ A. Cords of clear cells with rounded or polygonal shape and abundant clear
cytoplasm
/ B. Invasive papillary lesions with delicate connective tissue stalk covered with
epithelium resembling that lining the bladder
/ C. Small dark cells embedded in a finely fibrillar matrix with formation of
numerous rosettes
/ D. Triphasic pattern with tubule formation, spindle cells, and blastemal
elements
/ E. Uniform sheets of small round cells with scanty cytoplasm

Explanation - Q: 2.2 Close

The correct answer is D. Adequate sampling of most Wilms tumors will
detect the three distinct growth patterns that are most characteristic of this
tumor: less cellular tumor areas containing spindle cells; densely cellular
(blastemal) areas with closely packed small cells with scanty cytoplasm and
darkly blue nuclei; and areas with production of more mature-appearing
epithelium that may form occasional tubules. The tissues present are similar
to those present in the developing kidney, and may also include primitive
glomeruli. Wilms tumors may additionally contain heterologous elements,
such as skeletal muscle, smooth muscle, squamous or mucinous epithelium,
adipose tissue, cartilage, bone, or neural tissue.
Choice A suggests renal cell carcinoma.
Choice B suggests transitional cell carcinoma.
Choice C suggests neuroblastoma.
Choice E suggests Ewing sarcoma; neuroblastoma and some lymphomas
may also sometimes have a similar appearance.







A 2-year-old child is seen for a welI-child visit in a pediatric clinic. Abdominal
examination demonstrates a palpable, non-tender mass on the
Ieft side of the abdomen. The mother had no idea the mass was present and the
pediatrician did not note the presence of the mass at the
child's 18-month welI-child visit. Physical examination is otherwise unremarkable.
Question 4 of 5
Sometimes this child's disease occurs in association with certain congenital
anomalies. Children with which of the following abnormalities at
birth should be monitored for the development of this disease?
/ A. Aniridia
/ B. Coarctation of the aorta
/ C. Cystic hygroma
/ D. Parathyroid hyperplasia
/ E. Simian crease

Explanation - Q: 2.4 Close

The correct answer is A. The WAGR syndrome includes Wilms tumor,
aniridia (lack or defect of the iris), genital anomalies (gonadal dysgenesis,
hypospadias, cryptorchidism, or other genitourinary anomalies), and mental
retardation. Wilms tumor can also occur as part of the Beckwith-Wiedemann
syndrome with hemihypertrophy, and the Drash syndrome with nephropathy
and ambiguous genitalia.
Coarctation of the aorta (choice B) and cystic hygroma (choice C) are
associated with Turner syndrome, which may predispose for later
development of ovarian cancer.
Parathyroid hyperplasia (choice D) can be a component of multiple
endocrine neoplasia (MEN).
Simian crease (choice E) suggests Down syndrome, which is associated
with an increased incidence of leukemia.
A 2-year-old child is seen for a welI-child visit in a pediatric clinic. Abdominal
examination demonstrates a palpable, non-tender mass on the
Ieft side of the abdomen. The mother had no idea the mass was present and the
pediatrician did not note the presence of the mass at the
child's 18-month welI-child visit. Physical examination is otherwise unremarkable.
Question 5 of 5
Abnormalities of which of the following chromosomes have been linked to this
disease?
/ A. 3
/ B. 5
/ C. 11
/ D. 15
/ E. 21

Explanation - Q: 2.5 Close

The correct answer is C. Inactivation of the WT-1 Wilms tumor gene
(located at 11p13, and thought to encode a DNA-binding protein important in
fetal kidney development) has been reported in the tumor cells from many
Wilms tumor cases. Also, the WAGR syndrome involves a deletion of
chromosome 11p13 and the Beckwith-Wiedemann syndrome involves a
rearrangement of chromosome 11p15. The genetic basis of the Drash
syndrome has not been established. The other choices are distracters.


A 67-year-old man with an 18-year history of type 2 diabetes mellitus presents for
a routine physical examination. His temperature is 36.9 C
(98.5 F), his blood pressure is 158/98 mm Hg and his pulse is 82/minute and
regular. On examination, the physician notes a nontender,
pulsatile, mass in the mid-abdomen. A plain abdominal x-ray film with the patient
in the lateral position reveals spotty calcification of a
markedly dilated abdominal aortic walI.
Question 1 of 5
Which of the following is most likely pathogenetically related to this patient's
aortic disease?
/ A. Atherosclerosis
/ B. Cystic medial necrosis
/ C. Kawasaki disease
/ D. Mnckeberg arteriosclerosis
/ E. Syphilis




Explanation - Q: 3.1 Close

The correct answer is A. This patient has an abdominal aortic aneurysm
(AAA); these are most commonly the result of atherosclerosis. Hypertension
and cigarette smoking are also risk factors. AAA may be asymptomatic, or
may be associated with pain. Some aortic aneurysms are palpable as
pulsating masses in the abdomen. Abdominal aortic aneurysms typically
begin below the renal arteries and may extend well into the iliac system
bilaterally. Calcified atherosclerotic plaques on plain films of the abdomen
can be seen in the majority of cases. Rupture of an aortic aneurysm is
usually preceded by excruciating pain in the lower abdomen and back. The
mortality rate for abdominal aortic aneurysm repair performed electively is
about 2-5%, while that performed emergently after rupture of the aneurysm
has begun is 50%. Many patients with atherosclerotic aneurysms also have
severe coronary artery disease, and repair of the coronary arteries may be
needed before the patient is subjected to the risk of aortic aneurysm repair.
Cystic medial necrosis (choice B) is a risk factor for dissecting aneurysms
that typically do not widen the lumen of the aorta.
Kawasaki disease (choice C) can cause small aneurysms of the coronary
arteries.
Mnckeberg's arteriosclerosis (choice D) can cause focal calcification of
(usually medium-sized) arteries, but is not associated with aneurysm
formation.
Syphilis (choice E) is a now rare cause of aortic aneurysms that typically
involve the aortic root and ascending aorta.


A 67-year-old man with an 18-year history of type 2 diabetes mellitus presents for
a routine physical examination. His temperature is 36.9 C
(98.5 F), his blood pressure is 158/98 mm Hg and his pulse is 82/minute and
regular. On examination, the physician notes a nontender,
pulsatile, mass in the mid-abdomen. A plain abdominal x-ray film with the patient
in the lateral position reveals spotty calcification of a
markedly dilated abdominal aortic walI.
Question 2 of 5
Which of the following physiologic observations helps to account for the fact that
75% of the aneurysms of this patient's type are found in the
abdomen and only 25% principally involve the thorax?
/ A. Diastolic pressure is greater in the abdominal aorta in the supine position
/ B. Negative intrathoracic pressure reduces aortic wall tension in the thorax
/ C. The average blood flow in the abdominal aorta is greater than that in the
thoracic aorta
/ D. The average blood pressure in the abdominal aorta is higher than that in the
thoracic aorta
/ E. The average degree of turbulence in the thoracic aorta is higher than that in
the abdominal aorta

Explanation - Q: 3.2 Close

The correct answer is D. Increased blood pressure is a strong risk factor for
atherosclerosis, and humans pay a price for their erect sitting and standing
postures. In these postures, the abdominal aorta experiences the weight of a
column of blood added to the pressure produced by the heart. In the supine
posture, the pressures in the thoracic and abdominal aorta are similar. So, if
an average daily pressure is taken, the abdominal aorta tends to have a
significantly higher pressure than does the thoracic aorta.
Diastolic pressure (choice A) is actually greater in the thoracic aorta
compared to the abdominal aorta in the supine position. However, the
systolic blood pressure is greater in the abdominal aorta.
A negative intrathoracic pressure (choice B) would tend to increase
transmural pressure across the wall of the thoracic aorta, and thereby
increase wall tension and promote the development of aneurysms.
Blood flow (choice C) in the abdominal aorta is less than that in the thoracic
aorta, because some blood leaves the aorta through its thoracic branches.
Higher turbulence (choice E) tends to predispose for atherosclerosis, and
the flow in the abdominal aorta, with its many branches, tends to be more
turbulent than that in the thoracic aorta; this problem is exacerbated as
atherosclerotic disease advances.



A 67-year-old man with an 18-year history of type 2 diabetes mellitus presents for
a routine physical examination. His temperature is 36.9 C
(98.5 F), his blood pressure is 158/98 mm Hg and his pulse is 82/minute and
regular. On examination, the physician notes a nontender,
pulsatile, mass in the mid-abdomen. A plain abdominal x-ray film with the patient
in the lateral position reveals spotty calcification of a
markedly dilated abdominal aortic walI.
Question 3 of 5
The patient is taken to surgery and the abdominal aorta and proximal common
iliac arteries are replaced with a graft. Which of the following
aneurysm diameters is usually considered the threshold above which elective
surgery is recommended, unless contraindicated by other
disease?
/ A. 1 cm
/ B. 2 cm
/ C. 6 cm
/ D. 10 cm
/ E. 15 cm


Explanation - Q: 3.3 Close

The correct answer is C. This recommendation is made because larger
aneurysms have a much greater chance of rupture, and emergency repair
carries a high mortality rate. For aneurysms larger than 5 cm, the risk of
rupture is 5-10% per year

A 67-year-old man with an 18-year history of type 2 diabetes mellitus presents for
a routine physical examination. His temperature is 36.9 C
(98.5 F), his blood pressure is 158/98 mm Hg and his pulse is 82/minute and
regular. On examination, the physician notes a nontender,
pulsatile, mass in the mid-abdomen. A plain abdominal x-ray film with the patient
in the lateral position reveals spotty calcification of a
markedly dilated abdominal aortic walI.
Question 4 of 5
Following surgery, the patient is placed on a low-fat diet to reduce the risk of
continued progression of his atherosclerotic disease. A bile
acid sequestrant is added to interrupt enterohepatic circulation of bile acids.
Which of the following agents was most likely prescribed?
/ A. Atorvastatin
/ B. Cholestyramine
/ C. CIofibrate
/ D. Gemfibrozil
/ E. Lovastatin
Explanation - Q: 3.4 Close

The correct answer is B. Cholestyramine and colestipol are bile acid
sequestrants that bind bile acids in the intestine, thereby interrupting
enterohepatic circulation of bile acids. This has an indirect effect to enhance
LDL clearance and lower lipids in the blood.
Atorvastatin (choice A) and lovastatin (choice E) are lipid-lowering drugs
that competitively inhibit HMG-CoA reductase, an early step in cholesterol
biosynthesis.
Clofibrate (choice C) and gemfibrozil (choice D) are fibric acid derivatives
that may increase the activity of lipoprotein lipase.
A 67-year-old man with an 18-year history of type 2 diabetes mellitus presents for
a routine physical examination. His temperature is 36.9 C
(98.5 F), his blood pressure is 158/98 mm Hg and his pulse is 82/minute and
regular. On examination, the physician notes a nontender,
pulsatile, mass in the mid-abdomen. A plain abdominal x-ray film with the patient
in the lateral position reveals spotty calcification of a
markedly dilated abdominal aortic walI.
Question 5 of 5
The table shows values of vessel radius (r), intraluminal pressure (P), and wall
thickness (w) for both the normal aorta and an aortic aneurysm.



What effect does the aneurysm have on wall stress?
/ A. Wall stress decreases 10 fold
/ B. Wall stress decreases 2.5 fold
/ C. Wall stress decreases 5 fold
/ D. Wall stress increases 10 fold
/ E. Wall stress increases 2.5 fold
/ F. Wall stress increases 5 fold

Explanation - Q: 3.5 Close

The correct answer is E. According to the Law of LaPlace for a cylindrical
structure such as the aorta: wall stress (s) = (P x r)/w. Because P and w are
shown in the table to be unaffected by the aneurysm and because radius is
increased by 2.5 fold at the site of the aneurysm, it is clear that wall stress
has increased by 2.5 fold at the site of the aneurysm. This relationship
between vessel radius and wall tension can explain why the probability of
rupture increases as the aneurysm becomes larger.

A 3-year-old girl is seen in the emergency department with acute abdominal pain.
She has a 5-day history of vomiting and abdominal
distension. She has not passed stool during this time, and during the past day,
has been vomiting bilious materiaI. On physical examination,
she is lethargic, with a firm and tender abdomen, and peritoneal signs are
present. She is immediately referred for laparotomy for suspected
diagnoses of intussusception vs. volvulus. At surgery, approximately 20 cm of
small intestine is found to be markedly distended, and is
resected. The section contains a tightly knotted ball of nematodes that are about
15 to 35 cm in length. The worms have tapered ends without
hooks.
Question 1 of 5
The worms are most likely which of the following?



Explanation - Q: 4.1 Close

The correct answer is B. Ascaris lumbricoides is the only parasitic worm that is
likely to cause intestinal obstruction, and then only if the worm burden is high. The
description given of the worms in the case presentation is typical. All of the other
worms listed in the choices are also nematodes or roundworms.
Ankylostoma duodenale(choice A) is a small (approximately 1 cm) hookworm that
inhabits the small intestine and clings to the mucosa.
Enterobius vermicularis(choice C) is the pinworm. This is an approximately 1 cm
long worm that inhabits the large bowel (and appendix); the female deposits eggs on
the perianal skin.
Strongyloides stercoralis(choice D), or threadworm, is a 2.5 mm worm that lives in
the crypts of the small bowel and may cause chronic infection due to autoinfection. It
is the only nematode capable of increasing its numbers in a host. In
immunocompromised hosts, it can cause life-threatening disseminated infection.
Trichuris trichiura(choice E), or whipworm, is a 3-5 cm worm that lives on the
colorectal mucosa.

A 3-year-old girl is seen in the emergency department with acute abdominal pain.
She has a 5-day history of vomiting and abdominal
distension. She has not passed stool during this time, and during the past day,
has been vomiting bilious materiaI. On physical examination,
she is lethargic, with a firm and tender abdomen, and peritoneal signs are
present. She is immediately referred for laparotomy for suspected
diagnoses of intussusception vs. volvulus. At surgery, approximately 20 cm of
small intestine is found to be markedly distended, and is
resected. The section contains a tightly knotted ball of nematodes that are about
15 to 35 cm in length. The worms have tapered ends without
hooks.
Question 2 of 5
Which of the following best describes what would have been seen under the
microscope if the patient's stool had been analyzed for ova and
parasites?
/ A. Large oval eggs with a lateral spine
/ B. Large round to oval eggs, with a thick mammillated shells
/ C. Lemon-shaped eggs, with bipolar plugs,
/ D. Round eggs and proglottids filled with eggs
/ E. Small larvae

Explanation - Q: 4.2 Close

The correct answer is B. This is the correct description of fertilized Ascaris
eggs. Both fertilized and somewhat more elongated unfertilized eggs may be
seen; fertilized eggs may contain multiple cells under the thick wavy shell.
Choice A describes the eggs of the trematode (fluke) Schistosoma mansoni.
Choice C describes the eggs of Trichuris trichiura.
Choice D describes the eggs of the cestodes (tapeworms) Taenia saginata
and Taenia solium.
Choice E describes the larvae of Strongyloides stercoralis. Strongyloides
stercoralis has a rapid life cycle in its host. It is the only nematode whose
diagnostic form is a larva, not an egg.
A 3-year-old girl is seen in the emergency department with acute abdominal pain.
She has a 5-day history of vomiting and abdominal
distension. She has not passed stool during this time, and during the past day,
has been vomiting bilious materiaI. On physical examination,
she is lethargic, with a firm and tender abdomen, and peritoneal signs are
present. She is immediately referred for laparotomy for suspected
diagnoses of intussusception vs. volvulus. At surgery, approximately 20 cm of
small intestine is found to be markedly distended, and is
resected. The section contains a tightly knotted ball of nematodes that are about
15 to 35 cm in length. The worms have tapered ends without
hooks.
Question 3 of 5
If this child acquired her infection in the United States, in what region of the
nation does she most likely reside?
/ A. Desert Southwest
/ B. Midwest
/ C. Northeast
/ D. Pacific Northwest
/ E. Southeast

Explanation - Q: 4.3 Close

The correct answer is E. It is easy for medical students, residents, and
physicians to neglect parasitic diseases because they assume that the
American population is not likely to have them. However, it is thought that
more than 4 million individuals in the Untied States, most of whom are
immigrants from developing countries, are infected with Ascaris species,
predominately Ascaris lumbricoides. Most of these people have
asymptomatic infections. Ascaris can also be acquired in rural areas of the
Southeastern United States, where it is endemic. Worldwide, 1.4 billion
people are estimated to be infected.
A 3-year-old girl is seen in the emergency department with acute abdominal pain.
She has a 5-day history of vomiting and abdominal
distension. She has not passed stool during this time, and during the past day,
has been vomiting bilious materiaI. On physical examination,
she is lethargic, with a firm and tender abdomen, and peritoneal signs are
present. She is immediately referred for laparotomy for suspected
diagnoses of intussusception vs. volvulus. At surgery, approximately 20 cm of
small intestine is found to be markedly distended, and is
resected. The section contains a tightly knotted ball of nematodes that are about
15 to 35 cm in length. The worms have tapered ends without
hooks.
Question 4 of 5
Which of the following medications would be the most appropriate
pharmacotherapy for this patient?
/ A. Bithionol
/ B. Mebendazole
/ C. Metronidazole
/ D. Niclosamide
/ E. Praziquantel

Explanation - Q: 4.4 Close

The correct answer is B. Mebendazole is a systemically absorbed broad-
spectrum anthelminthic agent effective against Ascaris species, hookworm,
tapeworm, liver fluke, and pinworms.
Bithionol (choice A) is used to treat infections caused by Fasciola hepatica,
a tissue fluke.
Metronidazole (choice C) is used to treat infections caused by anaerobic
organisms, including the intestinal protozoa, Giardia lamblia, and Entamoeba
histolytica.
Niclosamide (choice D) is not available in the United States. It can be used
to treat tapeworm infections caused by T. saginata and D. latum. It is less
expensive than praziquantel.
Praziquantel (choice E) has broad-spectrum activity against most
trematodes and cestodes, with the exception of F. hepatica.




A 3-year-old girl is seen in the emergency department with acute abdominal pain.
She has a 5-day history of vomiting and abdominal
distension. She has not passed stool during this time, and during the past day,
has been vomiting bilious materiaI. On physical examination,
she is lethargic, with a firm and tender abdomen, and peritoneal signs are
present. She is immediately referred for laparotomy for suspected
diagnoses of intussusception vs. volvulus. At surgery, approximately 20 cm of
small intestine is found to be markedly distended, and is
resected. The section contains a tightly knotted ball of nematodes that are about
15 to 35 cm in length. The worms have tapered ends without
hooks.
Question 5 of 5
Part of the life cycle of this patient's parasite is a filarial stage in which larva,
hatched in the duodenum, penetrate the wall of the small
intestine. The passage of migrating larvae most commonly produces
symptomatic disease in which of the following organs?
/ A. Heart
/ B. Liver
/ C. Lungs
/ D. Pancreas
/ E. Stomach

Explanation - Q: 4.5 Close

The correct answer is C. Ascaris infection is acquired by ingestion of the
eggs, usually from contaminated soil. The ingested eggs hatch in the
duodenum to produce larvae, which cross the small intestinal wall to enter
the blood stream. They then pass through the heart to lodge in the lungs.
They leave the lung capillary bed to enter the airspaces. They then ascend
the bronchial tree into the oropharynx, where they are again swallowed. This
time, they return to the small intestine where they develop into mature worms
that can live in the host for up to two years. A gravid female worm may
produce up to 250,000 eggs daily, which are shed in stool. The passage of
the larvae through the lungs often produces cough and/or wheezing, and
may, in severe cases, produce fever, dyspnea, fleeting patchy pulmonary
infiltrates (Loeffler pneumonitis with prominent eosinophilia), and rarely
hemoptysis.
Passage through the heart (choice A) is usually asymptomatic, as the larvae
are small.
A single adult worm, but not usually larval forms, can migrate into the biliary
tree (choice B), leading to biliary colic, cholangitis, or gallstone formation;
obstructive jaundice uncommonly occurs.
The pancreas (choice D) and stomach (choice E) are not common sites for
complications of ascariasis.

A 27-year-old woman goes to an emergency room with severe abdominal pain.
She had previously experienced similar episodes of pain that
Iasted several hours to a few days, but this episode is the most severe. She has
also been experiencing nausea, vomiting, and constipation.
The physician is left with the impression that she is agitated and somewhat
confused, and an accurate history is difficult to elucidate. The
patient is sent for emergency laparotomy, but no pathology is noted at surgery.
Following the unrevealing surgery, an older surgeon
comments that he had once seen a similar case that was actually due to
porphyria.
Question 1 of 5
The porphyrias are biochemical abnormalities in which of the following
pathways?
/ A. GIycogen degradation
/ B. Heme synthesis
/ C. Lipoprotein degradation
/ D. Nucleotide degradation
/ E. Urea cycle


Explanation - Q: 1.1 Close

The correct answer is B. The porphyrias are a group of rare, related
diseases that have in common a block in the heme synthesis pathway. The
block is usually partial rather than complete, and thus many of these patients
have only intermittent symptoms. Most cases of porphyria present with either
a neurovisceral pattern (including both psychiatric symptoms and abdominal
pain) or with photosensitive skin lesions. These two patterns are associated
with different forms of porphyria.
Associate abnormalities of glycogen degradation (choice A) with the
glycogen storage diseases, such as von Gierke disease, Pompe disease,
and Forbes disease.
Associate abnormalities of lipoprotein degradation (choice C) with some
forms of hyperlipoproteinemia (notably Type I).
Associate abnormalities of nucleotide degradation (choice D) with gout and
Lesch-Nyhan syndrome.
Associate abnormalities of the urea cycle (choice E) with congenital
hyperammonemia, citrullinemia, and argininosuccinic acidemia.
A 27-year-old woman goes to an emergency room with severe abdominal pain.
She had previously experienced similar episodes of pain that
Iasted several hours to a few days, but this episode is the most severe. She has
also been experiencing nausea, vomiting, and constipation.
The physician is left with the impression that she is agitated and somewhat
confused, and an accurate history is difficult to elucidate. The
patient is sent for emergency laparotomy, but no pathology is noted at surgery.
Following the unrevealing surgery, an older surgeon
comments that he had once seen a similar case that was actually due to
porphyria.
Question 2 of 5
Following the surgery, the decision is made to screen for the porphyrias that
cause acute neurovisceral symptoms. Which of the following
tests would be most likely to be used?
/ A. Erythrocyte porphyrins
/ B. Total fecal porphyrins
/ C. Total plasma porphyrins
/ D. Total urinary porphyrins
/ E. Urinary porphobilinogen

Explanation - Q: 1.2 Close

The correct answer is E. The acute neurovisceral porphyrias are those that
tend to present with severe abdominal pain, often accompanied by
neuropsychiatric symptoms. The best tests to use for screening of these
diseases are urinary porphobilinogen (PBG, either random or 24 hour) and
urinary delta-aminolevulinic acid (ALA, either random or 24 hour).
Erythrocyte porphyrins (choice A) are used for follow-up in the
photosensitive types of porphyria.
Total fecal porphyrins (choice B) are used for follow-up evaluation after
screening tests for either the photosensitive porphyrias or the acute
neurovisceral porphyrias are positive.
Total plasma porphyrias (choice C) are useful for first line screening of the
photosensitive porphyrias, and are used for further evaluation after screening
in the acute neurovisceral porphyrias.
Total urinary porphyrins (choice D) are used for further evaluation after
screening for acute neurovisceral porphyrias.

A 27-year-old woman goes to an emergency room with severe abdominal pain.
She had previously experienced similar episodes of pain that
Iasted several hours to a few days, but this episode is the most severe. She has
also been experiencing nausea, vomiting, and constipation.
The physician is left with the impression that she is agitated and somewhat
confused, and an accurate history is difficult to elucidate. The
patient is sent for emergency laparotomy, but no pathology is noted at surgery.
Following the unrevealing surgery, an older surgeon
comments that he had once seen a similar case that was actually due to
porphyria.
uestion 3 of 5
Which of the following are the three most common forms of porphyria?
/ A. Acute intermittent porphyria, erythropoietic protoporphyria, and porphyria
cutanea tarda
/ B. Acute intermittent porphyria, hepatoerythropoietic porphyria, and variegate
porphyria
/ C. Congenital erythropoietic porphyria, delta-aminolevulinic acid dehydratase-
deficient porphyria, and hepatoerythropoietic porphyria
/ D. Erythropoietic protoporphyria, hereditary coproporphyria, and porphyria
cutanea tarda
/ E. Hereditary coproporphyria, variegate porphyria, and X-Iinked sideroblastic
anemia


Explanation - Q: 1.3 Close

The correct answer is A. The porphyrias are complex diseases that can
easily appear overwhelming. A very useful point to know (both clinically and
for the USMLE) is that the three most common forms are acute intermittent
porphyria, erythropoietic protoporphyria, and porphyria cutanea tarda. Acute
intermittent porphyria tends to present with acute neurovisceral symptoms.
Erythrocytic protoporphyria tends to present acutely with painful skin lesions.
Porphyria cutanea tarda tends to present with chronic blistering skin lesions.
The other types listed in various choices are also porphyrias, but are less
common.


A 27-year-old woman goes to an emergency room with severe abdominal pain.
She had previously experienced similar episodes of pain that
Iasted several hours to a few days, but this episode is the most severe. She has
also been experiencing nausea, vomiting, and constipation.
The physician is left with the impression that she is agitated and somewhat
confused, and an accurate history is difficult to elucidate. The
patient is sent for emergency laparotomy, but no pathology is noted at surgery.
Following the unrevealing surgery, an older surgeon
comments that he had once seen a similar case that was actually due to
porphyria.
uestion 4 of 5
This patient is found to have increased levels of both delta-aminolevulinic acid
(ALA) and porphobilinogen (PBG) in blood. Follow-up studies
demonstrate low PBG deaminase in erythrocytes. AIso, additional history is
elicited, revealing that the woman had started a very low
carbohydrate diet about one week before being admitted to the hospitaI. Which
of the following is the most likely diagnosis?
/ A. Acute intermittent porphyria
/ B. Congenital erythropoietic porphyria
/ C. Erythropoietic protoporphyria
/ D. Porphyria cutanea tarda
/ E. X-Iinked sideroblastic anemia


Explanation - Q: 1.4 Close

The correct answer is A. These laboratory findings are most consistent with
acute intermittent porphyria, which is due to PBG deaminase deficiency.
Patients usually, but not always, have a deficiency of erythrocyte PBG
deaminases. (Some cases have also been described in which the enzyme
deficiency is limited to liver.) The condition is an autosomal dominant
disorder that typically becomes symptomatic in women after puberty, and
then often only if a precipitating event (dieting, use of certain drugs,
premenstrual) is also present. Symptoms during the attacks can include
abdominal symptoms (pain, nausea, vomiting, constipation, diarrhea,
abdominal distension, ileus), which are thought to be due to the effects of
this condition on visceral nerves. Other symptoms that may be mediated
neurologically include incontinence, urinary retention, tachycardia,
diaphoresis, hypertension, muscle weakness, psychiatric symptoms,
seizures, and rarely, severe paralysis, respiratory insufficiency, and death.
Both intravenous glucose (oral is often inadequate due to poor intestinal
function) and exogenous heme supplementation can suppress the heme
biosynthetic mechanism, and tend to ameliorate the acute attack. Patients
should be cautioned to diet gently, as intense dieting can precipitate attacks.
Congenital erythropoietic porphyria (choice B) is characterized by severe
skin blistering that usually begins after birth, pink to dark-brown urine, normal
ALA and PBG, and increased porphyrins (primarily uroporphyrin I and
coproporphyrin I) in urine, plasma, and erythrocytes.
Erythropoietic protoporphyria (choice C) is characterized by cutaneous
photosensitivity that begins early in life and high protoporphyrin in
erythrocytes and bone marrow.
Porphyria cutanea tarda (choice D) is characterized by photosensitivity with
skin blistering, elevated plasma porphyrins, and elevated urine porphyrins
(mostly uroporphyrin and heptacarboxylporphyrin).
The very rare X-linked sideroblastic anemia (choice E), due to a deficiency
of delta-aminolevulinic acid synthase, can clinically resemble acute
intermittent porphyria, and is characterized by elevated levels of urinary ALA
and coproporphyrin.


A 27-year-old woman goes to an emergency room with severe abdominal pain.
She had previously experienced similar episodes of pain that
Iasted several hours to a few days, but this episode is the most severe. She has
also been experiencing nausea, vomiting, and constipation.
The physician is left with the impression that she is agitated and somewhat
confused, and an accurate history is difficult to elucidate. The
patient is sent for emergency laparotomy, but no pathology is noted at surgery.
Following the unrevealing surgery, an older surgeon
comments that he had once seen a similar case that was actually due to
porphyria.
Question 5 of 5
Which of the following drugs would be most likely to induce an attack of
abdominal pain in this patient?
/ A. Acetaminophen
/ B. Aspirin
/ C. Barbiturate
/ D. GIucocorticoid
/ E. Insulin

Explanation - Q: 1.5 Close

The correct answer is C. Some symptomatic episodes of acute porphyria
(including acute intermittent porphyria, hereditary coproporphyria, variegate
porphyria, and aminolevulinic acid dehydratase porphyria) are triggered by
drug ingestion, and administration of drugs to undiagnosed patients can
cause an acute exacerbation of an ongoing attack of acute porphyria. Drugs
considered unsafe for use in these patients notably include alcohol,
anticonvulsants, barbiturates, many other sedatives, and sulfonamide
antibiotics. Of particular concern are the sedative agents, since it may be
very tempting to give an obviously agitated patient a sedative to allow easier
examination of the patient. Many other drugs are also on the lists of
potentially dangerous drugs in these patients. Once the diagnosis is
established, the patient should be instructed to always inform her/his
physician of her condition, and ask that the safety of drugs prescribed in
patients with porphyria be checked. Many of the drugs that can induce or
exacerbate an attack of porphyria do so by increasing the activity of the
cytochrome P450 system, which indirectly triggers an increase in heme
biosynthesis. The other medications listed in the choices are "safe" in these
patients.





A 47-year-old woman presents to the emergency department with
cramping/colicky abdominal pain. The current episode of pain began
several hours ago, following a fatty meaI. The pain began slowly, and rose in
intensity to a plateau over the course of several hours. The
patient reports that she had had several other episodes of similar pain during the
past several months, with long intervening periods of
freedom from pain. On physical examination, she is noted to have tenderness to
deep palpation in the right upper quadrant of the abdomen
near the rib cage. The patient also reports that she is experiencing shoulder/back
pain at a site she identifies near the right lower scapula, but
no tenderness can be elicited during the back and shoulder examination.
Question 1 of 7
Which of the following organs is the most likely source of this woman's pain?
/ A. Appendix
/ B. Diaphragm
/ C. Esophagus
/ D. Gallbladder
/ E. Stomach

Explanation - Q: 2.1 Close

The correct answer is D. This woman most likely has gallstones.
Cholelithiasis, or the formation of calculi (gallstones) within the gallbladder, is
very common in the United States, with over 500,000 cholecystectomies
being performed yearly. While many cases of gallstone disease are
symptomatic, right upper quadrant pain with referral of the pain to the lower
right scapula should specifically suggest gallbladder disease. The pattern of
episodes of several hours of pain followed by long periods of freedom from
pain is also typical of symptomatic gallstone disease.
The appendix (choice A) would most likely cause lower abdominal pain.
Pain from irritation of the diaphragm (choice B) can cause right upper
quadrant pain and referred pain in the supraclavicular area (rather than the
subscapular pain of biliary colic). The absence of right upper quadrant
tenderness to palpation, and the history of pain after a fatty meal also argue
against this diagnosis.
Esophageal pain (choice C) related to regurgitation of gastric contents
(heartburn) can occur postprandially, but tends to radiate into the neck,
throat, or even face.
Peptic ulcer pain of gastric origin (choice E) is usually described as causing
burning, gnawing, or hunger, and may be relieved by eating.
A 47-year-old woman presents to the emergency department with
cramping/colicky abdominal pain. The current episode of pain began
several hours ago, following a fatty meaI. The pain began slowly, and rose in
intensity to a plateau over the course of several hours. The
patient reports that she had had several other episodes of similar pain during the
past several months, with long intervening periods of
freedom from pain. On physical examination, she is noted to have tenderness to
deep palpation in the right upper quadrant of the abdomen
near the rib cage. The patient also reports that she is experiencing shoulder/back
pain at a site she identifies near the right lower scapula, but
no tenderness can be elicited during the back and shoulder examination.
Question 2 of 7
Which of the following techniques would be most appropriate to demonstrate the
patient's most likely diagnosis?
/ A. Colonoscopy
/ B. CT scan of the abdomen
/ C. Esophagoduodenoscopy
/ D. MRI scan of the abdomen
/ E. UItrasonography
Explanation - Q: 2.2 Close

The correct answer is E. Real-time ultrasonography, with 98% sensitivity
and 95% specificity, is considered the method of choice for diagnosing
possible gallbladder stones.
Colonoscopy (choice A) and esophagoduodenoscopy (choice C) might be
helpful for excluding alternative diagnoses, but would not themselves
establish a diagnosis of gallstone disease.
CT (choice B) and MRI (choice D) scans of the abdomen are expensive
tests whose use is not warranted, since real-time ultrasonography performs
as well or better.

A 47-year-old woman presents to the emergency department with
cramping/colicky abdominal pain. The current episode of pain began
several hours ago, following a fatty meaI. The pain began slowly, and rose in
intensity to a plateau over the course of several hours. The
patient reports that she had had several other episodes of similar pain during the
past several months, with long intervening periods of
freedom from pain. On physical examination, she is noted to have tenderness to
deep palpation in the right upper quadrant of the abdomen
near the rib cage. The patient also reports that she is experiencing shoulder/back
pain at a site she identifies near the right lower scapula, but
no tenderness can be elicited during the back and shoulder examination.
Question 3 of 7
Following appropriate diagnostic studies, the patient is taken to the surgical suite.
During the surgery, the surgeon inserts his fingers from
right to left behind the hepatoduodenal ligament. As he does so, his fingers enter
which of the following?
/ A. Ampulla of Vater
/ B. Common bile duct
/ C. Epiploic foramen
/ D. Greater peritoneal sac
/ E. Portal vein

Explanation - Q: 2.3 Close

The correct answer is C. The space behind the stomach, hepatoduodenal
ligament, and hepatogastric ligament is the omental bursa. This space can
be entered by passing through the epiploic foramen of Winslow, as described
in the question stem.
The common bile duct enters the duodenum through the ampulla of Vater
(choice A).
The hepatoduodenal ligament contains the common bile duct (choice B), the
portal vein (choice E), and the hepatic artery.
The greater peritoneal sac (choice D) lies anterior to the stomach and
hepatoduodenal ligament.

A 47-year-old woman presents to the emergency department with
cramping/colicky abdominal pain. The current episode of pain began
several hours ago, following a fatty meaI. The pain began slowly, and rose in
intensity to a plateau over the course of several hours. The
patient reports that she had had several other episodes of similar pain during the
past several months, with long intervening periods of
freedom from pain. On physical examination, she is noted to have tenderness to
deep palpation in the right upper quadrant of the abdomen
near the rib cage. The patient also reports that she is experiencing shoulder/back
pain at a site she identifies near the right lower scapula, but
no tenderness can be elicited during the back and shoulder examination.
Question 4 of 7
During the cholecystectomy, the surgeon ligates the cystic artery. This is typically
a branch of which of the following?
/ A. Gastroduodenal artery
/ B. Left gastroepiploic artery
/ C. Right gastroepiploic artery
/ D. Right hepatic artery
/ E. Superior pancreaticoduodenal artery
Explanation - Q: 2.4 Close

The correct answer is D. The cystic artery is generally a branch of the right
hepatic artery.
The gastroduodenal artery (choice A) is a branch of the (common) hepatic
artery.
The left gastroepiploic artery (choice B) is a branch of the splenic artery.
The right gastroepiploic artery (choice C) is a branch of the gastroduodenal
artery.
The superior pancreaticoduodenal artery (choice E) is a branch of the
gastroduodenal artery.

A 47-year-old woman presents to the emergency department with
cramping/colicky abdominal pain. The current episode of pain began
several hours ago, following a fatty meaI. The pain began slowly, and rose in
intensity to a plateau over the course of several hours. The
patient reports that she had had several other episodes of similar pain during the
past several months, with long intervening periods of
freedom from pain. On physical examination, she is noted to have tenderness to
deep palpation in the right upper quadrant of the abdomen
near the rib cage. The patient also reports that she is experiencing shoulder/back
pain at a site she identifies near the right lower scapula, but
no tenderness can be elicited during the back and shoulder examination.


Question 5 of 7



Pathologic examination of the specimen removed by the surgeon demonstrates
the presence of numerous yellow stones (shown above).
These are most likely composed primarily of which of the following?
/ A. Bilirubinate
/ B. Calcium phosphate
/ C. Cholesterol
/ D. Cystine
/ E. Struvite

Explanation - Q: 2.5 Close

The correct answer is C. The stones are gallstones, and their yellow color
indicates that they are composed of cholesterol. Cholesterol stones are the
most common form of gallstones. Risk factors include female sex, multiparity,
obesity, increased age (female, fat, forty, and fertile) and North American
Indian race.
Bilirubinate (choice A) gallstones, which are usually associated with
hemolytic anemias, are less common, brown, rather than yellow, and often
faceted.
Calcium phosphate (choice B), cystine (choice D), and struvite (choice E)
composition can be seen in kidney stones.

A 47-year-old woman presents to the emergency department with
cramping/colicky abdominal pain. The current episode of pain began
several hours ago, following a fatty meaI. The pain began slowly, and rose in
intensity to a plateau over the course of several hours. The
patient reports that she had had several other episodes of similar pain during the
past several months, with long intervening periods of
freedom from pain. On physical examination, she is noted to have tenderness to
deep palpation in the right upper quadrant of the abdomen
near the rib cage. The patient also reports that she is experiencing shoulder/back
pain at a site she identifies near the right lower scapula, but
no tenderness can be elicited during the back and shoulder examination.
Question 6 of 7
If this patient had a small stone lodge near the ampulla of Vater, which of the
following complications would be most likely to occur?
/ A. Crohn disease
/ B. Diabetes mellitus
/ C. Pancreatitis
/ D. Peptic ulcer
/ E. Polyarteritis nodosa



Explanation - Q: 2.6 Close

The correct answer is C. A small gallstone obstructing the pancreatic
outflow is a well-known cause of acute pancreatitis. The other conditions
listed are not caused by gallstones.


A 47-year-old woman presents to the emergency department with
cramping/colicky abdominal pain. The current episode of pain began
several hours ago, following a fatty meaI. The pain began slowly, and rose in
intensity to a plateau over the course of several hours. The
patient reports that she had had several other episodes of similar pain during the
past several months, with long intervening periods of
freedom from pain. On physical examination, she is noted to have tenderness to
deep palpation in the right upper quadrant of the abdomen
near the rib cage. The patient also reports that she is experiencing shoulder/back
pain at a site she identifies near the right lower scapula, but
no tenderness can be elicited during the back and shoulder examination.
Question 7 of 7
If this patient had refused surgical treatment, which of the following would be the
most appropriate pharmacotherapy to provide definitive
treatment and thereby relieve associated pain?
/ A. Ampicillin
/ B. CIofibrate
/ C. Meperidine
/ D. Oxycodone
/ E. Ursodiol

Explanation - Q: 2.7 Close

The correct answer is E. The question is asking, "Which of the following will
eradicate a gallstone?" When a gallstone is eliminated the pain will
subsequently be eliminated. This question is NOT asking, "which of the
following is the most appropriate form of pain control?". Ursodiol
(ursodeoxycholic acid) is a hydrophilic bile acid that is used to dissolve small
(< 20 mm), non-calcified, radiolucent cholesterol gallstones in patients with
functioning gallbladders who cannot undergo (or refuse) cholecystectomy.
Analgesics and antibiotics, such as ampicillin (choice A), are administered
when appropriate, but do not help eradicate the stones.
Clofibrate (choice B) is an antihyperlipidemic that is associated with the
development of gallstones. High-risk patients, such as diabetics and the
elderly, should be watched closely.
As a side note, if this question were asking: "which of the following is the
most appropriate form of pain control in this patient", the most appropriate
answer would be meperidine. Meperidine (choice C) is the narcotic of choice
since it causes the least amount of spasm of the sphincter of Oddi. In other
words, meperidine is preferred over oxycodone (choice D).


A 64-year-old man with a history of coronary artery disease (CAD) comes to the
emergency department with the acute onset of severe,
constant, Iower abdominal pain and rectal bleeding. He reports that he previously
has had several episodes of similar, but less severe pain.
About 12 hours after the onset of pain, the patient began passing copious bright
red blood per rectum. He denies nausea, vomiting, sick
contacts, or foreign traveI. Initial physical examination reveals a distressed man,
who is afebrile, but tachypneic, with scant diffuse abdominal
tenderness to palpation. Rectal examination is positive for blood. Laboratory
studies reveal a metabolic acidosis with an elevated serum
Iactate.

Question 1 of 5

Which of the following is the most likely diagnosis?
/ A. Colon carcinoma
/ B. Infectious colitis
/ C. Inflammatory bowel disease
/ D. Ischemic colitis
/ E. Necrotizing enterocolitis

Explanation - Q: 3.1 Close

The correct answer is D. A patient with severe abdominal pain and rectal
bleeding with an unremarkable physical examination is likely suffering from
ischemic colitis. "Pain out-of-proportion to examination" is a classic finding for
ischemic colitis. The previous episodes of less severe pain represent
ischemic angina. An infarction has occurred, as indicated by the rise in
serum lactate secondary to the colon's anaerobic metabolism. The history of
coronary artery disease also suggests this diagnosis, as the atherosclerotic
processes that contribute to his CAD are also likely present in his abdominal
vasculature.
Colon cancer (choice A) would produce less acute symptoms, but
occasionally, colon cancer may present acutely with obstructive symptoms.
Patients may have bleeding and abdominal pain, but the pain is typically
intermittent and accompanied by nausea, vomiting, abdominal distention,
and absence of flatus.
Infectious colitis (choice B) is incorrect. While patients may have bleeding
and abdominal pain, nothing in the history suggests a disease of infectious
origin (no sick contacts or foreign travel). The acute onset also suggests a
vascular event, rather than an infectious one.
Inflammatory bowel disease (IBD) (choice C) is incorrect because while the
patient reports previous episodes, an elderly man with IBD would likely have
a chronic history of abdominal pain and bleeding.
Necrotizing enterocolitis (choice E) affects premature infants and would not
be relevant in this setting.


A 64-year-old man with a history of coronary artery disease (CAD) comes to the
emergency department with the acute onset of severe,
constant, Iower abdominal pain and rectal bleeding. He reports that he previously
has had several episodes of similar, but less severe pain.
About 12 hours after the onset of pain, the patient began passing copious bright
red blood per rectum. He denies nausea, vomiting, sick
contacts, or foreign traveI. Initial physical examination reveals a distressed man,
who is afebrile, but tachypneic, with scant diffuse abdominal
tenderness to palpation. Rectal examination is positive for blood. Laboratory
studies reveal a metabolic acidosis with an elevated serum
Iactate.
Question 2 of 5
The lactate produced from the anaerobic metabolism in the infarcted gut will
likely be which of the following?
/ A. Exhaled as a fruity odor
/ B. Incorporated into glycogen in the liver
/ C. Incorporated into myoglobin in muscle
/ D. Incorporated into urea in the urine
/ E. Secreted by the kidneys unchanged

Explanation - Q: 3.2 Close

The correct answer is B. Lactate is converted into glucose, and then
glycogen in the liver by a process know as the Cori cycle.
Choice A is incorrect, as lactate would not be exhaled. A fruity odor on the
breath would be a sign of ketoacidosis.
While some of the carbon from the lactate may be incorporated into peptides
via Krebs intermediates (e.g., choice C), the vast majority would be left as
carbohydrate.
Urea (choice D) represents a means of eliminating nitrogenous waste.
Choice E is wrong, as the kidneys would retain the lactate, rather than
excreting it.

A 64-year-old man with a history of coronary artery disease (CAD) comes to the
emergency department with the acute onset of severe,
constant, Iower abdominal pain and rectal bleeding. He reports that he previously
has had several episodes of similar, but less severe pain.
About 12 hours after the onset of pain, the patient began passing copious bright
red blood per rectum. He denies nausea, vomiting, sick
contacts, or foreign traveI. Initial physical examination reveals a distressed man,
who is afebrile, but tachypneic, with scant diffuse abdominal
tenderness to palpation. Rectal examination is positive for blood. Laboratory
studies reveal a metabolic acidosis with an elevated serum
Iactate.


Question 3 of 5
If this patient's disease were drug-induced, which of the following agents would
most likely be responsible?
/ A. Acetaminophen
/ B. Amiodarone
/ C. Cocaine
/ D. Dexamethasone
/ E. Nitroglycerin

Explanation - Q: 3.3 Close

The correct answer is C. Cocaine is a sympathomimetic drug that indirectly
acts on both the alpha and beta adrenergic receptors on the vasculature. As
such, cocaine may cause vasospasm in the abdominal vasculature leading
to infarction and ischemic colitis. Similar vasospastic events may occur in the
coronary vasculature, leading to myocardial infarction.
Acetaminophen (choice A) is an analgesic, and would not play a role in
producing ischemic colitis.
Amiodarone (choice B) is an antiarrhythmic, and would not contribute to
ischemic colitis.
Dexamethasone (choice D) is a steroidal anti-inflammatory drug. Not only
would this medication not cause ischemic colitis, it might mask the symptoms
due to its potent anti-inflammatory properties.
Nitroglycerin (choice E) is a venodilator, and would not contribute to
ischemic colitis. As a venodilator, nitroglycerin is used to treat coronary
ischemia by reducing cardiac preload.
A 64-year-old man with a history of coronary artery disease (CAD) comes to the
emergency department with the acute onset of severe,
constant, Iower abdominal pain and rectal bleeding. He reports that he previously
has had several episodes of similar, but less severe pain.
About 12 hours after the onset of pain, the patient began passing copious bright
red blood per rectum. He denies nausea, vomiting, sick
contacts, or foreign traveI. Initial physical examination reveals a distressed man,
who is afebrile, but tachypneic, with scant diffuse abdominal
tenderness to palpation. Rectal examination is positive for blood. Laboratory
studies reveal a metabolic acidosis with an elevated serum
Iactate.
Question 4 of 5
While the patient is in the emergency department, the pain becomes increasingly
severe. Several hours after his initial examination, the
patient becomes febrile and is now exquisitely tender to palpation. He writhes in
pain when the physician jostles the bed. Air is seen under
the diaphragm in an upright chest x-ray film. These new findings suggest which
of the following?
/ A. Abdominal aortic aneurysm
/ B. Bowel obstruction
/ C. Cholecystitis
/ D. Hypovolemia
/ E. Perforation with peritonitis

Explanation - Q: 3.4 Close

The correct answer is E. This patient has experienced a bowel perforation.
Air under the diaphragm in an upright chest film provides definitive evidence
that a hollow viscus has ruptured. Air near the liver on a left lateral decubitus
(patient lays with the left side down) is an alternative study to demonstrate
perforation. Spillage from the perforated bowel has irritated and inflamed the
peritoneum, resulting in peritonitis. Symptoms of peritonitis include extreme,
sharp pain exacerbated by jostling (patients often report that the bumpy ride
to the emergency department caused extreme pain). Patients will be
exquisitely tender to palpation and percussion and may have abdominal
rigidity. Fever typically accompanies peritonitis.
While an abdominal aortic aneurysm or AAA (choice A) presents as acute
abdominal pain, this pain is described as tearing and may radiate to the
back. A pulsatile abdominal mass may be palpated. The air on the chest film
is also inconsistent with AAA.
This patient does not have bowel obstruction (choice B). Signs and
symptoms of bowel obstruction include: nausea, vomiting, intermittent
abdominal pain, hypovolemia, abdominal distention, absence of flatus, and a
"step ladder" bowel pattern on abdominal films.
Cholecystitis (choice C) typically presents as right upper quadrant (RUQ)
pain, fever, and jaundice. Patients usually have a history of colicky RUQ
pain.
While the patient is at risk for hypovolemia (choice D), none of the
symptoms listed typify hypovolemia. Signs and symptoms of mild to
moderate hypovolemia include malaise, dry mouth, thirst, decreased skin
turgor, tachycardia, hypotension, and decreased urine output.
A 64-year-old man with a history of coronary artery disease (CAD) comes to the
emergency department with the acute onset of severe,
constant, Iower abdominal pain and rectal bleeding. He reports that he previously
has had several episodes of similar, but less severe pain.
About 12 hours after the onset of pain, the patient began passing copious bright
red blood per rectum. He denies nausea, vomiting, sick
contacts, or foreign traveI. Initial physical examination reveals a distressed man,
who is afebrile, but tachypneic, with scant diffuse abdominal
tenderness to palpation. Rectal examination is positive for blood. Laboratory
studies reveal a metabolic acidosis with an elevated serum
Iactate.
Question 5 of 5
Upon surgical exploration of the abdomen, the colon is dull and dusky from the
mid transverse colon to the rectum. The patient has occluded
which of the following vessels?
/ A. Celiac trunk
/ B. Cystic artery
/ C. External iliac artery
/ D. Inferior mesenteric artery
/ E. Superior mesenteric artery

Explanation - Q: 3.5 Close

The correct answer is D. The inferior mesenteric artery distributes blood to
the embryologic hindgut. This includes the distal 1/3 of the transverse colon
to the rectum. The rectum is spared because it receives circulation from the
inferior rectal artery (not mesenteric).
The celiac trunk (choice A) supplies the embryologic foregut. The first three
branches include the splenic artery, the left gastric artery, and the common
hepatic artery. This patient has no findings in this distribution.
The cystic artery (choice B) supplies the gall bladder. There are no gall
bladder findings in this case.
The external iliac artery (choice C) gives rise to the vessels of the lower
extremity. Symptoms of occlusion or stenosis might include buttock and thigh
pain exacerbated by walking. Severe stenosis might give patients buttock
and thigh pain, even at rest.
The superior mesenteric artery (choice E) supplies the embryologic hindgut.
This extends from the duodenum to the proximal 2/3 of the transverse colon.


A 45-year-old man goes to an emergency department because he is
experiencing severe abdominal pain, which is radiating straight through
to his back. The pain began several hours after an admitted alcoholic binge, and
has not changed in position, although it has become worse.
Question 1 of 5
Which of the following would be the most likely cause of this type of pain?
/ A. Acute appendicitis
/ B. Acute hepatitis
/ C. Acute pancreatitis
/ D. Chronic hepatitis
/ E. Myocardial infarction

Explanation - Q: 4.1 Close

The correct answer is C. The typical pain described occurs in
approximately 50% of patients with acute pancreatitis. Other patients may
have milder pain or even, uncommonly, pain first felt in the lower abdomen.
The pain of acute appendicitis (choice A) is often felt first as referred pain
near the umbilicus, with tenderness on palpation in the left lower quadrant.
Acute hepatitis (choice B) can cause pain referred to the right shoulder.
Chronic hepatitis (choice D) does not usually cause pain.
Myocardial infarction (choice E) can cause substernal pain and pain
radiating to the left shoulder.

A 45-year-old man goes to an emergency department because he is
experiencing severe abdominal pain, which is radiating straight through
to his back. The pain began several hours after an admitted alcoholic binge, and
has not changed in position, although it has become worse.
Question 2 of 5
In addition to alcohol use, which of the following is a common predisposing factor
for this patient's disease?
/ A. Biliary tract stones
/ B. Duodenal cancer
/ C. Gastric carcinoma
/ D. Kidney stones
/ E. Peptic ulcer


Explanation - Q: 4.2 Close

The correct answer is A. The overwhelmingly most common predisposing
factors for acute pancreatitis are gallstones (more specifically tiny ones that
lodge in the extrahepatic bile duct system) and alcohol abuse.
Rarely, nearby cancers (choices B and C) can occlude the pancreatic duct
system and cause a secondary acute pancreatitis.
Kidney stones (choice D) have no relationship with pancreatitis.
Peptic ulcers (choice E) that erode into the pancreas can uncommonly
secondarily inflame the pancreas.


A 45-year-old man goes to an emergency department because he is
experiencing severe abdominal pain, which is radiating straight through
to his back. The pain began several hours after an admitted alcoholic binge, and
has not changed in position, although it has become worse.
Question 3 of 5
Marked serum elevation of which of the following markers would most strongly
substantiate the likely diagnosis?
/ A. Acid phosphatase
/ B. Amylase
/ C. Aspartate aminotransferase
/ D. AIkaline phosphatase
/ E. Creatinine kinase

Explanation - Q: 4.3 Close

The correct answer is B. The usual markers for pancreatitis are amylase
and lipase. Marked elevation of amylase usually means either pancreatic
disease or salivary gland disease; lipase will be elevated in pancreatic
disease but not salivary gland disease. If you see elevated amylase on a
USMLE question, you should think of pancreatitis or salivary gland disease
(mumps, salivary gland stone). However, you should be aware, for your
general medical knowledge, that modest elevations of amylase can be seen
in a much wider variety of settings (often reflecting either subclinical
pancreatic damage or hemoconcentration of pancreatic enzymes), including
GI obstruction, mesenteric thrombosis and infarction, macroamylasemia (a
genetic condition with abnormal amylase), renal disease, ruptured tubal
pregnancy, lung cancer, acute alcohol ingestion, and following abdominal
surgery.
Associate acid phosphatase (choice A) with diseases involving the prostate
and, to lesser degrees, bone, the heart, platelets, and the liver.
Associate aspartate aminotransferase (choice C) with diseases of the heart,
muscle, liver, pancreas (though not as important for diagnosis as amylase
and lipase), and brain.
Associate alkaline phosphatase (choice D) with diseases of bone, liver, and
to lesser degrees, lung and heart.
Associate creatinine kinase (choice E) with diseases of the heart, muscle,
brain, and the general body (trauma, surgery).
A 45-year-old man goes to an emergency department because he is
experiencing severe abdominal pain, which is radiating straight through
to his back. The pain began several hours after an admitted alcoholic binge, and
has not changed in position, although it has become worse.
Question 4 of 5
The patient has a severe course that requires treatment in an ICU. CIinically, he
appears similar to patients with sepsis, with fever, elevated
white count, hypotension, increased pulse rate, shallow and rapid breathing,
oliguria, and a blunted sensorium, in addition to his pain and
abdominal tenderness. These clinical findings are most likely related to which of
the following?
/ A. Activation of the inflammatory cascade
/ B. AIcohol withdrawal symptoms
/ C. AIIergic reaction to alcohol
/ D. Drug toxicity effect
/ E. Secondary infection with mixed flora gut bacteria
Explanation - Q: 4.4 Close

The correct answer is A. Acute pancreatitis can either be relatively mild, or
a severe condition that may cause death. It is thought that, in severe cases,
leakage of enzyme-containing pancreatic secretions into the tissues/and or
blood stream causes cleavage of precursors, thus strongly activating the
complement and inflammatory cascades. These, in turn, produce abundant
cytokines, which worsen the symptoms. The clinical result is similar to
sepsis, with risk of multi-organ failure and death. The treatment of acute
pancreatitis is primarily supportive, and may include careful attention to fluid
resuscitation, oxygen supplementation, cardiovascular support, dialysis,
management of electrolyte abnormalities, pain control, and total parenteral
nutrition.
Alcohol allergy (choice C) or withdrawal (choice B) do not play any
additional part in most of these symptoms once the pancreatitis has
developed.
Infection (choice E) and drug toxicity (choice D) are also not a necessary
part of the clinical picture, although physicians may worry that the patient's
general clinical status is masking other, potentially more treatable, problems.

A 45-year-old man goes to an emergency department because he is
experiencing severe abdominal pain, which is radiating straight through
to his back. The pain began several hours after an admitted alcoholic binge, and
has not changed in position, although it has become worse.
Question 5 of 5
The patient's condition resolves in about two weeks, but he continues to drink
after leaving the hospitaI. When seen several years later, he
has had a number of similar episodes, and now has chronic severe abdominal
pain. CT scan demonstrates a single, smooth-walled, fluid
filled space in the tail of the pancreas, which can be reached by the radiologist
for CT-guided aspiration with an approach from the back. The
fluid aspirated is yellowish, clear, and acellular. Which of the following is the most
likely diagnosis?
/ A. Pancreatic microcystic adenoma
/ B. Pancreatic mucinous cystadenocarcinoma
/ C. Pancreatic mucinous cystadenoma
/ D. Pancreatic pseudocyst
/ E. Pancreatic solid-cystic tumor

Explanation - Q: 4.5 Close

The correct answer is D. Pancreatic pseudocyst is a fairly common
complication of both acute and chronic pancreatitis, and appears to develop
when trapping of pancreatic digestive juices (containing amylase, lipase, and
proteases) causes a "digestion" of part of the pancreas, leaving a fluid filled
cystic space. The term "pseudocyst", rather than "cyst", is used by purists
because the space does not have an epithelial lining, and is hence not a
"true cyst". Pseudocysts are usually solitary and typically measure 5-10 cm in
diameter. They can be surgically excised (and the surrounding tissue will
typically show evidence of chronic pancreatitis in long-standing cases) or
sometimes, if the anatomy is favorable, drained into adjacent hollow viscera.
Some are medically managed if small.
Most true neoplasms of the pancreas contain (often large numbers of)
smaller, multiple, cysts. These tumors can be benign or malignant, and the
ones with mucus-secreting epithelium (choices B and C) are more common
than those with a serous lining (choices A and E).

A 17-year-old boy is taken to the emergency department because he has
developed severe abdominal pain. The pain began abruptly
several hours previously, and was felt initially in the periumbilical region, but later
shifted to the right lower quadrant. The boy had initially felt
somewhat nauseous, but this has passed. On physical examination, he is noted
to have localized pain on cough and to be running a low-
grade fever.
Question 1 of 5
Examination of the abdomen demonstrates right lower quadrant tenderness at
the junction of the middle and outer thirds of the line joining the
umbilicus to the anterior superior spine of the iliac. This location is known as
which of the following?
/ A. Gubernaculum
/ B. Langer's line
/ C. Linea alba
/ D. McBurney's point
/ E. Tunica albuginea

Explanation - Q: 5.1 Close

The correct answer is D. The point described is McBurney's point, which
overlies the location of the appendix in most individuals.
The gubernaculum (choice A) is the fibrous cord that connects the primordial
testis or ovary to the anterolateral abdominal wall.
Langer's lines (choice B) are the cleavage lines of the skin.
The linea alba (choice C) is a sheet-like aponeurosis that covers the anterior
abdominal wall.
The tunica albuginea (choice E) is a tough fibrous coat that covers the testis.
A 17-year-old boy is taken to the emergency department because he has
developed severe abdominal pain. The pain began abruptly
several hours previously, and was felt initially in the periumbilical region, but later
shifted to the right lower quadrant. The boy had initially felt
somewhat nauseous, but this has passed. On physical examination, he is noted
to have localized pain on cough and to be running a low-
grade fever.
Question 2 of 5
Which of the following is the most likely diagnosis?
/ A. Appendicitis
/ B. Diverticulitis
/ C. Gallstones
/ D. Rectal ulcer
/ E. Renal colic

Explanation - Q: 5.2 Close

The correct answer is A. This patient has a typical presentation for
appendicitis, and the diagnosis is confirmed by the presence of localized
tenderness at McBurney's point.
Diverticulitis (choice B) is usually a disease of middle-aged or older
individuals and most commonly affects the left-lower quadrant.
Symptomatic gallstone disease (choice C) causes pain and tenderness in
the right upper quadrant.
Rectal ulcer (choice D) causes pain with stool movement, but does not
usually produce tenderness identifiable on abdominal examination.
Renal colic (choice E) usually produces flank or lower back pain.

A 17-year-old boy is taken to the emergency department because he has
developed severe abdominal pain. The pain began abruptly
several hours previously, and was felt initially in the periumbilical region, but later
shifted to the right lower quadrant. The boy had initially felt
somewhat nauseous, but this has passed. On physical examination, he is noted
to have localized pain on cough and to be running a low-
grade fever.
Question 3 of 5
The patient also exhibits an increase in pain in the right lower quadrant from the
passive extension of the right hip joint. This finding suggests
that the inflammation also involves which of the following?
/ A. BIadder
/ B. External oblique muscle
/ C. Femur
/ D. IIiopsoas muscle
/ E. Transverse abdominal muscle

Explanation - Q: 5.3 Close

The correct answer is D. This patient has a "positive psoas sign," which is
an increase in pain from passive extension of the right hip joint. This
maneuver stretches the iliopsoas muscle, which lies behind the appendix
and can become secondarily inflamed when the appendiceal inflammation
extends through the serosa. The psoas sign is clinically useful in both
confirming the appendix as the probable origin of the patient's pain, and
indicating that the inflammation is transmural and that the risk of rupture and
peritonitis is increased.
The bladder (choice A) is located more medially, and is usually not affected
by appendicitis.
The external oblique (choice B) and transverse abdominal (choice E)
muscles are in the anterior and lateral abdominal walls, and do not usually
become inflamed with appendicitis.
The femur (choice C) is moved during the extension of the right hip joint, but
is not the source of the pain.

A 17-year-old boy is taken to the emergency department because he has
developed severe abdominal pain. The pain began abruptly
several hours previously, and was felt initially in the periumbilical region, but later
shifted to the right lower quadrant. The boy had initially felt
somewhat nauseous, but this has passed. On physical examination, he is noted
to have localized pain on cough and to be running a low-
grade fever.
Question 4 of 5
The patient is prepared for immediate surgery. Cefotaxime is administered
before, during, and after surgery. The specimen, once removed,
is sent to the laboratory for pathology and bacteriologic culture. A malodorous
pus surrounds the serosa of the surgical specimen, and a
mixed gram-negative flora is cultured. Rapid enzyme tests for beta-Iactamase
production are positive. Which of the following drugs should be
added to the initial cefotaxime regimen?
/ A. Bacitracin
/ B. CIavulanic acid
/ C. CIindamycin
/ D. Isoniazid
/ E. Vancomycin

Explanation - Q: 5.4 Close

The correct answer is B. Clavulanic acid is a beta-lactamase inhibitor,
which when administered with beta lactam agents, irreversibly binds and
inactivates bacterial beta-lactamases, thereby permitting the companion drug
to disrupt bacterial cell wall synthesis. Suspected appendicitis is usually
treated with prompt appendectomy, since delay is associated with increased
risk of potentially life-threatening peritonitis and sepsis.
Bacitracin (choice A) is not correct, since this drug inhibits bacterial cell wall
synthesis by binding to and inhibiting the dephosphorylation of a membrane-
bound lipid pyrophosphate. Gram-negative bacteria are resistant to this
agent, and it would not have a synergistic effect if administered with a third
generation cephalosporin.
Clindamycin (choice C) is not correct, because this drug blocks protein
elongation by binding to the 50S ribosome. Although it is effective against
anaerobic gram-negative bacilli, it would not have a complementary effect
when administered with a third generation cephalosporin.
Isoniazid (choice D) is not correct because it inhibits the synthesis of mycolic
acids for the cell wall of actively dividing Mycobacteria. It would not be
effective in the flora of this patient's gut, nor would it act synergistically with
third generation cephalosporins.
Vancomycin (choice E) is not correct because it disrupts cell wall synthesis
in growing gram-positive bacteria. It would not be effective against the flora
of this patient's gut, nor would it act synergistically with third generation
cephalosporins.
A 17-year-old boy is taken to the emergency department because he has
developed severe abdominal pain. The pain began abruptly
several hours previously, and was felt initially in the periumbilical region, but later
shifted to the right lower quadrant. The boy had initially felt
somewhat nauseous, but this has passed. On physical examination, he is noted
to have localized pain on cough and to be running a low-
grade fever.
Question 5 of 5
The patient's postoperative recovery is uneventfuI, but 10 days after discharge,
he returns to his physician complaining of continuous low-
grade fever. An abscess is drained transrectally, and organisms are
cultured from the pus. Which of the following is an attribute of
this organism that makes it an important abscess former?
/ A. It is an anaerobe
/ B. It is an intracellular pathogen
/ C. Its endotoxin lacks 2,3-ketodeoxyoctonate
/ D. Mycolic acid
/ E. Prodigious capsule

Explanation - Q: 5.5 Close

The correct answer is E. Prevotella (Bacteroides) is a frequent cause of
abscesses in the intestinal tract because it is a normal flora organism and
produces a large capsule, which impedes phagocytosis.
Although the genus is anaerobic (choice A), it is not this attribute which
causes its formation of abscesses.
Prevotella is extracellular, not an intracellular pathogen (choice B).
Although Prevotella does indeed have this type of endotoxin (choice C), the
absence of this molecule decreases the toxicity of the toxin, and does not
contribute to its proclivity toward abscess formation.
Mycobacteria, and not other genera such as Prevotella, are known for their
long-chain fatty acids (mycolic acids; choice D).


The parents of a 13-month-old boy are contacted by a local social agency because the day
care they are using has reported them for
suspected child abuse. The abuse was suspected because the day care personnel thought
that the toddler had "too many bruises." The
parents deny the abuse, and promptly arrange to have their child seen by the chairman of
the pediatrics department in a clinic run by a
university medical center. On physical examination, the child is noted to have multiple
large bruises on his legs, arms, and buttocks. No skin
abrasions are seen, and no "pattern marks" (suggestive of being hit by an object such as a
belt buckle or rod) are seen. A blood smear isunremarkable. PTT is prolonged, and PT
and bleeding times are normaI.

Question 1 of 6

Which of the following is the most likely diagnosis?
/ A. Acute myelogenous leukemia
/ B. Disseminated intravascular coagulation
/ C. Hemophilia
/ D. Thrombocytopenia
/ E. Von Willebrand's disease



Explanation - Q: 1.1 Close

The correct answer is C. This child has hemophilia, which is an X-linked
recessive disorder of clotting. The severity of the symptoms experienced can
vary with the degree of enzymatic block in the production of the affected
clotting factor. Unfortunately, some cases may reach medical attention only
when the child begins to experience large numbers of falls when learning to
walk, and the parents are suspected of child abuse. The severity of the
symptoms can vary markedly from case to case, since even as little as 2-5%
of the normal levels of the missing clotting factor can markedly reduce
symptoms. Symptoms can include bruising, spontaneous bleeding, bleeding
into joints with associated pain and swelling, gastrointestinal and urinary tract
hemorrhage, and prolonged bleeding from cuts, tooth extractions, or surgery.
Acute myelogenous leukemia (choice A) and thrombocytopenia (choice D)
can also produce a tendency to bruise, but would not be associated with
normal peripheral smears.
Disseminated intravascular coagulation (choice B) is usually seen in patients
with other severe diseases, such as sepsis or complicated pregnancy.
Von Willebrand's disease (choice E) tends to cause oozing blood after injury
rather than bruising, and is associated with a long bleeding time.



The parents of a 13-month-old boy are contacted by a local social agency
because the day care they are using has reported them for
suspected child abuse. The abuse was suspected because the day care
personnel thought that the toddler had "too many bruises." The
parents deny the abuse, and promptly arrange to have their child seen by the
chairman of the pediatrics department in a clinic run by a
university medical center. On physical examination, the child is noted to have
multiple large bruises on his legs, arms, and buttocks. No skin
abrasions are seen, and no "pattern marks" (suggestive of being hit by an object
such as a belt buckle or rod) are seen. A blood smear is
unremarkable. PTT is prolonged, and PT and bleeding times are normaI.

Question 2 of 6

Deficiency of which of the following has been linked to this patient's condition?
/ A. Factor VII
/ B. Factor VIII
/ C. Factor X
/ D. Factor XII
/ E. Factor XIII

Explanation - Q: 1.2 Close

The correct answer is B. Hemophilia can be caused by a deficiency of
either factor VIII (classic hemophilia or hemophilia A) or factor IX (Christmas
disease or hemophilia B). The two variations are very similar clinically,
although the appropriate factor must be replaced when treating an acute
bleeding episode. Both of these factors are in the intrinsic coagulation
pathway. The other factors listed in the choices are not involved.


The parents of a 13-month-old boy are contacted by a local social agency
because the day care they are using has reported them for
suspected child abuse. The abuse was suspected because the day care
personnel thought that the toddler had "too many bruises." The
parents deny the abuse, and promptly arrange to have their child seen by the
chairman of the pediatrics department in a clinic run by a
university medical center. On physical examination, the child is noted to have
multiple large bruises on his legs, arms, and buttocks. No skin
abrasions are seen, and no "pattern marks" (suggestive of being hit by an object
such as a belt buckle or rod) are seen. A blood smear is
unremarkable. PTT is prolonged, and PT and bleeding times are normaI.

Question 3 of 6

This child's condition is due to a defective enzyme encoded on which of the
following chromosomes?
/ A. 13
/ B. 18
/ C. 21
/ D. X
/ E. Y


Explanation - Q: 1.3 Close

The correct answer is D. Both the hemophilia A and hemophilia B genes
are located on the X chromosome. While almost all affected individuals are
boys (rare exceptions are girls with a carrier mother and an affected father),
the Y chromosome (choice E) is not involved.
Associate chromosome 13 (choice A) with the trisomy known as Patau
syndrome.
Associate chromosome 18 (choice B) with the trisomy known as Edwards
syndrome.
Associate chromosome 21 (choice C) with the trisomy known as Down
syndrome.

The parents of a 13-month-old boy are contacted by a local social agency
because the day care they are using has reported them for
suspected child abuse. The abuse was suspected because the day care
personnel thought that the toddler had "too many bruises." The
parents deny the abuse, and promptly arrange to have their child seen by the
chairman of the pediatrics department in a clinic run by a
university medical center. On physical examination, the child is noted to have
multiple large bruises on his legs, arms, and buttocks. No skin
abrasions are seen, and no "pattern marks" (suggestive of being hit by an object
such as a belt buckle or rod) are seen. A blood smear is
unremarkable. PTT is prolonged, and PT and bleeding times are normaI.

Question 4 of 6

Which of the following can temporarily increase secretion of the needed factor in
some patients with a milder form of this disease?
/ A. Desmopressin
/ B. Epsilon-aminocaproic acid
/ C. Factor VIII concentrate
/ D. Factor IX concentrate
/ E. Fresh frozen plasma
Explanation - Q: 1.4 Close

The correct answer is A. Desmopressin can temporarily raise factor VIII
levels in a patient with mild classic hemophilia by causing the release of
factor VIII stored in the body. The use of desmopressin before dental work or
small surgical procedures may allow such procedures to be performed
without using factor VIII concentrates.
Epsilon-aminocaproic acid (choice B) is a anti-fibrinolytic agent used to slow
the degradation of blood clots, particularly in patients with hemophilia B.
Fresh frozen plasma (choice E) contains both factor VIII (choice C) and
factor IX (choice D), both of which are also available as concentrates. These
products are used to raise clotting factor levels, usually in patients who are
either actively bleeding or who are expected to be actively bleeding because
of surgery or dental procedures.

The parents of a 13-month-old boy are contacted by a local social agency
because the day care they are using has reported them for
suspected child abuse. The abuse was suspected because the day care
personnel thought that the toddler had "too many bruises." The
parents deny the abuse, and promptly arrange to have their child seen by the
chairman of the pediatrics department in a clinic run by a
university medical center. On physical examination, the child is noted to have
multiple large bruises on his legs, arms, and buttocks. No skin
abrasions are seen, and no "pattern marks" (suggestive of being hit by an object
such as a belt buckle or rod) are seen. A blood smear is
unremarkable. PTT is prolonged, and PT and bleeding times are normaI.

Question 5 of 6

The patient continues to experience persistent bleeding. Which of the following
would be the most appropriate pharmacotherapy?
/ A. Aminocaproic acid
/ B. Aspirin
/ C. Desmopressin
/ D. High-dose IV immune globulin
/ E. Prednisone

Explanation - Q: 1.5 Close

The correct answer is A. Aminocaproic acid is a synthetic hemostatic with a
specific antifibrinolysis action. The medication inhibits plasminogen activator
substance, which is concerned with the destruction of clots. This medication
does not control bleeding caused by a loss of vascular integrity.
Aminocaproic acid is indicated for control of excessive bleeding resulting
from systemic hyperfibrinolysis, well as for prevention of bleeding in
hemophiliacs undergoing a surgical procedure. It is contraindicated for use in
patients with severe renal impairment or active disseminated intravascular
clotting.
Aspirin (choice B) is absolutely contraindicated for use in this patient since it
would result in a more severe bleed.
Desmopressin is a synthetic analog of arginine vasopressin (choice C), the
naturally occurring human antidiuretic hormone (ADH). This agent is
generally used only in milder forms of the disease.
High-dose IV immune globulin (choice D) is most commonly used in the
treatment of ITP (idiopathic thrombocytopenic purpura) to rapidly raise the
platelet count.
Prednisone (choice E) is a glucocorticoid indicated for the treatment of
idiopathic thrombocytopenic purpura (IV dosing only) as well as in the
treatment of a variety of inflammatory conditions. It would be ineffective in
this patient.

The parents of a 13-month-old boy are contacted by a local social agency
because the day care they are using has reported them for
suspected child abuse. The abuse was suspected because the day care
personnel thought that the toddler had "too many bruises." The
parents deny the abuse, and promptly arrange to have their child seen by the
chairman of the pediatrics department in a clinic run by a
university medical center. On physical examination, the child is noted to have
multiple large bruises on his legs, arms, and buttocks. No skin
abrasions are seen, and no "pattern marks" (suggestive of being hit by an object
such as a belt buckle or rod) are seen. A blood smear is
unremarkable. PTT is prolonged, and PT and bleeding times are normaI.

Question 6 of 6

In approximately what year did effective screening of blood products begin to
limit the transmission of HIV and hepatitis B in these patients?
/ A. 1955
/ B. 1965
/ C. 1975
/ D. 1985
/ E. 1995

Explanation - Q: 1.6 Close

The correct answer is D. This is clinically important information, because
hemophiliac children who were diagnosed after 1985 have had very little
chance of developing AIDS or hepatitis as a complication of their disease
(they may still get exposures by other routes, e.g., sexual), while these were
major problems in patients who were treated earlier. Fortunately, recently
developed recombinant DNA factor VIII products are coming into increasing
use, and offer the chance of providing truly adequate hemostatic control
without any increased viral risk.

Following a viral illness, a previously healthy 3-year-old child develops multiple
petechiae and purpura, and is noted to be oozing blood from
the mouth. Prothrombin and activated partial thromboplastin times are within
normal limits. Review of a peripheral smear demonstrates that
platelet counts are markedly decreased, but all the platelets that are present
appear morphologically normaI. The remainder of the blood
smear and additional bone marrow studies are normaI.

Question 1 of 4

Which of the following is the most likely diagnosis?
/ A. Disseminated intravascular coagulation
/ B. Hemophilia A
/ C. Hemophilia B
/ D. Idiopathic thrombocytopenic purpura
/ E. Von Willebrand disease

Explanation - Q: 1.1 Close

The correct answer is D. Idiopathic thrombocytopenic purpura (ITP), also
known as immune thrombocytopenic purpura, now that the immunologic
basis of the disease has been better defined, is a hemorrhagic disorder that
can present in two very different forms in children and adults. Children (peak
ages 2-4 years) tend to present with acute, self-limited thrombocytopenia
that often follows a viral (or immunization) trigger. In contrast, adults (peak
ages 20-50 years) tend to have an insidious development of
thrombocytopenia that may persist for long periods. In both types of ITP, the
hemorrhagic diathesis is the result of a pure thrombocytopenia that does not
affect prothrombin (PT) and activated partial thromboplastin (PTT) times
because the blood clotting factors are present in normal quantities.
In disseminated intravascular coagulation (choice A), platelets are often
decreased as a result of consumption, but PT and PTT are prolonged.
In hemophilia A and B (choices B and C), platelets are present in normal
numbers, PT is normal, and PTT is long.
In von Willebrand disease (choice E), platelet counts are normal, but platelet
function is decreased because of decreased or abnormal von Willebrand
factor.

Following a viral illness, a previously healthy 3-year-old child develops multiple
petechiae and purpura, and is noted to be oozing blood from
the mouth. Prothrombin and activated partial thromboplastin times are within
normal limits. Review of a peripheral smear demonstrates that
platelet counts are markedly decreased, but all the platelets that are present
appear morphologically normaI. The remainder of the blood
smear and additional bone marrow studies are normaI.

Question 2 of 4

Which of the following tests would be most helpful in confirming the probable
diagnosis?
/ A. D-dimer levels
/ B. Factor VIII concentration
/ C. Factor IX concentration
/ D. IgG autoantibodies on platelet surfaces
/ E. Total plasma von Willebrand factor antigen

Explanation - Q: 1.2 Close

The correct answer is D. While ITP is mostly a diagnosis of exclusion, the
presence of IgG autoantibodies on the platelet surface can be an important
confirmatory test result.
High D-dimer levels (choice A) suggest disseminated intravascular
coagulation.
Low factor VIII (choice B) suggests hemophilia A.
Low factor IX (choice C) suggests hemophilia B.
Low or abnormal von Willebrand factor antigen (choice E) suggests von
Willebrand disease.


Following a viral illness, a previously healthy 3-year-old child develops multiple
petechiae and purpura, and is noted to be oozing blood from
the mouth. Prothrombin and activated partial thromboplastin times are within
normal limits. Review of a peripheral smear demonstrates that
platelet counts are markedly decreased, but all the platelets that are present
appear morphologically normaI. The remainder of the blood
smear and additional bone marrow studies are normaI.

Question 3 of 4

Most patients with this condition have antibodies to which of the following?
/ A. PIatelet ADP
/ B. PIatelet membrane cholesterol
/ C. PIatelet membrane glycoproteins
/ D. PIatelet membrane lipids
/ E. PIatelet thromboxane A2

Explanation - Q: 1.3 Close

The correct answer is C. The autoantibodies are usually directed against
platelet membrane glycoproteins.
ADP (choice A) and thromboxane A2 (choice E) are involved in platelet
aggregation.
Cholesterol (choice B) and lipids (choice D) are components of virtually all
mammalian plasma membranes.

Following a viral illness, a previously healthy 3-year-old child develops multiple
petechiae and purpura, and is noted to be oozing blood from
the mouth. Prothrombin and activated partial thromboplastin times are within
normal limits. Review of a peripheral smear demonstrates that
platelet counts are markedly decreased, but all the platelets that are present
appear morphologically normaI. The remainder of the blood
smear and additional bone marrow studies are normaI.

Question 4 of 4

Approximately what percentage of children with this condition will undergo
spontaneous remission?
/ A. Less than 5%
/ B. 15%
/ C. 30%
/ D. 60%
/ E. More than 80%

Explanation - Q: 1.4 Close

The correct answer is E. More than 80% of childhood cases of ITP undergo
spontaneous remission, which frequently occurs within 2 months.
Spontaneous remission is uncommon (2%) in adults, but more than 60% will
eventually recover with treatment. Treatment in children is controversial;
treatment in adults can involve splenectomy, glucocorticoids, and
intravenous IgG. Approximately 1% of children with ITP and 5% of adults
with ITP die from hemorrhagic complications, including intracranial
hemorrhage.

A 23-year-old man is admitted to the hospital through the emergency department
with probable appendicitis. He has been having right lower
quadrant abdominal pain for several days, which has been becoming
increasingly worse. His temperature 39.2 C (102.6 F), blood pressure
is 80/40 mm Hg, pulse is 120/min, and respirations are 35/min. The abdomen is
rigid with guarding. Multiple petechiae and purpura are
present, and the patient is oozing blood from his oral mucosa. According to the
patient's wife, he has not had bleeding problems in the past.

Question 1 of 5

The fact the abdomen is rigid with guarding suggests which of the following?
/ A. Colon cancer
/ B. Diverticulitis
/ C. Liver failure
/ D. Peritonitis
/ E. Shock

Explanation - Q: 2.1 Close

The correct answer is D. The usual reason for a patient to have a rigid
abdomen is that peritonitis is present, and is causing severe pain related to
peritoneal nerve fiber stimulation. The probable cause of the peritonitis is a
ruptured appendix. This patient is also probably in shock (choice E), as
indicated by the hypotension with increased respirations and heart rate, but
this would not cause the abdominal guarding. The other answers are
distracters.

A 23-year-old man is admitted to the hospital through the emergency department
with probable appendicitis. He has been having right lower
quadrant abdominal pain for several days, which has been becoming
increasingly worse. His temperature 39.2 C (102.6 F), blood pressure
is 80/40 mm Hg, pulse is 120/min, and respirations are 35/min. The abdomen is
rigid with guarding. Multiple petechiae and purpura are
present, and the patient is oozing blood from his oral mucosa. According to the
patient's wife, he has not had bleeding problems in the past.

Question 2 of 5

Given this patient's presentation, which of the following is the most likely cause of
his petechiae, purpura, and mucosal blood oozing?
/
A. Disseminated intravascular coagulation
/ B. Hemophilia A
/ C. Idiopathic thrombocytopenic purpura
/ D. Von Willebrand disease
/ E. Wiskott-AIdrich syndrome

Explanation - Q: 2.2 Close

The correct answer is A. You should suspect disseminated intravascular
coagulation (DIC) in patients who are seriously ill with other disease who
then develop evidence of a coagulopathy. In this case, sepsis is the probable
inciting event. DIC is thought to occur in 30-50% of patients with sepsis, and
may, in many cases, be triggered by a reaction to gram-negative or
staphylococcal cell wall material. Other settings in which DIC can be a
complication include major trauma, obstetric complications, acute myelocytic
leukemias, disseminated carcinomas, burns, massive transfusions, acute
hepatic failure, myocardial infarction, and inflammatory conditions (e.g.,
ulcerative colitis, rheumatoid arthritis, Crohn disease, sarcoidosis).
The other conditions listed in the choices would not be expected to be
specifically associated with sepsis, although for medical/legal and safety
reasons, supporting diagnostic tests should be ordered to exclude previously
undiagnosed disease.
Hemophilia (choice B) is a hereditary coagulopathy with X-linked genetics
that typically presents in early childhood with extensive tissue hemorrhages
and hemoarthroses following minor trauma.
Idiopathic thrombocytopenic purpura (choice C) is an acquired autoimmune
disease characterized, in adults, by chronic coagulopathy with petechiae,
purpura, and mucosal bleeding. It is not usually associated with any other
systemic diseases, such as this patient's appendicitis and probable sepsis.
Von Willebrand disease (choice D) is hereditary, with usually autosomal
dominant genetics. It typically causes prolonged menstrual bleeding and
prolonged bleeding from small cuts and surgical procedures. Petechiae and
ecchymoses are less often seen.
Wiskott-Aldrich syndrome (choice E) is a hereditary disease with X-linked
genetics, thrombocytopenia, eczema, and recurrent infection that tends to
present in children with bloody diarrhea.
A 23-year-old man is admitted to the hospital through the emergency department
with probable appendicitis. He has been having right lower
quadrant abdominal pain for several days, which has been becoming
increasingly worse. His temperature 39.2 C (102.6 F), blood pressure
is 80/40 mm Hg, pulse is 120/min, and respirations are 35/min. The abdomen is
rigid with guarding. Multiple petechiae and purpura are
present, and the patient is oozing blood from his oral mucosa. According to the
patient's wife, he has not had bleeding problems in the past.

Question 3 of 5

Which of the following findings on screening laboratory tests would be most
consistent with the probable diagnosis?
/ A. Decreased platelet count, increased prothrombin time, increased activated
partial thromboplastin time
/ B. Increased platelet count, increased prothrombin time, normal activated
partial thromboplastin time
/ C. Increased platelet count, normal prothrombin time, increased activated
partial thromboplastin time
/ D. Normal platelet count, increased prothrombin time, increased activated
partial thromboplastin time
/ E. Normal platelet count, normal prothrombin time, normal activated partial
thromboplastin time

Explanation - Q: 2.3 Close

The correct answer is A. Platelet count is invariably decreased (and usually
obvious on peripheral smear) in DIC, and this is one of the more reliable
screening tests for the condition (although it does not exclude many other
diagnoses). Both prothrombin time and activated partial thromboplastin times
are often prolonged, but you should be aware that they might each be
shortened or normal as well in DIC. Thus, these two commonly performed
tests are actually not as useful as they might appear. The other choices
listed are wrong because the platelet counts are not decreased.

A 23-year-old man is admitted to the hospital through the emergency department
with probable appendicitis. He has been having right lower
quadrant abdominal pain for several days, which has been becoming
increasingly worse. His temperature 39.2 C (102.6 F), blood pressure
is 80/40 mm Hg, pulse is 120/min, and respirations are 35/min. The abdomen is
rigid with guarding. Multiple petechiae and purpura are
present, and the patient is oozing blood from his oral mucosa. According to the
patient's wife, he has not had bleeding problems in the past.
Question 4 of 5
For confirmation of the probable diagnosis, the emergency department physician
orders a D-dimer test. D-dimer is which of the following?
/ A. A clotting factor in the common coagulation pathway
/ B. A clotting factor in the extrinsic coagulation pathway
/ C. A clotting factor in the intrinsic coagulation pathway
/ D. A factor found in the complement pathway
/ E. A fibrin degradation product

Explanation - Q: 2.4 Close

The correct answer is E. A variety of specialized tests have been
developed to help in the diagnosis of disseminated intravascular coagulation.
Unfortunately, many of these are only available in major medical centers or
through reference laboratories, which markedly limits their usefulness in
emergency rooms elsewhere. One of the more available and useful of these
tests is the D-dimer tests, which measures a fibrin degradation product that is
made when a cross-linked clot (indicating the presence of thrombin to form
the clots) is lysed by plasmin. One way to understand disseminated
intravascular coagulation is to think of it as primarily a thrombotic problem,
with excessive clot formation and then lysis, which leads secondarily to a
hemorrhagic problem as platelets and clotting factors are consumed. High D-
dimer levels indicate that clots are forming and then lysing at an unusual rate
in the body, and thus, together with a low platelet count, suggest that DIC is
present. Functionally, DIC can be subdivided into acute and chronic
presentations, which tend to occur in somewhat different settings. In the
acute presentations, the hemorrhagic processes often dominate the clinical
difficulties, while in the chronic presentations, the clotting tendencies often
dominate the picture. The D-dimers are not in themselves factors (e.g.,
substances to be consumed) in either the coagulation or complement
pathways.

A 23-year-old man is admitted to the hospital through the emergency department
with probable appendicitis. He has been having right lower
quadrant abdominal pain for several days, which has been becoming
increasingly worse. His temperature 39.2 C (102.6 F), blood pressure
is 80/40 mm Hg, pulse is 120/min, and respirations are 35/min. The abdomen is
rigid with guarding. Multiple petechiae and purpura are
present, and the patient is oozing blood from his oral mucosa. According to the
patient's wife, he has not had bleeding problems in the past.
Question 5 of 5
Which of the following would be most effective in diminishing the rate at which
clot formation is occurring?
/ A. Aminocaproic acid
/ B. Heparin
/ C. PIatelet transfusion
/ D. Red cell transfusion
/ E. Tranexamic acid

Explanation - Q: 2.5 Close

The correct answer is B. The therapy of disseminated intravascular
coagulation is complex and problematic, and, frankly, not always successful.
The patients are often already critically ill, and the DIC they have developed
often contributes to major organ failure. The basic problem in therapy is how
to treat simultaneous bleeding and clotting tendencies. Paradoxically, while
the bleeding tendency may be appearing to dominate the clinical picture,
treatment with the anticoagulant heparin may actually help the patient by
decreasing the rate at which the clotting factors are being consumed. This
must, of course, be done very carefully, since an "overshoot" may
exacerbate the patient's problems. Blood components including platelet
transfusions (choice C), red cell transfusions (choice D), and cryoprecipitate
are also sometimes used to restore the blood to a closer to normal state. As
a last resort when other measures have failed, some physicians will also try
antifibrinolytic agents such as aminocaproic acid (choice A) and tranexamic
acid (choice E) to try to keep clots from dissolving and exacerbating the
bleeding tendency.


A 7-year-old girl is evaluated by a pediatrician for a possible bleeding disorder.
The girI's father and grandmother have both had life-Iong
histories of easy bruising. On physical examination, the child is noted to have
multiple small bruises over her body, and the mother reports
that she will frequently bleed for most of an hour following a small cut. The child
has also had numerous nosebleeds that were stopped only
when the nose was packed with facial tissue. CBC is normal on all measured
values. The peripheral smear shows the usual number and
morphology of platelets. Template bleeding time is longer than normaI.
Prothrombin time is within the reference range. Activated partial
thromboplastin time is slightly longer than normaI.
Question 1 of 5
Which of the following is the most likely diagnosis?
/ A. Disseminated intravascular coagulation
/ B. Hemophilia A
/ C. Hemophilia B
/ D. Idiopathic thrombocytopenic purpura
/ E. Von Willebrand disease

Explanation - Q: 3.1 Close

The correct answer is E. Von Willebrand disease is a usually autosomal
dominant condition in which there is either a quantitative or qualitative
abnormality of von Willebrand factor (VWF) production. You should also be
aware that the disease can uncommonly be autosomal recessive or acquired
(associated with Wilms tumor, congenital heart disease, systemic lupus
erythematosus, seizures treated with valproic acid, and hypothyroidism). The
presentation and laboratory findings illustrated are typical. The disease is
life-long, and patients may also present with menorrhagia, post-operative
bleeding, postpartum bleeding, or gingival bleeding. Activated partial
thromboplastin time may be either normal or prolonged, depending on the
degree to which the intrinsic blood coagulation pathway has been inhibited
by decreased binding of factor VIII to von Willebrand factor.
Disseminated intravascular coagulation (choice A) and idiopathic
thrombocytopenic purpura (choice D) are both acquired conditions.
Hemophilia A and B (choice B and C) have X-linked genetics.

A 7-year-old girl is evaluated by a pediatrician for a possible bleeding disorder.
The girI's father and grandmother have both had life-Iong
histories of easy bruising. On physical examination, the child is noted to have
multiple small bruises over her body, and the mother reports
that she will frequently bleed for most of an hour following a small cut. The child
has also had numerous nosebleeds that were stopped only
when the nose was packed with facial tissue. CBC is normal on all measured
values. The peripheral smear shows the usual number and
morphology of platelets. Template bleeding time is longer than normaI.
Prothrombin time is within the reference range. Activated partial
thromboplastin time is slightly longer than normaI.
Question 2 of 5
Multimers of all sizes of von Willebrand's factor form complexes in plasma with
which of the following?
/ A. Factor V
/ B. Factor VI
/ C. Factor VIII
/ D. Factor IX
/ E. Factor X
Explanation - Q: 3.2 Close

The correct answer is C. Normal plasma levels of clotting factor VIII are
maintained by complexing with multimers of Von Willebrand factor. This
protects factor VIII from degradation and helps to deliver it to the site of
injury. This is the contribution VWF makes to the intrinsic pathway of
coagulation. The other factors listed do not complex with VWF.


A 7-year-old girl is evaluated by a pediatrician for a possible bleeding disorder.
The girI's father and grandmother have both had life-Iong
histories of easy bruising. On physical examination, the child is noted to have
multiple small bruises over her body, and the mother reports
that she will frequently bleed for most of an hour following a small cut. The child
has also had numerous nosebleeds that were stopped only
when the nose was packed with facial tissue. CBC is normal on all measured
values. The peripheral smear shows the usual number and
morphology of platelets. Template bleeding time is longer than normaI.
Prothrombin time is within the reference range. Activated partial
thromboplastin time is slightly longer than normaI.
Question 3 of 5
Very large von Willebrand's factor multimers contribute most to which of the
following platelet activities?
/ A. Normal adherence of platelets to damaged blood vessels
/ B. Normal budding of platelets from megakaryocytes
/ C. Normal digestion of platelets during clot lysis
/ D. Normal discharge of granules from platelets
/ E. Normal transport of platelets in the blood

Explanation - Q: 3.3 Close

The correct answer is A. The typical platelet life span begins in the bone
marrow when platelets bud off from megakaryocytes, and are released to
float freely in the blood stream. When a damaged blood vessel is
encountered, VWF acts as an adhesive bridge between the platelets and the
damaged subendothelium at the site of vascular injury. The platelets
subsequently contract and release granules to help in formation of the clot.
Eventually, the clot lyses, and macrophages consume the platelet debris.
A 7-year-old girl is evaluated by a pediatrician for a possible bleeding disorder.
The girI's father and grandmother have both had life-Iong
histories of easy bruising. On physical examination, the child is noted to have
multiple small bruises over her body, and the mother reports
that she will frequently bleed for most of an hour following a small cut. The child
has also had numerous nosebleeds that were stopped only
when the nose was packed with facial tissue. CBC is normal on all measured
values. The peripheral smear shows the usual number and
morphology of platelets. Template bleeding time is longer than normaI.
Prothrombin time is within the reference range. Activated partial
thromboplastin time is slightly longer than normaI.
Question 4 of 5
Which of the following is the binding site for the von Willebrand's factor multimers
on the platelets?
/ A. ADP
/ B. Factor VIII
/ C. GIycoprotein 1b
/ D. Thromboxane A2
/ E. Prostaglandin l2

Explanation - Q: 3.4 Close

The correct answer is C. Von Willebrand factor binds on platelets to the
specific receptor glycoprotein 1b when acting as a bridge between the
platelets and the site of vascular injury.
ADP (choice A) is released by damaged erythrocytes and helps VWF to
adhere to the exposed collagen. It is not, however, the binding factor that
connects the VWF to the platelets.
Factor VIII (choice B) does not participate directly in the platelet portion of
blood clot formation.
Thromboxane A
2
(choice D) promotes subendothelial exposure after vascular
injury and induces platelet aggregation.
Prostaglandin I
2
(choice E) inhibits subendothelial exposure after minor
vascular injury.

A 7-year-old girl is evaluated by a pediatrician for a possible bleeding disorder.
The girI's father and grandmother have both had life-Iong
histories of easy bruising. On physical examination, the child is noted to have
multiple small bruises over her body, and the mother reports
that she will frequently bleed for most of an hour following a small cut. The child
has also had numerous nosebleeds that were stopped only
when the nose was packed with facial tissue. CBC is normal on all measured
values. The peripheral smear shows the usual number and
morphology of platelets. Template bleeding time is longer than normaI.
Prothrombin time is within the reference range. Activated partial
thromboplastin time is slightly longer than normaI.
Question 5 of 5
In an individual with one of the milder forms of the disease presented in this
case, which of the following medications could be used to
increase von Willebrand's factor and factor VIII after treatment?
/ A. Aspirin
/ B. Aminocaproic acid
/ C. Desmopressin
/ D. Ibuprofen
/ E. Naproxen


Explanation - Q: 3.5 Close

The correct answer is C. Desmopressin is a synthetic analogue of
antidiuretic hormone that can be administered intravenously, intranasally, or
subcutaneously, and is frequently used as the primary treatment for bleeding
in individuals with mild von Willebrand disease. Its function in this disease is
to cause release of VWF from endothelial storage sites, so its use is
reserved for acute treatment of bleeding symptoms (including home
treatment with the high dose intranasal preparation), rather than as a routine
medication. Factor VIII concentrates that also contain VWF in high molecular
weight form are used in the treatment of patients with the more severe forms
of Von Willebrand disease. With treatment, the majority of patients,
particularly those with milder forms of Von Willebrand disease, have normal
life spans.
Aminocaproic acid (choice B) is an inhibitor of fibrinolysis that is used to
maintain clots in some individuals with von Willebrand disease.
Drugs with platelet inhibiting activities, including aspirin (choice A), ibuprofen
(choice D), and naproxen (choice E), should be avoided in patients with
disorders of hemostasis.



A child is taken to a pediatrician because the mother is concerned that the child
has been losing, rather than gaining weight, and has been
having numerous infections. After a complete physical examination with
screening serum chemistry and blood studies, the physician
suspects that the child has a malignancy.
Question 1 of 5
Which of the following is the most common malignancy of childhood?
/ A. Ewing sarcoma
/ B. Leukemia
/ C. Neuroblastoma
/ D. Retinoblastoma
/ E. Wilms tumor


Explanation - Q: 1.1 Close

The correct answer is B. Leukemia is the most common malignancy of
childhood. The other malignancies listed are also characteristically childhood
diseases, but occur less frequently.
Ewing sarcoma (choice A) can involve bone or soft tissues;
Neuroblastoma (choice C) can involve the adrenal gland or the extra-
adrenal sympathetic chain.
Retinoblastoma (choice D) involves the eye, and Wilms tumor (choice E)
involves the kidney.
A child is taken to a pediatrician because the mother is concerned that the child
has been losing, rather than gaining weight, and has been
having numerous infections. After a complete physical examination with
screening serum chemistry and blood studies, the physician
suspects that the child has a malignancy.
Question 2 of 5
The complete blood count shows anemia, thrombocytopenia, and a white count
of 110,000/mm3. The peripheral smear contains numerous
blasts, which are smalI, intensely blue cells with homogeneous chromatin,
regular nuclear shape, small or absent nucleolus, and scanty blue
cytoplasm. This patient's disease most likely will be classified as which of the
following types?
/ A. L1
/ B. L2
/ C. L3
/ D. M3
/ E. M7

Explanation - Q: 1.2 Close

The correct answer is A. These blasts cells are the lymphoid L1 cells, and
the child has ALL, type L1. L1 is one of the French-American-British
classifications for lymphoblastic leukemia, which also includes L2 and L3
types.
L2 (choice B) cells are large and heterogenous blast class with
heterogeneous chromatin, irregular nuclear shape, and large nucleolus.
L3 (choice C) cells are large and homogeneous cells whose most prominent
feature is cytoplasmic vacuolization that may overlie the nucleus.
A French-American-British classification has also been used for acute
myeloid leukemias, and different types are given different M designations.
M3 (choice D) is acute promyelocytic leukemia, and the cells have abundant
cytoplasm that often contains numerous Auer rods (small rod like structures).
M7 (choice E) is megakaryoblastic leukemia, and the cells are very large
and maybe multinucleated, resembling megakaryocytes.


A child is taken to a pediatrician because the mother is concerned that the child
has been losing, rather than gaining weight, and has been
having numerous infections. After a complete physical examination with
screening serum chemistry and blood studies, the physician
suspects that the child has a malignancy.
Question 3 of 5
Which of the following is the most common form of leukemia in childhood?
/ A. Acute lymphoblastic leukemia
/ B. Acute myeloblastic leukemia
/ C. Chronic lymphocytic leukemia
/ D. Chronic myelogenous leukemia
/ E. Erythroleukemia


Explanation - Q: 1.3 Close

The correct answer is A. Acute lymphoblastic leukemia is the most
common form of leukemia in children, and accounts for nearly 1/3 of all
pediatric cancers.

A child is taken to a pediatrician because the mother is concerned that the child
has been losing, rather than gaining weight, and has been
having numerous infections. After a complete physical examination with
screening serum chemistry and blood studies, the physician
suspects that the child has a malignancy.
Question 4 of 5
What is the peak age of incidence of the most common form of this disease in
children?
/ A. Less than 6 months
/ B. 8 -14 months
/ C. 2-5 years
/ D. 10-12 years
/ E. More than 15 years

Explanation - Q: 1.4 Close

The correct answer is C. While acute lymphoblastic leukemia can occur at
any age, its peak age of incidence is 2 to 5 years.
A child is taken to a pediatrician because the mother is concerned that the child
has been losing, rather than gaining weight, and has been
having numerous infections. After a complete physical examination with
screening serum chemistry and blood studies, the physician
suspects that the child has a malignancy.
Question 5 of 5
What is the cure rate of the most common form of this disease in children?
/ A. Less than 5%
/ B. 10-25%
/ C. 35-50%
/ D. 70-80%
/ E. More than 95%


Explanation - Q: 1.5 Close

The correct answer is D. With modern chemotherapeutic regimens, we
have now achieved a 70-80% cure rate of acute lymphoblastic leukemia. The
medical regimens with which we treat these children may include
corticosteroids (prednisone, dexamethasone), anti-neoplastic agents
(vincristine, asparaginase, daunorubicin, 6-mercaptopurine, cytarabine,
etoposide, cyclophosphamide), and prophylactic antimicrobials
(sulfamethoxazole and trimethoprim, nystatin, clotrimazole, itraconazole).
Radiotherapy is also sometimes used in these patients. Complications of
acute lymphoblastic leukemia or its treatment can include suppression of
normal bone marrow elements (predisposing for anemia, neutropenia, and
thrombocytopenia), tumor lysis syndrome (which produces hyperuricemia
and electrolyte abnormalities and can be prevented or ameliorated with
hydration and allopurinol), neurotoxicity (which may cause a mild mental
retardation and poor emotional control), and secondary malignancies.
A 22-year-old man has been having fatigue and headaches. On physical
examination, the patient is noted to be pale and has a petechial
rash. A complete blood count and peripheral smear show markedly decreased
numbers of platelets, red blood cells, granulocytes,
monocytes, and reticulocytes. The erythrocyte morphology is normaI. Bone
marrow aspiration and biopsy show a marrow with 10% cellularity,
the rest of the marrow being occupied principally by fat cells.
Question 1 of 5
Which of the following is the most likely diagnosis?
/ A. Aplastic anemia
/ B. Folate deficiency anemia
/ C. Iron deficiency anemia
/ D. Sickle cell anemia
/ E. Thalassemia

Explanation - Q: 2.1 Close

The correct answer is A. This patient most likely has aplastic anemia, which
is defined as an anemia that involves all cell lines produced in the marrow.
The underlying problem is an impaired ability of the most primitive stem cells
in the bone marrow (from which all the blood cell lines except lymphocytes
are derived) to reproduce. Patients may present with symptoms related to
anemia (pallor, headache, palpitations, dyspnea, or fatigue),
thrombocytopenia (mucosal bleeding or petechiae), or neutropenia
(infections, mouth and pharyngeal ulcerations). There is a double peak of
incidence, in the early 20's and then in older adulthood.
Folate deficiency (choice B) produces a megaloblastic anemia, and altered
neutrophils with hypersegmentation of the nucleus may be seen, but the
marrow will not be hypocellular.
Iron deficiency anemia (choice C) tends to be microcytic, and does not affect
cell lines other than erythrocytes.
Sickle cell anemia (choice D) has bizarre-shaped erythrocytes and affects
only the erythrocyte line.
Thalassemia (choice E) is characterized by hypochromic erythrocytes that
may have a "target" appearance, and does not affect cell lines other than the
erythrocytic line.

A 22-year-old man has been having fatigue and headaches. On physical
examination, the patient is noted to be pale and has a petechial
rash. A complete blood count and peripheral smear show markedly decreased
numbers of platelets, red blood cells, granulocytes,
monocytes, and reticulocytes. The erythrocyte morphology is normaI. Bone
marrow aspiration and biopsy show a marrow with 10% cellularity,
the rest of the marrow being occupied principally by fat cells.
Question 2 of 5
Which of the following is noted for sometimes causing this patient's condition?
/ A. Asbestos
/ B. Benzene
/ C. Lead
/ D. Mercury
/ E. Methyl alcohol


Explanation - Q: 2.2 Close

The correct answer is B. Approximately 80% of cases of aplastic anemia
are acquired, with the remainder being congenital. Among the acquired
cases, often no inciting cause can be identified, and an autoimmune basis
appears probable. However, in some cases, an inciting cause can be
identified, and in these, it is important to recognize and if possible remove
the cause. Benzene is the chemical most commonly cited as being
associated with development of aplastic anemia.
Associate asbestos (choice A) with pulmonary fibrosis, mesothelioma, and
lung cancer.
Associate lead poisoning (choice C) with a hypochromic, microcytic anemia,
abdominal discomfort, and CNS effects.
Associate mercury poisoning (choice D) with (depending upon the route of
exposure) GI disturbance, pneumonitis, renal failure, and CNS involvement.
Associate methyl alcohol (choice E) with visual system and central nervous
system damage.


A 22-year-old man has been having fatigue and headaches. On physical
examination, the patient is noted to be pale and has a petechial
rash. A complete blood count and peripheral smear show markedly decreased
numbers of platelets, red blood cells, granulocytes,
monocytes, and reticulocytes. The erythrocyte morphology is normaI. Bone
marrow aspiration and biopsy show a marrow with 10% cellularity,
the rest of the marrow being occupied principally by fat cells.
Question 3 of 5
Which of the following medications has been associated with the development of
this patient's condition?
/ A. Amyl nitrate
/ B. Aspirin
/ C. Chloramphenicol
/ D. CIofazimine
/ E. Dapsone


Explanation - Q: 2.3 Close

The correct answer is C. Drugs noted for causing aplastic anemia include
chloramphenicol, phenylbutazone, and gold. Cases triggered by idiosyncratic
reaction to drugs appear not to involve an immune mechanism, but may be a
result of direct toxicity, possibly involving altered detoxification.
Amyl nitrate (choice A) does not have any hematologic side effects.
Aspirin (choice B) can cause leukopenia, thrombocytopenia, and
prolongation of bleeding time, but not aplastic anemia.
Clofazimine (choice D) does not have any hematologic side effects.
Dapsone (choice E) can cause dose-related hemolysis in patients with or
without glucose-6-phosphatase deficiency.

A 22-year-old man has been having fatigue and headaches. On physical
examination, the patient is noted to be pale and has a petechial
rash. A complete blood count and peripheral smear show markedly decreased
numbers of platelets, red blood cells, granulocytes,
monocytes, and reticulocytes. The erythrocyte morphology is normaI. Bone
marrow aspiration and biopsy show a marrow with 10% cellularity,
the rest of the marrow being occupied principally by fat cells.
Question 4 of 5
With supportive care alone, this patient's condition is estimated to have which of
the following mortality rates?
/ A. Less than 5%
/ B. 10-25%
/ C. 30-40%
/ D. 50-60%
/ E. More than 70%


Explanation - Q: 2.4 Close

The correct answer is E. Supportive care consists primarily of appropriate
transfusions to replace life-threateningly low red cell counts or platelet
counts. Supportive care alone is no longer considered adequate therapy,
since aplastic anemia has a greater than 70% mortality rate if that is the only
therapy offered.
A 22-year-old man has been having fatigue and headaches. On physical
examination, the patient is noted to be pale and has a petechial
rash. A complete blood count and peripheral smear show markedly decreased
numbers of platelets, red blood cells, granulocytes,
monocytes, and reticulocytes. The erythrocyte morphology is normaI. Bone
marrow aspiration and biopsy show a marrow with 10% cellularity,
the rest of the marrow being occupied principally by fat cells.
Question 5 of 5
If treated with immunosuppression, the five year survival rate is approximately
which of the following?
/ A. 5%
/ B. 25%
/ C. 50%
/ D. 75%
/ E. 95%

Explanation - Q: 2.5 Close

The correct answer is D. Patients treated with immunosuppression have an
estimated 75% 5-year-survival rate, and those lucky individuals who have
matched sibling donor bone marrow transplant have a more than 90% 5-
year-survival. Colony stimulating cytokines (G-CSF and GM-CSF) are now
commercially available, and can also have great value in stimulating any
residual growth capacity the stem cells have.
A complete blood count (CBC) and a blood smear are taken from a patient who
has been complaining of fatigue. The CBC shows a white
count of 200,000/mm3, with differential count including 70% segmented
neutrophils and 15% band forms. The absolute numbers of basophils
and eosinophils are increased somewhat. Review of the peripheral smear does
not show any blast forms.
Question 1 of 8
Which of the following is the most likely diagnosis?
/ A. Acute lymphocytic leukemia
/ B. Acute myelogenous leukemia
/ C. Chronic lymphocytic leukemia
/ D. Chronic myelogenous leukemia
/ E. Hodgkin disease

Explanation - Q: 3.1 Close

The correct answer is D. This patient most likely has chronic myelogenous
leukemia (CML). This is a cancer of pluripotent marrow stem cells
characterized by a proliferation, predominantly of granulocytes, although
other cell lines (megakaryocytes, monocytes, erythrocytes, and even some T
and B cell lines) may also be increased.
Blast cells would be seen in the peripheral smear in acute myelogenous
leukemia (choice B) and acute lymphocytic leukemia (choice A).
In chronic lymphocytic leukemia (choice C), the numbers of lymphocytes are
increased and the numbers of other blood cells may be decreased.
Hodgkin disease (choice E) may show a modest increase in neutrophils and
eosinophils in the blood, but would not have the dramatic increase seen in
this patient.

A complete blood count (CBC) and a blood smear are taken from a patient who
has been complaining of fatigue. The CBC shows a white
count of 200,000/mm3, with differential count including 70% segmented
neutrophils and 15% band forms. The absolute numbers of basophils
and eosinophils are increased somewhat. Review of the peripheral smear does
not show any blast forms.
Question 2 of 8
Which of the following is the median age for presentation of this disease?
/ A. 1 year
/ B. 5 years
/ C. 25 years
/ D. 45 years
/ E. 75 years

Explanation - Q: 3.2 Close

The correct answer is D. Chronic myelogenous leukemia is primarily a
disease of middle age, with a median age of 45 years. The comparatively
few cases occurring in the elderly are more likely to be aggressive than those
occurring in middle-aged adults.
A complete blood count (CBC) and a blood smear are taken from a patient who
has been complaining of fatigue. The CBC shows a white
count of 200,000/mm3, with differential count including 70% segmented
neutrophils and 15% band forms. The absolute numbers of basophils
and eosinophils are increased somewhat. Review of the peripheral smear does
not show any blast forms.
Question 3 of 8
This patient's disease can be confused in some settings with polycythemia vera.
Which of the following tests would be most helpful in
distinguishing between these two diseases?
/ A. Leukocyte alkaline phosphatase
/ B. Myelin basic protein
/ C. Protein C activity
/ D. ScI-70
/ E. Tartrate resistant acid phosphatase


Explanation - Q: 3.3 Close

The correct answer is A. While to the novice, polycythemia vera and
chronic myelogenous leukemia appear to be obviously different diseases,
both cause an increase in many marrow cell lines, and ambiguous cases do
exist. Leukocyte alkaline phosphatase is a helpful test to perform in these
settings, because it is characteristically low in chronic myelogenous
leukemia, but high in polycythemia vera. Also, polycythemia vera does not
usually have the Philadelphia chromosome.
Myelin basic protein (choice B) is used in the diagnosis of multiple sclerosis.
Protein C (choice C) is involved in the clotting mechanism.
Scl-70 (choice D) is an antibody often present in scleroderma.
Associate tartrate-resistant acid phosphatase (choice E) positivity with hairy
cell leukemia.

A complete blood count (CBC) and a blood smear are taken from a patient who
has been complaining of fatigue. The CBC shows a white
count of 200,000/mm3, with differential count including 70% segmented
neutrophils and 15% band forms. The absolute numbers of basophils
and eosinophils are increased somewhat. Review of the peripheral smear does
not show any blast forms.
Question 4 of 8
Which of the following organs is most commonly significantly enlarged in these
patients?
/ A. Liver
/ B. Pancreas
/ C. Stomach
/ D. Spleen
/ E. Thymus

Explanation - Q: 3.4 Close

The correct answer is D. Splenomegaly is common in patients with chronic
myelogenous leukemia, and in more than half of the patients, is palpable
more than 5 cm below the costal margin at the time of diagnosis.
Splenomegaly may be a very helpful sign on physical examination, since it is
uncommon in reactive leukocytosis. Hepatomegaly (choice A) can also
occur, but is less common. The other answers are distracters.

A complete blood count (CBC) and a blood smear are taken from a patient who
has been complaining of fatigue. The CBC shows a white
count of 200,000/mm3, with differential count including 70% segmented
neutrophils and 15% band forms. The absolute numbers of basophils
and eosinophils are increased somewhat. Review of the peripheral smear does
not show any blast forms.
Question 5 of 8
Which of the following translocations is very common in this patient's disease?
/ A. t(8;14)
/ B. t(8;21)
/ C. t(9;22)
/ D. t(12;21)
/ E. t(15;17)

Explanation - Q: 3.5 Close

The correct answer is C. The characteristic chromosomal translocation of
chronic myelogenous leukemia is t(9;22)(q34;q11). This translocation results
in a distinctive abnormal chromosome sometimes called the Philadelphia
chromosome, and is seen in 90-95% of CML cases. The Philadelphia
translocation is also seen in 20-25% of adults with acute lymphoblastic
leukemia, 2% of adults with acute myeloid leukemia, and 2-10% of childhood
acute lymphoblastic leukemia. Some authors think that these cases may
represent variants of chronic myelogenous leukemia in which the "blast
transformation" (which commonly occurs 3-5 years after diagnosis in
untreated patients with chronic myelogenous leukemia) either develops very
early in the disease, or later, but in a patient in whom the chronic phase had
been missed clinically. The Philadelphia translocation is not usually
encountered in children with acute myeloid leukemia or in adults with chronic
lymphocytic leukemia.
T(8;14) (choice A) is a translocation with unfavorable prognosis found in
some cases of acute lymphoblastic (L3 form) leukemia.
T(8;21) (choice B) is a translocation with favorable prognosis found in some
cases of acute myelogenous leukemia (M2 form).
T(12;21) (choice D) is a translocation with favorable prognosis found in
some cases of acute lymphoblastic (L1 form) leukemia.
T(15;17) (choice E) is a translocation with favorable prognosis found in
some cases of acute myelogenous leukemia (M3 form).
A complete blood count (CBC) and a blood smear are taken from a patient who
has been complaining of fatigue. The CBC shows a white
count of 200,000/mm3, with differential count including 70% segmented
neutrophils and 15% band forms. The absolute numbers of basophils
and eosinophils are increased somewhat. Review of the peripheral smear does
not show any blast forms.
Question 6 of 8
This translocation joins which of the following two genes?
/ A. BCR and ABL
/ B. E2A and PBX1
/ C. myc and lgH
/ D. myc and kappa light chain
/ E. myc and lambda light chain

Explanation - Q: 3.6 Close

The correct answer is A. The t(9;22) Philadelphia translocation links the
Abelson oncogene (c-ABL) (normally on chromosome 9) to the BCR region
of chromosome 22. The resulting gene product is a new, chimeric, protein
called BCR-ABL, which has tyrosine kinase activity. In some manner still not
well understood, this gene product then produces the clinical problems we
diagnosis as CML.
E2A and PBX1 (choice B) describes t(1;19), seen in some cases of pre-B-
cell ALL, which links the E2A gene product (a basic helix-loop-helix
transcription factor) with the homeobox gene PBX1.
The myc oncogene and the immunoglobulin heavy chain IgH (choice C) are
joined in a t(8;14) translocation commonly seen in Burkitt's lymphoma and
type L3 acute lymphoblastic leukemia.
The translocations described in choices D and E also occur with lower
frequency in Burkitt's lymphoma and L3 acute lymphoblastic leukemia; in
these cases, the translocations produced are, respectively, t(2;8) and t(8;22).


A complete blood count (CBC) and a blood smear are taken from a patient who
has been complaining of fatigue. The CBC shows a white
count of 200,000/mm3, with differential count including 70% segmented
neutrophils and 15% band forms. The absolute numbers of basophils
and eosinophils are increased somewhat. Review of the peripheral smear does
not show any blast forms.
Question 7 of 8
Which of the following specifically inhibits the abnormal gene product produced
by the translocation seen in this disease?
/ A. Busulfan
/ B. Hydroxyurea
/ C. Imatinib mesylate
/ D. Interferon alfa-2a
/ E. Interferon alfa-2b

Explanation - Q: 3.7 Close

The correct answer is C. Imatinib mesylate (Gleevec) is an important new
drug that specifically targets the abnormal gene product (a tyrosine kinase)
of the BCR-ABL translocation. This new drug is well absorbed after oral
administration and can be used to treat both the chronic phase and blast
crisis of CML. It also shows promise in potentially being used in autologous
(self-to-self) bone marrow transplant, where it may be possible to clear the
transplant of leukemic cells in vitro with chemotherapy before reinjecting the
transplant. The other agents listed in the choices are also used in the
chemotherapy of CML.
Busulfan (choice A) is an alkylating agent, hydroxyurea (choice B) inhibits
DNA synthesis, and recombinant forms of interferons (choices D and E) can
help induce remissions in CML.

A complete blood count (CBC) and a blood smear are taken from a patient who
has been complaining of fatigue. The CBC shows a white
count of 200,000/mm3, with differential count including 70% segmented
neutrophils and 15% band forms. The absolute numbers of basophils
and eosinophils are increased somewhat. Review of the peripheral smear does
not show any blast forms.
Question 8 of 8
The cure rate with patients who have this condition and undergo bone marrow
transplant is now estimated to be which of the following?
/ A. 5%
/ B. 15%
/ C. 25%
/ D. 50%
/ E. 80%


Explanation - Q: 3.8 Close

The correct answer is D. In the past, the average life expectancy of CML
patients has been 3-5 years, with most patients dying after a blast crisis
develops. The blast crisis may produce either clinical acute lymphoblastic
leukemia or acute myeloid leukemia. This was historically true despite
chemotherapy and any apparent improvements in blood counts with
chemotherapy. Bone marrow transplant is becoming an increasingly
preferred treatment modality in those cases in which a suitable donor can be
found, because it now has a cure rate of approximately 50%. The mortality
rate of this procedure is 10-20% or less if a matched sibling donor is used
and increases to 30-40% if an unrelated donor is used.


A 17-year-old African American with type 1 diabetes spends a weekend camping
with friends. A day after returning, he is brought into the
emergency department because he is in diabetic ketoacidosis. In the emergency
room, he is noted to be jaundiced. The laboratory calls the
emergency department physician because the plasma has a red color.
Question 1 of 5
Which of the following enzymatic deficiencies would most likely cause a
hemolytic episode such as this man is experiencing?
/ A. Acid sphingomyelinase deficiency
/ B. GIucocerebrosidase deficiency
/ C. GIucose-6-phosphate dehydrogenase deficiency
/ D. Ferrochelatase deficiency
/ E. Phytanic acid alpha-oxidase deficiency


Explanation - Q: 4.1 Close

The correct answer is C. Of the enzyme deficiencies listed, only glucose-6-
phosphate dehydrogenase deficiency (G-6-PD) would cause hemolysis. The
cells most likely to be affected are usually the older erythrocytes. Most
patients with this condition have long asymptomatic intervals and only
undergo hemolysis when a metabolic stress occurs. While elementary
discussions of this condition usually stress the ingestion of fava beans or the
taking of medicines (e.g., sulfonamides, antimalarial drugs) as triggers of
hemolysis, you should be aware that in real life in the United States, the most
common triggers are fever, acute viral and bacterial infections, and diabetic
ketoacidosis. Once the diagnosis of G6PD deficiency is suspected, the
actual enzyme activity can be assayed in the laboratory to establish the
diagnosis.
Acid sphingomyelinase deficiency (choice A) produces the lysosomal
storage disease Niemann-Pick disease.
Glucocerebrosidase deficiency (choice B) produces the lipid storage
disease Gaucher disease.
Ferrochelatase deficiency (choice D) produces erythropoietic protoporphyria.
Phytanic acid alpha-oxidase deficiency (choice E) produces the storage
disease Refsum disease.
A 17-year-old African American with type 1 diabetes spends a weekend camping
with friends. A day after returning, he is brought into the
emergency department because he is in diabetic ketoacidosis. In the emergency
room, he is noted to be jaundiced. The laboratory calls the
emergency department physician because the plasma has a red color.
Question 2 of 5
Which of the following is the approximate incidence of this enzymatic defect in
African-American males?
/ A. Less than 0.1%
/ B. 1%
/ C. 10%
/ D. 30%
/ E. 90%


Explanation - Q: 4.2 Close

The correct answer is C. The incidence of glucose-6-phosphate deficiency
in American black males is thought to be about 12%. The condition can also
be found in lower frequency in black females (who either have both X
chromosomes involved or who have one affected X chromosome with the
other inactivated as a Barr body in a high percentage of the body). G6PD
deficiency has a worldwide distribution, but occurs most frequently in tropical
and subtropical areas of the Eastern hemisphere. Populations that have a
significant frequency of G6PD deficiency include Brazilian Blacks, people
from the lowlands of the Mediterranean basin, especially those of Greek or
Sardinian descent, Kurdish Jews and the Southern Chinese. Most affected
individuals are asymptomatic. However, individuals from ethnic backgrounds
with high frequencies of G6PD deficiency should be checked for it if
treatment of an illness requires a medication known to precipitate hemolysis.
G6PD deficiency should be also be considered in the differential diagnosis of
any nonimmune hemolytic anemia.

A 17-year-old African American with type 1 diabetes spends a weekend camping
with friends. A day after returning, he is brought into the
emergency department because he is in diabetic ketoacidosis. In the emergency
room, he is noted to be jaundiced. The laboratory calls the
emergency department physician because the plasma has a red color.
Question 3 of 5
Which of the following is a product of the enzyme for which the patient has a
deficiency?
/ A. FADH2
/ B. NAD+
/ C. NADH
/ D. NADP+
/ E. NADPH

Explanation - Q: 4.3 Close

The correct answer is E. Glucose-6-phosphate dehydrogenase is the
committed step of the hexose monophosphate shunt pathway (pentose
phosphate pathway). The enzymes of this pathway are present in the
cytoplasm of all cells, but are especially prevalent in adipose and liver cells.
The hexose monophosphate shunt pathway makes ribose-5-phosphate for
nucleotide synthesis, interconverts 6 carbon sugars and 5 carbon sugars,
and produces NADPH. NADPH, the reduced form of nicotinamide adenine
dinucleotide phosphate, is used in reductive biosynthetic reactions such as
fatty acid synthesis, and in the regeneration of glutathione by glutathione
reductase. Glutathione has three main roles in a cell: it keeps the protein
sulfhydryl groups of cysteines reduced, it reduces peroxides, and it maintains
hemoglobin in the reduced state.
NADP+ (choice D) is also involved in this reaction, but as a substrate, rather
than a product.
The various forms of NAD (choices B and C) and FAD (choice A) do not
take part.

A 17-year-old African American with type 1 diabetes spends a weekend camping
with friends. A day after returning, he is brought into the
emergency department because he is in diabetic ketoacidosis. In the emergency
room, he is noted to be jaundiced. The laboratory calls the
emergency department physician because the plasma has a red color.
Question 4 of 5
Regeneration of which of the following in a reduced form is impaired in
individuals with this disorder?
/ A. Acetaldehyde
/ B. AcetyI CoA
/ C. Biotin
/ D. GIutathione
/ E. GIycogen


Explanation - Q: 4.4 Close

The correct answer is D. Reduced glutathione is an effective scavenger of
dangerous oxidative metabolites; regeneration of reduced glutathione is
accomplished by glutathione reductase with the aid of NADPH, a product of
the hexose monophosphate shunt pathway. Red blood cells that are deficient
for G6PD, and are exposed to oxidants, can be depleted of their store of
glutathione. The lack of glutathione leads to the oxidation of the sulfhydryl
groups on hemoglobin, causing the formation of methemoglobin and then
denatured globin, which forms insoluble masses called Heinz bodies that are
attached to the RBC membrane. The overall outcome is that the red blood
cells become rigid, making them susceptible to destruction by the
reticuloendothelial system.
The other answers are distracters.

A 17-year-old African American with type 1 diabetes spends a weekend camping
with friends. A day after returning, he is brought into the
emergency department because he is in diabetic ketoacidosis. In the emergency
room, he is noted to be jaundiced. The laboratory calls the
emergency department physician because the plasma has a red color.
Question 5 of 5
In a Nigerian population, the frequency of males with G6PD deficiency is 0.2.
Assuming the population is in Hardy-Weinberg equilibrium at
this locus, what is the frequency of homozygous females?
/ A. 0.02
/ B. 0.04
/ C. 0.2
/ D. 0.32
/ E. 0.45


Explanation - Q: 4.5 Close

The correct answer is B. Glucose 6-phosphate dehydrogenase deficiency
is an X-linked recessive trait. A male is hemizygous for the X chromosome,
and thus has only one copy of each trait located on that chromosome. The
frequency of an X-linked recessive in males is thus equal to the frequency of
the allele in the population. From this, we know that q= 0.2 and p = 0.8. A
woman has two copies of each gene on the X chromosome, so the equation
for Hardy Weinberg equilibrium is the same as for autosomal traits. In this
case, a homozygous recessive female would occur at a frequency of q
2
or
0.04.
Choice A, 0.02, is incorrect. If a person remembered that G6PD was more
frequent in males, but didn't know how to use the equations to get the true
estimate, he or she might guess this answer.
Choice C, 0.2, makes the assumption that the trait is autosomal, and so the
frequency of affected males and affected females are equal.
Choice D, 0.32, assumes that q=0.01, and then calculates the frequency of
carrier females (2pq).
Choice E, 0.45 is a distracter

A follow-up physical examination is performed on a 7-year-old child with known
celiac disease, which has been difficult to controI. A complete
blood count is done as part of the evaluation, which demonstrates a moderate
anemia. Review of the peripheral blood smear shows
numerous enlarged, hypochromic erythrocytes. Some of the neutrophils show
hypersegmentation of the nuclei, with up to 8 Iobes, rather than
the usuaI 3.
Question 1 of 5
Deficiency of which of the following would most likely cause this child's anemia?
/ A. Iron
/ B. Folate
/ C. Vitamin A
/ D. Vitamin C
/ E. Vitamin K

Explanation - Q: 5.1 Close

The correct answer is B. The combination of enlarged, hypochromic
erythrocytes with hypersegmented neutrophils indicates that the patient has
a megaloblastic anemia. Of the causes listed in the choices, only folate
deficiency will produce these changes. Folate deficiency can also be caused
by inadequate intake (elderly, alcoholics, infants), increased requirements
(pregnancy, infancy, high metabolic states), impaired utilization
(methotrexate therapy), or increased loss (hemodialysis).
Iron deficiency (choice A) usually produces a microcytic, hypochromic
anemia.
Vitamin A deficiency (choice C) causes night blindness.
Vitamin C deficiency (choice D) causes scurvy and poor wound healing.
Vitamin K deficiency (choice E) causes a bleeding tendency.

A follow-up physical examination is performed on a 7-year-old child with known
celiac disease, which has been difficult to controI. A complete
blood count is done as part of the evaluation, which demonstrates a moderate
anemia. Review of the peripheral blood smear shows
numerous enlarged, hypochromic erythrocytes. Some of the neutrophils show
hypersegmentation of the nuclei, with up to 8 Iobes, rather than
the usuaI 3.
Question 2 of 5
Deficiency of which the following can also cause this patient's type of anemia?
/ A. Calcium
/ B. Magnesium
/ C. Vitamin B12
/ D. Vitamin D
/ E. Vitamin E

Explanation - Q: 5.2 Close

The correct answer is C. Vitamin B12 deficiency can also cause
megaloblastic anemia.
Calcium and vitamin D deficiencies (choices A and D) can cause
osteoporosis and rickets.
Magnesium deficiency (choice B) is usually seen in settings in which
patients are very ill for other reasons, but appears to cause GI symptoms,
personality change, and tetany.
Vitamin E (choice E) is an antioxidant. Deficiency may produce mild
hemolytic anemia or neurologic (spinocerebellar) disease.

A follow-up physical examination is performed on a 7-year-old child with known
celiac disease, which has been difficult to controI. A complete
blood count is done as part of the evaluation, which demonstrates a moderate
anemia. Review of the peripheral blood smear shows
numerous enlarged, hypochromic erythrocytes. Some of the neutrophils show
hypersegmentation of the nuclei, with up to 8 Iobes, rather than
the usuaI 3.
Question 3 of 5
The substance most likely deficient in this child is normally absorbed primarily at
which of the following sites?
/ A. Colon
/ B. Distal ileum
/ C. Duodenum and jejunum
/ D. Esophagus
/ E. Stomach


Explanation - Q: 5.3 Close

The correct answer is C. Folate is absorbed in the proximal small bowel
and vitamin B12 is absorbed in the ileum (choice B). Thus poorly controlled
celiac disease, which principally affects the proximal small intestine, is more
likely to cause folate deficiency than B12 deficiency.
The esophagus (choice D) and stomach (choice E) are not major absorptive
sites; and the colon (choice A) principally absorbs fluid and electrolytes.
A follow-up physical examination is performed on a 7-year-old child with known
celiac disease, which has been difficult to controI. A complete
blood count is done as part of the evaluation, which demonstrates a moderate
anemia. Review of the peripheral blood smear shows
numerous enlarged, hypochromic erythrocytes. Some of the neutrophils show
hypersegmentation of the nuclei, with up to 8 Iobes, rather than
the usuaI 3.
Question 4 of 5
The two substances that can cause this type of anemia if deficient, are necessary
for synthesis of which of the following?
/ A. AcetyI CoA
/ B. ADP
/ C. ATP
/ D. NADPH
/ E. Tetrahydrofolate (THF)

Explanation - Q: 5.4 Close

The correct answer is E. Vitamin B12 is a cofactor in the activation of folic
acid to tetrahydrofolate (THF). The THF is necessary for DNA base
synthesis, and careful evaluation will demonstrate megaloblastic changes in
all 3 hematopoietic lines. The other answers are distracters.
A follow-up physical examination is performed on a 7-year-old child with known
celiac disease, which has been difficult to controI. A complete
blood count is done as part of the evaluation, which demonstrates a moderate
anemia. Review of the peripheral blood smear shows
numerous enlarged, hypochromic erythrocytes. Some of the neutrophils show
hypersegmentation of the nuclei, with up to 8 Iobes, rather than
the usuaI 3.
Question 5 of 5
Deficiency of vitamin B12 can produce damage to which of the following?
/ A. Cardiovascular system
/ B. Central nervous system
/ C. Female reproductive system
/ D. Hepatobiliary system
/ E. Urinary system

Explanation - Q: 5.5 Close

The correct answer is B. Vitamin B12 functions in transfer of 1-carbon
fragments, and has a role in other pathways besides DNA synthesis. Vitamin
B12 deficiency can also cause demyelination and myelopathy, with resultant
CNS degeneration. The other choices are distracters.


_ .
A 2-year-old African American child is evaluated by a pediatrician because the
mother noticed that the child had been behaving listlessly for
several months. Office hematocrit is 29%. A peripheral blood smear is shown
above.
Question 1 of 6
Which of the following is the most likely diagnosis?
/ A. AIpha thalassemia major
/ B. Beta thalassemia major
/ C. Beta thalassemia minor
/ D. Sickle cell anemia
/ E. Sickle cell trait


Explanation - Q: 6.1 Close

The correct answer is D. This child has sickle cell anemia. This is an
autosomal recessive, chronic hemolytic anemia characterized by sickle- or
crescent-shaped erythrocytes. Almost all cases are individuals with at least
some African ancestry. In African Americans, the prevalence of sickle cell
anemia is about 0.3%.
The various thalassemias (choices A, B, and C) can be excluded because
the peripheral smear findings would include a reference to or a description of
target cells. In questionable cases, or when the possibility of coexisting
disease is present, hemoglobin electrophoresis can distinguish between the
various diseases noted in the choices.
Sickle cell trait (choice E), or the heterozygote and milder form of sickle cell
disease, can be excluded because the patient is frankly anemic with a very
low hematocrit. Sickle cell trait has a prevalence in African Americans of
about 8-13%. These individuals are usually asymptomatic, but may develop
clinical disease in unusual situations (such as with severe infections).


Question 2 of 6
The disease is caused by a nucleotide substitution at position +70 in the gene
region. The mutation also destroys a restriction endonuclease
site from +66 to +72 in the gene. Using a probe that binds just upstream from the
mutation, restriction patterns from his mother and from his
father were obtained and are shown at the left on the diagram below.





Which of the patterns on the right most likely represents that of the child?
/ A. A
/ B. B
/ C. C
/ D. D
/ E. E

Explanation - Q: 6.2 Close

The correct answer is E. The mutation that causes the disease also
destroys a restriction enzyme site. Loss of a restriction site would produce a
longer restriction fragment that would be retained higher on the gel. Thus, in
both parents' patterns, the upper band must be cut from the chromosome
bearing the mutation. The affected child would be homozygous for this
mutation and show only the upper band in his pattern. The band is thicker,
indicating more material. The same size restriction fragment is cut from both
his chromosomes.
Choice A shows a homozygous normal individual.
Choice B shows an individual with a band representing a shorter fragment
not seen in either parent.
Choice C shows an individual with a band representing a longer fragment
not seen in either parent. If the mutation involved a triplet repeat expansion,
a pattern like this might be generated. It is not correct for the mutation
described in the stem.
Choice D shows a carrier.

Question 3 of 6
The prevalence of this disease in African Americans is about 1/625. The boy's
uncle has been tested and knows that he is a carrier of the
mutation involved. If he marries an African American woman, what is the risk
their first child will be affected with this disease?
/ A. 1/2500
/ B. 1/1200
/ C. 1/125
/ D. 1/100
/ E. 1/50


Explanation - Q: 6.3 Close

The correct answer is E. If the prevalence of the disease (q
2
) were 1/625,
then the carrier frequency (2pq) in the same population would be 2(1/25) or
2/25

The risk of having an affected child is then: 2/25 x 1/4 = 2/100 or 1/50. The
risk is the same for each child, regardless of birth order.

Question 4 of 6
Compared to the prevalence in African Americans (1/625), the prevalence of this
disease in Africans may be as high as 1/50. Which of the
following genetic mechanisms contributes to this disparity?
/ A. Founder effect
/ B. Gene flow
/ C. Genetic drift
/ D. Incomplete penetrance
/ E. Increased mutation rate in Africans as compared to African Americans.


Explanation - Q: 6.3b Close

The correct answer is E. If the prevalence of the disease (q
2
) were 1/625,
then the carrier frequency (2pq) in the same population would be 2(1/25) or
2/25

The risk of having an affected child is then: 2/25 x 1/4 = 2/100 or 1/50. The
risk is the same for each child, regardless of birth order.


Question 4 of 6
Compared to the prevalence in African Americans (1/625), the prevalence of this
disease in Africans may be as high as 1/50. Which of the
following genetic mechanisms contributes to this disparity?
/ A. Founder effect
/ B. Gene flow
/ C. Genetic drift
/ D. Incomplete penetrance
/ E. Increased mutation rate in Africans as compared to African Americans.

Explanation - Q: 6.4 Close

The correct answer is B. Gene flow refers to the exchange of genes among
populations. When two populations, previously isolated reproductively, begin
to intermarry, gene frequencies within both populations will change due to
this flow, eventually reaching new equilibria. In either of the populations,
some gene frequencies will increase and some will decrease. Another cause
of the higher prevalence of sickle cell disease in Africans is the continued
selective pressure of malaria, not present in the U.S. Natural selection is not
given as an option in this question.
A founder effect (choice A) refers to the introduction of a mutation into a
rapidly expanding population by an individual who was part of a small
ancestral group. Both populations in this question share the same genetic
origin (Africa) and carry the same mutation. A founder effect would not
account for this disparity in gene frequency.
Genetic drift (choice C) refers to the random fluctuations of gene
frequencies in small populations. Some genes can increase in frequency,
others may decrease, when compared to the gene frequencies seen in larger
populations. Gene frequencies are statistical measurements, and small
populations are more likely to vary from the statistical mean than large
populations. Consider a family in which both parents are carriers of an
autosomal recessive disease-producing gene. Statistics indicate that if the
family has enough children, 25% will be AA, 50% will be Aa, and 25% will be
aa. If the family only has 4 children, there is some random chance that 2 will
be affected (e.g. 50% rather than 25%)
Incomplete penetrance (choice D) refers to the fact that not all individuals
with the disease-producing genotype have the disease (phenotype). The
same mutation should show nearly identical penetrance in two populations,
and would not account for the differences described in this question.
Mutation rates in large populations tend to be the same (compare to choice
E), and do not account for major differences in gene frequencies.
Question 5 of 6
Which of the following viruses most commonly causes aplastic crises in patients
with this disease?
/ A. BK virus
/ B. Human herpes virus 8
/ C. Human papilloma virus 18
/ D. JC virus
/ E. Parvovirus B19


Explanation - Q: 6.5 Close

The correct answer is E. Sickle cell patients are vulnerable to a variety of
potentially life-threatening clinical crises. Important causes of aplastic crises
(also called hematologic crises), in which the marrow fails to produce
adequate numbers of erythrocytes, include parvovirus B19 infection and
folate deficiency. For your personal information, although the question is
usually asked about sickle cell anemia, these two inciting agents can also
induce exacerbation of anemia in individuals with thalassemia and a variety
of other hereditary red cell disorders. Simple transfusions can be used to
treat aplastic crises.
BK virus (choice A) causes renal disease in immunocompromised hosts.
Human herpes virus 8 (choice B) has been implicated in Kaposi sarcoma.
Human papilloma virus 18 (choice C) is an important cause of cervical
dysplasia and cancer.
JC virus (choice D) causes progressive multifocal leukoencephalopathy.

Question 6 of 6
At age 5, the boy and his mother take a trip in a friend's small plane. On landing,
the boy complains of very severe pain involving his bones
and abdomen. Which of the following problems most likely developed?
/ A. Aplastic crisis
/ B. Hematologic crisis
/ C. Infectious crisis
/ D. Osteomyelitis
/ E. Vasoocclusive crisis

Explanation - Q: 6.6 Close

The correct answer is E. This patient probably developed a vasoocclusive
crisis, which occurs when there is an exacerbation of red cell sickling. The
increased sickling leads to obstruction of the microcirculation with sickled
erythrocytes, which in turn produces ischemia with its accompanying severe
pain (analogous to the pain of angina and myocardial infarction). The pain
most frequently involves bones, abdomen, and/or chest. Involvement of the
nervous system can produce focal neurologic signs and symptoms.
Involvement of the chest can produce acute chest syndrome (due to
microvascular occlusion in the pulmonary capillary bed), and is the major
cause of death in children less than 5 years of age who have sickle cell
disease. Precipitating causes for vasooclusive crises include cold weather
(which induces vasospasm), hypoxia (as in this patient who flew in an
unpressurized airplane), infection, dehydration, acidosis, alcohol intoxication,
emotional stress, and pregnancy. Vasoocclusive crises are usually treated
with hydration and analgesia. In severe cases, or if acute chest syndrome
develops, exchange blood transfusion is sometimes used to reduce the
number of circulating sickled cells. Important triggers for aplastic or
hematologic crises (choices A and B) are discussed in the explanation to
the preceding question. Infections (choice C), which can include
osteomyelitis (choice D), are usually related to these patients' functional
asplenia (due to splenic ischemia and infarction) and resultant defective
immunity. Encapsulated organisms (Haemophilus influenzae and
Streptococcus pneumoniae) are a particular vulnerability, but there is also an
increased vulnerability (apparently related to altered serum IgM levels,
impaired opsonization, and poor complement pathway function) to some
other organisms including Mycoplasma pneumoniae, Salmonella
typhimurium, Staphylococcus aureus, and Escherichia coli.

A 62-year-old woman consults her physician because she has been having
headaches, dizziness, and tinnitus. The patient also reports that
her skin seems to be a great deal more itchy than several years ago, and that the
episodes of itchiness are sometimes triggered by a hot
shower. Physical examination demonstrates hypertension, splenomegaly, and a
ruddy complexion seen best in the face, palms, and nail
beds. The patient did not appear to be dehydrated.
Question 1 of 4
The patient has an erythrocyte count of 6.7 million/mm3. Which of the following
studies would be helpful in establishing that an absolute
increase in erythrocyte mass has occurred?
/ A. Free erythrocyte protoporphyrin
/ B. Hematocrit
/ C. Hemoglobin
/ D. Partial thromboplastin time
/ E. Radioactive chromium labeling

Explanation - Q: 7.1 Close

The correct answer is E. The dilution of small numbers of
51
Cr-labelled
erythrocytes in the patient's blood can be used to calculate the patient's red
cell mass. This is important in cases of erythrocytosis, because it allows the
exclusion of hemoconcentration effects (relative erythrocytosis) not related to
a true increase in red cell mass.
Free erythrocyte protoporphyrin (choice A) is used in the evaluation of the
porphyrias.
Hematocrit (choice B) and hemoglobin (choice C) cannot distinguish
between a relative and an absolute erythrocytosis.
Partial thromboplastin time (choice D) is used in the evaluation of clotting
disorders.
A 62-year-old woman consults her physician because she has been having
headaches, dizziness, and tinnitus. The patient also reports that
her skin seems to be a great deal more itchy than several years ago, and that the
episodes of itchiness are sometimes triggered by a hot
shower. Physical examination demonstrates hypertension, splenomegaly, and a
ruddy complexion seen best in the face, palms, and nail
beds. The patient did not appear to be dehydrated.
Question 2 of 4
If the erythrocytosis is related to ectopic production of erythropoietin, which of the
following tumors would be the most likely source of the
erythropoietin?
/ A. Adenocarcinoma of the breast
/ B. Endometrial adenocarcinoma
/ C. Hepatocellular carcinoma
/ D. Renal cell carcinoma
/ E. Serous cystadenoma of the ovary


Explanation - Q: 7.2 Close

The correct answer is D. Erythropoietin is normally produced in the kidneys,
and renal cell carcinoma can produce it ectopically, producing a form of
secondary erythrocytosis. The other answers are distracters.
A 62-year-old woman consults her physician because she has been having
headaches, dizziness, and tinnitus. The patient also reports that
her skin seems to be a great deal more itchy than several years ago, and that the
episodes of itchiness are sometimes triggered by a hot
shower. Physical examination demonstrates hypertension, splenomegaly, and a
ruddy complexion seen best in the face, palms, and nail
beds. The patient did not appear to be dehydrated.
Question 3 of 4
In addition to the increased red cell count, this patient has a leukocytosis of 15 x
103per microliter, with increases in neutrophils, eosinophils,
and basophils. Her platelet count is 450 x 103per microliter. Which of the
following is most likely to be contributing to this woman's pruritus?
/ A. Basophils
/ B. Eosinophils
/ C. Erythrocytes
/ D. Neutrophils
/ E. PIatelets

Explanation - Q: 7.3 Close

The correct answer is A. This patient's disease is closely related to chronic
myelogenous leukemia, and increases in a variety of cell lines are seen,
although the most striking increase is in the erythrocyte line. Basophils and
mast cells are interrelated cell types that can both release histamine and a
variety of vasoactive chemicals and cytokines. An occasionally helpful clinical
clue is that itchiness not related to an obvious rash can be due to increased
numbers of these cell lines.
High eosinophil (choice B) counts might be associated with itchiness, but in
non-allergic diseases, the high mast cell and basophil counts are more likely
to cause symptoms.
Erythrocytes (choice C), neutrophils (choice D), and platelets (choice E) do
not contain granules that would be likely to cause itchiness.

A 62-year-old woman consults her physician because she has been having
headaches, dizziness, and tinnitus. The patient also reports that
her skin seems to be a great deal more itchy than several years ago, and that the
episodes of itchiness are sometimes triggered by a hot
shower. Physical examination demonstrates hypertension, splenomegaly, and a
ruddy complexion seen best in the face, palms, and nail
beds. The patient did not appear to be dehydrated.
Question 4 of 4
If this patient has a normal arteriaI O2 saturation level and low erythropoietin,
which of the following is the most likely diagnosis?
/ A. Chronic lung disease
/ B. Chronic myelogenous leukemia
/ C. Essential thrombocythemia
/ D. Polycythemia vera
/ E. Tumor-associated erythrocytosis


Explanation - Q: 7.4 Close

The correct answer is D. Polycythemia vera is an idiopathic chronic
myeloproliferative disorder that is characterized by an increased red cell
mass. For the diagnosis to be formally made, the patient must have either all
major criteria for diagnosis, or the first two major criteria plus any two minor
criteria. Major criteria include (1) a red cell mass equal to or greater than 36
mL/kg in men and 32 mL/kg in women; (2) an arterial O
2
saturation greater
than 92%; and (3) splenomegaly (related to extramedullary hematopoiesis).
Minor criteria include (1) thrombocytosis greater than 400 x 10
3
/microliter;
leukocytosis greater than 12 x 10
3
/microliter; leukocyte alkaline phosphatase
activity greater than 100 in the absence of fever and infection (this helps
exclude chronic myelogenous leukemia, choice B); and serum B12 greater
than 900 pg/mL or unsaturated B12-binding capacity greater than 2200
pg/mL (B12 abnormality is common in polycythemia vera). This condition is
closely related to both chronic myelogenous leukemia and essential
thrombocythemia (choice C), in that all cell lines produced in the marrow
tend to be increased, although in the case of polycythemia vera, the
predominant increase is in the erythrocyte line. Patients are usually treated
with recurrent phlebotomy (removal of blood as for a donation) to prevent
complications related to hyperviscosity of the blood (venous thrombosis,
stroke, hepatic portal vein thrombosis, angina pectoris, intermittent
claudication). These patients are also vulnerable to bleeding complications
(related to abnormal platelets), peptic ulcer disease (related to high
histamine levels from basophils and mast cells), and pruritus. Some cases of
polycythemia vera eventually "burn out" and the patient becomes anemic and
requires transfusions. Patients are also vulnerable to developing secondary
leukemias as either a direct complication of their disease or as a
complication of therapy.
Chronic lung disease (choice A) severe enough to cause erythrocytosis
would not show a normal O
2
saturation.
Tumor-associated erythrocytosis (choice E) would show a high
erythropoietin.

A 3-year-child is taken to a physician for a routine physical examination. On
questioning about any problems she may have noticed that
concern her, the mother comments that the child does not seem to be feeling
well much of the time. She has noticed that the child is irritable,
sleeps poorly, and is often constipated. Physical examination is unremarkable.
Office hematocrit shows a mild anemia.
Question 1 of 6
The physician considers the possibility that the child has an environmental
illness. Which of the following is the most common environmental
illness of children in the United States?
/ A. Arsenic poisoning
/ B. Bismuth poisoning
/ C. Copper poisoning
/ D. Insecticide poisoning
/ E. Lead poisoning

Explanation - Q: 8.1 Close

The correct answer is E. Lead poisoning is thought to be the most common
environmental illness of children in this country. A high index of suspicion is
warranted, because no pathognomic symptoms exist and the physical
findings are usually negative. Symptoms that can be seen are often vaguely
neurologic (irritability, either sleeplessness or excessive lethargy, poor
appetite, headaches, vague changes in activity level). Abdominal pain with or
without vomiting can also be seen.
Arsenic poisoning (choice A) is less common than lead poisoning in this
country, and is mostly likely to be the result of either ground-water
contaminated by flowing through arsenic bearing rocks, or ingestion of rat
poison.
Bismuth (choice B) is used industrially and is not usually found in
environments to which children are exposed.
Copper (choice C) is relatively nontoxic, although it can contribute to liver
disease in patients with abnormal copper metabolism (e.g., Wilson's
disease).
Insecticide poisoning (choice D) can occur in children with some frequency
since insecticides are common in households, but this is less frequent than
lead poisoning.
A 3-year-child is taken to a physician for a routine physical examination. On
questioning about any problems she may have noticed that
concern her, the mother comments that the child does not seem to be feeling
well much of the time. She has noticed that the child is irritable,
sleeps poorly, and is often constipated. Physical examination is unremarkable.
Office hematocrit shows a mild anemia.
Question 2 of 6
The child's family is poor, and lives in an apartment built in the 1930's and never
substantively remodeled. Rodents and insects are common
in the building, as are pesticides used to try to eliminate them. If the child does
have the most common environmental illness in US children,
which of the following would be the most likely source of the poison?
/ A. Crumbling sidewalk fragments
/ B. OId metal doorknob
/ C. OId paint chips
/ D. Rat poison
/ E. Roach poison


Explanation - Q: 8.2 Close

The correct answer is C. In the past, the interior paint used on houses often
contained lead, which provided a very white color to the paint (to which other
colors could be added to produce vibrant paints). These paints are no longer
in use, but there is enough very old housing still present in this country that
lead poisoning remains a significant problem. The old paint often chips off
and the chips may be ingested by toddlers. The affected children have often
been bottle-fed for protracted periods and have developed some degree of
pica (ingestion of non-food substances). Lead was also commonly used in
plumbing pipes in early houses as well. In black, non-Hispanic children living
in homes built before 1946, the prevalence of lead poisoning is almost 22%.
The rates drop to 13.7% in those living in homes built from 1946 to 1973, and
then to 3.4% in homes built after 1973. Rates of lead poisoning are lower
among Mexican-American children (13%, 2.3% and 1.6%, respectively) and
white, non-Hispanic children (5.6%, 1.4%, and 1.5%, respectively). Lead
poisoning from paints also is occasionally a problem when decorative dishes
are used to contain acid foods (the classic example is an imported Mexican
pitcher used for orange juice) that leak the lead. Also, exposures can come
from some folk remedies and from adults working in lead-related
occupations.
The crumbling sidewalk fragments (choice A) and the old metal doorknob
(choice B) are probably non-toxic.
Rat poison (choice D) can contain arsenic and roach poison (choice E) can
contain organophosphate insecticides.
A 3-year-child is taken to a physician for a routine physical examination. On
questioning about any problems she may have noticed that
concern her, the mother comments that the child does not seem to be feeling
well much of the time. She has noticed that the child is irritable,
sleeps poorly, and is often constipated. Physical examination is unremarkable.
Office hematocrit shows a mild anemia.
Question 3 of 6
Which of the following is traditionally associated with this child's disease, but is
actually now uncommonly seen?
/ A. Aphthous ulcer on inside of cheek
/ B. Dark line on the gums
/ C. Fissures on the tongue
/ D. Hairy leukoplakia of the tongue
/ E. Pyogenic granuloma of gums


Explanation - Q: 8.3 Close

The correct answer is B. The "lead-line," which is due to lead deposition
along the gums, is traditionally associated with lead poisoning, but has
become uncommon because we are picking up most cases of lead poisoning
earlier, when the body load of lead is smaller. The physician should look for it
in suspected cases of lead poisoning, even though he or she probably won't
find it.
Aphthous ulcers (choice A), commonly called "canker sores," are idiopathic
benign oral ulcers that can occur with emotional distress, or unrelated to any
obvious inciting causes.
Fissures on the tongue (choice C) can be seen with a variety of nutritional
deficiencies.
Hairy leukoplakia (choice D) is a distinctive oral lesion (fuzzy white tongue)
seen in some AIDS patients.
Pyrogenic granuloma (choice E) produces a ball-like red mass lesion, and
can occur sporadically or in association with pregnancy.
A 3-year-child is taken to a physician for a routine physical examination. On
questioning about any problems she may have noticed that
concern her, the mother comments that the child does not seem to be feeling
well much of the time. She has noticed that the child is irritable,
sleeps poorly, and is often constipated. Physical examination is unremarkable.
Office hematocrit shows a mild anemia.
Question 4 of 6
This child's disease often produces an anemia that on peripheral smear most
closely resembles which of the following?
/ A. Folate deficiency
/ B. Hereditary spherocytosis
/ C. Iron deficiency
/ D. Sickle cell anemia
/ E. Vitamin B12 deficiency


Explanation - Q: 8.4 Close

The correct answer is C. The anemia of lead toxicity is a microcytic,
hypochromic anemia that closely resembles that of iron deficiency on the
peripheral blood smear. While the gold standard for diagnosing lead
poisoning is the whole blood lead level, the free erythrocyte protoporphyrin
level is often used to demonstrate the degree of biological abnormalities that
exists. Lead interferes with ferrochelatase, the enzyme helps to incorporate
iron into the protoporphyrin molecule in hemoglobin synthesis. This is why
the anemia of lead poisoning clinically resembles that of iron deficiency - the
iron is present, but the body cannot use it.
Folate deficiency (choice A) and vitamin B12 deficiency (choice E) both
produce macrocytic anemia.
In hereditary spherocytosis (choice B), all of the erythrocytes are small
spheres that lack the biconcave shape typical of normal erythrocyte.
In sickle cell anemia (choice D), even when the patient is not in a sickling
crisis, at least a few markedly deformed sickle cells will be seen.

Question 5 of 6


A peripheral blood smear is examined (above). Which of the following diagnostic
features is present in the smear?
/ A. Acanthocytes
/ B. Auer rods
/ C. Basophilic stippling
/ D. Hairy cells
/ E. Sickle cells


Explanation - Q: 8.5 Close

The correct answer is C. Basophilic stippling causes round, dark-blue
granules in red blood cells on smears stained with brilliant cresyl blue (the
granules are more red-purple on Wright/Giemsa stains). The granules are
precipitated ribosomes and mitochondria, and are usually caused by an
impairment of maturation in the bone marrow. Causes include lead
poisoning, exposure to some drugs, severe burns, anemias, and septicemia.
Acanthocytes (choice A) are "star-like" red cells with irregularly spaced
projections that can be found in abetalipoproteinemia and some liver
diseases.
Auer rods (choice B) are elongated, bluish-red rods composed of fused
lysosomal granules. They are found in the cytoplasm of cells in the
myelocytic and monoblastic lines in patients with acute myelogenous
leukemia.
Hairy cells (choice D) are seen only in hairy cell leukemia and are white cells
with fine, irregular pseudopods ("hairs") and immature nuclear features.
Sickle cells (choice E) are seen in sickle cell anemia, not lead poisoning.

A 3-year-child is taken to a physician for a routine physical examination. On
questioning about any problems she may have noticed that
concern her, the mother comments that the child does not seem to be feeling
well much of the time. She has noticed that the child is irritable,
sleeps poorly, and is often constipated. Physical examination is unremarkable.
Office hematocrit shows a mild anemia.
Question 6 of 6
Which of the following is an oral drug that can be used to treat this disorder in
children?
/ A. Amantadine
/ B. Succimer
/ C. Sulfasalazine
/ D. Tolbutamide
/ E. Tubocurarine

Explanation - Q: 8.6 Close

The correct answer is B. Lead poisoning is treated with lead chelating
agents to increase urinary secretion. Succimer is the only one of these
agents which has been approved for oral use in children; D-penicillamine is
also commonly used, but has never been formally approved for this use by
the FDA. Other chelating agents that are sometimes used in severe acute
lead poisoning include dimercaprol (IM) and calcium disodium edetate (IM or
IV).
Amantadine (choice A) is an antiviral agent active against influenza A virus.
Sulfasalazine (choice C) is used in rheumatoid arthritis.
Tolbutamide (choice D) is a sulfonylurea used as an oral hypoglycemic
agent.
Tubocurarine (choice E) is a skeletal muscle relaxant.


A 55-year-old man consults a physician because he has been experiencing
chronic weakness and fatigue. Physical examination is notable
for the presence of petechiae and a massively enlarged spleen. A peripheral
blood smear was sent, which on review demonstrates anemia,
thrombocytopenia, modest neutropenia, and the presence of small numbers of
unusual white blood cells. These blood cells are small cells
with a moderate amount of cytoplasm that are covered with distinctive
cytoplasmic projections that appear to be long microvilli.
Question 1 of 5
Which of the following is the most likely diagnosis?
/ A. Chronic myelogenous leukemia
/ B. Erythroleukemia
/ C. Hairy cell leukemia
/ D. Hodgkin's lymphoma
/ E. Metastatic oat cell carcinoma

Explanation - Q: 9.1 Close

The correct answer is C. Hairy cell leukemia is a chronic leukemia that
often has an indolent course. It can present with weakness and fatigue due
to anemia, bleeding due to thrombocytopenia, or fever and infections due to
neutropenia. Almost every patient has splenomegaly, which is massive in 4/5
of cases. The male to female ratio is 5:1 and the patients are usually middle
aged with a median age of 52. The other conditions listed would not have
"hairy cells" in the peripheral blood.

A 55-year-old man consults a physician because he has been experiencing
chronic weakness and fatigue. Physical examination is notable
for the presence of petechiae and a massively enlarged spleen. A peripheral
blood smear was sent, which on review demonstrates anemia,
thrombocytopenia, modest neutropenia, and the presence of small numbers of
unusual white blood cells. These blood cells are small cells
with a moderate amount of cytoplasm that are covered with distinctive
cytoplasmic projections that appear to be long microvilli.
Question 2 of 5
Which of the following tests could confirm this diagnosis?
/ A. 5-hydroxyindoleacetic acid
/ B. Free erythrocyte protoporphyrin
/ C. Homovanillic acid
/ D. Myelin basic protein
/ E. Tartrate-resistant acid phosphatase


Explanation - Q: 9.2 Close

The correct answer is E. Strong positivity for tartrate-resistant acid
phosphatase (TRAP) is a classic finding in hairy cell leukemia cells, and you
may see an item about this on a USMLE question. However, you should also
be aware that this test is no longer available at many centers.
5-hydroxyindoleacetic acid (choice A) is a serotonin derivative used in
diagnosing carcinoid tumors.
Free erythrocyte protoporphyrin (choice B) is used in the evaluation of
porphyrias.
Homovanillic acid (choice C) is a urinary catecholamine derivative that is
used in diagnosis of pheochromocytomas.
Myelin basic protein (choice D) is used in diagnosing multiple sclerosis.
A 55-year-old man consults a physician because he has been experiencing
chronic weakness and fatigue. Physical examination is notable
for the presence of petechiae and a massively enlarged spleen. A peripheral
blood smear was sent, which on review demonstrates anemia,
thrombocytopenia, modest neutropenia, and the presence of small numbers of
unusual white blood cells. These blood cells are small cells
with a moderate amount of cytoplasm that are covered with distinctive
cytoplasmic projections that appear to be long microvilli.
Question 3 of 5
Which of the following immunologic markers is considered to have high
sensitivity and specificity for this patient's disease?
/ A. BIy-7
/ B. CD3
/ C. CD19
/ D. CD20
/ E. CD21



Explanation - Q: 9.3 Close

The correct answer is A. Immunophenotyping of peripheral blood has
mostly replaced the (less sensitive) TRAP test for diagnosis, and is thought
to be capable of making the correct diagnosis in this setting in over 90% of
cases, even when only very small numbers of circulating leukemia cells are
present. Monoclonal Bly-7 is both sensitive and specific for hairy cell
leukemia cells.
These cells also express CD19 (choice C) and CD20 (choice D), but these
are common pan-B cell antigens and are not specific for hairy cell leukemia.
They do not express the late B cell antigen CD21 (choice E) or the T-cell
antigen CD3 (choice B). A last useful tip is that they also stain for CD22 at a
higher intensity than normal B cells.
A 55-year-old man consults a physician because he has been experiencing
chronic weakness and fatigue. Physical examination is notable
for the presence of petechiae and a massively enlarged spleen. A peripheral
blood smear was sent, which on review demonstrates anemia,
thrombocytopenia, modest neutropenia, and the presence of small numbers of
unusual white blood cells. These blood cells are small cells
with a moderate amount of cytoplasm that are covered with distinctive
cytoplasmic projections that appear to be long microvilli.
Question 4 of 5
The cells described are abnormal cells in which of the following cell lines?
/ A. B-Iymphocytes
/ B. Eosinophils
/ C. Macrophages
/ D. Neutrophils
/ E. T-Iymphocytes

Explanation - Q: 9.4 Close

The correct answer is A. Hairy cell leukemia cells are an abnormal type of
mature B lymphocytes. Unlike many leukemias, these cells are often present
in circulating blood in small numbers, although involvement of the spleen is
more prominent. Bone marrow aspiration is often unsuccessful due to a dry
tap; core biopsy will show the tumor cells with abundant cytoplasm infiltrating
a fine fibrillar network. The hairy cell leukemia cells are sensitive to purine
analogues (cladribine) and interferons. Granulocyte colony stimulating factor
(filgrastim) is used to reduce the neutropenia that may produce life-
threatening susceptibility to infections (gram-negative rods, atypical
mycobacteria, disseminated fungus, and Pneumocystis). Patients often
experience long-term remissions with chemotherapy, and late relapses (after
5-10 years) often respond a second time to the same chemotherapy. The
other choices listed are distracters.

A 55-year-old man consults a physician because he has been experiencing
chronic weakness and fatigue. Physical examination is notable
for the presence of petechiae and a massively enlarged spleen. A peripheral
blood smear was sent, which on review demonstrates anemia,
thrombocytopenia, modest neutropenia, and the presence of small numbers of
unusual white blood cells. These blood cells are small cells
with a moderate amount of cytoplasm that are covered with distinctive
cytoplasmic projections that appear to be long microvilli.
Question 5 of 5
Which of the following is the most appropriate pharmacotherapy?
/ A. CIadribine
/ B. Cyclophosphamide
/ C. FIudarabine
/ D. Methotrexate
/ E. 6-Mercaptopurine


Explanation - Q: 9.5 Close

The correct answer is A. The treatment of hairy cell leukemia (HCL) has
dramatically improved with the development of newer, more effective
antineoplastic agents, such as cladribine. Cladribine is considered to be the
treatment of choice for this condition. This is a relatively nontoxic drug that
provides benefit in approximately 95% of all cases and a complete remission
in more than 80% of these cases. In general, response rates are long-lasting,
with few patients relapsing in the first several years. Interferon and
splenectomy are rarely used today to treat this condition. With respect to the
pharmacology of this agent, cladribine is indicated for the treatment of active
HCL as defined by clinically significant anemia, neutropenia,
thrombocytopenia, or disease related symptoms. Non-labeled uses include
treatment of advanced cutaneous T-cell lymphoma, chronic lymphocytic
leukemia, non-Hodgkin lymphomas, acute myeloid leukemia, and
autoimmune hemolytic anemia.
Cyclophosphamide (choice B) is an antineoplastic commonly used in the
treatment of a variety of malignant diseases, such as Hodgkin disease as
well as treatment of several types of leukemia. This medication is an
alkylating agent related to the nitrogen mustards. It suppresses cell-mediated
and humoral immunity as to interfere with "recall responses", causes
lymphopenia of B and T cells, and reduces serum immunoglobulin levels.
Fludarabine (choice C) is an antimetabolite antineoplastic agent indicated
for the treatment of chronic lymphocytic leukemia (CLL). Although not
approved for hairy cell leukemia, this agent has been used with some
success in the treatment of this condition. Fludarabine is specifically a
fluorinated nucleotide analog of the antiviral agent, vidarabine. This agent
appears act by inhibiting DNA polymerase alpha, ribonucleotide reductase,
and DNA primase, thus inhibiting DNA synthesis.
Methotrexate (choice D) is an agent that competitively inhibits dihydrofolic
acid reductase and is also used in antineoplastic therapeutic treatment
regimens, such as ALL, cancers of the head, neck, and breast, usually in
combination with other therapies. It is also used to treat severe, active,
classic or definite rheumatoid arthritis in adults who have an insufficient
response with conventional therapies, and for severe, recalcitrant, disabling
psoriasis.
6-Mercaptopurine (choice E), otherwise known as mercaptopurine or 6-MP,
is a purine analog antimetabolite indicated for the treatment of remission
induction and maintenance therapy of acute lymphocytic leukemia (ALL) as
well as treatment of acute myelogenous (and acute myelomonocytic)
leukemia.

A college student is brought to the student health center at the urging of his
roommate. He has been missing class because he needs to
check the room lock many times before he can leave. Once he starts to ride his
bicycle to class, he frequently returns several times to lock the
door. He repeats this ritual every morning and often when he leaves the house.
He misses his appointments and his academic performance
suffers. His hands are chafed.
Question 1 of 4
Which of the following is the most likely diagnosis?
/ A. Generalized anxiety disorder
/ B. Obsessive compulsive disorder
/ C. Panic disorder
/ D. Paranoid personality disorder
/ E. Posttraumatic stress disorder

Explanation - Q: 1.1 Close

The correct answer is B. Patients with obsessive compulsive disorder
(OCD) suffer from obsessive thoughts and compulsive behaviors that impair
everyday function. Obsessions are defined as recurrent and persistent
thoughts, impulses, or images that are intrusive, inappropriate, and cause
anxiety and distress. Patients realize that these thoughts and images are a
product of their own mind and they will attempt to suppress them. In addition
to obsessions, patients experience compulsions. Compulsions are repetitive
behaviors or mental acts that a person performs in accordance with an
obsession. The behaviors are aimed at reducing distress or preventing some
dreaded event or situation. Locking doors is a common compulsion, and thus
this patient meets the diagnostic criteria for obsessive compulsive disorder.
Generalized anxiety disorder (choice A) is characterized by excessive
anxiety and apprehensive expectation for a period greater than 6 months.
Patients experience anxiety, cognitive vigilance, autonomic hyperactivity,
motor tension, irritability, and poor concentration. Compulsions are not a part
of this disorder.
Panic disorder (choice C) is characterized by episodes of panic. Patients
have a discrete period of intense fear with tachycardia, palpitations,
sweating, trembling, shortness of breath, chest pain and tightening,
abdominal discomfort, fear of dying, and paresthesias.
Paranoid personality disorder (choice D) is characterized by enduring
patterns of personality characterized by mistrust and suspiciousness of
people.
Posttraumatic stress disorder (choice E) is an anxiety disorder that develops
around a traumatic event. Symptoms revolve around the event and include
reexperiencing of the trauma, psychic numbing, and increased autonomic
arousal.
A college student is brought to the student health center at the urging of his
roommate. He has been missing class because he needs to
check the room lock many times before he can leave. Once he starts to ride his
bicycle to class, he frequently returns several times to lock the
door. He repeats this ritual every morning and often when he leaves the house.
He misses his appointments and his academic performance
suffers. His hands are chafed.
Question 2 of 4
The patient's incessant door locking is an example of which of the following?
/ A. Compulsion
/ B. Delusion
/ C. Magical thinking
/ D. Obsession
/ E. Paranoid ideation

Explanation - Q: 1.2 Close

The correct answer is A. Compulsions are repetitive behaviors or mental
acts that patients perform in accordance with an obsession (choice D). It is
important to realize that obsessions are the mental processes and that
compulsions are the actions or behaviors.
Delusions (choice B) are fixed false beliefs that are not culturally accepted.
(People that believe in Santa Claus are not deluded.)
Magical thinking (choice C) is a mental process, not a behavior, like door
locking. Patients with magical thinking believe they have special, "magical"
capacities that others do not have. Thus door locking does not exemplify
magical thinking.
Door locking does not exemplify paranoid ideation (choice E). However,
paranoid ideation, or the patient's belief that others are out to harm him/her,
may provide the source of anxiety to drive the compulsion.
A college student is brought to the student health center at the urging of his
roommate. He has been missing class because he needs to
check the room lock many times before he can leave. Once he starts to ride his
bicycle to class, he frequently returns several times to lock the
door. He repeats this ritual every morning and often when he leaves the house.
He misses his appointments and his academic performance
suffers. His hands are chafed.
Question 3 of 4
Which of the following is the most appropriate pharmacotherapy for this patient?
/ A. CIozapine
/ B. Desipramine
/ C. FIuoxetine
/ D. Haloperidol
/ E. Lorazepam


Explanation - Q: 1.3 Close

The correct answer is C. The effectiveness of selective serotonin reuptake
inhibitors (SSRIs) in treating OCD has contributed significant indirect
evidence to the role of the serotonergic system in the pathophysiology of
OCD. It is hypothesized that dysregulation of this neurotransmitter could
contribute to the repetitive obsessions and ritualistic behaviors. This
hypothesis is also supported by the relative ineffectiveness of noradrenergic
antidepressants, such as desipramine.
Clozapine (choice A) is an atypical antipsychotic, and would not play a role
in the treatment of OCD unless psychotic features were noted.
Desipramine (choice B) is a noradrenergic antidepressant, and as noted
above, has no effect on OCD symptoms.
Haloperidol (choice D) is an antipsychotic drug that has no effect on OCD
symptoms unless psychotic features were noted.
Lorazepam (choice E) is a benzodiazepine that is used to treat acute
agitation. It would not be used to treat OCD.

A college student is brought to the student health center at the urging of his
roommate. He has been missing class because he needs to
check the room lock many times before he can leave. Once he starts to ride his
bicycle to class, he frequently returns several times to lock th
door. He repeats this ritual every morning and often when he leaves the house.
He misses his appointments and his academic performance
suffers. His hands are chafed.
Question 4 of 4
According to psychoanalytic theory, this patient's disorder develops when
defense mechanisms fail to contain the patient's anxiety. One
defense mechanism employed in this patient's constellation of symptoms is
reaction formation. Which of the following is an example of
reaction formation?
/ A. A man ignores the fact that his spouse is cheating on him and they invest in
a house together
/ B. A man in the intensive care unit becomes infantile and unruly
/ C. A man in the intensive care unit tells his nurse, "You that are the best nurse
l have ever seen-unlike those horrible nurses yesterday who
made me wait for pain medicine. Those nurses were horrible and l never want to
see them again."
/ D. A man who is extremely angry with his spouse treats her gently and kindly
/ E. A promiscuous man accuses his spouse of being unfaithful to him

Explanation - Q: 1.4 Close

The correct answer is D. Obsessional patients often show the defense
mechanism of reaction formation. Reaction formation is when affects are
transformed into their opposites and ambivalence is resolved in the opposite
manner from which it arises. This man resolves his anger with his wife by
creating the opposite affect.
Choice A exemplifies the defense mechanism known as denial. Denial is the
invalidation of an unpleasant or unwanted piece of information. He denies
that his marriage is compromised, and continues investing in it.
Choice B exemplifies regression. When regression is employed, patients
return to an earlier level of functioning. This patient's infantile behavior
represents regression. Regression is often seen in medically ill patients.
Choice C exemplifies splitting. In splitting, aspects of mental content are kept
separate. The man has overidealized those who met his needs, and
devalued those who frustrate him. This defense is often seen in patients with
borderline personality disorder.
Choice E exemplifies projection. In projection, a person rids him/herself of
unacceptable thoughts by attributing them to others. While this rids the
affected individual of the unwanted affect, he/she then lives in a world of
others who harbor the unacceptable material. This is often seen in paranoid
patients.


A 32-year-old married lawyer presents to the emergency department with a
complaint of "having a heart attack." He explains he was "doing
nothing particular" at home about 45 minutes ago when he began having chest
pain with shortness of breath and nausea. His symptoms
peaked within ten minutes, and he "knew this was the big one." His wife noted he
was "shaking and sweaty." His wife immediately brought
him to the hospitaI. He has no significant past medical history, takes no
medications, and denies substance use. His family medical history is
significant for a paternal grandfather that "died of a massive heart attack" at age
56. Physical examination reveals an anxious diaphoretic
man taking short, shallow breaths. Vital signs, cardiac auscultation, ECG, and
cardiac enzymes are completely normaI. The patient has been
to the emergency department 5 times in the last 6 weeks and apologizes for "the
million dollar workup," but explains "every time it happens l
just know l am doomed to die."
Question 1 of 6
Which of the following is the most likely diagnosis?
/ A. Agoraphobia
/ B. Generalized anxiety disorder
/ C. Malingering
/ D. Panic disorder
/ E. Phobia


Explanation - Q: 2.1 Close

NONE AVAILABLE

A 32-year-old married lawyer presents to the emergency department with a
complaint of "having a heart attack." He explains he was "doing
nothing particular" at home about 45 minutes ago when he began having chest
pain with shortness of breath and nausea. His symptoms
peaked within ten minutes, and he "knew this was the big one." His wife noted he
was "shaking and sweaty." His wife immediately brought
him to the hospitaI. He has no significant past medical history, takes no
medications, and denies substance use. His family medical history is
significant for a paternal grandfather that "died of a massive heart attack" at age
56. Physical examination reveals an anxious diaphoretic
man taking short, shallow breaths. Vital signs, cardiac auscultation, ECG, and
cardiac enzymes are completely normaI. The patient has been
to the emergency department 5 times in the last 6 weeks and apologizes for "the
million dollar workup," but explains "every time it happens l
just know l am doomed to die."
Question 2 of 6
Which of the following criteria would serve to exclude the most likely diagnosis?
/ A. Panic attacks beginning during sleep
/ B. Panic attacks beginning while driving
/ C. Panic attacks occurring at work and home
/ D. Panic attacks occurring "out of the blue"
/ E. Panic attacks occurring with caffeine intake

Explanation - Q: 2.2 Close

The correct answer is E. Panic attacks resulting from substances
(especially stimulants) or general medical conditions are not considered
panic disorder. Myocardial infarction, hypothyroidism, and carcinoid
syndrome should be ruled out.
Panic attacks may begin while driving, or awaken patients from sleep
(choices A and B).
Panic attacks may occur anywhere, including work and home (choice C).
Panic attacks ONLY occurring with the trigger of being in open spaces are
diagnostic of agoraphobia (a phobia).
In panic disorder, panic attacks are unprecipitated ("out of the blue"; choice
D). Panic attacks triggered by a feared event or object are seen in phobias.
A 32-year-old married lawyer presents to the emergency department with a
complaint of "having a heart attack." He explains he was "doing
nothing particular" at home about 45 minutes ago when he began having chest
pain with shortness of breath and nausea. His symptoms
peaked within ten minutes, and he "knew this was the big one." His wife noted he
was "shaking and sweaty." His wife immediately brought
him to the hospitaI. He has no significant past medical history, takes no
medications, and denies substance use. His family medical history is
significant for a paternal grandfather that "died of a massive heart attack" at age
56. Physical examination reveals an anxious diaphoretic
man taking short, shallow breaths. Vital signs, cardiac auscultation, ECG, and
cardiac enzymes are completely normaI. The patient has been
to the emergency department 5 times in the last 6 weeks and apologizes for "the
million dollar workup," but explains "every time it happens l
just know l am doomed to die."
uestion 3 of 6
Which of the following would be the most appropriate pharmacotherapy?
/ A. CIozapine
/ B. Disulfiram
/ C. FIuoxetine
/ D. Lithium
/ E. Risperidone


Explanation - Q: 2.3 Close

The correct answer is C. The selective serotonin reuptake inhibitors
(SSRIs) are considered first line treatment for panic disorder. Buspirone and
the benzodiazepines are also used.
Clozapine (choice A) is an atypical antipsychotic reserved for treatment of
refractory schizophrenia, due to the risk of agranulocytosis and myocarditis.
Disulfiram (choice B) is used as an adjunct treatment to maintain sobriety.
Patients ingesting alcohol while taking this medicine become ill, due to
accumulation of acetaldehyde.
Lithium (choice D) is a first line treatment for bipolar disorder, and can be
used to augment antidepressant medicines.
Risperidone (choice D) is an atypical antipsychotic medication with
prominent D2 blockade. It is used to treat psychotic conditions, such as
schizophrenia
A 32-year-old married lawyer presents to the emergency department with a
complaint of "having a heart attack." He explains he was "doing
nothing particular" at home about 45 minutes ago when he began having chest
pain with shortness of breath and nausea. His symptoms
peaked within ten minutes, and he "knew this was the big one." His wife noted he
was "shaking and sweaty." His wife immediately brought
him to the hospitaI. He has no significant past medical history, takes no
medications, and denies substance use. His family medical history is
significant for a paternal grandfather that "died of a massive heart attack" at age
56. Physical examination reveals an anxious diaphoretic
man taking short, shallow breaths. Vital signs, cardiac auscultation, ECG, and
cardiac enzymes are completely normaI. The patient has been
to the emergency department 5 times in the last 6 weeks and apologizes for "the
million dollar workup," but explains "every time it happens l
just know l am doomed to die."
Question 4 of 6
Years later, the patient develops a "fear of flying" and is started in once-weekly
therapy for systematic desensitization. Which of the following
forms of psychotherapy is systematic desensitization?
/ A. Behavioral psychotherapy
/ B. Cognitive psychotherapy
/ C. Family psychotherapy
/ D. Group psychotherapy
/ E. Psychoanalytic psychotherapy


Explanation - Q: 2.4 Close

The correct answer is A. Behavioral therapies are based on the learning
theory (operant and classical conditioning). If the anxiety is uncoupled from
the situation, the avoidant behavior will decrease. In systematic
desensitization, the patient constructs a hierarchy of images, and gradually
works to tolerate imagining the most fearful situation. Behavioral
psychotherapy explains behavior as being shaped by reward or punishment,
unlike cognitive psychotherapy which posits behavior as secondary to the
way a person thinks.
Cognitive psychotherapy (choice B) is based on the premise that behavior
can be changed by challenging errors in thinking (cognitive distortions).
"Homework" is used to ascertain the underlying (negative) assumptions.
Family psychotherapy (choice C) is based on the theory that a family is a
system striving to maintain homeostasis, which leads to behaviors.
Group psychotherapy (choice D) is based on many theories, and techniques
include identification and universalization.
Psychoanalytic psychotherapy (choice E) is an intensive type of therapy,
usually 4-5 times per week; the goal is for the patient to develop insight into
unconscious conflicts, and become more aware of the underlying causes of
behavior.
Also, it is not uncommon for a patient to develop more than one anxiety
disorder (panic disorder and a phobia).
A 32-year-old married lawyer presents to the emergency department with a
complaint of "having a heart attack." He explains he was "doing
nothing particular" at home about 45 minutes ago when he began having chest
pain with shortness of breath and nausea. His symptoms
peaked within ten minutes, and he "knew this was the big one." His wife noted he
was "shaking and sweaty." His wife immediately brought
him to the hospitaI. He has no significant past medical history, takes no
medications, and denies substance use. His family medical history is
significant for a paternal grandfather that "died of a massive heart attack" at age
56. Physical examination reveals an anxious diaphoretic
man taking short, shallow breaths. Vital signs, cardiac auscultation, ECG, and
cardiac enzymes are completely normaI. The patient has been
to the emergency department 5 times in the last 6 weeks and apologizes for "the
million dollar workup," but explains "every time it happens l
just know l am doomed to die."
Question 5 of 6
A full cardiac workup of this patient is most likely to reveal which of the following?
/ A. Coronary vasospasm
/ B. Ebstein's anomaly
/ C. Mitral valve prolapse
/ D. Myocarditis
/ E. QTc prolongation

Explanation - Q: 2.5 Close

The correct answer is C. As many as 50% of patients with panic disorder
also have mitral valve prolapse. A beta blocker may alleviate some
symptoms.
Coronary vasospasm (choice A) occurs with cocaine use and usually
presents with the signs and symptoms of a myocardial infarction (with a
positive urine toxicology screen for cocaine).
Ebstein's anomaly (choice B) is a rare, albeit notorious, congenital defect
associated with prenatal exposure to lithium.
Myocarditis (choice D) has occurred with clozapine, which received a "black
box" warning in the PDR in 2002.
QTc prolongation (choice E) can occur with antipsychotic medicines and
tricyclic antidepressants. Torsades may develop.
A 32-year-old married lawyer presents to the emergency department with a
complaint of "having a heart attack." He explains he was "doing
nothing particular" at home about 45 minutes ago when he began having chest
pain with shortness of breath and nausea. His symptoms
peaked within ten minutes, and he "knew this was the big one." His wife noted he
was "shaking and sweaty." His wife immediately brought
him to the hospitaI. He has no significant past medical history, takes no
medications, and denies substance use. His family medical history is
significant for a paternal grandfather that "died of a massive heart attack" at age
56. Physical examination reveals an anxious diaphoretic
man taking short, shallow breaths. Vital signs, cardiac auscultation, ECG, and
cardiac enzymes are completely normaI. The patient has been
to the emergency department 5 times in the last 6 weeks and apologizes for "the
million dollar workup," but explains "every time it happens l
just know l am doomed to die."
Question 6 of 6
The patient returns to the emergency department in a "bizarre state." Friends
report he has been "Iaughing like a loon at all the wrong things,"
"Iooking over his shoulder in public places," and repeatedly questioning their
motives. Examination reveals tachycardia, scleral injection, and
a dry cough. Use of which of the following substances is most likely to explain
this patient's symptoms?
/ A. AIprazolam
/ B. Caffeine
/ C. Cannabis
/ D. Methamphetamine
/ E. Pseudoephedrine

Explanation - Q: 2.6 Close

The correct answer is C. Inappropriate laughter, paranoia, and tachycardia,
scleral injection, and a dry cough are all associated with cannabis use.
Alprazolam (choice A) may cause sedation, slurred speech, disinhibition and
ataxia. Patients seem "drunken."
Caffeine use (choice B) may initially present with panic attacks, but when
carefully questioned, the patient will report caffeine intake (coffee, tea,
chocolate, cocoa, over-the-counter cold medications) and usually develops
headaches (during withdrawal) from caffeine. Caffeine intoxicated patients
could develop paranoia and tachycardia, but cannabis use explains all the
listed symptoms.
Methamphetamine (choice D) and pseudoephedrine (choice E) are
stimulants. Patients may initially present with panic attacks, and can develop
paranoia and tachycardia, but the additional history of inappropriate laughter
and scleral injection indicates cannabis use. Amphetamine abuse mimics
symptoms of schizophrenia.

A 30-year-old man is brought to the emergency department by police, who
arrested him because he was in the parking lot of a local malI,
yelling "I am a golden god" as he stepped in front of moving cars. When
questioned about his identity, he talks incessantly in a rapid fashion
and threatens to "unleash God's wrath on those who do not submit." He reports
that he has not slept in a week, and does not need sleep. He
has spent the last week preparing for a secret government mission that only the
president knows about. When asked if he ever hears God
talking to him, he says, "Of course not, I am God!" Police report that he has had
several prior arrests for reckless driving and lewd acts. On
examination, he is disheveled and malodorous. It is nearly impossible to get any
further history, as he rapidly paces about and mutters, "I
must go, I must go" over and over again.
Question 1 of 7
Which of the following is the most likely preliminary diagnosis?
/ A. Antisocial personality disorder
/ B. Bipolar disorder
/ C. Borderline personality disorder
/ D. Post traumatic stress disorder
/ E. Schizophrenia

Explanation - Q: 3.1 Close

The correct answer is B. This patient is displaying many of the diagnostic
criteria for bipolar disorder. He has had a distinct period of abnormally and
persistently elevated mood lasting at least one week. In addition, his
thoughts are grandiose ("I am a golden god"), he has a decreased need for
sleep, and he has pressured and excessive speech. His arrest history
indicates possible previous sexual indiscretions, which are a hallmark of
bipolar disorder (excessive involvement in pleasurable activities that have a
high potential for painful consequences). His pacing indicates psychomotor
agitation, also a hallmark of bipolar disorder. This patient probably has a
history of severe depressive episodes, but the occurrence of a single manic
episode allows the diagnosis of bipolar disorder to be made.
Antisocial personality disorder (choice A) refers to a long-standing pattern of
socially irresponsible behavior that reflects a disregard for the rights of
others. These individuals were formerly called psychopaths, and generally
lack a conscience. Many people with this disorder engage in unlawful acts.
The most pervasive characteristic is a lack of remorse for the harm they
cause others.
Borderline personality disorder (choice C) refers to a lifelong pattern of
unstable affect and self-image with erratic behavior. Borderlines have
interpersonal relationships that are intense, but very unstable ("love-hate"
relationships). They are prone to self-mutilation and the defense mechanism
of splitting. But they have a life of chaos, not a sense of grandeur and power,
so the diagnosis does not fit this case.
Posttraumatic stress disorder (choice D) is an anxiety disorder that develops
surrounding a traumatic event. Symptoms revolve around the event and
include reexperiencing of the trauma, avoidance of associated stimuli,
psychic numbing, and increased autonomic arousal.
While the psychotic symptoms of mania and schizophrenia (choice E) may,
at times, be difficult to distinguish, the grandiose content of this man's
symptoms suggest that he is having a manic episode. Note that his
comments that God does not talk to him can be taken as a denial of auditory
hallucinations.
A 30-year-old man is brought to the emergency department by police, who
arrested him because he was in the parking lot of a local malI,
yelling "I am a golden god" as he stepped in front of moving cars. When
questioned about his identity, he talks incessantly in a rapid fashion
and threatens to "unleash God's wrath on those who do not submit." He reports
that he has not slept in a week, and does not need sleep. He
has spent the last week preparing for a secret government mission that only the
president knows about. When asked if he ever hears God
talking to him, he says, "Of course not, I am God!" Police report that he has had
several prior arrests for reckless driving and lewd acts. On
examination, he is disheveled and malodorous. It is nearly impossible to get any
further history, as he rapidly paces about and mutters, "I
must go, I must go" over and over again.
Question 2 of 7
Before a definitive diagnosis can be made, which of the following should be
performed?
/ A. Cranial nerve exam
/ B. CT scan of the head
/ C. EIectroencephalogram
/ D. Magnetic resonance imaging of the head
/ E. Toxicological screen

Explanation - Q: 3.2 Close

The correct answer is E. Intoxication with a number of agents such as
amphetamines, cocaine, or other sympathomimetics can mimic mania seen
in bipolar disorder. Other possibilities to consider are antidepressant
medications, thyroid hormone replacements, hyperthyroidism, and other
neurologic conditions. It is often difficult to distinguish the mania of bipolar
disorder from the mania of substance abuse. Often the two are comorbid
conditions as patients "treat" their condition. If a manic episode is substance-
induced, it cannot contribute to a diagnosis of bipolar disorder.
Cranial nerve exam (choice A) cannot be performed on an uncooperative
patient and would likely not contribute to reaching a diagnosis in this patient.
CT scan of the head (choice B) would not likely aid in the diagnosis of this
patient unless something in the patient's medical history suggests an organic
cause (e.g., frontal neoplasm).
An electroencephalogram (choice C) would not contribute to the diagnosis of
this patient as there is no evidence of seizures.
MRI (choice D) would not contribute to the diagnosis for the same reasons a
CT scan would not.

A 30-year-old man is brought to the emergency department by police, who
arrested him because he was in the parking lot of a local malI,
yelling "I am a golden god" as he stepped in front of moving cars. When
questioned about his identity, he talks incessantly in a rapid fashion
and threatens to "unleash God's wrath on those who do not submit." He reports
that he has not slept in a week, and does not need sleep. He
has spent the last week preparing for a secret government mission that only the
president knows about. When asked if he ever hears God
talking to him, he says, "Of course not, I am God!" Police report that he has had
several prior arrests for reckless driving and lewd acts. On
examination, he is disheveled and malodorous. It is nearly impossible to get any
further history, as he rapidly paces about and mutters, "I
must go, I must go" over and over again.
Question 3 of 7
This patient is started on lithium to stabilize his mood. BIood levels of lithium
need to be closely monitored as therapeutic levels are close to
toxic levels. The ratio of toxic dose to therapeutic dose is known as which of the
following?
/ A. Fractional elimination constant
/ B. Half life
/ C. Loading dose
/ D. Therapeutic index
/ E. Volume of distribution

Explanation - Q: 3.3 Close

The correct answer is D. The therapeutic index of a drug is the ratio of the
toxic dose to therapeutic dose. For a drug with a small therapeutic index,
care must be taken not to overdose the patient. Small, stepwise increases in
dosing can aid in finding the therapeutic dose without experiencing toxicity.
A 30-year-old man is brought to the emergency department by police, who
arrested him because he was in the parking lot of a local malI,
yelling "I am a golden god" as he stepped in front of moving cars. When
questioned about his identity, he talks incessantly in a rapid fashion
and threatens to "unleash God's wrath on those who do not submit." He reports
that he has not slept in a week, and does not need sleep. He
has spent the last week preparing for a secret government mission that only the
president knows about. When asked if he ever hears God
talking to him, he says, "Of course not, I am God!" Police report that he has had
several prior arrests for reckless driving and lewd acts. On
examination, he is disheveled and malodorous. It is nearly impossible to get any
further history, as he rapidly paces about and mutters, "I
must go, I must go" over and over again.
Question 4 of 7
Which of the following is a known adverse effect of lithium?
/ A. Agranulocytosis
/ B. AItered judgement
/ C. Aplastic anemia
/ D. Hypothyroidism
/ E. Male infertility


Explanation - Q: 3.4 Close

The correct answer is D. Patients on lithium chronically may develop
iatrogenic hypothyroidism. (Approx. 5% of patients taking the drug > 18
months.) Lithium exerts this effect by interfering with the synthesis and
release of thyroid hormone. Thus any patient experiencing prolonged
depressive symptoms while taking lithium needs to have his/her thyroid
assessed.
Agranulocytosis (choice A) is commonly reported for patients taking the
atypical antipsychotic drug clozapine or carbamazepine, which is sometimes
employed as a second-line treatment for bipolar disorder.
Choice B is incorrect. Neurological side effects of lithium include tremor,
choreoathetosis, ataxia, motor hyperactivity, dysarthria, and aphasia.
Lithium is not known to cause aplastic anemia (choice C). Aplastic anemia is
a serious potential adverse effect of the mood stabilizer carbamazepine.
Carbamazepine would present an alternative to lithium in this patient, but a
periodic blood count must be performed to assess for aplastic anemia.
Lithium is not known to affect fertility in males (choice E) or females.
However, lithium has strong teratogenic effects (Ebstein's anomaly of the
tricuspid valve) and should NOT be given to any woman who is pregnant or
even thinking about becoming pregnant.
A 30-year-old man is brought to the emergency department by police, who
arrested him because he was in the parking lot of a local malI,
yelling "I am a golden god" as he stepped in front of moving cars. When
questioned about his identity, he talks incessantly in a rapid fashion
and threatens to "unleash God's wrath on those who do not submit." He reports
that he has not slept in a week, and does not need sleep. He
has spent the last week preparing for a secret government mission that only the
president knows about. When asked if he ever hears God
talking to him, he says, "Of course not, I am God!" Police report that he has had
several prior arrests for reckless driving and lewd acts. On
examination, he is disheveled and malodorous. It is nearly impossible to get any
further history, as he rapidly paces about and mutters, "I
must go, I must go" over and over again.
Question 4 of 7
Which of the following is a known adverse effect of lithium?
/ A. Agranulocytosis
/ B. AItered judgement
/ C. Aplastic anemia
/ D. Hypothyroidism
/ E. Male infertility

Explanation - Q: 3.5 Close

The correct answer is B. Benzodiazepines are frequently used to treat
acute agitation and can help manage acute mania until lithium can exert its
effects. Benzodiazepines potentiate the inhibitory effect on the CNS neurons
by binding to GABA receptors and increasing the frequency of the opening of
chloride channels in response to GABA stimulation. The net effect is CNS
depression and reduction in the patient's agitation.
Barbiturates bind the GABA receptor and prolong the duration of opening of
chloride channels in response to GABA (choice A). This acts to suppress the
CNS.
Lorazepam has no effect on dopamine receptors. Many antipsychotic
medications act by inhibiting dopamine receptors (choice C).
Lorazepam does not effect the release of epinephrine from the adrenal
medulla (choice D).
As noted above, lorazepam has no effect on dopamine receptors (choice E).

- - -
A 30-year-old man is brought to the emergency department by police, who
arrested him because he was in the parking lot of a local malI,
yelling "I am a golden god" as he stepped in front of moving cars. When
questioned about his identity, he talks incessantly in a rapid fashion
and threatens to "unleash God's wrath on those who do not submit." He reports
that he has not slept in a week, and does not need sleep. He
has spent the last week preparing for a secret government mission that only the
president knows about. When asked if he ever hears God
talking to him, he says, "Of course not, I am God!" Police report that he has had
several prior arrests for reckless driving and lewd acts. On
examination, he is disheveled and malodorous. It is nearly impossible to get any
further history, as he rapidly paces about and mutters, "I
must go, I must go" over and over again.
Question 6 of 7
The patient later reaches a steady state level of lithium that produces toxic side
effects. If he decides to discontinue his medication, how long
would it take for his lithium blood levels to reach 25% of his original steady state
levels assuming a half life of 22 hours for lithium?
/ A. 11 hours
/ B. 22 hours
/ C. 33 hours
/ D. 44 hours
/ E. 55 hours
/ F. 66 hours
/ G. 77 hours
/ H. 88 hours


Explanation - Q: 3.6 Close

The correct answer is D. Lithium, like most drugs follows first-order kinetics,
which means a constant percent of the drug is eliminated per unit time. His
drug levels will decrease by 50% every half-life. Therefore, they will be 50%
of original levels after one half-life, 25% after two half-lives, 12.5% after three
half-lives, etc. Two half-lives is 22 x 2 = 44 hours.

A 30-year-old man is brought to the emergency department by police, who
arrested him because he was in the parking lot of a local malI,
yelling "I am a golden god" as he stepped in front of moving cars. When
questioned about his identity, he talks incessantly in a rapid fashion
and threatens to "unleash God's wrath on those who do not submit." He reports
that he has not slept in a week, and does not need sleep. He
has spent the last week preparing for a secret government mission that only the
president knows about. When asked if he ever hears God
talking to him, he says, "Of course not, I am God!" Police report that he has had
several prior arrests for reckless driving and lewd acts. On
examination, he is disheveled and malodorous. It is nearly impossible to get any
further history, as he rapidly paces about and mutters, "I
must go, I must go" over and over again.
Question 7 of 7
A screening test is developed for assessing vulnerability to developing this
condition. A sample of 10,000 people between the ages of 18 to
24 is recruited from the general population and given the screening test. Of this
sample, 200 individuals are identified as likely to develop the
condition. The sample is tracked over the next twenty years. Forty of the original
sample, although none of those selected by the test, are lost
to follow-up. A total of 100 people from the sample eventually were diagnosed
with this condition, of which 90 were correctly identified by the
screening test. Based on this study, the positive predictive value of the screening
test is best estimated as which of the following?
/ A. 45%
/ B. 60%
/ C. 75%
/ D. 90%
/ E. 100%

Explanation - Q: 3.7 Close

The correct answer is A. Positive predictive value assesses the proportion
of those identified as having the condition that actually end up developing the
condition. In this case, 90 of the original 200 identified by the test developed
the disorder. (90/200 = 45%). Note the test has a sensitivity (ability to detect
disease) of 90%. (90 of the 100 people who actually developed the condition
were correctly identified by the screening test)

A 25-year-old man presents to the emergency department with multiple
lacerations to both wrists. He says he has a history of "every
diagnosis in the book, Doc." The patient reports several recent stressors,
including being fired from his job after "Iosing it" with a "rotten
customer," financial problems, and a fight with his girlfriend three hours ago.
When asked about suicidal ideation, the patient responds with
"would you want this life?" With permission, the doctor speaks with the man's
psychiatrist. His psychiatrist describes a long-standing pattern
of unstable relationships, career changes, and extreme mood swings with erratic
sleep patterns. The patient has taken multiple overdoses in
the past, usually when his psychiatrist is out of town. After the doctor listens
several minutes to the patient empathetically, the man responds
with multiple compliments, proclaiming the doctor is "the best doctor ever." An
hour later the doctor is summoned by the nurses to again see
the patient. The man is sullen and angry "You don't even care what happens to
me. You never did; none of you do, and you just let me sit here
forever. What kind of lousy doctor are you? You're an embarrassment to your
profession."
Question 1 of 6
Which of the following is the most likely diagnosis?
/ A. Antisocial personality disorder
/ B. Borderline personality disorder
/ C. Histrionic personality disorder
/ D. Narcissistic personality disorder
/ E. Paranoid personality disorder

Explanation - Q: 4.1 Close

The correct answer is B. Borderline personality disorder is characterized by
a pattern of instability in 1) relationships (fights with girlfriend and customers),
2) self image (career changes may be one), 3) affect (mood swings), AND
marked impulsivity (multiple overdoses). Another clue is the frantic efforts to
avoid abandonment (overdoses precede separation from doctor, wrist
slashing after break up with girlfriend). Persons with borderline personality
are prone to rages and complain of chronic feelings of emptiness.
Antisocial personality disorder (choice A) is characterized by a pervasive
pattern of disregard for the rights of others.
Histrionic personality disorder (choice C) is characterized by a pervasive
pattern of excessive emotionality and attention-seeking. These individuals
are usually the "life of the party," and may be associated with "creating a
scene." They are not self-destructive, like persons with borderline personality
disorder.
Narcissistic personality disorder (choice D) is characterized by a pervasive
pattern of grandiosity, need for admiration, and lack of empathy. They exhibit
a stable self-image and are not self-destructive like persons with borderline
personality disorder.
Paranoid personality disorder (choice E) is characterized by a pervasive
pattern of distrust and suspiciousness.
A 25-year-old man presents to the emergency department with multiple
lacerations to both wrists. He says he has a history of "every
diagnosis in the book, Doc." The patient reports several recent stressors,
including being fired from his job after "Iosing it" with a "rotten
customer," financial problems, and a fight with his girlfriend three hours ago.
When asked about suicidal ideation, the patient responds with
"would you want this life?" With permission, the doctor speaks with the man's
psychiatrist. His psychiatrist describes a long-standing pattern
of unstable relationships, career changes, and extreme mood swings with erratic
sleep patterns. The patient has taken multiple overdoses in
the past, usually when his psychiatrist is out of town. After the doctor listens
several minutes to the patient empathetically, the man responds
with multiple compliments, proclaiming the doctor is "the best doctor ever." An
hour later the doctor is summoned by the nurses to again see
the patient. The man is sullen and angry "You don't even care what happens to
me. You never did; none of you do, and you just let me sit here
forever. What kind of lousy doctor are you? You're an embarrassment to your
profession."
uestion 2 of 6
The doctor repeatedly reassures the patient that he deserves the best care and
his doctors are working on it. The patient refuses to accept
the reassurance, and begins yelling loudly. The doctor screams at the patient to
be quiet. Later, he says " I Iet him have it, because what he
REALLY needed was some tough love for once." The doctor is using which of the
following defense mechanisms?
/ A. Denial
/ B. Isolation
/ C. Rationalization
/ D. Sublimation
/ E. Suppression


Explanation - Q: 4.2 Close

The correct answer is C. Rationalization, which is providing a logical reason
(what the patient needs) for a behavior, is usually employed to avoid being
blamed (e.g., for unprofessional behavior).
Denial (choice A) is usually used to avoid awareness of a painful reality, and
is often seen in patients given the news of a fatal illness, or in patients
confronted about substance abuse.
Isolation (choice B) splits the thought from the feeling, and can be seen
when doctors discuss "interesting cases" or "severe pathology" in completely
intellectual terms.
Sublimation (choice D) is replacing an unacceptable wish with a more
acceptable one. In this example, the doctor might have "taken out his
aggression" in the exercise room.
Suppression (choice E) is consciously deciding to remove an idea or feeling
from awareness. "I'm not going to think about that."
A 25-year-old man presents to the emergency department with multiple
lacerations to both wrists. He says he has a history of "every
diagnosis in the book, Doc." The patient reports several recent stressors,
including being fired from his job after "Iosing it" with a "rotten
customer," financial problems, and a fight with his girlfriend three hours ago.
When asked about suicidal ideation, the patient responds with
"would you want this life?" With permission, the doctor speaks with the man's
psychiatrist. His psychiatrist describes a long-standing pattern
of unstable relationships, career changes, and extreme mood swings with erratic
sleep patterns. The patient has taken multiple overdoses in
the past, usually when his psychiatrist is out of town. After the doctor listens
several minutes to the patient empathetically, the man responds
with multiple compliments, proclaiming the doctor is "the best doctor ever." An
hour later the doctor is summoned by the nurses to again see
the patient. The man is sullen and angry "You don't even care what happens to
me. You never did; none of you do, and you just let me sit here
forever. What kind of lousy doctor are you? You're an embarrassment to your
profession."
Question 3 of 6
Which coping mechanism is illustrated by the statement, "You are the best doctor
ever, the rest of the staff is cold and heartless."?
/ A. Denial
/ B. Projection
/ C. Repression
/ D. Splitting
/ E. Suppression

Explanation - Q: 4.3 Close

The correct answer is D. Splitting is a primitive defense that oversimplifies
all relationships into "good" and "bad." The self, others, and situations are
completely polarized into one category or the other. Persons with borderline
personality disorder have difficulty tolerating ambivalence (concurrent
positive and negative feelings).
Denial (choice A) is the outright rejection of information. "I do not have
cancer."
Projection (choice B) is attributing one's traits/feelings to another person.
"You're mad at me." When, in fact, I am mad at you.
Repression (choice C) is unconscious exclusion of thoughts/feelings, "What
anger? I was never angry."
Suppression (choice E) is active exclusion of thoughts/feelings from
consciousness, "I'm not going to think about that right now."
A 25-year-old man presents to the emergency department with multiple
lacerations to both wrists. He says he has a history of "every
diagnosis in the book, Doc." The patient reports several recent stressors,
including being fired from his job after "Iosing it" with a "rotten
customer," financial problems, and a fight with his girlfriend three hours ago.
When asked about suicidal ideation, the patient responds with
"would you want this life?" With permission, the doctor speaks with the man's
psychiatrist. His psychiatrist describes a long-standing pattern
of unstable relationships, career changes, and extreme mood swings with erratic
sleep patterns. The patient has taken multiple overdoses in
the past, usually when his psychiatrist is out of town. After the doctor listens
several minutes to the patient empathetically, the man responds
with multiple compliments, proclaiming the doctor is "the best doctor ever." An
hour later the doctor is summoned by the nurses to again see
the patient. The man is sullen and angry "You don't even care what happens to
me. You never did; none of you do, and you just let me sit here
forever. What kind of lousy doctor are you? You're an embarrassment to your
profession."
Question 4 of 6
This patient is started on trazodone to help with sleep. Common side effects of
trazodone include which of the following?
/ A. Drowsiness, dizziness, fatigue, and fatal liver failure
/ B. Drowsiness, dizziness, hypertension, and nervousness
/ C. Drowsiness, dizziness, hypotension, and priapism
/ D. Drowsiness, dizziness, nervousness, and seizures
/ E. Drowsiness, nervousness, GI distress and sexual dysfunction

Explanation - Q: 4.4 Close

The correct answer is C. This question illustrates the fact that many
antidepressant drugs have similar side effects, but often have a certain
particular side effect worth knowing. The clue to choice C (trazodone) is
priapism, a painful sustained erection. It is a medical emergency!
The clue to choice A (nefazodone) is fatal liver failure; this drug now has a
"black box" warning in the PDR.
The clue to choice B (venlafaxine) is hypertension, specifically diastolic
hypertension.
The clue to choice D (bupropion) is seizures. DO NOT give this medicine to
patients at risk for seizures (e.g., metabolic derangement, head injury).
The clues to choice E (any and all SSRIs) are GI distress and sexual
dysfunction, which are very troublesome side effects. Inquire about sexual
dysfunction in all patients taking SSRIs.


A 25-year-old man presents to the emergency department with multiple
lacerations to both wrists. He says he has a history of "every
diagnosis in the book, Doc." The patient reports several recent stressors,
including being fired from his job after "Iosing it" with a "rotten
customer," financial problems, and a fight with his girlfriend three hours ago.
When asked about suicidal ideation, the patient responds with
"would you want this life?" With permission, the doctor speaks with the man's
psychiatrist. His psychiatrist describes a long-standing pattern
of unstable relationships, career changes, and extreme mood swings with erratic
sleep patterns. The patient has taken multiple overdoses in
the past, usually when his psychiatrist is out of town. After the doctor listens
several minutes to the patient empathetically, the man responds
with multiple compliments, proclaiming the doctor is "the best doctor ever." An
hour later the doctor is summoned by the nurses to again see
the patient. The man is sullen and angry "You don't even care what happens to
me. You never did; none of you do, and you just let me sit here
forever. What kind of lousy doctor are you? You're an embarrassment to your
profession."
Question 5 of 6
The doctor calls the insurance company to authorize admission for "mood
stabilization." When asked, the patient says he does not want to
harm himself at this point. The insurance company denies authorization for an
inpatient admission. The doctor determines the patient's
presentation is too despondent and hopeless to be safe. The most appropriate
intervention is for the doctor to call the insurance company
back and do which of the following?
/ A. Say "The patient is a danger to himself in my opinion, get your supervisor on
the phone now."
/ B. Say "The patient is a danger to himself in my opinion, Iet me explain my
reasoning."
/ C. Say "The patient says he is actively suicidal now.", even though he did not
/ D. Tell the patient to say he is suicidaI, then say "The patient says he is
actively suicidal now."
/ E. "This is unacceptable. Get your supervisor on the phone now, or you will be
exposing yourself to legal liability."

Explanation - Q: 4.5 Close

The correct answer is B. The FIRST intervention is for the doctor to calmly
explain the reasoning involved in the assessment.
Choice A is an unnecessary beginning to the conversation; calmly asking for
the supervisor later (if needed) would be the appropriate response.
Choice C is lying. It's illegal, for one. Contacting the attending, the board, the
administrator, etc., may help the doctor get the patient's needs met through
honest means.
Choice D is illegal and unethical, and the doctor will ultimately suffer for
modeling to the patient "we can make deals."
Choice E is a threat and implies coercion, at the very least it could damage
the doctor's professional reputation.
A 25-year-old man presents to the emergency department with multiple
lacerations to both wrists. He says he has a history of "every
diagnosis in the book, Doc." The patient reports several recent stressors,
including being fired from his job after "Iosing it" with a "rotten
customer," financial problems, and a fight with his girlfriend three hours ago.
When asked about suicidal ideation, the patient responds with
"would you want this life?" With permission, the doctor speaks with the man's
psychiatrist. His psychiatrist describes a long-standing pattern
of unstable relationships, career changes, and extreme mood swings with erratic
sleep patterns. The patient has taken multiple overdoses in
the past, usually when his psychiatrist is out of town. After the doctor listens
several minutes to the patient empathetically, the man responds
with multiple compliments, proclaiming the doctor is "the best doctor ever." An
hour later the doctor is summoned by the nurses to again see
the patient. The man is sullen and angry "You don't even care what happens to
me. You never did; none of you do, and you just let me sit here
forever. What kind of lousy doctor are you? You're an embarrassment to your
profession."
Question 6 of 6
The nurse calls to tell the doctor that the patient has taken an overdose in the
emergency department. When the doctor arrives, the patient
has slurred speech and is sedated. Within five minutes the patient is sleeping, his
respiratory rate is 10/min and he responds minimally to
painful stimuli. The family tells the doctor that the man takes clonazepam at
home for nocturnal myoclonus. The most appropriate
pharmacologic intervention is to administer which of the following?
/ A. Dextrose
/ B. FIumazenil
/ C. Naloxone
/ D. Naltrexone
/ E. Thiamine


Explanation - Q: 4.6 Close

The correct answer is B. The history is consistent with respiratory
depression secondary to benzodiazepine overdose. Flumazenil is a
benzodiazepine receptor antagonist.
Dextrose (choice A) treats hypoglycemia.
Naloxone (choice C), an opioid antagonist, reverses opioid overdose.
Thiamine, naloxone, and dextrose are often given to patients who are "found
down" and present to the emergency department in a coma without any
history.
Naltrexone (choice D) blocks the effects of opiates via opioid antagonism
(people cannot "get high"). It is used to prevent relapse in a previously opioid
dependent individual.
Thiamine (choice E) prevents Wernicke-Korsakoff syndrome.

A 69-year-old white man visits a physician in the outpatient clinic for the first time.
Over the past 5 months, he reports increasing lethargy,
weight loss, and crying "for no reason." The patient had always been an
optimistic person, but today he feels "detached from everything." He
describes his mood "as if there was a pane of glass between me and the rest of
the world and l don't think I'm going to make it. My family and
friends are like cardboard cutouts." His speech is slow and methodicaI,
punctuated by frequent sighs. The patient has also lost interest in
watching movies, which had been his favorite pastime. He also admits that his
drinking has become a problem over the past few weeks, and
he currently consumes a bottle of white wine every evening. He recently lost his
job and is currently filing for divorce, which would end a
seventeen-year marriage. His son has attention-deficit disorder, for which he is
prescribed amphetamine. The patient was hospitalized for
major depression three years ago. The patient's previous doctor had started him
on a medication, but he is unable to recall the name or
anything about it except that he is not supposed to eat cheese, aged meats, or
chocolate while taking it. On physical examination, the patient
appears emaciated. His abdomen is very distended, with hepatomegaly 3 cm
below the right costal margin. He also has a slight tremor. His
gait is normaI. His latest calcium level is 10.3 mg/dL.
Question 1 of 5
Which of the following is the most likely diagnosis at this time?
/ A. Acute stress disorder
/ B. Adjustment disorder
/ C. AIcohol abuse
/ D. Bipolar disorder
/ E. Hypercalcemia
/ F. Unipolar disorder


Explanation - Q: 5.1 Close

The correct answer is F. The patient's symptoms of weight loss, lethargy,
tearfulness, hopelessness ("I'm not going to make it"), and depersonalization
("world through a pane of glass; family and friends are like cardboard
cutouts") for at least one month meet the criteria for unipolar disorder (major
depression). Even more specific are his loss of interest in favorite activities,
as well as the feelings of sadness. The symptoms have been going on for at
least two weeks and represent a change from previous functioning, which
defines depression. Three stressors in his life are job loss, the upcoming
divorce. and his son's attention-deficit disorder. The lifetime rate of major
depression in men in the US is about 10%.
Acute stress disorder (choice A) is the consequence of the experience of a
traumatic event outside the realm of normal human experience. Neither
divorce nor job loss fits this criterion. Symptoms of acute stress disorder
(ASD) must also include reexperiencing the event as dreams, recollections of
flashback, and avoidance of associated stimuli, along with diffuse other
symptoms such as irritability, sleep disruption, and difficulty concentrating.
Adjustment disorder (choice B) is a dysfunctional change in behavior within
three months of an identifiable stressor. The dysfunction can only last 6
months after the stressor has ended. An adjustment disorder cannot be a
grief response AND the diagnosis only applies if no other Axis I diagnosis
can be used. Given the patient's current symptoms and previous treatment
history, the criteria for this diagnosis are not met.
Alcohol abuse (choice C) is probably occurring as a coping mechanism for
the depression, and is unlikely to be a specific cause of the depressive
symptoms. Ascites, hepatomegaly, tremor, macrocytosis (raised mean
corpuscular volume, and increased liver enzymes are suggestive of chronic
alcohol use.
Bipolar disorder (choice D) can have a similar presentation to unipolar
disorder when in the depressive phase. The differential is based on being
able to identify one or more manic episodes in the patient's past. Because
none are presented here, there is no evidence for this diagnosis.
Hypercalcemia (choice E) may present with symptoms of confusion,
polyuria, polydipsia, and abdominal pain. This patient has none of these
symptoms and, in addition, his calcium levels are actually normal.

A 69-year-old white man visits a physician in the outpatient clinic for the first time.
Over the past 5 months, he reports increasing lethargy,
weight loss, and crying "for no reason." The patient had always been an
optimistic person, but today he feels "detached from everything." He
describes his mood "as if there was a pane of glass between me and the rest of
the world and l don't think I'm going to make it. My family and
friends are like cardboard cutouts." His speech is slow and methodicaI,
punctuated by frequent sighs. The patient has also lost interest in
watching movies, which had been his favorite pastime. He also admits that his
drinking has become a problem over the past few weeks, and
he currently consumes a bottle of white wine every evening. He recently lost his
job and is currently filing for divorce, which would end a
seventeen-year marriage. His son has attention-deficit disorder, for which he is
prescribed amphetamine. The patient was hospitalized for
major depression three years ago. The patient's previous doctor had started him
on a medication, but he is unable to recall the name or
anything about it except that he is not supposed to eat cheese, aged meats, or
chocolate while taking it. On physical examination, the patient
appears emaciated. His abdomen is very distended, with hepatomegaly 3 cm
below the right costal margin. He also has a slight tremor. His
gait is normaI. His latest calcium level is 10.3 mg/dL.
Question 2 of 5
Which of the following medications is the patient most likely currently taking?
/ A. Amitriptyline
/ B. Chlorpromazine
/ C. FIuoxetine
/ D. Lorazepam
/ E. Phenelzine

Explanation - Q: 5.2 Close

The correct answer is E. Monoamine oxidase inhibitors (MAOIs) include
phenelzine and tranylcypromine. MAOIs have the potential for severe side
effects when taken with sympathomimetic medications or tyramine-
containing foods (e.g., cheeses, red wines, beers, meats, fruits, beans, liver,
yeast extracts) because they can cause a hypertensive crisis. MAOIs can
also cause orthostatic hypotension, nausea, insomnia, and sexual
dysfunction.
Tricyclic antidepressants include amitriptyline (choice A) and nortriptyline.
They take anywhere from 2 to 6 weeks to take effect. They have
anticholinergic side-effects including dry mouth, blurred vision, constipation,
ileus, urinary retention, and even delirium.
Neuroleptics such as chlorpromazine (choice B) reduce psychotic symptoms
that result from a number of illnesses, including schizophrenia, bipolar
disorder, major depressive disorder with psychotic features, psychosis
secondary to stimulant drugs, and organic psychoses from Alzheimer
disease or Huntington disease.
Selective serotonin reuptake inhibitors (SSRIs) include fluoxetine (choice C).
They have a reduced side-effect profile and are effective in depression
treatment. The risk of overdose is low. Their main pharmacologic effect is to
block the presynaptic serotonin uptake site. Increasing the availability of
serotonin in the synaptic cleft is thought to improve depressive symptoms.
A benzodiazepine such as lorazepam (choice D) is primarily used for anxiety
rather than depression. Adverse reactions include sedation, dizziness,
weakness, unsteady gait, headache, and sleep disturbance.

A 69-year-old white man visits a physician in the outpatient clinic for the first time.
Over the past 5 months, he reports increasing lethargy,
weight loss, and crying "for no reason." The patient had always been an
optimistic person, but today he feels "detached from everything." He
describes his mood "as if there was a pane of glass between me and the rest of
the world and l don't think I'm going to make it. My family and
friends are like cardboard cutouts." His speech is slow and methodicaI,
punctuated by frequent sighs. The patient has also lost interest in
watching movies, which had been his favorite pastime. He also admits that his
drinking has become a problem over the past few weeks, and
he currently consumes a bottle of white wine every evening. He recently lost his
job and is currently filing for divorce, which would end a
seventeen-year marriage. His son has attention-deficit disorder, for which he is
prescribed amphetamine. The patient was hospitalized for
major depression three years ago. The patient's previous doctor had started him
on a medication, but he is unable to recall the name or
anything about it except that he is not supposed to eat cheese, aged meats, or
chocolate while taking it. On physical examination, the patient
appears emaciated. His abdomen is very distended, with hepatomegaly 3 cm
below the right costal margin. He also has a slight tremor. His
gait is normaI. His latest calcium level is 10.3 mg/dL.
Question 3 of 5
To gather more details about the patient's current state of mind, which of the
following questions would be most appropriate for the physician
to ask at this time?
/ A. " Are you taking your antidepressant medications as your previous doctor
instructed?"
/ B. " Have you ever made plans to kill yourself?"
/ C. " Is there any history of depression in your family?"
/ D. " On a depression scale of one to ten, how depressed do think you feel right
now?"
/ E. " On average, how much alcohol do you drink in one day?"

Explanation - Q: 5.3 Close

The correct answer is B. At this stage, it is crucial to ask a direct question
about suicidal ideation, particularly because this is the first time the physician
is meeting the patient. In addition, the patient is a white male over the age of
50 who has already been hospitalized once for major unipolar depression,
which places him at a greater risk for suicide. The suicide rate among people
who have been hospitalized at least once for unipolar depression has been
estimated to be 15%. A physician is the last person patients have seen
before taking their own life in 10% of suicides. Asking this question as part of
the mental status examination also gives information on how severe the
depression is. If the patient has a plan about how they will take their own life,
then this suggests that they are more likely to follow through with their plan.
Compliance with medication (choice A) is an important cause of depression
relapse. It has been estimated that fewer than 10% of people suffering from
unipolar major depression are likely to be receiving a full therapeutic dose of
medication. More than 20% of patients fail to fill the first prescription they
receive for major depression, and the majority of patients who do begin
treatment discontinue the medication within 14 weeks, usually due to side
effects.
Depression in the family (choice C) is a risk factor for recurrent depression.
Pursuing this line of questioning is useful for the long-term management of
depression but does not address any major life-threatening issues. Other risk
factors for recurrent depression include a history of multiple episodes
(patients with 3 or more prior episodes have at least a 90% recurrence rate),
depression associated with dysthymia, onset after age 60, long duration of
individual episodes, poor symptom control during therapy, comorbid anxiety
disorder, or substance abuse.
Objective analysis of depression such as a depression score of 1 to 10
(choice D) is also useful in the long term care of patients with depression. In
doing so, the physician is able to objectively document the effectiveness of
the patient's antidepressant therapy. Another useful method is counting the
number of improvements in the quality of the patient's life, e.g., more family
interaction, being able to go to church, returning back to work. For this to be
useful, it is important to get an idea of where the patient is at in the initial
visit. It does not, however, take precedence over asking about suicidal
ideation.
Alcoholic intake (choice E) is an important question to ask since the patient
appears to have signs of alcoholism (hepatomegaly, increasing abdominal
girth as an indication of ascites, weight loss, and tremor). However, it does
not necessarily have to be addressed on the initial visit. Suicidal risk is a
more ominous life-threatening event in this instance. Accurate answers can
be derived when asking the patient to describe their drinking habits during
the course of the day rather than asking about alcohol intake as a single
numerical quantity.

A 69-year-old white man visits a physician in the outpatient clinic for the first time.
Over the past 5 months, he reports increasing lethargy,
weight loss, and crying "for no reason." The patient had always been an
optimistic person, but today he feels "detached from everything." He
describes his mood "as if there was a pane of glass between me and the rest of
the world and l don't think I'm going to make it. My family and
friends are like cardboard cutouts." His speech is slow and methodicaI,
punctuated by frequent sighs. The patient has also lost interest in
watching movies, which had been his favorite pastime. He also admits that his
drinking has become a problem over the past few weeks, and
he currently consumes a bottle of white wine every evening. He recently lost his
job and is currently filing for divorce, which would end a
seventeen-year marriage. His son has attention-deficit disorder, for which he is
prescribed amphetamine. The patient was hospitalized for
major depression three years ago. The patient's previous doctor had started him
on a medication, but he is unable to recall the name or
anything about it except that he is not supposed to eat cheese, aged meats, or
chocolate while taking it. On physical examination, the patient
appears emaciated. His abdomen is very distended, with hepatomegaly 3 cm
below the right costal margin. He also has a slight tremor. His
gait is normaI. His latest calcium level is 10.3 mg/dL.
Question 4 of 5
The physician decides to discontinue his current medication and prescribes
sertraline instead. Sertraline directly affects which of the
following neurotransmitters?
/ A. Acetylcholine
/ B. Dopamine
/ C. Epinephrine
/ D. Norepinephrine
/ E. Serotonin


Explanation - Q: 5.4 Close

The correct answer is E. Sertraline specifically blocks the reuptake of
serotonin into the presynaptic axon terminal. This enhances serotonin
activation and brings about a cascade of events ultimately resulting in a
reduced sensitivity of presynaptic autoreceptors for serotonin and reduced
serotonin synthesis. The most common adverse reactions to the SSRIs are
gastrointestinal (especially nausea), neuropsychiatric (particularly headache
and tremor), and changes in sexual functioning. SSRIs also treat anxious
depression, dysthymia, and atypical depression.
Acetylcholine (choice A) has been cited as the main neurotransmitter
involved in Alzheimer dementia. Drugs that utilize this concept are tacrine
and donepezil, which work in the brain as cholinesterase inhibitors at the
neuronal synapse. By inhibiting the cholinesterase enzyme, they increase
the level of acetylcholine and aid in maintaining mental function, although
they do not stop the degeneration of cholinergic cells. Sertraline has no
effect on the levels of acetylcholine.
Dopamine (choice B) is found in both small and large neuronal pathways in
the CNS. The latter include the nigrostriatal pathway involved, in the etiology
of Parkinson disease and mesolimbic/mesocortical pathways, implicated in
psychosis. L-dopa, which is converted to dopamine, is the primary
replacement therapy in Parkinson disease. Dopamine antagonists are used
in the treatment of psychosis. Dopaminergic input to the chemoreceptor
trigger zone is the basis of the use of the agonist apomorphine as an emetic
to treat poisoning, and the use of antagonists as antiemetics. Hypothalamic
dopaminergic neurons inhibit prolactin secretion and lead to the use of
agonists in inhibiting lactation. There are a number of drugs that interact
presynaptically with dopamine terminals including reserpine, amphetamine,
MAO inhibitors, and cocaine.
Epinephrine (choice C) is a neurotransmitter, and a hormone. It stimulates
alpha1-, alpha2-, beta1-, and beta2-adrenergic receptors in a dose-related
fashion. It is the initial drug of choice for treating bronchoconstriction and
hypotension resulting from anaphylaxis as well as all forms of cardiac arrest.
It is useful in managing reactive airway disease, but beta-adrenergic agents
are often used initially because of their convenience and oral inhalation
route. Epinephrine is not a neurotransmitter specifically affected by any
antidepressants currently available.
Norepinephrine (choice D), like serotonin, is a neurotransmitter that may
induce depression if depleted. There are several antidepressants that
increase norepinephrine levels:


A 69-year-old white man visits a physician in the outpatient clinic for the first time.
Over the past 5 months, he reports increasing lethargy,
weight loss, and crying "for no reason." The patient had always been an
optimistic person, but today he feels "detached from everything." He
describes his mood "as if there was a pane of glass between me and the rest of
the world and l don't think I'm going to make it. My family and
friends are like cardboard cutouts." His speech is slow and methodicaI,
punctuated by frequent sighs. The patient has also lost interest in
watching movies, which had been his favorite pastime. He also admits that his
drinking has become a problem over the past few weeks, and
he currently consumes a bottle of white wine every evening. He recently lost his
job and is currently filing for divorce, which would end a
seventeen-year marriage. His son has attention-deficit disorder, for which he is
prescribed amphetamine. The patient was hospitalized for
major depression three years ago. The patient's previous doctor had started him
on a medication, but he is unable to recall the name or
anything about it except that he is not supposed to eat cheese, aged meats, or
chocolate while taking it. On physical examination, the patient
appears emaciated. His abdomen is very distended, with hepatomegaly 3 cm
below the right costal margin. He also has a slight tremor. His
gait is normaI. His latest calcium level is 10.3 mg/dL.
Question 5 of 5
Should this particular patient commit suicide, the likeliest method of suicide is
which of the following?
/ A. A drug overdose
/ B. By running his car in a closed garage for an extended period of time
/ C. Cutting his wrists
/ D. Hanging himself
/ E. With a firearm


Explanation - Q: 5.5 Close

The correct answer is E. Firearms were the commonest method of suicide
used by persons aged 65 years or older representing 71% of suicides. In
1998, firearms were the most common method of suicide by both males and
females, accounting for 78% of male and 35% of female suicides in this age
group.
Overdose with liquids, pills or gas (choice A), 17%, and suffocation (choice
D),11%, represents the other two most common methods of suicide used by
persons aged 65 years or older. Elderly patients make fewer attempts per
completed suicide, have a higher-male-to-female ratio than other groups,
have often visited a health-care provider before their suicide, and have more
physical illnesses. It is estimated that 20% of elderly (over 65 years) persons
who commit suicide visited a physician within 24 hours of their act, 41%
visited within a week of their suicide and 75% have been seen by a physician
within one month of their suicide. The suicide rate of white males rises
dramatically after age 65 and accounts for the majority of all suicides in the
elderly.
In 2000, the death rate by car exhaust fumes (choice B) was less than 1 per
100 000 per year.
Between the age group of 65 to 70 years, the mortality rate from suicide by a
cut or stab (choice C) was 0.32 deaths per 100 000 per year.


A 62-year-old man is seen in an emergency department because of severe pain
in his lower back, Ieft buttock and the posterolateral aspect
of his left leg. The pain began acutely after he lifted his 8-year-old granddaughter
into the back of a truck, and he describes it as "shooting" in
nature. When examined by the physician, he has curled into a fetal position,
which he says relieves the pain. Attempts to extend the man's
spine cause a marked exacerbation of the pain.

Question 1 of 5

On physical examination, the physician notes that spasm is occurring in the large
muscles on the posterior aspect of the thigh. Which of the
following muscles is most likely involved?
/ A. Adductor longus
/ B. Biceps femoris
/ C. Gastrocnemius
/ D. Sartorius
/ E. Soleus

Explanation - Q: 1.1 Close

The correct answer is B. The biceps femoris is the only muscle listed that is
in the posterior compartment of the thigh.
The adductor longus (choice A) and the sartorius (choice D) are in the
anterior compartment of the thigh.
The gastrocnemius (choice C) and the soleus (choice E) are in the posterior
compartment of the leg below the knee.


A 62-year-old man is seen in an emergency department because of severe pain
in his lower back, Ieft buttock and the posterolateral aspect
of his left leg. The pain began acutely after he lifted his 8-year-old granddaughter
into the back of a truck, and he describes it as "shooting" in
nature. When examined by the physician, he has curled into a fetal position,
which he says relieves the pain. Attempts to extend the man's
spine cause a marked exacerbation of the pain.
Question 2 of 5
More detailed examination demonstrates foot drop with weakness of the anterior
tibiaI, posterior tibiaI, and peroneal muscles. Sensory loss
is demonstrated over the anterior shin and dorsal foot. These findings suggest a
radiculopathy at which of the following cord levels?
/ A. C-7
/ B. L-5
/ C. S-3
/ D. T-9
/ E. T-12

Explanation - Q: 1.2 Close

The correct answer is B. The findings are typical for involvement at the L-5
level. Most ruptured disks involve either the lumbar area (L-5 and S-1 most
common) or the cervical area (C-6 and C-7 most commonly). Compression
by a disk of the cervical cord can produce a spastic paresis of the lower
limbs; compression of the cauda equina can cause urine retention or
incontinence.
C-7 (choice A) supplies the back of the shoulders, down the back of the
arm, past the elbow, to the back of the second and third fingers.
S-3 (choice C) supplies a small area on the buttocks.
T-9 (choice D) supplies a band on the abdomen and back that passes above
the umbilicus.
T-12 (choice E) supplies a similar band that passes below the umbilicus.


A 62-year-old man is seen in an emergency department because of severe pain
in his lower back, Ieft buttock and the posterolateral aspect
of his left leg. The pain began acutely after he lifted his 8-year-old granddaughter
into the back of a truck, and he describes it as "shooting" in
nature. When examined by the physician, he has curled into a fetal position,
which he says relieves the pain. Attempts to extend the man's
spine cause a marked exacerbation of the pain.

Question 3 of 5

PIain spinal x-ray films show disk space narrowing in the involved area of the
spine, and herniation with protrusion of the nucleus pulposus of
one disk is demonstrated by CT scan. The normal location of the disk is between
which of the following?
/ A. The body of the superior vertebrae and the body of the inferior vertebrae
/ B. The inferior articular process of the superior vertebrae and the superior
articular process of the inferior vertebrae
/ C. The pedicle of the superior vertebrae and the pedicle of the inferior
vertebrae
/ D. The superior articular process of the superior vertebrae and the inferior
articular process of the inferior vertebrae
/ E. The vertebral canal of the superior vertebrae and the vertebral canal of the
inferior vertebrae

Explanation - Q: 1.3 Close

The correct answer is A. The disk lies between the bodies of the vertebrae,
cushioning the bones to allow for smooth motion. When too much pressure is
placed on the spine, a disk can bulge or rupture (herniated disk). The pain of
disk herniation may begin abruptly after an obvious inciting event (as in this
case) or may be insidious in onset. In many patients, the pain is worsened by
Valsalva maneuver, coughing, laughing, or straining at stool. Patients without
symptoms of spinal cord compression, cauda equina compression, or severe
neurologic deficits related to peripheral nerve root involvement are often
treated conservatively, since up to 95% recover without surgery. Surgical
repair is used in symptomatic patients, or those with more severe or
persistent cases.
The superior and inferior articular processes (choices B and D) form the
surfaces for the joints between the posterior aspects of the vertebrae.
The vertebral canal (choice E) contains the spinal cord, and the pedicle
(choice C) contributes to its posterior margin.

A 62-year-old man is seen in an emergency department because of severe pain
in his lower back, Ieft buttock and the posterolateral aspect
of his left leg. The pain began acutely after he lifted his 8-year-old granddaughter
into the back of a truck, and he describes it as "shooting" in
nature. When examined by the physician, he has curled into a fetal position,
which he says relieves the pain. Attempts to extend the man's
spine cause a marked exacerbation of the pain.

Question 4 of 5

The nucleus pulposus is composed of which of the following?
/ A. Cancellous bone
/ B. Compact bone
/ C. EIastic fibrocartilage
/ D. Hyaline cartilage
/ E. White fibrocartilage

Explanation - Q: 1.4 Close

The correct answer is E. The disk contains a soft, centrally located nucleus
pulposus, surrounded by the protective shell of the annulus fibrosis. The
nucleus pulposus is composed of white fibrocartilage.
The disk contains no bone (choices A and B).
Elastic fibrocartilage (choice C) is found in the larynx, epiglottis, and ear.
The disk has a thin outer layer of hyaline cartilage (choice D), but this does
not involve the nucleus pulposus.
A 62-year-old man is seen in an emergency department because of severe pain
in his lower back, Ieft buttock and the posterolateral aspect
of his left leg. The pain began acutely after he lifted his 8-year-old granddaughter
into the back of a truck, and he describes it as "shooting" in
nature. When examined by the physician, he has curled into a fetal position,
which he says relieves the pain. Attempts to extend the man's
spine cause a marked exacerbation of the pain.

Question 5 of 5

A principal component of the intervertebral disk is collagen. Cross-Iinking of
collagen to form a more stable structure requires the use of a
vitamin C-dependent enzyme that can hydroxylate which of the following amino
acids in procollagen?
/ A. Asparagine
/ B. Cysteine
/ C. Proline
/ D. Serine
/ E. Valine

Explanation - Q: 1.5 Close

The correct answer is C. The amino acids proline and lysine in collagen are
hydroxylated by two vitamin C-dependent enzymes, prolyl- and lysyl
hydroxylase, respectively. Hydroxyproline plays an essential role in stable
triple helix formation. Cysteine residues form disulfide bonds also important
for a stable collagen structure, but hydroxylation plays no role in this process.
The other amino acids listed are not hydroxylated in the process of collagen
cross-linking.

A 40-year-old man consults a physician because of chronic lumbar discomfort.
On physical examination, Iarge tender bilateral masses are
felt deep in his lower back, below his rib cage, and to either side of the vertebral
column. Urinalysis demonstrates mild proteinuria and
microscopic hematuria without red cell casts. CT scan demonstrates massive
bilateral enlargement of the kidneys with near total
replacement of the renal cortex by roughly spherical cysts of varying sizes.
Question 1 of 5
Which of the following is the most likely diagnosis?
/ A. Autosomal dominant polycystic kidney disease
/ B. Autosomal recessive polycystic kidney disease
/ C. Cystic renal dysplasia
/ D. Medullary sponge kidney
/ E. Nephronophthisis-uremic medullary cystic disease complex

Explanation - Q: 2.1 Close

The correct answer is A. This patient most likely has the autosomal
dominant (adult) form of polycystic kidney disease. This relatively common
condition causes massive bilateral enlargement of the kidneys by mid-
adulthood, and affects approximately 1 in 1000 individuals. It may present as
an incidental finding on ultrasound (even in children) or with symptoms
related to the effects of the cysts (lumbar discomfort, hematuria, urinary tract
infection) or uremia. The cysts arise from dilatation of tubules throughout the
nephron, are characteristically spherical, unless compressed by adjacent
cysts, and are separated by residual functioning renal tissue.
In contrast to the autosomal dominant form of polycystic kidney disease, the
autosomal recessive form (choice B) tends to present in childhood, causes
less massive renal enlargement, and tends to have a combination of small
spherical cysts and elongated dilated channels that occur radially at right
angles to the cortical surface.
Cystic renal dysplasia (choice C) is an often unilateral developmental
malformation of the kidney that is characterized by a fairly small number of
large cysts with intervening tissue that may contain abnormal tissues such as
cartilage and undifferentiated mesenchyme.
Medullary sponge kidney (choice D) is characterized by multiple cystic
dilations confined to the renal medulla, rather than cortex.
The nephronophthisis-uremic medullary cystic disease complex (choice E) is
a group of progressive renal disorders that are usually identified in childhood
and present with medullary cysts with tubular atrophy and fibrosis in the
cortex.
A 40-year-old man consults a physician because of chronic lumbar discomfort.
On physical examination, Iarge tender bilateral masses are
felt deep in his lower back, below his rib cage, and to either side of the vertebral
column. Urinalysis demonstrates mild proteinuria and
microscopic hematuria without red cell casts. CT scan demonstrates massive
bilateral enlargement of the kidneys with near total
replacement of the renal cortex by roughly spherical cysts of varying sizes.
Question 2 of 5
Which of the following sites would be most likely to additionally contain cysts in
this patient's condition?
/ A. Adrenal gland
/ B. Cerebral cortex
/ C. Liver
/ D. Ovary
/ E. Thyroid

Explanation - Q: 2.2 Close

The correct answer is C. In addition to the massive cystic replacement of
the kidneys, patients with autosomal dominant polycystic disease also have
a tendency to form smaller numbers of cysts at other sites. Approximately
40% of these patients have one or more (usually asymptomatic) hepatic
cysts; less frequently, cysts are seen in the spleen, pancreas, and lungs. The
sites listed in the other choices do not have a notable predilection for
developing cysts.
A 40-year-old man consults a physician because of chronic lumbar discomfort.
On physical examination, Iarge tender bilateral masses are
felt deep in his lower back, below his rib cage, and to either side of the vertebral
column. Urinalysis demonstrates mild proteinuria and
microscopic hematuria without red cell casts. CT scan demonstrates massive
bilateral enlargement of the kidneys with near total
replacement of the renal cortex by roughly spherical cysts of varying sizes.
Question 3 of 5
There is a specific link between this patient's disease and which of the following
conditions?
/ A. Berry aneurysm
/ B. Lymphedema
/ C. Polyarteritis nodosa
/ D. Temporal arteritis
/ E. Thromboangiitis obliterans

Explanation - Q: 2.3 Close

The correct answer is A. Up to 1/3 of patients with adult polycystic kidney
disease may have berry aneurysms (roughly spherical dilatations) of the
arteries of the circle of Willis at the base of the brain. These may bleed
intracranially, with significant risk of death. It is thought that the same defect
(possibly an abnormally weak basement membrane) that predisposes for the
organ cysts also predisposes for berry aneurysm formation. Other
cardiovascular anomalies that these patients may have include aortic root
dilation and mitral valve prolapse.
Lymphedema (choice B) is an accumulation of excess lymphatic fluid, which
may occur in a congenital form or secondary to malignant or other disease
that occludes lymphatic channels.
Polyarteritis nodosa (choice C) is probably an autoimmune disease, with
focal inflammation of medium sized muscular arteries. It can be seen
idiopathically, or following infection or medication use.
Temporal arteritis (choice D) is a chronic inflammatory disease of large
blood vessels, particularly those of the head. Temporal arteritis is often
associated with polymyalgia rheumatica.
Thromboangiitis obliterans (choice E) is a vascular disease of small and
medium-sized arteries and veins that is most commonly associated with
smoking.

A 40-year-old man consults a physician because of chronic lumbar discomfort.
On physical examination, Iarge tender bilateral masses are
felt deep in his lower back, below his rib cage, and to either side of the vertebral
column. Urinalysis demonstrates mild proteinuria and
microscopic hematuria without red cell casts. CT scan demonstrates massive
bilateral enlargement of the kidneys with near total
replacement of the renal cortex by roughly spherical cysts of varying sizes.

Question 4 of 5

5 years after the diagnosis is established, the patient develops persistent
hypertension secondary to his renal disease. The hypertension is
treated with lisinopriI. This agent acts at which of the following steps?
/ A. CIeavage of angiotensin l
/ B. Increase of sodium and fluid retention
/ C. Induction of aldosterone secretion
/ D. Secretion of angiotensin substrate
/ E. Secretion of rennin

Explanation - Q: 2.4 Close

The correct answer is A. Hypertension is very common among patients with
autosomal dominant polycystic disease, and apparently develops, in large
part, because the cysts impair blood flow to enough renal glomeruli to trigger
the angiotensin-renin system. ACE inhibitors (e.g., captopril, lisinopril,
enalapril, benazepril, fosinopril, moexipril, perindopril, quinapril, ramipril, and
trandolapril) are often effective anti-hypertensive agents in these patients
because they interrupt the stimulation of this pathway. More specifically,
ACE-inhibitors block the conversion of angiotensin I to angiotensin II, a step
that is normally performed by angiotensin converting enzyme (ACE) as blood
circulates through the lung. The renin-angiotensin system is activated when
reduced blood pressure or distal tubular sodium triggers the secretion of
renin (choice E) by the juxtaglomerular cells of the kidney. The circulating
renin cleaves angiotensin substrate (secreted by the liver, choice D) to
angiotensin I. Following cleavage of angiotensin I to angiotensin II by ACE,
angiotensin II triggers both vasoconstriction and aldosterone secretion
(choice C), which then causes increased sodium and fluid retention (choice
B).
A 40-year-old man consults a physician because of chronic lumbar discomfort.
On physical examination, Iarge tender bilateral masses are
felt deep in his lower back, below his rib cage, and to either side of the vertebral
column. Urinalysis demonstrates mild proteinuria and
microscopic hematuria without red cell casts. CT scan demonstrates massive
bilateral enlargement of the kidneys with near total
replacement of the renal cortex by roughly spherical cysts of varying sizes.
Question 5 of 5
Fifteen years after diagnosis, the patient develops chronic renal failure. This
would be indicated most directly by rising serum levels of which
of the following?
/ A. Amylase
/ B. Bilirubin
/ C. Creatinine
/ D. Potassium
/ E. Sodium

Explanation - Q: 2.5 Close

The correct answer is C. Chronic renal failure is common among older
patients with autosomal dominant polycystic kidney disease, and if it
becomes sufficiently severe, may be treated with hemodialysis or kidney
transplant. Renal failure is usually monitored clinically with serum levels of
creatinine and urea.
Amylase (choice A) is a marker for pancreatic damage.
Bilirubin (choice B) is a marker for biliary tree damage and hemolysis.
Serum potassium (choice D) may be either normal or moderately elevated in
renal failure, but can be altered by many conditions and is a less useful
monitoring tool than creatinine.
Serum sodium (choice E) can be normal or reduced in renal failure, but can
also be altered by many other conditions, and so is a less useful monitoring
tool than creatinine.
A 20-year-old man presents to his family physician with a 1-year history of low
back pain and stiffness that is worse in the morning. He reports
that the pain is improved with activity and a warm shower. He now pinpoints most
of his symptoms to his sacroiliac joints. Physical
examination reveals decreased chest expansion on inspiration. X-ray films reveal
bilateral symmetric sacroiliac erosion and bony fusion
(bamboo spine) of part of the lumbar spine.
Question 1 of 5
Which of the following is the most likely diagnosis?
/ A. Ankylosing spondylitis
/ B. Enteropathic arthritis
/ C. Juvenile arthritis
/ D. Psoriatic arthritis
/ E. Reiter syndrome

Explanation - Q: 3.1 Close

The correct answer is A. This patient most likely has ankylosing spondylitis,
an inflammatory arthropathy of the vertebral column. This disorder is most
common in young men, and its peak incidence is at 20 years of age. Greater
than 90% of patients are positive for HLA-B27. Patients usually complain of
pain that is helped with activity and/or a warm shower. There is classically
bilateral sacroiliac involvement.
Enteropathic arthritis (choice B) is seen in patients with inflammatory bowel
diseases such as ulcerative colitis and Crohn disease.
Juvenile arthritis (choice C) is a general term for chronic inflammatory
arthritis in children.
Psoriatic arthritis (choice D) is seen in 7% of patients with psoriasis. HLA-
B27 is positive in many patients with psoriatic arthritis affecting the spine.
Reiter syndrome (choice E) is a triad of seronegative polyarthritis,
conjunctivitis and nonspecific urethritis. It is most commonly seen in men,
and is associated with venereal disease exposure or bacillary dysentery.
Approximately 90% of patients are HLA-B27 positive.
A 20-year-old man presents to his family physician with a 1-year history of low
back pain and stiffness that is worse in the morning. He reports
that the pain is improved with activity and a warm shower. He now pinpoints most
of his symptoms to his sacroiliac joints. Physical
examination reveals decreased chest expansion on inspiration. X-ray films reveal
bilateral symmetric sacroiliac erosion and bony fusion
(bamboo spine) of part of the lumbar spine.
Question 2 of 5
Which of the following genetic markers is most commonly associated with this
condition?
/ A. HLA-B5
/ B. HLA-B8
/ C. HLA-B18
/ D. HLA-B27
/ E. HLA-DR5
Explanation - Q: 3.2 Close

The correct answer is D. Over 90% of patients with ankylosing spondylitis
are HLA-B27 positive. HLA-B27 is also found in patients with Reiter
syndrome and psoriatic arthritis.
HLA-B5 (choice A) is associated with congenital adrenal hyperplasia.
HLA-B8 (choice B) is associated with autoimmune adrenalitis, celiac
disease, Graves disease, and Hashimoto thyroiditis.
HLA-B18 (choice C) is associated with Hodgkin disease.
HLA-DR5 (choice E) is associated with Hashimoto thyroiditis and juvenile
arthritis.

A 20-year-old man presents to his family physician with a 1-year history of low
back pain and stiffness that is worse in the morning. He reports
that the pain is improved with activity and a warm shower. He now pinpoints most
of his symptoms to his sacroiliac joints. Physical
examination reveals decreased chest expansion on inspiration. X-ray films reveal
bilateral symmetric sacroiliac erosion and bony fusion
(bamboo spine) of part of the lumbar spine.
Question 3 of 5
What cardiac condition is most commonly associated with this patient's
diagnosis?
/ A. Aortic regurgitation
/ B. Aortic stenosis
/ C. Ischemia
/ D. Mitral regurgitation
/ E. Mitral stenosis

Explanation - Q: 3.3 Close

The correct answer is A. Approximately 10% of patients with ankylosing
spondylitis develop a characteristic aortic valve lesion. The aortic ring
becomes dilated and the valve leaflets become scarred. Inflammatory lesions
develop on the valve ring, resulting in aortic regurgitation.
The other diagnoses are possible, but are not the most common form of
cardiac involvement in these patients.
A 20-year-old man presents to his family physician with a 1-year history of low
back pain and stiffness that is worse in the morning. He reports
that the pain is improved with activity and a warm shower. He now pinpoints most
of his symptoms to his sacroiliac joints. Physical
examination reveals decreased chest expansion on inspiration. X-ray films reveal
bilateral symmetric sacroiliac erosion and bony fusion
(bamboo spine) of part of the lumbar spine.
Question 4 of 5
The patient later falls, and then complains of low back pain. X-ray films reveal a
fracture of the lumbar spine. On physical examination, he has
Iow back tenderness and is unable to extend his great toe on the right. Which of
the following nerve roots was most likely injured?
/ A. L2
/ B. L3
/ C. L4
/ D. L5
/ E. S1
Explanation - Q: 3.4 Close

The correct answer is D. This patient is unable to extend his great toe,
which indicates impairment of the action of the long toe extensors,
specifically the extensor hallucis longus (EHL). The EHL is innervated by the
L5 nerve root.
The L2 nerve root (choice A) innervates the hip flexors, for example the
iliopsoas.
The L3 nerve root (choice B) innervates the knee extensors, such as the
quadriceps.
The L4 nerve root (choice C) innervates the ankle dorsiflexors, such as the
tibialis anterior.
The S1 nerve root (choice E) innervates the ankle plantar flexors, such as
the gastrocnemius and soleus.
A 20-year-old man presents to his family physician with a 1-year history of low
back pain and stiffness that is worse in the morning. He reports
that the pain is improved with activity and a warm shower. He now pinpoints most
of his symptoms to his sacroiliac joints. Physical
examination reveals decreased chest expansion on inspiration. X-ray films reveal
bilateral symmetric sacroiliac erosion and bony fusion
(bamboo spine) of part of the lumbar spine.
Question 5 of 5
A 24-year-old man is seen later that day in the same clinic with complaints of
dysuria and pain in his knee and ankle joints. On examination,
he has a mild conjunctivitis bilaterally. Which of the following genetic markers is
most commonly associated with this patient's disease?
/ A. HLA-B5
/ B. HLA-B8
/ C. HLA-B18
/ D. HLA-B27
/ E. HLA-DR5

Explanation - Q: 3.5 Close

The correct answer is D. The patient most likely has Reiter syndrome,
another spondyloarthropathy that affects young men. Reiter syndrome is
characterized classically by the triad of seronegative polyarthritis,
conjunctivitis and urethritis. HLA-B27 is found in 60-85% of patients with
Reiter syndrome and is also found in patients with psoriatic arthritis.
HLA-B5 (choice A) is associated with congenital adrenal hyperplasia.
HLA-B18 (choice C) is associated with Hodgkin disease.
HLA-B8 (choice B) is associated with autoimmune adrenalitis, celiac
disease, Graves disease and Hashimoto thyroiditis.
HLA-DR5 (choice E) is associated with Hashimoto thyroiditis and juvenile
arthritis.















A 67-year-old woman is brought to the hospital complaining of severe back pain
after falling out of a chair. A Iateral film of the spine is
obtained; the T6-T8 region is shown above. Laboratory studies demonstrate a
normocytic, normochromic anemia with a normal white blood
cell count, but a markedly elevated erythrocyte sedimentation rate (ESR). A
peripheral blood smear demonstrates rouleaux formation.
Question 1 of 5
Rouleaux formation suggests which of the following?
/ A. High calcium content in serum
/ B. High globulin content in serum
/ C. High sodium content in serum
/ D. Low potassium content in serum
/ E. Low urea content in serum

Explanation - Q: 4.1 Close

The correct answer is B. Rouleaux formation refers to the stacking of
erythrocytes (distinguished from agglutination) seen when there is a relative
or absolute hypergammaglobulinemia. It can be seen when there is excess
globulin production, as in multiple myeloma and Waldenstrm's
macroglobulinemia, and also in liver disease, when there is a loss of enough
albumin to leave a relative hypergammaglobulinemia. The increased
rouleaux formation in this patient also led to a markedly elevated erythrocyte
sedimentation rate (ESR), since the rouleaux settle out of blood much faster
than do individual erythrocytes. Rouleaux formation is unrelated to the
electrolyte or urea content of the serum.
Hypercalcemia (choice A) is a frequent finding in multiple myeloma due to
the numerous osteolytic bone lesions, and the resulting release of calcium
into the blood, but it does not contribute to rouleaux formation.
Sodium (choice C) and potassium (choice D) concentrations in the serum
do not affect the ESR or rouleaux formation.
Urea levels are often elevated, rather than decreased (choice E), in this
condition, secondary to glomerulopathy or tubulointerstitial nephritis.


Question 2 of 5

Serum electrophoresis demonstrates a spike of protein in the gamma zone.
When the electrophoresed proteins are then reacted with
specific antisera, the product is characterized. Which of the following antisera are
most likely to positively react with the excess proteins
being produced?
/ A. Anti-alpha and anti-gamma isotypes
/ B. Anti-epsilon and anti-kappa isotypes
/ C. Anti-gamma and anti-Iambda isotypes
/ D. Anti-kappa and anti-Iambda isotypes
/ E. Anti-mu and anti-delta isotypes

Explanation - Q: 4.2 Close

The correct answer is C. The most common antibody produced in multiple
myeloma is IgG, and the most common light chain produced is lambda light
chain (normal immunoglobulin light chains may be of the kappa or lambda
types). Therefore, this product would react with antisera prepared against the
gamma isotype (heavy chain constant domain determinants) and the lambda
isotype (light chain constant domain determinants).
In multiple myeloma, the antibodies are monoclonal. Thus, it is possible for
anti-alpha OR anti-gamma isotype antisera to react with the product, but not
both (choice A).
Although it is possible for a monoclonal plasma cell myeloma to produce IgE
heavy chains and kappa light chains simultaneously (choice B), it is not the
most likely type of antibody to be produced.
A plasma cell myeloma cannot produce two light chains simultaneously, e.g.,
kappa and lambda isotypes (choice D).
Mu and delta isotypes (choice E) are both heavy chain isotypes, and only
one would be produced by a monoclonal plasma cell myeloma.




Question 3 of 5



Biopsy of one of the lytic bone lesions reveals nodules with the microscopic
appearance shown above. Which of the following cellular
markers would most likely characterize these cells?
/
A. CD5 and surface immunoglobulin M
/ B. CD10 and surface immunoglobulin M
/ C. CD38 and cytoplasmic immunoglobulin
/ D. TdT and CD1
/ E. TdT and CD19

Explanation - Q: 4.3 Close

The correct answer is C. The photomicrograph shows a typical area of
myeloma. The typical appearance of a myeloma is that of sheet-like masses
of cells resembling normal plasma cells, intermixed with other cytologic
variants such as plasmablasts and multinucleated cells. The markers that
identify plasma cells in tissues are surface CD38 molecules and cytoplasmic
immunoglobulin.
CD5 and surface immunoglobulin M (choice A) are the most likely markers
to be found in the malignant cells of chronic lymphocytic leukemia.
CD10 and surface immunoglobulin M (choice B) describes the most likely
markers found in the malignant cells of Burkitt lymphoma.
TdT and CD1 (choice D) are the most likely markers to be found in the
neoplastic cells of acute lymphoblastic leukemia of the T cell type.
Remember that TdT is a marker only found on the most primitive of
lymphocyte precursors. It would never be found in a fully differentiated cell
tumor such as multiple myeloma.
TdT and CD19 (choice E) are the most likely markers to be found in the
neoplastic cells of acute lymphoblastic leukemia of the B cell type.


Question 4 of 5
The presence of Bence-Jones protein in the urine of this patient would put him at
risk for development of disease that is most closely related
to which of the following?
/ A. AIzheimer disease
/ B. Familial amyloidotic neuropathy
/ C. Hemodialysis-associated amyloidosis
/ D. Primary amyloidosis
/ E. Reactive systemic amyloidosis

Explanation - Q: 4.4 Close

The correct answer is D. Multiple myeloma is usually accompanied by the
presence of free immunoglobulin light chains (Bence Jones protein) in the
serum and urine. These proteins eventually build up to toxic levels and
precipitate in the extracellular spaces as amyloid fibrils (AL type). This is
considered a primary amyloidopathy, since its development is not secondary
to an infectious/inflammatory process.
Alzheimer disease (choice A) is the most common non-infectious
amyloidopathy in the U.S. Amyloid in this disorder is formed of amyloid beta
protein cleaved from amyloid precursor protein, and thus is not closely
related to the proteinaceous fibrils of AL (amyloid of light chain
immunoglobulin molecules).
In familial amyloidotic neuropathy (choice B), the amyloid material is
composed of transthyretin.
In hemodialysis-associated amyloidosis (choice C), the precursor to the
amyloid fibril is beta-2 microglobulin.
Reactive systemic amyloidosis (choice E) is a secondary amyloidosis
resulting from chronic inflammatory conditions. The fibril precursor in this
case would be SAA (serum amyloid A protein).

Question 5 of 5
Which of the following conditions is likely to accompany this disease?
/ A. Decreased resistance to
/ B. Decreased resistance to lnfluenza A virus
/ C. Decreased resistance to
/ D. Decreased serum concentrations of C1, C4, and C2
/ E. Increased incidence of bacteremia

Explanation - Q: 4.5 Close

The correct answer is C. Patients with multiple myeloma generally suffer
from a decrease of humoral immune responses, although their cell-mediated
responses remain relatively normal. Of the options, the protective response
to Streptococcus pneumoniae is the one that is most strongly mediated by
antibodies, primarily of the IgA type.
Resistance to fungi (e.g., Candida albicans, choice A) and parasites is
primarily through cell-mediated mechanisms.
Resistance to intracellular pathogens such as the enveloped influenza A
virus (choice B) is primarily maintained by cell-mediated mechanisms.
C1, C4, and C2 (choice D) are components of the classical complement
cascade that are used up when complement is activated by complexes of
antigen and antibody (IgG and IgM). Since the myeloma protein reflects
proliferation of a single clone, it is unlikely that the myeloma antibody would
ever encounter the antigenic determinant that fits its idiotype and activate
complement.
Recurrent Neisseria bacteremia (choice E) usually results from deficiencies
in the late components of the complement cascade (C5-8), which are
genetic, and not present in multiple myeloma.
A 32 year-old woman is brought to the emergency department by her husband.
He states that his wife suddenly developed severe right back
pain approximately three hours ago. The pain is sharp in nature, radiates towards
her groin, and comes and goes. She vomits when she
arrives at the emergency department. She has no fevers or chills, and states that
she has had no prior similar episodes. She complains of
urinary urgency and frequency, but denies dysuria or hematuria. Her last bowel
movement was yesterday and normaI. She does not
remember the date of her last menses. The patient states that when the pain is
most severe, she is unable to catch her breath. As the
physician enters the room to evaluate the patient, he observes that she is tossing
and turning, and unable to get comfortable. On examination,
her temperature is 37.8 C (100.1 F) with otherwise normal vital signs. Heart and
lung examination are within normal limits. Abdominal
examination reveals decreased bowel sounds diffusely with mild right-sided
abdominal pain. There are no peritoneal signs. There is severe
costovertebral angle tenderness on the right side only.
Question 1 of 6
These symptoms are most consistent with which of the following?
/ A. Acute appendicitis
/ B. Acute cholecystitis
/ C. Biliary colic
/ D. Ectopic pregnancy
/ E. Renal colic

Explanation - Q: 5.1 Close

The correct answer is E. This patient is exhibiting a classic picture of renal
colic. This term describes the pain that is caused by the stretching of the
collecting system or ureter, and/or distention of the renal capsule. Urinary
obstruction (usually from a calcification) is the main mechanism responsible
for renal colic. As the stone moves down the ureter, local pain is referred to
the distribution of the ilioinguinal nerve and the genital branch of the
genitofemoral nerve, thus explaining the pain she is feeling in her groin.
Severity and location of the pain can vary from patient to patient due to stone
size, location, degree of obstruction, and acuteness of obstruction.
In contrast to renal colic, patients with acute appendicitis (choice A) have
peritoneal irritation and therefore prefer to remain perfectly still. Movement
irritates the peritoneal inflammation, making their pain worse. Abdominal
examination will reveal peritoneal signs.
Acute cholecystitis (choice B) has an onset of pain that is similar to biliary
colic (see below), however, the pain persists and may be unremitting for
several days. Progression of inflammation causes gallbladder distention,
leading to inflammation of the peritoneum; therefore the patient is most
comfortable lying still.
Biliary colic (choice C) is due to the presence of an impacted stone in the
cystic duct, or Hartmann pouch, or from passage of a stone through the duct.
It is characterized by a rapid increase in intensity of pain, a plateau of
discomfort that lasts for several hours, and a gradual decrease in intensity.
Pain is felt in the right upper quadrant or midepigastrium. This colic usually
occurs after meals. There are no associated urinary complaints.
Patients with an ectopic pregnancy (choice D) have lower abdominal pain
and irregular or absent menses. They do not complaint of back/flank pain.
On examination, there is discrete tenderness over the lower abdomen.



A 32 year-old woman is brought to the emergency department by her husband.
He states that his wife suddenly developed severe right back
pain approximately three hours ago. The pain is sharp in nature, radiates towards
her groin, and comes and goes. She vomits when she
arrives at the emergency department. She has no fevers or chills, and states that
she has had no prior similar episodes. She complains of
urinary urgency and frequency, but denies dysuria or hematuria. Her last bowel
movement was yesterday and normaI. She does not
remember the date of her last menses. The patient states that when the pain is
most severe, she is unable to catch her breath. As the
physician enters the room to evaluate the patient, he observes that she is tossing
and turning, and unable to get comfortable. On examination,
her temperature is 37.8 C (100.1 F) with otherwise normal vital signs. Heart and
lung examination are within normal limits. Abdominal
examination reveals decreased bowel sounds diffusely with mild right-sided
abdominal pain. There are no peritoneal signs. There is severe
costovertebral angle tenderness on the right side only.
Question 2 of 6
If the source of this patient's pain is found to be obstructive, chemical analysis of
the obstruction would most likely reveal which of the
following?
/ A. Bilirubin
/ B. Calcium oxalate
/ C. Cholesterol
/ D. Cystine
/ E. Uric acid

Explanation - Q: 5.2 Close

The correct answer is B. The source of pain is a renal calcification. There
are a variety of components that make up a renal stone, however, in the
United States, calcium oxalate is the most common type of kidney stone.
Bilirubin (choice A) is present in pigmented gallstones. These are the most
common types of gallbladder calculi worldwide. They are characterized by
their high concentration of bilirubin and their low cholesterol content.
Cholesterol gallstones (choice C) are the most common form of gallbladder
calculi in the United States. Neither cholesterol nor bilirubin are present in
renal stones.
Cystine stones (choice D) are relatively rare. They form in acidic urine and
are secondary to an inborn error of metabolism (cystinuria) resulting in
abnormal intestinal mucosal absorption and renal tubular absorption of
dibasic amino acids including cystine, ornithine, lysine, and arginine.
Uric acid stones (choice E) comprise less than 5% of all urinary calculi.
There is a higher incidence in patients with gout or myeloproliferative
diseases, and those treated for malignant conditions with cytotoxic drugs.
Acidic pH of the urine is necessary for their formation.
Question 3 of 6
If the patient instead was found to have a struvite stone, which of the following
would be a likely associated finding?
/ A. Acidic pH of the urine
/ B. E. coli
/ C. EIevated uric acid levels
/ D. Low urinary ammonium levels
/ E. Urinary tract infection
Explanation - Q: 5.3 Close

The correct answer is E. Struvite stones are composed of magnesium
ammonium phosphate (MAP). They are found most commonly in women,
and may recur rapidly. They are associated with infection with urea-splitting
organisms such as Proteus, Pseudomonas, Providencia, Klebsiella,
Staphylococci, and Mycoplasma. The high ammonium concentration derived
from the urea-splitting organisms results in an alkaline urinary pH. The
urinary pH of a patient with a MAP stone ranges from 6.8 to 8.3 and is rarely
below 7.0 (compare with choice A). The normal urinary pH is 5.85.
E. coli(choice B) is a frequent cause of urinary tract infections. However, it is
not a urea-splitting organism and therefore is not associated with struvite
stones.
Elevated uric acid levels (choice C) are associated with uric acid stones.
Uric acid stones compromise less than 5% of all urinary calculi.
As previously stated, the ammonium levels of patients with struvite stones is
elevated, not decreased (choice D).

Question 4 of 6
Ketorolac is given intravenously to the patient. To which of the following drug
classes does this agent belong?
/ A. Antibiotic
/ B. Antidiuretic
/ C. Nonsteroidal anti-inflammatory drug
/ D. Opioid
/ E. Steroid

Explanation - Q: 5.4 Close

The correct answer is C. Ketorolac (Toradol) is a nonsteroidal anti-
inflammatory agent (NSAID) and is the only NSAID approved for parenteral
use in the US. Ketorolac can dramatically improve the pain of renal colic and
can be administered either IM or IV. In severe cases, ketorolac can be used
in combination with opioid analgesics. Ketorolac has minimal side effects,
however, it can increase prothrombin time when used in conjunction with
anticoagulants, increase phenytoin levels and methotrexate toxicity, and is
potentiated by probenecid. This agent is contraindicated in patients with
renal failure, peptic ulcer disease, or recent GI bleeding.
The use of antibiotics (choice A) in patients with kidney stones is
controversial. Antibiotic use is justified if there is evidence of a UTI. Whereas
only 3% of patients treated for renal colic have been shown to develop new
UTIs, an infection can seriously complicate the patient's clinical outcome.
The antidiuretic (choice B) desmopressin (DDAVP), a synthetic analogue of
vasopressin, has been shown to substantially reduce the pain of patients
with renal colic. Its mechanism of action is thought to be reduction of
intraureteral pressure, but it may also relax ureteral and renal pelvic
musculature.
Opioids (choice D) are an important part of acute renal colic therapy.
Morphine, meperidine, and butorphanol are the agents most commonly used.
Prednisone, an oral corticosteroid (choice E), is a potent anti-inflammatory
agent that can reduce local edema in the ureter.
Question 5 of 6
The patient continues to have nausea and vomiting. Which of the following is the
most appropriate pharmacotherapy?
/ A. Diphenoxylate
/ B. Lansoprazole
/ C. Loperamide
/ D. Metoclopramide
/ E. Sucralfate

Explanation - Q: 5.5 Close

The correct answer is D. Patients with acute renal colic frequently have
nausea and vomiting, therefore antiemetics frequently have a role in the
treatment of such patients. The antiemetic effect of metoclopramide results
from its ability to block central dopamine receptors. Clinical trials have
additionally shown metoclopramide to be effective in relief of the pain of renal
colic. Other antiemetic medications are also used, such as promethazine,
hydroxyzine, and prochlorperazine.
Diphenoxylate (choice A) and loperamide (choice C) are antidiarrheal
agents.
Lansoprazole (choice B) decreases gastric acid production by irreversibly
inhibiting the H+/K+ ATPase located in the luminal membrane of parietal
cells. This agent, and similar agents such as omeprazole, are used to treat
Zollinger-Ellison syndrome and gastroesophageal reflux disorder (GERD).
Sucralfate (choice E), or aluminum sucrose sulfate, polymerizes in the acid
environment of the stomach. The polymer then forms a protective coating
over ulcer beds, thus accelerating the healing of peptic ulcers and reducing
their rate of recurrence.

Question 6 of 6

For this stone to pass spontaneously from this patient, it must travel the entire
length of the ureter. Which of the following correctly describes
the anatomical position of the right ureter?
/ A. It crosses underneath the common iliac artery
/ B. It lies medial to the inferior vena cava
/ C. It sits on the quadratus lumborum muscle
/ D. It travels between the inferior vena cava and the descending abdominal
aorta
/ E. The right gonadal vessels cross over the ureter

Explanation - Q: 5.6 Close

The correct answer is E. The gonadal vessels roughly parallel the ureter
through much of its retroperitoneal extent. These vessels obliquely cross
over the ureter from medial to lateral before entering the renal pelvis.
Normally, the gonadal arteries are branches of the abdominal aorta. The
right gonadal vein drains directly into the vena cava while the left gonadal
vein drains into the left renal vein.
At no point does either ureter go underneath the iliac vessels (choice A).
The ureter crosses above the iliac vessels at approximately the bifurcation of
the internal and external iliac arteries.
The inferior vena cava and abdominal aorta are both retroperitoneal
structures. The aorta runs slightly on the patient's left side and the inferior
vena cava runs slightly on the patient's right side. Along its path, the right
ureter remains lateral to the inferior vena cava (choices B and D). Rarely,
the ureter may cross underneath the vena cava in its superior location. This
is called a retrocaval ureter.
In its retroperitoneal location, the ureter (on either side) is related posteriorly
to the psoas muscle. The quadratus lumborum (choice C) runs lateral to the
psoas muscle and is not related to the ureter.


A 23-year-old woman complains to her physician of chronic flatulence, abdominal
cramping, and being bloated. She delivered a healthy baby
3 months previously, and she states that her symptoms began several months
into her pregnancy. She had expected them to disappear after
delivery, but they did not. On further questioning, the woman reports that she has
been having alternating diarrhea and constipation. Her stool
appears dark and oily to her, and floats in the toilet bowI. She feels hungry all of
the time, and has been losing weight despite eating a great
deaI.
Question 1 of 5
This woman's symptoms are most suggestive of which of the following?
/ A. Biliary tract disease
/ B. Cancer of the gastrointestinal tract
/ C. Granulomatous infectious disease
/ D. Malabsorption
/ E. Psychiatric disease

Explanation - Q: 1.1 Close

NONE AVAILABLE

Question 2 of 5
The woman's physician initially suggests that the patient avoid milk products, but
her symptoms fail to improve. This finding is consistent with
which of the following conditions?
/ A. Adenomatous polyps
/ B. Appendicitis
/ C. Celiac disease
/ D. Cystic fibrosis
/ E. UIcerative colitis

Explanation - Q: 1.2 Close

The correct answer is C. Flatulence, bloating, and abdominal cramping
often reflect bacterial gas production as a result of utilization of unabsorbed
nutrients by the bacteria within the gut. On a practical basis, lactose
intolerance is the most common cause of malabsorption with flatulence, and
a trial of avoidance of lactose-containing milk products was reasonable. If
this fails to correct the problem, then more serious gastrointestinal (usually
small intestinal) disease must be considered. Of the list given in the choices,
only celiac disease and cystic fibrosis (choice D) commonly cause
malabsorption, and cystic fibrosis almost always presents in childhood.
Adenomatous polyps (choice A) are usually asymptomatic, but may cause
bleeding per rectum.
Appendicitis (choice B) usually presents with fever and right lower quadrant
abdominal pain.
Ulcerative colitis (choice E) can cause alternating diarrhea and constipation,
but would not usually cause significant malabsorption, since the small bowel
is not significantly involved.
Question 3 of 5
Which of the following is usually considered to be the most accurate way of
establishing this patient's likely diagnosis?
/ A. BIood culture
/ B. Jejunal biopsy
/ C. Nasal biopsy
/ D. Rectal biopsy
/ E. Stool for ova and parasites

Explanation - Q: 1.3 Close

The correct answer is B. Jejunal biopsy demonstrating flattened mucosal
villi is considered the gold standard for demonstrating celiac disease. The
patient should have active disease (e.g., be on a normal diet and
symptomatic) at the time of biopsy in order for the diagnosis to be
established. Some clinicians also simply do a trial of gluten-free diet, to avoid
subjecting the patient to biopsy. You should also be aware that endomysial
antibody (EMA) titers in serum are relatively sensitive and specific for celiac
disease, and have been proposed (although still not widely accepted) as a
screening tool for celiac disease.
The disease does not appear to have an infectious basis, and so blood
culture (choice A) and stool examination for ova and parasites (choice E)
would not be helpful.
Celiac disease is usually confined to the small intestine, so nasal (choice C)
and rectal (choice D) biopsies would not be helpful.
Question 4 of 5
Which of the following is an example of a food that the patient should avoid?
/ A. Apple
/ B. Bread
/ C. Carrot
/ D. Lettuce
/ E. Steak
Explanation - Q: 1.4 Close

The correct answer is B. Celiac disease appears to be the result of an
immunologically mediated toxic reaction to gluten that occurs in genetically
susceptible individuals. The gluten proteins can be found in all forms of
wheat (including durum, semolina, spelt, kamut, einkorn, and faro), as well as
related grains including barley, triticale, rye, and possibly, oats. Breads are
consequently an obvious food to avoid, but patients should be warned that
the essentially complete avoidance of these grain products may require that
they prepare most of their own foods, since a surprising variety of
commercially prepared foods contain small amounts of flour or other grain
products. Even products like vinegars, alcohols, and pharmaceuticals may
have gluten-containing additives. Celiac disease often presents in childhood,
but some individuals (such as this patient) remain either asymptomatic or
undiagnosed until some event in adulthood (such as pregnancy, child birth,
surgery, viral infection, or severe emotional stress) causes an acute
exacerbation of their disease. Consequently, the absence of a life-long
history of problems should not be used to exclude the possibility of celiac
disease in a symptomatic patient.
Fresh fruits (choice A), vegetables (choices C and D), and meats (choice
E) prepared at home without additives can be eaten safely.

Question 5 of 5
Several years later, the patient cheats on her dietary restrictions and develops a
blistering, intensely itchy skin rash that has a symmetrical
distribution and is found on her elbows, knees, and buttocks. This is most likely
which of the following?
/ A. Dermatitis herpetiformis
/ B. Herpes simplex
/ C. Herpes zoster
/ D. Molluscum contagiosum
/ E. Psoriasis
Explanation - Q: 1.5 Close

The correct answer is A. Dermatitis herpetiformis is a skin condition known
to be associated with celiac disease, and, interestingly, may also be the
presenting complaint for celiac disease. The diagnosis can be confirmed by
skin biopsy with demonstration of IgA in the biopsy tissues. More than 85%
of patients with dermatitis herpetiformis also have (obviously symptomatic or
not) gluten sensitivity, and removal of gluten from the diet may improve the
skin disease. Untreated or poorly treated celiac disease is also associated
with anemia (due to iron, folate, or vitamin B12 deficiency), osteoporosis,
vitamin K deficiency with risk of hemorrhage, nervous system disorders
secondary to nutrient deficiencies, pancreatic insufficiency, and intestinal
lymphomas. Other disorders, in addition to dermatitis herpetiformis with
which celiac disease is associated, include diabetes mellitus, thyroid
disease, lupus, IgA nephropathy, primary biliary cirrhosis, and less commonly
chronic active hepatitis, scleroderma, myasthenia gravis, Addison disease,
rheumatoid arthritis, Sjgren syndrome, and Down syndrome.
Herpes simplex (choice B) and herpes zoster (choice C) are infectious
causes of vesicular skin disease not specifically associated with celiac
disease.
Molluscum contagiosum (choice D) is due to a viral infection that induces the
formation of raised papules with central craters ("small volcanoes").
Psoriasis (choice E) is a predominately autoimmune skin condition
characterized by scaly patch formation.


During a routine examination of a 2-year-old child, the mother mentions to the
pediatrician that the child has been bloated and has passed
Iarge amounts of gas for the last several months. On further questioning, the
mother also reports that the child has been having intermittent,
but frequent episodes of watery malodorous diarrhea with abdominal cramps and
sometimes fever, which began shortly after the child was
moved to a new day care center. Other family members have also had similar
symptoms, but of shorter duration, which have since resolved.
Physical examination of the child is remarkable only for hyperactive bowel
sounds.
Question 1 of 5
Which of the following is the most likely pathogen?


Explanation - Q: 2.1 Close

The correct answer is D. All of the protozoa listed in the choices are
intestinal parasites that can cause chronic diarrhea. Giardia lamblia is the
most frequent cause of protozoal diarrhea in North America. The case history
illustrates the typical course in children who acquire the infection in a day
care setting and then go on to develop chronic infection. Giardia lamblia is an
interesting protozoal parasite whose life cycle alternates between trophozoite
and cyst stages. While most bacterial infections require inoculating doses of
hundreds to thousands of organisms or more to produce disease, giardiasis
requires ingestion of only as few as 10 or less organisms in the cyst form
(research studies have shown infection after ingestion of a single cyst). In
practice, this means that the infection is very highly communicable in a day
care or home setting, since it is difficult to remove all of the cysts from one's
hands after handling a contaminated diaper. The use of gloves and taking
care to keep children from playing with their diapers can be helpful in
controlling the spread of the disease. Giardiasis can also be acquired
through ingestion of contaminated water (it will resist chlorination of less than
8 hours but can be killed with boiling or removed with micropore filtration),
and so can also be seen among hikers and as traveler's diarrhea. The
reservoirs appear to be beavers, bears, dog, cats, and humans.


Question 2 of 5

A thorough stool exam for ova and parasites is most likely to reveal which of the
following?
/ A. Acid fast oocyst that are 5 microns in diameter
/ B. Acid fast oocysts that are 10 microns in diameter
/ C. Oval cysts with up to 4 nuclei
/ D. Round cysts containing no more than 4 nuclei
/ E. Round cysts, including some with 5 to 8 nuclei

Explanation - Q: 2.2 Close

The correct answer is C. The cysts of Giardia lamblia are oval, have
prominent cell walls, and up to 4 nuclei. A diagnosis of giardiasis can be
made by finding either the trophozoites and/or the cysts in a stool sample.
However, organisms are only found in 50% of cases if only one stool sample
is used. If three stool samples obtained on three different days are used, the
detection rate is approximately 90%. If stools are negative, Giardia can be
detected in duodenal material that can be sampled using the Enterotest. A
gelatin capsule that is attached to a nylon string is swallowed. After 4 to 6
hours, the string is removed and examined under the microscope for
trophozoites.
Choice A describes the oocysts of Cryptosporidium parvum, a common
cause of mild diarrheal illness. It causes incurable, protracted diarrhea in
AIDS patients. The diagnosis of cryptosporidiosis is made by finding oocysts
in the feces using a modified acid fast or auramine stain.
Choice B describes the oocysts of Cyclospora cayetanensis, a protozoa that
has recently been identified as a cause of diarrheal illness. In 1995 and
1996, it caused major outbreaks in the United States that were later traced to
contaminated raspberries from Guatemala. Diagnosis of cyclosporiasis can
be made by finding the acid fast oocyst in stool. Its oocyst is much larger
than that of Cryptosporidium parvum, and it is important to differentiate
between them since Cyclospora can be treated with trimethoprim-
sulfamethoxazole and other antibiotics, whereas there is no effective
treatment for Cryptosporidium parvum.
Choice D describes the cysts of Entamoeba histolytica. The diagnosis of
intestinal infection with Entamoeba histolytica can be made by finding either
trophozoites or cysts in the stool. The cysts of Entamoeba histolytica are
round, have refractile walls and contain up to four nuclei.
Choice E describes the cysts of Entamoeba coli, a nonpathogenic
protozoan. Its cysts can be distinguished from the cysts of Entamoeba
histolytica because they contain 5 to 8 nuclei.
Question 3 of 5
How long, after exposure to this organism, do most people who develop clinical
disease exhibit symptoms?
/ A. 1-2 days
/ B. 3-5 days
/ C. 1-3 weeks
/ D. 4-6 weeks
/ E. 2-3 months

Explanation - Q: 2.3 Close

The correct answer is C. Most patients who develop clinical disease
become symptomatic 1-3 weeks after cyst ingestion. Many patients have
asymptomatic infections, and it appears that some degree of immunity is
often acquired after exposure, since clinical infection is more common in
children than in adults after known exposures. Many patients who develop
clinical illness have disease that lasts only 1 to 2 weeks, but chronic cases
also occur, and are an important source for spread of the infection.
Symptoms can include diarrhea, abdominal cramps, pale and greasy stools,
fatigue, bloating, and weight loss. The mechanism by which the organisms
produce disease is still poorly understood, but they are known to colonize
(sometimes very heavily) the surface of the small intestine without invasion
into the mucosa. Some of the symptoms may be due to an acquired (and
reversible) lactase deficiency related to malfunction of the intestinal
epithelium.
Question 4 of 5
Which of the following drugs would be most effective in the treatment of this
child?
/ A. Diloxanide furoate
/ B. Iodoquinol
/ C. Metronidazole
/ D. Paromycin
/ E. Trimethoprim-sulfamethoxazole

Explanation - Q: 2.4 Close

The correct answer is C. It is important to establish an accurate diagnosis
in protozoal intestinal infections, because different organisms have markedly
different drug sensitivities and no "shot-gun" therapy is likely to be effective.
Metronidazole (Flagyl) is effective against Giardia (and also Entamoeba); it is
not officially licensed for this use in the United States but is commonly used
anyway. Alternative agents include furazolidone (less effective than
metronidazole) and oral quinacrine (no longer available in the United States
because of severe side effects).
Diloxanide furoate (choice A) and iodoquinol (choice B) and are sometimes
used in the treatment of amebiasis.
Paromycin (choice D) can be used to treat cryptosporidiosis.
Trimethoprim-sulfamethoxazole (choice E) can be used to treat isosporiasis
and cyclosporiasis.
Question 5 of 5
This disease would most likely contribute to the patient's death if which of the
following diseases were also present?
/ A. AIDS
/ B. Crohn disease
/ C. Goodpasture's syndrome
/ D. Measles
/ E. Minimal change disease
Explanation - Q: 2.5 Close

The correct answer is A. Patients who have had rectal contact during sex
with an infected partner may easily acquire giardiasis. If these patients also
have AIDS, a particularly overwhelming infection may occur, with essentially
"wall-to-wall" colonization of the intestinal mucosal surface with Giardia. In
these severe cases, the malabsorption that is produced may be sufficiently
severe to induce malnutrition, which will exacerbate the AIDS patient's
already high vulnerability to other infectious disease. The diseases listed in
the other choices are distracters that have no particular link to giardiasis.










A 62-year-old man complains to his physician that he has noticed a marked
increase in the amount of flatulence he experiences. He has also
been experiencing intermittent, but increasing abdominal pain, and has had a 20
pound weight loss in the last month. On physical
examination, he seems to be slightly jaundiced. AIkaline phosphatase and
bilirubin are increased. CT scan demonstrates a mass in the head
of the pancreas.
Question 1 of 4
Approximately how many Americans in the United States are diagnosed with this
disorder each year?
/ A. 280
/ B. 2,800
/ C. 28,000
/ D. 280,000
/ E. 2,800,000

Explanation - Q: 3.1 Close

The correct answer is C. Approximately 28,000 patients in the United
States are diagnosed with pancreatic cancer yearly, and nearly that number
also die of it yearly. Pancreatic cancer is a deadly disease, with one of the
highest mortalities of any cancer, many patients dying within one year of
diagnosis. The underlying problem is that pancreatic cancer tends to not
produce symptoms until after metastases to nearby critical structures (lymph
nodes, liver, celiac plexus, superior mesenteric vessels, Ligament of Treitz,
portal vein) have occurred. At least some pancreatic cancers appear to have
a genetic basis. The National Familial Pancreas Cancer Registry now has
over 250 families with two or more members with pancreatic cancer. Also,
the second familial breast cancer gene, BRCA2 (in both men and women),
appears to be important, and may account for the increased incidence of
pancreatic cancer seen in Ashkenazi Jews. Other rare syndromes that may
have pancreatic cancer as a component include Peutz-Jeghers syndrome,
familial melanoma, hereditary colon cancer (the form without polyps), and
hereditary pancreatitis.
Question 2 of 4
Which of the following is an important risk factor for developing this disorder?
/ A. Aflatoxin exposure
/ B. AIcohol use
/ C. Caffeine ingestion
/ D. Cigarette smoking
/ E. Hepatitis B infection

Explanation - Q: 3.2 Close

The correct answer is D. Cigarette smoking appears to be a very important
risk factor for pancreatic carcinoma, but interestingly, neither alcohol use
(choice B) nor caffeine ingestion (choice C) have been implicated. Other
risk factors include older age, race (more common in African-Americans and
some Jewish groups than Caucasians), gender (men more than women, but
may just reflect smoking rates), chronic pancreatitis, diabetes mellitus,
gastric resection, and diet (bad: meats, cholesterol, fried foods, nitrosamines;
good: fruit, vegetables).
Exposure to the fungal product aflatoxin (choice A) and hepatitis B (choice
E) are risk factors for liver, but not pancreatic, cancers.
Question 3 of 4
In patients with this man's condition with intractable pain referred to the back, the
network of nerves around the aorta is sometimes blocked
with alcohoI. This network is known as which of the following?
/ A. Celiac plexus
/ B. Choroid plexus
/ C. Esophageal plexus
/ D. Hepatic plexus
/ E. Mesenteric plexus
Explanation - Q: 3.3 Close

The correct answer is A. The celiac plexus contains the nerves around the
aorta that may be stimulated by either pressure or direct involvement by
tumor, and so produce pain.
The choroid plexus (choice B) is a vascular plexus of the eye, and is also a
vascular plexus extending into the ventricles.
The esophageal plexus (choice C) refers to the nerve plexus around the
esophagus.
The hepatic plexus (choice D) is the division of the celiac plexus that
accompanies the hepatic artery and portal vein to the liver.
There are actually two mesenteric plexuses (choice E), which accompany
the superior and inferior mesenteric arteries.

Question 4 of 4
Which of the following surgical methods is used most often to treat patients with
small tumors of the head of the pancreas with no evidence of
metastasis?
/ A. Billroth l procedure
/ B. Billroth ll procedure
/ C. Roux-en-Y gastric bypass
/ D. Vertical banded gastroplasty
/ E. Whipple procedure
Explanation - Q: 3.4 Close

The correct answer is E. While you do not need to know a great deal about
surgery for the Step 1 USMLE examination, a few operations may be
mentioned. Among these is the Whipple procedure, also known as a
pancreaticoduodenectomy. In this surgical procedure, the duodenum,
proximal pancreas, gallbladder, and sometimes, the distal stomach are
resected. The remaining pancreas, biliary tree, and stomach are then
reattached to the small intestine. The Whipple procedure is only performed in
the relatively small percentage of patients who may have resectable disease.
Tumors of the tail of the pancreas might, in theory, be more easily resected,
but this area tends to not produce any signs or symptoms until late in the
disease, after metastasis has occurred. Pancreatic cancers that are not
resectable can be treated with chemotherapy and radiation therapy.
Immunotherapy using a vaccine based on the patient's own cancer cells is
also being tried experimentally, and shows promise. An important thing to
remember when treating these patients (and other poor prognosis patients)
is that long-term survivors have occurred, and that even if death eventually
intervenes, a survival of several years may be very much worthwhile to both
the patient and his family (particularly if young children are indirectly
involved).
The Billroth I and II procedures (choices A and B ) are used to treat gastric
carcinomas and intractable peptic ulcers of the duodenum and stomach.
While you could argue that the gastric resection part of the Whipple
procedure resembles a Billroth II operation, you should pick the Whipple
procedure if both answers are available, because it is much more specific for
pancreatic carcinoma.
The Roux-en-Y gastric bypass (choice C) and vertical banded gastroplasty
(choice D) are used to treat obesity by reducing the effective stomach
volume.
A 14-year-old girl asks her pediatrician if there is anything she can do about the
large amounts of gas she passes during class at schooI. She
states that her flatulence is worse in the afternoon, and she often has diarrhea
when she goes home from schooI. Her symptoms generally
subside by morning, and she thinks she is better on weekends. She has been
having these problems for four or five years, but has the
impression that they are worse now than when she was younger.
Question 1 of 5
The physician suspects food intolerance. Statistically, intolerance to which of the
following would be most likely in this patient's age group and
with her history?
/ A. Fructose
/ B. Galactose
/ C. Lactose
/ D. Maltose
/ E. Sucrose

Explanation - Q: 4.1 Close

The correct answer is C. Lactose intolerance is the most common form of
intolerance to sugars. Some authors argue that it is often not even really a
"disease," but rather a natural consequence of the maturation of the small
intestine, with resulting reduction in the levels of mucosal lactase.
Caucasians tend to have a lower incidence of symptomatic problems than
many other races (e.g., Blacks, Asians and Native Americans). Impaired
absorption of the other sugars listed in the choices can also occur, but is
rare.
Question 2 of 5
A consulting dietician identifies a major food group that should be avoided by the
young patient and further cautions that avoiding these foods
may put her at risk for a secondary dietary deficiency. If the patient removes the
offending food from her diet, she is at greatest risk of
developing a deficiency of which of the following?
/ A. Calcium
/ B. Chloride
/ C. Iron
/ D. Potassium
/ E. Sodium

Explanation - Q: 4.2 Close

The correct answer is A. Lactose is found predominately in milk and milk
products. Aged cheeses and butter have lower concentrations of lactose and
may be tolerated by many individuals. Complete removal of milk products
from the diet puts the individual at greatest risk for developing calcium
deficiency, because milk products are a major source of calcium. Three easy
ways to prevent the deficiency are to encourage the individual to drink fruit
juice products supplemented with calcium, to use lactose-free milk products,
and to chew calcium-containing antacid tablets. Lactase enzyme is also
available in forms that can be added to milk (usually hours before drinking) or
taken as tablets before eating a lactose-containing meal.
Table salt is the usual source of sodium and chloride (choices B and E).
Meats are the most important dietary source of iron (choice C).
Dietary deficiencies of potassium (choice D) are usually only seen if severe
restriction of all fruits and vegetables is present or if the patient is taking a K-
losing diuretic.
Question 3 of 5
This patient's diarrhea would be most accurately described as which of the
following
/ A. Diarrhea related to increased intestinal motility
/ B. Diarrhea related to short gut syndrome
/ C. Exudative diarrhea
/ D. Osmotic diarrhea
/ E. Secretory diarrhea

Explanation - Q: 4.3 Close

The correct answer is D. While there is some overlap in mechanisms in
some cases of diarrhea, it can be helpful to conceptualize diarrhea into
categories based on the mechanism accounting for the diarrhea. In this case,
the lactose sugar that is retained in the gut markedly increases the
osmolarity of the gut contents, which provides a physiologic force that tends
to hold fluid in the gut.
Irritable bowel syndrome is a good example of a type of diarrhea due to
increased intestinal motility (choice A).
The diarrhea seen in infants after recovery from necrotizing enterocolitis is a
good example of diarrhea related to a short gut (choice B).
The diarrhea seen in patients with invasive amebiasis is an example of an
exudative diarrhea (choice C).
The toxin-mediated diarrhea seen in cholera is a good example of a
secretory diarrhea (choice E).

Question 4 of 5
The undigested carbohydrate passing into the colon will induce enzymes for its
metabolism in resident E. Coli. The mechanism that induces
gene expression for these enzymes most directly involves the carbohydrate
/ A. binding to a repressor protein
/ B. binding to an activator protein
/ C. binding to an enhancer element associated with the gene region
/ D. decreasing cAMP within the E. Coli
/ E. increasing cAMP within the E.Coli

Explanation - Q: 4.4 Close

The correct answer is A. The enzymes required to metabolize lactose are
encoded by the lactose operon in E. coli. Lactose induces gene expression
by binding to a repressor protein and interfering with repressor binding to the
operator region of the DNA. Lack of repression contributes to inducing
expression of the genes. E. coli carries out lactose fermentation, producing
hydrogen gas that may be exhaled and detected in the breath of individuals
who are lactose intolerant.
Lactose has no direct effect on the cAMP concentration (choices D and E).
cAMP concentrations are more directly controlled by the extracellular
concentration of glucose. In response to a low extracellular glucose
concentration, cAMP increases, binds to an activator protein (choice B) that
in turn binds to a CAP region (similar to a eukaryotic enhancer) (choice C) in
the DNA. This mechanism does not directly involve lactose.


Question 5 of 5



The result of a large population study to determine the adult distribution of
deficiency of the intestinal enzyme that would normally metabolize
the nutrient in question is shown in the graph above. Given this data, if one
wanted to determine the heritability of this deficiency, the most
appropriate test would be which of the following?
/ A. A concordance study in twins
/ B. Calculation of the Hardy-Weinberg distribution
/ C. Calculation of the linkage dysequilibrium
/ D. Log of the odds (LOD) score calculation
/ E. Spectral karyotype

Explanation - Q: 4.5 Close

The correct answer is A. The graph shows a distribution characteristic of a
multifactorial trait. If one wanted to determine the contribution of genes
(heritability) versus non-genetic factors (diet, environment, etc.), a
concordance study in monozygotic (MZ) versus dizygotic (DZ) twins would
be appropriate. Heritability is calculated as (C
MZ
- C
DZ
)/(1- C
DZ
). It is not
necessary to memorize the calculation but rather to understand the concept
of heritability and concordance studies in twins.
The Hardy-Weinberg equation (choice B) is most commonly used to
calculate carrier frequencies in recessive disease. It can also be used to
calculate the disease prevalence from the carrier frequency. Although it can
be applied to dominant genetic diseases, there is no carrier status in these
conditions and the equation is not typically used in these cases.
Linkage dysequilibrium exists if specific combinations of alleles at two loci
are seen together more often than expected by chance. Calculation of
linkage dysequilibrium (choice C) is most commonly used in mapping genes.
A LOD score calculation (choice D) is used to identify linkage between two
genetic loci. A common usage is in establishing linkage between a disease
phenotype and a known chromosomal marker in an effort to map the gene
involved.
A spectral karyotype (choice E) is used to assess cytogenetic abnormalities
such as trisomies, deletions, and translocations.


A 41-year-old woman comes to the physician 3 weeks after a vaginal delivery,
complaining of a lump in her right breast. She states that over
the past few days, she has noticed increasing redness in the area and that the
site is painful and feels somewhat firm to her. She also
complains of fever and chills. She is concerned because she is currently breast-
feeding her child. She has no medical or surgical history. She
uses acetaminophen occasionally for headaches and is allergic to sulfa drugs.
Her temperature is 37.8 C (100.1 F), blood pressure is
110/70 mm Hg, pulse is 98/minute, and respirations are 12/minute. The right
breast has an area of erythema and edema that is tender with
no fluctuance.
Question 1 of 4
Which of the following is the most likely diagnosis?
/ A. Breast carcinoma
/ B. Eczema
/ C. Fibroadenoma
/ D. Mastitis
/ E. Trauma

Explanation - Q: 1.1 Close

The correct answer is D. Mastitis represents a parenchymatous infection of
the mammary glands. It is most often seen in postpartum women who are
breast-feeding, and the symptoms typically appear 3-4 weeks postpartum.
Most women with mastitis complain of pain in the breast with an area of
redness and "hardness." Women also often have fever and chills, myalgias
and arthralgias, and tachycardia. Examination shows erythema, edema, and
tenderness.
Patients with breast carcinoma (choice A) often present with an
asymptomatic mass. While it is possible for patients with breast cancer to
have erythema and edema of the breast and tenderness on exam, this
presentation in a postpartum, breast-feeding woman is most consistent with
mastitis.
Eczema (choice B) does not present with a lump, as this patient has. It is a
superficial disorder involving the top part (epidermis and upper dermis) of the
skin only. It is also characterized by scale with erythema and not associated
with fever, chills, and systemic symptoms like this patient has.
Fibroadenoma (choice C) represents a proliferation of fibrous tissue in the
breast. It is the most common tumor in young women. Patients with
fibroadenoma typically present with complaints of a breast lump. The mass is
usually small, unilateral, firm, and freely mobile. Patients with fibroadenoma
do not usually present with erythema, edema, pain in the breast, and
systemic signs of infection.
Trauma (choice E) to the breast can lead to a condition called fat necrosis.
Patients with breast trauma usually complain of a firm area of the breast,
sometimes mobile, and occasionally with induration. However, patients with
fat necrosis from breast trauma usually recall a prior incident of trauma.
Question 2 of 4
Histological examination of diagnostic tissue from this patient would reveal which
of the following?
/ A. A Iocalized area of acute inflammation
/ B. Cellular, fibroblastic stroma enclosing cystic spaces lined by epithelium
/ C. Edema fluid within the intercellular spaces of the epidermis
/ D. Hemorrhage enclosed within the tissue
/ E. Strands of infiltrating tumor cells

Explanation - Q: 1.2 Close

The correct answer is A. This patient has a presentation that is most
consistent with mastitis, which represents an acute localized infection.
Histologic evaluation would, therefore, reveal a localized area of acute
inflammation with edema and neutrophil emigration.
Cellular, fibroblastic stroma enclosing cystic spaces lined by epithelium
(choice B) would be the histology of a fibroadenoma and not mastitis.
Edema fluid within the intercellular spaces of the epidermis (choice C) is the
histologic finding in eczema.
Hemorrhage enclosed within a tissue (choice D) describes a hematoma and
would be the expected histologic finding in a patient with trauma to the breast
with a resultant hematoma. This patient's presentation is more consistent
with mastitis than trauma.
Strands of infiltrating tumor cells (choice E) would be the expected finding in
a patient with certain types of carcinoma of the breast. This is not the
histologic finding in mastitis.

Question 3 of 4
Which of the following is most likely responsible for this pathologic process?



Explanation - Q: 1.3 Close

The correct answer is E. Staphylococcus aureus is a catalase-positive,
coagulase-positive, and beta-hemolytic organism that is the most common
cause of mastitis. The source of the organism is almost always from the
nursing infant's oropharynx. Enterotoxin F, or Toxic Shock Syndrome Toxin,
has been reported to cause toxic shock syndrome in some patients with
mastitis caused by Staphylococcus aureus.
Blunt force injury (choice A) to the breast might be expected to cause a
hematoma or fat necrosis. This patient has a presentation that is consistent
with mastitis, and not traumatic injury to the breast.
Chlamydia trachomatis(choice B) is an obligate intracellular organism. It is
most commonly found in the genital tract and is associated with cervicitis and
pelvic inflammatory disease in women, urethritis in men, and pneumonia and
conjunctivitis in newborns. It is not normally associated with mastitis.
Hormonal exposure (choice C) is not considered causative of mastitis. There
is some evidence that hormonal exposure may contribute to the development
of breast cancer. This patient, however, has a presentation more consistent
with mastitis than breast cancer.
Neisseria gonorrhoeae(choice D) is a gram-negative coccus that can cause
cervicitis, pelvic inflammatory disease, arthritis, pharyngitis, and urethritis. It
can also cause neonatal conjunctivitis. It is not commonly associated with
mastitis.

Question 4 of 4
The patient is started on dicloxacillin. This medication works via which of the
following mechanisms?
/ A. BIocking cell wall synthesis
/ B. Inhibition of bacterial dihydrofolate reductase
/ C. Inhibition of bacteriaI DNA gyrase
/ D. Inhibition of protein synthesis
/ E. Inhibition of resorption of sodium and chloride

Explanation - Q: 1.4 Close

The correct answer is A. Dicloxacillin belongs to the general class of
penicillin antibiotics. Penicillins interfere with bacterial cell wall synthesis by
binding to bacterial penicillin binding proteins, resulting in eventual bacterial
cell lysis. Bacterial resistance to penicillins results when bacterial beta-
lactamases disrupt the beta-lactam ring contained within these antibiotics.
Dicloxacillin (like methicillin and nafcillin) is synthesized to be resistant to
beta-lactamases. However, resistance to these antibiotics is increasing as
well. If a patient with mastitis does not respond to dicloxacillin, bacterial
resistance should be suspected and vancomycin should be used.
Inhibition of bacterial dihydrofolate reductase (choice B) is the mechanism of
action of trimethoprim and pyrimethamine.
Inhibition of bacterial DNA gyrase (choice C) is the mechanism of action of
the fluoroquinolones and quinolones.
Inhibition of protein synthesis (choice D) is the mechanism of action of the
lincosamines (clindamycin, lincomycin). These drugs bind the 50S subunit of
ribosomes to inhibit the bacterial protein synthesis.
Inhibition of resorption of sodium and chloride (choice E) is the mechanism
of furosemide (a loop diuretic).

A 37-year-old woman undergoes a routine breast examination. During the breast
examination, the physician is aware that the skin of the
breast moves together with the underlying breast tissue, rather than being
obviously separate from it.
Question 1 of 6
The breast tissue is normally attached to the overlying skin via which of the
following?
/ A. Cooper's ligaments
/ B. Cruciate ligaments
/ C. Falciform ligament
/ D. Poupart's ligaments
/ E. Rhomboid ligaments

Explanation - Q: 2.1 Close

The correct answer is A. The suspensory ligaments of Cooper are fibrous
condensations of connective tissue stroma that attach the mammary gland to
the dermis of the overlying skin. These are particularly prominent in the
superior aspect of the breast, and help to support the breast tissue.
The cruciate ligaments(choice B) are in the knee.
The falciform ligament (choice C) attaches the peritoneum to the liver.
Poupart's ligament (choice D) is an alternative name for the inguinal
ligament.
The rhomboid ligament (choice E) is another name for the costoclavicular
ligament.
Question 2 of 6
Careful examination of the central depressed area of the nipple demonstrates
multiple small openings. These openings are from which of the
following?
/ A. Areola
/ B. Lactiferous ducts
/ C. Montgomery's glands
/ D. Sweat glands
/ E. Terminal ductules

Explanation - Q: 2.2 Close

The correct answer is B. The lactiferous ducts open into the nipples. The
area of each of these ducts immediately below the nipple is usually dilated,
forming a lactiferous sinus, which can store a droplet of milk that helps to
initiate the baby's sucking reflex during nursing.
The areola (choice A) is the ring of darkly pigmented skin around the nipple.
Montgomery's glands (choice C) are modified eccrine glands (described
incorrectly by some authors as sebaceous glands) that provide oil and
moisture for the skin of the nipple and areola. They open into the areola in
small tubercles rather than the nipple.
Sweat glands (choice D) are common in the skin of the breast generally, but
are too small to be able to seen by the unaided eye.
The terminal ductules (choice E) of the breast system are at the deep end of
the duct system of the breasts, and receive milk from the lobular tissue.
Question 3 of 6
The physician identifies a palpable mass in one breast, and the patient is
scheduled for a "Iumpectomy." The reason that "Iumpectomies" for
breast lumps can be safely performed in some patients is that the breast is
divided into multiple lobes, each of which contains a separate
duct system with connecting lobules. The normal breast usually contains how
many lobes?
/ A. 2 to 3
/ B. 5 to 7
/ C. 10 to 12
/ D. 15 to 25
/ E. 30 to 50

Explanation - Q: 2.3 Close

The correct answer is D. The normal breast contains 15 to 25 lobes. Each
lobe can be thought of as having an "inverted tree" composed of a "trunk"
made of the lactiferous duct, "branches" made of smaller ducts that feed into
the lactiferous duct, and "leaves" made of lobular tissue. Depending on the
clinical setting, surgeons will also sometimes excise the duct system under
the nipple along with the lump in the breast.

Question 4 of 6
The surgical specimen is sent fresh from the surgical suite to the laboratory for
frozen section examination. Before cutting into the specimen,
the pathologist makes a careful gross examination, which demonstrates that
most of the specimen has replacement of the normally fatty
breast tissue with strands of dense, white, firm tissue. In a few areas, roughly
spherical lesions up to 3-cm diameter with a bluish hue to them
are seen. Palpation of these areas produces a fluctuant sensation. Which of the
following is the most likely diagnosis based on the gross
evaluation?
/ A. Breast cancer
/ B. Changes of pregnancy
/ C. Fibroadenoma
/ D. Fibrocystic disease
/ E. Mastitis

Explanation - Q: 2.4 Close

The correct answer is D. While a careful pathologist will wait for appropriate
frozen or permanent tissue histologic examination, the description given in
the question stem is most suggestive of fibrocystic disease. The fibrous part
of the lesion forms the dense, white tissue strands, while the blue lesions are
what are called "blue-domed cysts. The blue color comes from the presence
of darkly colored fluid (which usually means old hemorrhage) within the cyst.
The interior cyst wall is usually smooth. These blue-domed cysts are a
favorite of examiners because they produce a distinctive gross picture and
should be specifically associated with fibrocystic disease, but you should be
aware that in real life they do not occur with anything near the frequency of
fibrocystic disease without obvious large cysts grossly (but many smaller
cysts on microscopic examination). Fibrocystic disease of the breast is a very
common lesion, and a frequent source of palpable lumps in the breast, which
may require further evaluation through either excisional biopsy (as in this
case), Tru-cut needle biopsy (producing a thin core about 1 cm long), or
needle aspiration (producing fluid or cells for cytology).
While a diagnosis of breast cancer should obviously be confirmed
microscopically before telling the clinician, invasive breast cancer (choice A)
can be suspected when there is a hard, white, nodular area of the breast that
has irregular (but often reasonably well-defined - in contrast to the fibrotic
areas of fibrocystic disease) boundaries.
The changes of pregnancy (choice B) cannot be reliably picked up on gross
examination of the breast.
Fibroadenoma (choice C), like breast cancer, tends to produce a well-
defined mass lesion, but it typically has smooth borders and may have a
slightly gray and slightly mucoid (e.g., shiny or oily appearing) surface on
cross-section.
Long-standing mastitis (choice E) can cause breast fibrosis, but will not
cause blue-domed cyst formation. More acutely, mastitis can produce
abscesses (which appear white rather than blue).
Question 5 of 6
Frozen section examination demonstrates fibrosis and cystic spaces. AIso seen
are areas of compressed glands with a lobular orientation.
The glands are lined by a single layer of epithelial cells with oval nuclei and
regular arrangement. No true invasion of glands into the adjacent
stroma is seen. This patient probably has which of the following?
/ A. Atypical ductal hyperplasia
/ B. Ductal carcinoma in situ
/ C. Lobular carcinoma in situ
/ D. Sclerosing adenosis
/ E. Usual ductal hyperplasia

Explanation - Q: 2.5 Close

The correct answer is D. The lesion described is sclerosing adenosis. The
tip-offs in the description are the references to compressed glands and
lobular orientation. Sclerosing adenosis is a common component of
fibrocystic disease, and occurs when fibrosis distorts the normal lobular
architecture. The result can be some fairly bizarre, but completely benign,
compressed glands that may mimic carcinoma on both frozen and
permanent sections. Low power examination is often helpful, as this
emphasizes the lobular character of the lesion.
Lesions actually involving the epithelium of the duct system typically have
multiple layers of cells and range from usual ductal hyperplasia (choice E,
with low risk of invasive carcinoma and characterized by the presence of
both myoepithelial cells and epithelial cells within the duct) through atypical
ductal hyperplasia (choice A, with medium risk of invasive carcinoma and
characterized by ductal carcinoma in situ-like features only involving portions
of a duct) to ductal carcinoma in situ (choice B, with relatively high risk of
invasive carcinoma and characterized by clearly abnormal features such as
loss of myoepithelial cells and formation of cribriform patterns involving
complete cross-sections of ducts).
Lobular carcinoma in situ (choice C) typically produces lobular units whose
lumina are completely filled with epithelial cells.
In practice, while you may be asked to distinguish classic examples of the
different lesions mentioned in the choices on examinations, you should be
aware that this whole area can be very problematic in real-life microscopic
examinations of breast tissue, and one piece of breast tissue sent to different
experts in breast pathology may be returned with a variety of diagnoses.


Question 6 of 6
Which of the following breast lesions is considered to have the greatest potential
for eventual progression to a malignant lesion?
/ A. Apocrine metaplasia
/ B. BIue dome cyst
/ C. Epithelial hyperplasia
/ D. Fat necrosis
/ E. Fibrosis


Explanation - Q: 2.6 Close

The correct answer is C. While fibrocystic disease may have many
components, including cyst formation (choice B), apocrine metaplasia
(choice A, a benign alteration of cyst epithelium to resemble that of apocrine
sweat glands), sclerosing adenosis, and fibrosis (choice E), only the
epithelial hyperplasia (usual, atypical, or carcinoma in situ) is thought to
indicate significant premalignant (or malignant, for carcinoma in situ)
potential. For this reason, most pathologists pay particular attention to the
epithelial lining of the ducts and lobules when evaluating breast biopsy
specimens with fibrocystic disease. Fibrocystic breasts without any evidence
of epithelial changes do not appear to have any significant increased risk of
progression to breast cancer. (You should, however, be aware that a
fibrocystic breast may make both breast palpation and mammography more
difficult and make it more likely to miss a small lesion.)
Fat necrosis (choice D) is seen after breast trauma, and has no significant
malignant potential.


A 47-year-old man presents to his physician because he has noticed that his
right breast is noticeably larger than his left breast. Breast
examination demonstrates that the right breast is diffusely enlarged. The breast
tissue is freely mobile and without distinct masses. The
patient reports that the breast has been slowly enlarging over a period of several
years.
Question 1 of 7
Which of the following is the most likely diagnosis?
/ A. Ductal carcinoma in situ
/ B. Fibroadenoma
/ C. Gynecomastia
/ D. Invasive ductal carcinoma
/ E. Lobular carcinoma in situ

Explanation - Q: 3.1 Close

The correct answer is C. The most common cause for male breast
evaluation in the United States is gynecomastia, or benign breast
enlargement. Gynecomastia may involve one or both breasts. Male breasts
can also develop ductal carcinoma in situ (choice A), invasive ductal
carcinoma (choice D), and fibroadenoma (choice B), but these lesions are
much less common than gynecomastia.
The glands in male breasts do not have lobular tissue, so lobular carcinoma
in situ (choice E) does not occur.

Question 2 of 7
AIso noted on physical examination is the presence of multiple spider angiomata.
These are most closely associated with disease of which
of the following organs?
/ A. Liver
/ B. Prostate
/ C. Stomach
/ D. Testes
/ E. Thyroid

Explanation - Q: 3.2 Close

The correct answer is A. Spider angiomas are small vascular lesions with
small blood vessels radiating from a central point. They are specifically
associated with liver disease, particularly due to alcohol abuse. They are not
specifically associated with diseases of the other organs listed in the choices.


Question 3 of 7

The presence of the spider angiomas should specifically trigger a question to the
patient about his history of use of which of the following?
/ A. AIcohol
/ B. Caffeine
/ C. Cigarettes
/ D. Heroin
/ E. Marijuana

Explanation - Q: 3.3 Close

The correct answer is A. Spider angiomas are most often associated with
liver disease related to alcohol abuse. Abuse of the other items listed in the
choices does not predispose for spider angioma formation.

Question 4 of 7
Which of the following is the most likely mechanism causing a relative excess of
hormone leading to the breast enlargement in this patient?
/ A. Decreased production of testosterone secondary to primary hypogonadism
/ B. Drug that inhibits testosterone synthesis
/ C. Drug with estrogen-Iike activity
/ D. Increased peripheral conversion of androgens to estrogens
/ E. Increased production of estrogen by a cancer

Explanation - Q: 3.4 Close

The correct answer is D. Peripheral conversion of androgens (testosterone
and androstenedione) to estrogens occurs mainly in adipose tissue, muscle,
and skin. In patients with chronic liver disease, malnutrition, and
hyperthyroidism, this peripheral conversion is increased, and may be
associated with feminization (seen as changes in hair distribution, body fat
distribution, and breast size).
Conditions that cause primary or secondary hypogonadism (choice A) can
cause gynecomastia by the mechanisms of decreased production and/or
action of testosterone. These conditions can include Klinefelter syndrome,
congenital anorchia, testicular trauma or torsion, viral orchitis (e.g., mumps),
pituitary tumors, and renal failure.
Drugs that can cause gynecomastia by inhibiting testosterone synthesis
(choice B) or action include ketoconazole, metronidazole, cisplatin,
spironolactone, and cimetidine.
Drugs that can cause gynecomastia because of their estrogen-like activity
(choice C) include diethylstilbestrol, digitalis, and estrogen-containing foods
and cosmetics.
Gynecomastia can also be seen as a consequence of increased estrogen
production by some tumors (choice E), including testicular tumors and
cancers secreting ectopic hCG (from lung, kidney, GI tract, and extragonadal
germ cell tumors). Gynecomastia can also occur as a normal physiologic
variant, particularly during puberty and in older men.

Question 5 of 7
Which of the following enzymes converts androgens to estrogens?
/ A. ALA synthase
/ B. Aromatase
/ C. Branching enzyme
/ D. MethylmalonyI CoA mutase
/ E. PRPP synthetase

Explanation - Q: 3.5 Close

The correct answer is B. The enzyme aromatase is found in adipose tissue
(and hence is increased in fat people), muscle, and skin. It acts on
androgens to form estrogens by adding double bonds to make a benzene-
like ring. It is this enzyme whose activity is relatively increased in liver
disease.
ALA synthase (choice A) is an important early enzyme in heme synthesis.
Branching enzyme (choice C) is used in glycogen formation.
Methylmalonyl CoA mutase (choice D) is involved in the propionic acid
pathway leading to synthesis of succinyl CoA.
PRPP synthetase (choice E) occurs in purine synthesis.






Question 6 of 7

Which of the following is a genetic syndrome associated with this patient's
condition and a 10- to 20-fold increased incidence of breast
cancer?
/ A. Cushing syndrome
/ B. Down syndrome
/ C. Hashimoto disease
/ D. KIinefelter syndrome
/ E. Turner syndrome

Explanation - Q: 3.6 Close

The correct answer is D. The genetic condition Klinefelter syndrome
(47,XXY) is fairly unique among the causes of gynecomastia in that it is
accompanied by an increased incidence of male breast cancer. In most
types of patients with gynecomastia, the incidence of breast cancer is not
increased, and there are no adverse medical complications of the
gynecomastia (although social and psychological problems may occur).
Gynecomastia is usually only treated (with surgery) in patients in whom the
breast either continues to enlarge or is personally troubling to the patient.
Gynecomastia may occur in Cushing syndrome (choice A) and
hyperthyroidism related to Hashimoto disease (choice C), but these are not
genetic diseases.
Gynecomastia is not usually a feature of Down syndrome (choice B), or
Turner syndrome (choice E).
Question 7 of 7
The normal male breast differs from the normal female breast in which of the
following ways?
/ A. Adipose tissue is absent
/ B. Dermis is absent
/ C. Ductal tissue is absent
/ D. Lobular tissue is absent
/ E. Muscle is absent

Explanation - Q: 3.7 Close

The correct answer is D. The normal male breast (and the male breast with
gynecomastia), unlike the normal female breast, lacks lobular tissue. Both
male and female breasts contain ductal tissue (choice C), adipose tissue
(choice A), dermis (choice B), and small amounts of smooth muscle tissue
(choice E).


A 53-year-old woman consults a physician after discovering a mass in her breast.
Physical examination demonstrates a 1.5-cm diameter,
firm mass lesion in the upper, outer quadrant of her right breast. The mass is
surgically removed and pathologic examination of tissue
obtained at surgery reveals invasive breast cancer.

Question 1 of 5

What percentage of breast masses are discovered by the patient, rather than by
mammography or physician examination of the breasts?
/ A. 5%
/ B. 20%
/ C. 50%
/ D. 80%
/ E. 95%

Explanation - Q: 1.1 Close

The correct answer is D. Breast cancer accounts for the greatest number of
new cancer cases in women each year. Mammography is the screening
method used to detect subclinical breast cancer-the stage at which breast
cancer is least likely to have spread, but about 80% of breast masses are
discovered initially by the patient, which it is why it is important to continue to
stress breast self-examination.


Question 2 of 5

While about 75% of the lymphatic fluid from the breast drains first to the axilla,
most of the remaining lymphatic fluid drains first to which of the
following groups of lymph nodes?
/ A. Anterior internal thoracic nodes
/ B. Internal inferior thoracic nodes
/ C. Lateral intercostal nodes
/ D. Superior mediastinal nodes
/ E. Tracheobronchial nodes

Explanation - Q: 1.2 Close

The correct answer is A. The anterior internal thoracic nodes, also known
as the internal mammary nodes, are a pair of chained lymph nodes running
superiorly to inferiorly along the chest wall near both sides of the sternum.
They are inaccessible for surgical removal during mastectomy, but may
contain metastatic breast cancer. Rarely, the lateral intercostal nodes
(choice C) may contain metastatic breast cancer.
The inferior internal thoracic nodes (choice B) drain the liver and diaphragm.
The superior mediastinal nodes (choice D) drain the trachea, esophagus,
and heart.
The tracheobronchial nodes (choice E) drain the lung.
Question 3 of 5
Which of the following will most likely be identified at pathologic examination of
this woman's breast mass?
/ A. Ductal carcinoma
/ B. Lobular carcinoma
/ C. Medullary carcinoma
/ D. Paget disease
/ E. Tubular carcinoma

Explanation - Q: 1.2 Close

The correct answer is A. The anterior internal thoracic nodes, also known
as the internal mammary nodes, are a pair of chained lymph nodes running
superiorly to inferiorly along the chest wall near both sides of the sternum.
They are inaccessible for surgical removal during mastectomy, but may
contain metastatic breast cancer. Rarely, the lateral intercostal nodes
(choice C) may contain metastatic breast cancer.
The inferior internal thoracic nodes (choice B) drain the liver and diaphragm.
The superior mediastinal nodes (choice D) drain the trachea, esophagus,
and heart.
The tracheobronchial nodes (choice E) drain the lung.
Question 3 of 5
Which of the following will most likely be identified at pathologic examination of
this woman's breast mass?
/ A. Ductal carcinoma
/ B. Lobular carcinoma
/ C. Medullary carcinoma
/ D. Paget disease
/ E. Tubular carcinoma

Explanation - Q: 1.3 Close

The correct answer is A. There is a cumulative risk in women of developing
breast cancer of 1 in 8 by age 95; 1/3 to 1/2 of the patients die of the
disease. Breast cancer may occur in a ductal or a lobular pattern. Invasive
ductal carcinoma, not otherwise specified, is the most common histological
type of invasive breast cancer. Risk factors for breast cancer include positive
family history, early menarche, late menopause, late first pregnancy, and
history of in situ or invasive breast cancer. Women who develop breast
cancer before age 35 tend to have more aggressive disease. Two breast
cancer genes, BRCA1 and BRCA2, have been identified; 5% of women with
breast cancer carry one or the other of these genes. Male breast cancer is
much less common than female, but has a high mortality rate
Lobular carcinoma (choice B) is the second most common type of breast
cancer. It may occur bilaterally in some patients.
Medullary carcinoma (choice C) and tubular carcinoma (choice E) are
histological variants of ductal carcinoma with statistically better prognoses
than ductal carcinoma, not otherwise specified.
Paget disease (choice D) is the presence of individual adenocarcinoma cells
within the skin overlying a breast cancer.

Question 4 of 5
Immunohistochemical examination of paraffin-embedded sections through the
tumor demonstrates that it stains for estrogen receptors (ER)
and progesterone receptors (PR). This finding specifically suggests that the
tumor may respond to which of the following drugs?
/ A. 5-FIuorouracil
/ B. Cyclophosphamide
/ C. Doxorubicin
/ D. Methotrexate
/ E. Tamoxifen

Explanation - Q: 1.4 Close

The correct answer is E. Treatment with adjuvant tamoxifen for 5 years in
ER positive tumors can reduce the risk of death by 25% in both pre- and
postmenopausal women with or without axial lymph node involvement.
Breast cancers that express the Her-2/neu receptor may respond to a new
drug, trastuzumab, which is a monoclonal antibody directed against the
receptor. The other drugs listed are all used for breast cancer chemotherapy,
but work because they are cytotoxic rather than because they affect the
hormonal response of the cancer.


Question 5 of 5

The patient's physician suggests that she immediately begin chemotherapy
treatment. The patient dismisses his suggestion and says, "I do
not need any medicine, all l need is bedrest." This statement is most consistent
with which of the following responses to illness?
/ A. Acceptance
/ B. Anger
/ C. Bargaining
/ D. Denial
/ E. Grieving

Explanation - Q: 1.5 Close

The correct answer is D. Denial is a coping mechanism to defend against
overwhelming anxiety. Pathologic and extreme denial can interfere with
accurate diagnosis, impede treatment, and consequently perpetuate the
disease state. Denial is common in the early stages of dealing with a terminal
illness and is not necessarily pathologic. Less extreme forms of denial may
even serve the patient in positive ways. "I'm as strong now as I was when I
was 20 and I'm gonna make it." Notably, the stages of grieving over the loss
of a loved one (bereavement) are very similar to grieving over the loss of
one's health (stages of dying).
Acceptance (choice A) is a realistic perspective concerning the
consequences of illness. "Coming to terms" with the illness restores
emotional equilibrium and patients appear to return to their baseline
personality and emotional functioning.
Anger (choice B) is often directed at fate, God, themselves, their caretakers,
and their families and, if taken to the extreme, may result in isolation from
much needed support.
Bargaining (choice C) entails promises to buy additional time.
Grieving (choice E) is a process of changing affective states over time and
includes five stages as described by Elisabeth Kubler-Ross (denial, anger,
bargaining, depression, and acceptance). Denial, anger, anxiety, depression,
and dependence can all be abnormal responses to illness (when extreme).


A 23-year-old woman comes to the physician because of a lump in her right
breast. She states that she first noted the lump about a year ago
and that it has seemed to enlarge over the past year. She notes some occasional
tenderness in the area, usually at the same time during her
menstrual cycle. She has no medical problems. She had an appendectomy at the
age of 18. She takes no medications and is allergic to
penicillin. Examination of the breast demonstrates a freely mobile, smoothly
contoured, discrete mass in the upper outer quadrant of the
breast. UItrasonography demonstrates a smooth mass with circumscribed
margins and homogeneous echo pattern, consistent with a solid
Iesion.

Question 1 of 4

Which of the following is the most likely diagnosis?
/ A. Breast abscess
/ B. Fibroadenoma
/ C. Fibrocystic breast changes
/ D. Mastitis
/ E. Pregnancy

Explanation - Q: 2.1 Close

The correct answer is B. Fibroadenomas are the most common breast
lesions found in women under 25 years of age. Fibroadenomas are the
second most common cause of benign breast lesions (second only to
fibrocystic changes) in women of all ages. Patients with a fibroadenoma
typically present complaining of a palpable lump, often with some gradual
growth. There may be some occasional cyclic tenderness. Management is
with biopsy or close observation. If the lesion is palpable, increasing in size,
or psychologically disturbing, biopsy should be performed. If the woman is
less than 25 years of age with small fibroadenomas that appear "classic" by
imaging, then expectant management with careful continued observation can
be considered.
A breast abscess (choice A) can also present as a lump in the breast.
However, an abscess represents a localized collection of pus resulting from
an infection. Therefore, patients with a breast abscess will often have
erythema, edema, pain, and tenderness around the area of the mass. Such
patients may also have systemic signs of infection, including fever and
tachycardia. This patient has no evidence of infection.
Fibrocystic breast changes (choice C) are the most common, benign
condition of the breast. They can be present in young women, become more
common as a woman approaches the menopause, and often regress during
and after the menopause. The most common symptoms are pain and
tenderness, and the masses are usually bilateral. Mammography and
ultrasound of the breast often reveal the fibrocystic changes.
Mastitis (choice D) is an infection of the breast. It can occur in any woman,
but most often occurs in lactating women during the postpartum period.
Patients with mastitis will often present with tenderness and erythema of the
breast along with fever. Treatment is with antibiotics.
Pregnancy (choice E) is associated with a number of changes in the breast,
especially as the breast prepares for lactation. Fibroadenomas may grow
rapidly during pregnancy, but the primary diagnosis, and hence the best
answer, is still fibroadenoma

Question 2 of 4

Histological examination of diagnostic tissue from this patient would reveal which
of the following?
/ A. A classic cribriform pattern with neoplastic epithelial cells
/ B. Cystically dilated ducts plus stromal fibrosis
/ C. Irregular steatocytes and intervening necrotic material and inflammatory
cells
/ D. Lobular hypertrophy
/ E. Proliferating ducts and stromal cells

Explanation - Q: 2.2 Close

The correct answer is E. Fibroadenomas have a typical microscopic
appearance. The predominant feature is the fibroblastic stroma. This is a
delicate, cellular, fibroblastic stroma resembling intralobular stroma. Within
this fibroblastic stroma are seen proliferating ducts. These ducts are usually
compressed and are lined by benign-appearing epithelium. If the margin
includes surrounding tissue beyond the fibroadenoma, compressed breast
connective tissue forming a "capsule" to the mass may be seen as well.
A classic cribriform pattern with neoplastic epithelial cells (choice A) is what
would be revealed by histological examination of an intraductal carcinoma of
the breast. The epithelium in a fibroadenoma is benign-appearing.
Histologic examination of a biopsy specimen from a patient with fibrocystic
breast changes would demonstrate cystically dilated ducts plus stromal
fibrosis (choice B).
Irregular steatocytes and intervening necrotic material and inflammatory cells
(choice C) describes the findings on pathologic evaluation of a biopsy
specimen from a patient with fat necrosis. Fat necrosis is most commonly
caused by trauma, but can also occur after surgery or radiation therapy.
Lobular hypertrophy (choice D) is seen in pregnant women. This lobular
hypertrophy occurs during the pregnancy to allow for lactation in the
postpartum period.

Question 3 of 4
If the histologic examination revealed similar findings as in this patient, but
demonstrated increased cellularity, an elevated mitotic rate,
stromal overgrowth, and infiltrative borders, then which of the following is the
most likely diagnosis?
/ A. Fat necrosis
/ B. Fibrocystic changes
/ C. Mastitis
/ D. Normal breast tissue
/ E. Phyllodes tumor

Explanation - Q: 2.2 Close

The correct answer is E. Fibroadenomas have a typical microscopic
appearance. The predominant feature is the fibroblastic stroma. This is a
delicate, cellular, fibroblastic stroma resembling intralobular stroma. Within
this fibroblastic stroma are seen proliferating ducts. These ducts are usually
compressed and are lined by benign-appearing epithelium. If the margin
includes surrounding tissue beyond the fibroadenoma, compressed breast
connective tissue forming a "capsule" to the mass may be seen as well.
A classic cribriform pattern with neoplastic epithelial cells (choice A) is what
would be revealed by histological examination of an intraductal carcinoma of
the breast. The epithelium in a fibroadenoma is benign-appearing.
Histologic examination of a biopsy specimen from a patient with fibrocystic
breast changes would demonstrate cystically dilated ducts plus stromal
fibrosis (choice B).
Irregular steatocytes and intervening necrotic material and inflammatory cells
(choice C) describes the findings on pathologic evaluation of a biopsy
specimen from a patient with fat necrosis. Fat necrosis is most commonly
caused by trauma, but can also occur after surgery or radiation therapy.
Lobular hypertrophy (choice D) is seen in pregnant women. This lobular
hypertrophy occurs during the pregnancy to allow for lactation in the
postpartum period.

Question 3 of 4
If the histologic examination revealed similar findings as in this patient, but
demonstrated increased cellularity, an elevated mitotic rate,
stromal overgrowth, and infiltrative borders, then which of the following is the
most likely diagnosis?
/ A. Fat necrosis
/ B. Fibrocystic changes
/ C. Mastitis
/ D. Normal breast tissue
/ E. Phyllodes tumor

Explanation - Q: 2.3 Close

The correct answer is E. Phyllodes tumors are similar to fibroadenomas in
that they arise from intralobular stroma. Furthermore, on pathologic
evaluation, low-grade phyllodes tumors can resemble fibroadenomas.
However, there are important differences. First, most phyllodes tumors
present in the sixth decade, whereas fibroadenomas most commonly present
in young women. Also, while most phyllodes tumors are low-grade tumors
that only rarely metastasize, some are aggressive high-grade lesions that
commonly recur locally and do metastasize hematogenously. These
aggressive lesions are often called cystosarcoma phyllodes. Some phyllodes
tumors are small, while others may be large enough to involve virtually the
entire breast. Grossly, these tumors often have leaf-like projections off of
them. On histologic evaluation, the keys to distinguishing between
fibroadenoma and phyllodes tumor are the increased cellularity, enhanced
mitotic rate, stromal overgrowth, nuclear pleomorphism, and infiltrative
borders that are seen in phyllodes tumors and are absent in fibroadenomas.
Fat necrosis (choice A) demonstrates necrotic fat cells that are surrounded
by lipid-filled macrophages and an infiltration of neutrophils. It does not
resemble a fibroadenoma.
Fibrocystic changes (choice B) are characterized by cysts, and do not
closely resemble fibroadenomas, as low-grade phyllodes tumors do.
Mastitis (choice C) is an infection of the breast, usually by Staphylococcus
aureus. It is characterized by acute inflammation and does not resemble
fibroadenoma.
Normal breast tissue (choice D) is not characterized by a pattern similar to
fibroadenoma, except with increased cellularity, elevated mitotic rate, stromal
overgrowth, and infiltrative borders. These are characteristics of phyllodes
tumors.

Question 4 of 4
If the patient were instead found to have an aggressive breast carcinoma with a
poor prognosis, the Kubler-Ross model predicts that she will
go through which of the following stages?
/ A. Acceptance, anger, ambivalence, deniaI, depression
/ B. BIues, depression, psychosis, treatment, resolution
/ C. DeniaI, anger, bargaining, depression, acceptance
/ D. DeniaI, anger, psychosis, homicide, suicide
/ E. Realization, infantilization, socialization, condemnation

Explanation - Q: 2.4 Close

The correct answer is C. Elisabeth Kubler-Ross is the psychiatrist who
authored the groundbreaking "On Death and Dying" in 1969. She was born in
Switzerland, but moved to the United States in the 1950s, where she worked
with dying patients. Her model identified five stages that occur when
individuals are confronted with death: 1) Denial, 2) Anger, 3) Bargaining, 4)
Depression, and 5) Acceptance. While she originally described this process
as it relates to facing death, others have used these 5 steps to describe
reaction to grief or loss. While these stages are useful to understand and
contemplate the experience of grief or dying, it is important to recognize that
not all people will go through these steps. Those who do go through each of
these steps may also not go through them in the "order" described by the
Kubler-Ross model. No model is perfect for explaining the intricacies of every
different human being's response to grief or death. The Kubler-Ross model
does provide a nice framework and starting point for understanding these
difficult issues.
None of the other choices correctly describes the stages proposed by
Kubler-Ross.


A 45-year-old woman presents to her primary care physician complaining of
fatigue, weight gain, and shortness of breath. She has always
been an active athlete, but in the past 2 weeks, has found it impossible to jog for
more than a few minutes, after which she feels tired and
winded. She feels like her appetite is normal or has even declined, but she
notices that she has gained 15 pounds and her pants and shoes
no longer fit welI. She has very little energy, and is sleeping poorly, with
occasional difficulty breathing at night. She denies any pain, fever, or
chills. Review of her chart reveals an up-to-date health screening including a
normal baseline mammogram, a normaI Pap smear in the last
year, and total cholesterol of 165 mg/dL two years ago. On physical examination,
she appears comfortable, has a temperature of 36.8 C
(98.2. F), blood pressure of 135/68 mm Hg, pulse of 90/min, and respiratory rate
of 24/min. She appears fatigued but not in acute distress,
and her skin appears normaI. Expiratory wheezes are heard at the bases of both
lungs. Her heart has a normaI-sounding S1 and S2, with a
II/IV soft holosystolic murmur heard best at the apex of the heart. Her abdomen
is modestly distended, and her ankles are edematous. A
chest x-ray film reveals cardiomegaly as well as increased vascular markings in
the lung beds and bilateral small pleural effusions.
Laboratory studies show:
Question 1 of 5
Which of the following is the most likely diagnosis?
/ A. Acute leukemia
/ B. Cardiomyopathy
/ C. Fibromyalgia
/ D. Hypothyroidism
/ E. Major depressive disorder


Explanation - Q: 1.1 Close

The correct answer is B. This woman has many of the classic symptoms of
heart failure, with symptoms of both poor forward cardiac output (fatigue,
poor appetite) and of vascular congestion in both the right and left atria
(edema, abdominal distension that may be ascites, cardiomegaly, pulmonary
vascular congestion and effusions seen on chest x-ray, dyspnea with
exertion, and paroxysmal nocturnal dyspnea.)
Acute leukemia (choice A) is a potential cause of fatigue, poor energy, and
poor nutritional status (which can cause edema and pleural effusion). Usually
some abnormality will be apparent, most commonly pancytopenia, due to
replacement of bone marrow with leukemic cells; the leukocyte count may be
elevated due to the presence of leukemic cells in the peripheral blood. They
often present with bleeding or infectious complications of pancytopenia.
Anemia could potentially cause a murmur due to elevated cardiac output, but
an acute leukemia would not typically cause cardiomegaly or pulmonary
edema.
Fibromyalgia (choice C) is a potential cause of fatigue, poor energy, and
poor sleep, especially in women ages 25-45: its principal sign, however, is
diffuse musculoskeletal pain and stiffness, with characteristic tender trigger
points. It is not consistent with this patient's chest x-ray abnormalities or
cardiac and lung findings.
Based on examination, this patient could certainly have hypothyroidism
(choice D). Symptoms are usually insidious in onset and include fatigue,
poor appetite with weight gain, poor sleep and possibly, obstructive sleep
apnea. Patients often complain of constipation, cold intolerance, stiffness
and muscle cramping, as well as decreased intellectual activity. Severe
hypothyroidism can result in cardiomegaly, pericardial effusion, and
symptoms of cardiac failure. The skin often appears dry, rough, and doughy
in texture. The normal TSH, however, makes hypothyroidism in this patient
very unlikely: The TSH is nearly always elevated, as most hypothyroidism is
primary, which means the pituitary is secreting maximal TSH in an attempt to
stimulate a hypofunctional thyroid gland. Rarely, TSH may be normal or
depressed (even undetectable) in pituitary or hypothalamic failure. To rule
this out, one might test first for T4 and T3 levels. Normal levels of these, in
conjunction with the normal TSH, would rule out hypothyroidism as a cause
of this clinical presentation.
Major depression (choice E) should always be in the differential for a patient
who presents with disturbances in sleep, appetite, and energy, and can also
result in weight loss or gain. These "vegetative signs" of depression may be
the presenting abnormality in a depressed patient who does not note a mood
disturbance themselves. One should also ask about depressed mood,
anhedonia (loss of interest in or inability to take pleasure in activities the
person normally enjoys), an inability to concentrate and carry on usual
intellectual activities, feelings of worthlessness or guilt, and suicidal ideation.
Depression cannot, however, on its own, produce the physical findings this
patient has, which taken together, are worrisome for some physiologic
abnormality.
Question 2 of 5
Which of the following is the most likely cause of the patient's murmur?
/ A. Aortic insufficiency
/ B. Aortic stenosis
/ C. High-output flow murmur
/ D. Mitral regurgitation
/ E. Mitral stenosis
/ F. Pulmonic insufficiency
/ G. Pulmonic stenosis
/ H. Tricuspid regurgitation
/ I. Tricuspid stenosis


Explanation - Q: 1.2 Close

The correct answer is D. Mitral regurgitation is characterized by a
holosystolic murmur heard best at the apex, often with a blowing sound,
which may radiate to the axilla.
The murmur of aortic insufficiency (choice A) is a decrescendo diastolic
murmur. Remember that the aortic valve is open during systole; a systolic
murmur, then, cannot represent regurgitant aortic flow due to an improperly
closed valve.
Aortic stenosis (choice B) does produce a systolic murmur caused by
turbulent flow across a narrowed aortic valve during systole. This murmur is
usually a crescendo-decrescendo murmur, often with a harsh quality, and is
characteristically heard best at the base of the heart; it may radiate to the
carotids as well.
High-output states (choice C) can cause a similar soft systolic murmur to
that described here. However, this patient's history is most consistent with
cardiac failure, which is a low-output state.
Mitral stenosis (choice E) causes a murmur due to turbulent low-velocity flow
during diastolic filling of the left ventricle through a narrowed mitral orifice.
This results in a soft diastolic murmur heard best at the apex. Remember that
the mitral valve is closed during systole, therefore, an abnormal mitral sound
in systole must be the sound of abnormal regurgitant flow through a closed
valve.
The right-sided murmurs are less common, similar in quality, and usually less
loud than the left-sided murmurs (given that pressures on the right are
usually lower):
Pulmonic insufficiency (choice F), when audible, therefore causes a soft
diastolic murmur at the right upper sternal border.
Pulmonic stenosis (choice G) causes a crescendo-decrescendo systolic
murmur also heard at the base of the heart.
Tricuspid regurgitation (choice H) causes a holosystolic murmur at the left
lower or right lower sternal border.
Tricuspid stenosis (choice I) when audible, is a diastolic murmur heard best
at the same location
Question 3 of 5
BIood in the pulmonary veins is at the same pressure (during all phases of the
cardiac cycle) as blood in which of the following?
/ A. Aorta
/ B. Left atrium
/ C. Left ventricle
/ D. Right atrium
/ E. Right ventricle

Explanation - Q: 1.3 Close

The correct answer is B. The pressures in two chambers, which are not
separated by a closed valve, will be equal. The pulmonary vein empties into
the left atrium, and no valve separates the two chambers, therefore the
pressures are equal in all phases of the cardiac cycle. This patient's
pulmonary vascular congestion is likely due to elevated pulmonary venous
pressure, which is, in turn, likely due to elevated left atrial pressures.
Pressures in the aorta (choice A) will be higher than pressures in the
pulmonary veins during the cardiac cycle.
The left ventricle (choice C) is separated from the left atrium and the
pulmonary veins by the mitral valve. The pulmonary veins and the left atrium
are at the same pressure as the left ventricle during diastole, when the mitral
valve is open. With complete mitral insufficiency, the pulmonary veins are
completely exposed to left ventricular pressures during systole, resulting in
severe pulmonary edema.
The right atrium (choice D) is not in communication with the pulmonary
veins, being separated from them by, in sequence, the tricuspid valve, the
right ventricle, the pulmonic valve, the pulmonary arterial system, and the
pulmonary capillary bed.
The right ventricle (choice E), during systole, is at the same pressure as the
pulmonary artery, not the pulmonary veins. During diastole, the pulmonary
arterial pressure exceeds right ventricular pressure, and the valve is closed.


Question 4 of 5
To improve her shortness of breath, the patient is given furosemide. What is the
molecular mechanism and site of action of this drug?
/ A. ADH antagonism of in the collecting ducts
/ B. AIdosterone antagonism in the distal tubule
/ C. BIockade of sodium reabsorption in the proximal tubule
/ D. BIockade of sodium transport in the distal tubule
/ E. Inhibition of carbonic anhydrase in the proximal tubule
/ F. Inhibition of sodium-potassium-chloride cotransport in the loop of Henle

Explanation - Q: 1.4 Close

The correct answer is F. The Na-K-2Cl cotransporter in the loop of Henle
operates via an ATP-dependent sodium-potassium exchange pump in the
cell that creates a gradient for sodium diffusion from the urine space into the
cell. This maintains the sodium concentration gradient of the renal medulla.
Furosemide is the most commonly used loop diuretic; it acts by blocking the
action of the cotransporter in the thick ascending limb of the loop of Henle.
ADH antagonism (choice A) is not an important diuretic drug mechanism,
however, certain drugs, most notably lithium, inhibit ADH's action, resulting in
nephrogenic diabetes insipidus.
Aldosterone promotes the reabsorption of sodium in the late distal tubule and
collecting system and promotes the excretion of potassium. Aldosterone
receptor antagonism (choice B) is the mechanism of action of potassium-
sparing diuretics such as spironolactone.
Sodium reabsorption in the proximal tubule (choice C) is a largely passive
process, which is coupled to the transport of organic solutes and anions and
also to chloride transport, via both transcellular and paracellular
mechanisms.
The thiazide diuretics work primarily by blocking sodium transport in the early
portion of the distal tubule (choice D).
Acetazolamide inhibits carbonic anhydrase (choice E), preventing the
luminal transformation of bicarbonate into CO
2
, which diffuses back into the
cell. Inhibition of this enzyme increases both bicarbonate and sodium
concentrations in the urine, resulting in high urine pH and metabolic acidosis.
Question 5 of 5
What important physiologic effect will starting this patient on an angiotensin-
converting-enzyme inhibitor achieve?
/ A. Decrease in arteriolar resistance, resulting in less resistance to forward
cardiac output
/ B. Decrease in cardiac filling pressures, resulting in less pulmonary congestion
/ C. Increase in arteriolar resistance, resulting in improved blood pressure
/ D. Increase in left-ventricular end-diastolic volume, improving stroke volume
via Starling forces
/ E. Increase in myocardial contractility, resulting in improved stroke volume
/ F. Stabilization of myocardial membranes, resulting in reduced risk of
arrhythmia
Explanation - Q: 1.5 Close

The correct answer is A. In cardiac failure, the juxtaglomerular apparatus
releases renin in response to low blood pressure or low flow states. Renin
cleaves angiotensinogen into angiotensin I, which is then cleaved by
angiotensin-converting enzyme (ACE) into angiotensin II. Angiotensin II is a
potent vasoconstrictor and increases blood pressure. This, however,
increases the resistance against which the heart must pump, thereby
reducing cardiac output. By reducing angiotensin II activity, systemic
vascular resistance (normally high in cardiac failure, in an attempt to maintain
blood pressure in the presence of low flow) is reduced, permitting the heart
to eject more volume against a lower aortic pressure. This is often described
as "afterload reduction" and is the mainstay of therapy in congestive heart
failure. Paradoxically, blood pressure may not change: the reduced
resistance, by permitting increased flow, may result in no net change in
pressure. This is most easily understood as a physiologic manifestation of
Ohm's law: V = IR. In electricity, this law means that voltage is equal to
current times resistance. Blood pressure is analogous to voltage, cardiac
output to current flow, and the resistance in this case is the resistance of the
systemic vasculature.
Reduction of cardiac filling pressures (choice B) or "preload," is also an
important aspect of the treatment of heart failure. In heart failure, the heart
operates at high filling pressures and high left ventricular end-diastolic
volume (LVEDV) because both aldosterone and ADH promote the retention
of fluid in response to low forward flow and decreased effective circulating
volume. The result is vascular congestion in the pulmonary veins due to the
high LV diastolic pressure, resulting in symptomatic pulmonary edema. By
reducing this preload, congestive symptoms can be relieved, and LVEDV
can be reduced without significant loss of stroke volume. ACE inhibitors,
however, do not reduce preload: drugs that do this are nitrates (which act as
venodilators) and diuretics.
Increasing arteriolar resistance (choice C) in heart failure increases the
"afterload" against which the heart must eject and does not improve cardiac
output.
Increasing LVEDV (choice D) is usually helpful in hypovolemia or other
states in which inadequate volume is available to the heart, thereby limiting
cardiac output. This happens in the portion of the Starling curve at low
LVEDV, where an increase in LVEDV results in a large increase in stroke
volume. Patients in symptomatic heart failure like this patient operate at very
high LVEDV and benefit from its reduction.
Increasing myocardial contractility (choice E) is beneficial in heart failure,
and is the mechanism of action of inotropic drugs. This is not a mechanism of
ACE inhibitors.
Prevention of arrhythmia (choice F) is also important in heart failure, as the
dilated heart is vulnerable to both atrial and ventricular arrhythmias. This is
not a direct action of ACE inhibitors, however.

A 23-year-old man presents to the urgent care clinic complaining of severe throat
pain, fever, chills, and diffuse joint pains. He first developed
symptoms two weeks ago and was evaluated by another physician at the same
clinic. A throat culture was done, and the patient was given a
prescription for antibiotics that he did not filI. He now returns with a worsening of
his symptoms. He has since developed severe joint pain and
swelling, which first affected his right wrist, then spread to both knees, and now
has also affected his left ankle. He also complains of
moderate to severe chest discomfort and shortness of breath. His temperature is
38.7 C (101.6 F), blood pressure is 118/86 mm Hg, pulse
is 104/min, and respirations are 20/min. There is an exudate on his oropharynx
and bilateral anterior cervical lymphadenopathy. On lung
examination, there are bibasilar crackles, and the cardiac examination reveals
tachycardia, but a normal rhythm and no murmurs or rubs.
Examination of his joints reveals synovitis in his right wrist, Ieft ankle, and both
knees. A c `. ... . ... 1 .. . .
;.. 1 `....`.``-..--.....`..```..`-.-`.--.`
Question 1 of 5
:
Which of the following is the most likely cause of this patient's cardiac findings?
/ A. Acute myocardial infarction
/ B. Aortic dissection
/ C. Mitral regurgitation
/ D. Myocarditis
/ E. Wolff-Parkinson-White (WPW) syndrome

Explanation - Q: 2.1 Close

The correct answer is D. The patient has myocarditis, which is an
inflammation of the cardiac muscle. It is most commonly the result of an
infectious process. Signs and symptoms can range from an asymptomatic
state to arrhythmias, heart failure, and death. The patient often has an
antecedent infection, and in this case, he had an exudative pharyngitis.
Acute myocardial infarction (choice A) usually presents with severe
squeezing left-sided chest pain that can radiate down the left arm. Patients
are generally middle-aged, and can have risk factors for cardiac disease
such as hypertension, diabetes, hypercholesterolemia, or a history of
tobacco use. The electrocardiogram can vary from nonspecific T wave
changes to ST segment elevation.
Aortic dissection (choice B) would present as sudden onset of severe chest
pain, which often radiates to the back. Patients can have hypotension,
depending on the severity of the dissection, or hypertension, which is often a
predisposing factor. Patients can also have unequal pulses in their
extremities, if the dissection affects one of the major arteries branching off
the aortic arch.
Mitral regurgitation (choice C) is a result of mitral valve insufficiency, in
which there is a regurgitant flow of blood across the mitral valve, from the left
ventricle, into the left atrium, during systole. It is often due to rheumatic heart
disease, but can also result from mitral valve prolapse, or papillary muscle
rupture. Physical examination should reveal a holosystolic murmur, heard
best at the apex.
Wolff-Parkinson-White (WPW) syndrome (choice E) is a ventricular
preexcitation syndrome associated with an atrioventricular bypass track.
Patients often have paroxysmal tachycardias, and an electrocardiogram will
often reveal a shortened PR interval, a delta wave, and a wide QRS
complex.
Question 2 of 5
What underlying condition can explain the patient's upper respiratory as well as
cardiac and joint signs and symptoms?
/ A. Acute rheumatic fever
/ B. Budd-Chiari syndrome
/ C. Ebstein's anomaly
/ D. Sjgren syndrome
/ E. Takayasu arteritis

Explanation - Q: 2.2 Close

The correct answer is A. Acute rheumatic fever is an inflammatory disorder
that affects multiple systems. There are five major criteria for rheumatic
fever: carditis, migratory polyarthritis, subcutaneous nodules, Sydenham
chorea, and erythema marginatum. There are also minor criteria: fever,
arthralgia, elevated acute phase reactants, and a prolonged PR interval.
Budd-Chiari syndrome (choice B) is an occlusion of the major hepatic veins,
which leads to congestive liver disease. Patients often have abdominal pain,
jaundice, and hepatomegaly.
Ebstein's anomaly (choice C) is due to an anomalous attachment of the
tricuspid leaflets, and results in downward displacement of the tricuspid valve
into the right ventricle. This results in tricuspid regurgitation. Symptoms can
vary from cyanosis to arrhythmias.
Sjgren syndrome (choice D) is an autoimmune disorder characterized by
inflammatory changes in glands, producing dry eyes and dry mouth.
Takayasu arteritis (choice E) is a vasculitis syndrome that affects medium to
large arteries, in particular, the aortic arch and its branches. It is also known
as "pulseless disease" because patients have weak or absent pulses in their
upper extremities. It primarily affects young Asian females.

Question 3 of 5
Which of the following test results would help confirm the most likely diagnosis?
/ A. EIevated antinuclear antibody
/ B. Low anti-deoxyribonuclease B titer
/ C. Low anti-hyaluronidase titer
/ D. Low anti-streptolysin O titer
/ E. Throat culture positive for group A streptococci

Explanation - Q: 2.3 Close

The correct answer is E. To meet criteria for the diagnosis of rheumatic
fever, patients must have either two major, or 1 major and 2 minor criteria,
plus evidence of an antecedent streptococcal infection. A throat culture
positive for group A streptococci would fulfill the criteria in the presence of
myocarditis and migratory polyarthritis.
Elevated antinuclear antibody (choice A) is not associated with rheumatic
fever. In the appropriate clinical setting, it is helpful in the diagnosis of
rheumatologic disorders such as systemic lupus erythematous.
Anti-streptolysin O (choice D), anti-deoxyribonuclease B (choice B), and
anti-hyaluronidase (choice C) are all streptococcal antibody tests. In the
setting of rheumatic fever associated with a recent group A streptococcal
infection, the titers for these antibody tests would be elevated (in the
absence of infection, they may actually be undetectable). A significant titer of
any of these antibody tests would meet criteria for the documentation of an
antecedent streptococcal infection.
Question 4 of 5
A biopsy of the affected cardiac tissue would most likely show which of the
following?
/ A. Angiosarcoma
/ B. Aschoff body
/ C. Atheromas
/ D. Hyperplastic arteriolosclerosis
/ E. Libman-Sacks lesions
Explanation - Q: 2.4 Close

The correct answer is B. The Aschoff body is the classic lesion of
rheumatic fever. It is an area of focal interstitial myocardial inflammation. It is
characterized by large cells, known as Anitschkow myocytes, and Aschoff
cells, which are multinucleated giant cells.
Angiosarcoma (choice A), a rare malignant tumor affecting the vascular
tissue, can occur in the skin, breast, liver, or musculoskeletal system.
Atheromas (choice C) are fibrous plaques within the intima of arteries. They
are a finding of atherosclerosis.
Hyperplastic arteriolosclerosis (choice D) is characterized by concentric,
laminated thickening of arteriolar walls. It often occurs in the kidneys, and
may lead to malignant nephrosclerosis.
Libman-Sacks lesions (choice E) are small vegetations that occur on
valvular heart tissue. They can occur on either side of the valve, and are
associated with endocarditis in systemic lupus erythematous.
uest|on 0
The patient continues to deteriorate, he develops worsening heart failure, and
requires transfer to the intensive care unit for use of an
inotropic agent to increase his cardiac output. Which of the following agents
would most likely be used?
/ A. Benazepril
/ B. Diltiazem
/ C. Dobutamine
/ D. Metoprolol
/ E. Phenylephrine


Explanation - Q: 2.5 Close

The correct answer is C. Dobutamine is a positive inotropic agent used in
severe cases of heart failure that require inotropic support.
Benazepril (choice A) is an angiotensin converting enzyme inhibitor.
Medications in this class can be used in heart failure to decrease afterload,
but they do not have any direct affect on cardiac tissue.
Diltiazem (choice B) and metoprolol (choice D) are both negative inotropic
agents. When used in the setting of acute heart failure, the patient's course
can worsen, although beta blockers such as metoprolol and carvedilol (mixed
alpha and beta blocker) are sometimes cautiously used in some patients with
CHF.
Phenylephrine (choice E) is an alpha-receptor agonist. It causes
vasoconstriction, and is used in severe cases of hypotension.

A 78-year-old man had been previously active, but found that his health was
declining. Over a four-month period, his ability to perform even
very minimal exercise, such as walking around his yard, declined precipitously.
The family took him from doctor to doctor, none of whom were
initially able to figure out what was wrong with him. Because of the patient's age,
most of the physicians that the family consulted were
unwilling to do much other than to listen to the family's story and then run a few
screening tests. In some ways, he acted as if he were in
congestive heart failure, but he initially had no evidence of fluid overload and his
lungs were clear. The cardiac profile on chest X-ray was
slightly enlarged. His ECG studies were interpreted as within the normal range
for his age. Angiography studies showed no evidence of
significant coronary artery occlusion. Pulmonary function studies were
unrevealing.
Question 1 of 6
Following a Thanksgiving meaI, the patient's condition worsened markedly over
the next few hours, and he was taken to an emergency
department. At that point, the patient was in obvious, severe, congestive heart
failure with evidence of fluid overload and pulmonary edema.
Intravenous furosemide was started, which over the next few hours markedly
improved his clinical condition. Furosemide is classified as
which of the following?
/ A. Carbonic anhydrase inhibitor
/ B. Loop diuretic
/ C. Osmotic diuretic
/ D. Potassium-sparing diuretic
/ E. Thiazide diuretic

Explanation - Q: 3.1 Close

The correct answer is B. Large, salty, holiday meals are notorious for
setting off (potentially fatal) exacerbations of what might have been
previously mild congestive failure. There are a number of drugs with diuretic
activity that can increase the amount of urine that is produced.
Pharmacologists subclassify these drugs based on the mechanisms by
which they act. Furosemide is a diuretic that is commonly used in the hospital
setting in intravenous form to rapidly reduce the degree of fluid overload
present in a patient in severe congestive heart failure. This diuretic acts by
inhibiting the Na/K/2Cl cotransporter on the luminal membrane of the thick
ascending portion of the loop of Henle. It is consequently classified as a loop
diuretic, as is ethacrynic acid, which has a similar mechanism of action.
Carbonic anhydrase inhibitors (choice A), such as acetazolamide and
dorzolamide, act on the proximal convoluted tubule to reduce Na
+
resorption
secondary to an inhibition of CO
2
formation with resulting decreased
intracellular bicarbonate and H
+
levels.
Osmotic diuretics (choice C), such as mannitol, inhibit water reabsorption
throughout the nephron.
Potassium-sparing diuretics (choice D), such as spironolactone, amiloride,
and triamterene, act at the level of the collecting tubules and ducts by acting
as aldosterone receptor antagonists.
Thiazide diuretics (choice E), such as hydrochlorothiazide, indapamide, and
metolazone, inhibit the Na/Cl cotransporter in the distal convoluted tubule.
Question 2 of 6
The patient is seen the following morning by a cardiologist. The cardiologist does
a very careful physical examination. He notes that the heart
sounds appear distant. He then has the patient lie at an angle of 30 to 45
degrees, and does a careful examination of the right jugular pulse,
which he finds very worrisome. The pulse is both very elevated and shows
dramatic x and y descents. Further, he notes that the venous
distention paradoxically increases during inspiration. This last finding is
sometimes called which of the following?
/ A. Chvostek's sign
/ B. Corrigan's sign
/ C. Homans' sign
/ D. KussmauI's sign
/ E. Murphy's sign


Explanation - Q: 3.2 Close

The correct answer is D. The sign described is Kussmaul's sign. The act of
inflating the lungs during inspiration lowers the pressure in the chest while
increasing that in the abdomen, drawing blood from the abdomen into the
chest (and increased abdominal pressure helps to directly drive blood toward
the chest). If the right atrium cannot fill, then the jugular venous pressure
rises paradoxically (not so much from blood flow from the head as from the
abdomen, because the inferior vena cava and superior vena cava are
functionally connected through the right atrium). Kussmaul's sign is seen in
patients who have non-compliant right ventricles. It can also be seen in
patients with severe ascites (which increases the intra-abdominal pressure).
This case illustrates the importance of considering the jugular venous pulse
as well as the arterial pulse, since the cardiologist was able to find a number
of significant findings pertaining to the jugular venous pulse, which other
physicians had missed. The jugular venous pressure can be used at the
bedside to estimate the right atrial filling pressure. The jugular venous
pressure is estimated by measuring the height of the visible venous pulse
above the sternal angle, and then adding 5 cm (corresponding to how far
below the sternum the right atrium is located). The jugular venous waveform
has an A wave, which is followed by an X descent, then a V wave, and finally
a Y descent. The A wave (first rise in pressure) reflects the right atrial
contraction, while the X-descent reflects right atrial diastole, and then early
right ventricular systole. The V wave is the second major positive wave, and
reflects continued venous inflow into the right atrium in opposition to a closed
mitral valve. The following Y-descent is the negative deflection that occurs
when the tricuspid valve opens in early diastole.
Chvostek's sign (choice A) is seen in tetany, and is a facial muscle spasm
occurring when the facial nerve is tapped anterior to the external auditory
meatus.
Corrigan's sign (choice B), which suggests aortic regurgitation, is a full, hard
arterial pulse, which is followed by a sudden collapse.
Homans' sign (choice C) is pain at the back of the knee or calf when the
ankle is dorsiflexed, and suggests venous thrombosis of the leg.
Murphy's sign (choice E) is pain on palpation of the right subcostal area
during inspiration, and is frequently seen in acute cholecystitis.
Question 3 of 6
This patient most likely has which of the following?
/ A. Acute myocarditis
/ B. Congestive cardiomyopathy
/ C. OId left ventricle myocardial infarction
/ D. Recent left ventricle myocardial infarction
/ E. Restrictive cardiomyopathy
Explanation - Q: 3.3 Close

The correct answer is E. The "distant" heart sounds and jugular venous
pulse findings both suggest that this patient has restrictive cardiomyopathy
that is limiting the heart's ability to fill during diastole and is also impairing
ventricular contraction. Other findings that may be encountered on physical
examination in patients with restrictive cardiomyopathy include S3 and/or S4
heart sounds, occasional mitral or tricuspid regurgitation murmurs, and, if the
patient is in secondary congestive failure, peripheral edema and pulmonary
rales. Restrictive cardiomyopathy is relatively rare and the findings on
physical examination are subtle, and consequently this patient's history of
missed diagnosis is unfortunately not all that uncommon. Underlying causes
of restrictive cardiomyopathy include endomyocardial fibrosis, Loeffler
eosinophilic endomyocardial disease, hemochromatosis, amyloidosis,
sarcoidosis, scleroderma, carcinoid heart disease, and glycogen storage
disease. Patients typically present at an advanced stage of the disease, and
may have symptoms of angina, shortness of breath, peripheral edema, and
ascites with abdominal discomfort (related to pooling of blood in the liver and
other abdominal organs). Once the diagnosis is suspected,
echocardiography typically demonstrates normal to symmetrically thickened
heart chamber walls with rapid early-diastolic filling and slow late-diastolic
filling (the cardiac chambers are acting more or less like poorly distensible
plastic bags). Cardiac catheterization will more or less repeat the
observations seen in the analysis of the jugular venous pulse, typically
showing elevated ventricular end-diastolic pressure, normal to slightly
decreased ejection fraction, and prominent x and y descents.
Acute myocarditis (choice A) can cause congestive cardiomyopathy (choice
B), but the heart is usually larger and the constrictive findings seen in this
case would not be present.
While recent and old myocardial infarctions affecting the right ventricle may
produce similar jugular venous findings to those seen in this case, left
ventricular infarction (choices C and D) would not impair right ventricular
filling and contraction.

Question 4 of 6
An endomyocardial biopsy is performed, which demonstrates eosinophilic
acellular deposits within the myocardial biopsy. When recut,
histological sections are stained with Congo red and viewed under polarized
light, and the deposits appear bright green. These deposits are
most likely to be composed of which of the following?
/ A. Amyloid
/ B. Fibrin
/ C. Hemosiderin
/ D. Melanin
/ E. Uric acid


Explanation - Q: 3.4 Close

The correct answer is A. Amyloid deposits are suspected when
hematoxylin and eosin-stained histological sections show extracellular
eosinophilic deposits. The presence of amyloidosis is confirmed when the
characteristic "apple-green birefringence" on Congo red stain is
demonstrated.
Fibrin deposits (choice B) are also red on hematoxylin and eosin stain, but
show no fluorescence with Congo red stain.
Hemosiderin (choice C) causes yellow brown deposits; melanin (choice D)
causes brown-black deposits; and uric acid (choice E) causes yellow
crystalline deposits.
Question 5 of 6
Which of the following features of proteins is most likely responsible for the bright
green appearance of the Congo red-stained materiaI?
/ A. Beta pleated sheet configuration
/ B. Calcium binding
/ C. Iron containing heme moiety
/ D. Multiple alpha helices
/ E. Presence of multiple subunits

Explanation - Q: 3.5 Close

The correct answer A. It was originally assumed by biochemists that
amyloid was always composed of the same material. It came as something
of a shock when antibody techniques were developed that demonstrated that
the antigenicity of amyloid in different clinical settings varied markedly. The
common feature these proteins shared that accounted for both the affinity for
Congo red and their characteristic regular fibrillar structure on electron
microscopy turned out to be that the proteins all have a beta pleated sheet
tertiary (secondary according to some biochemical purists) configuration,
best demonstrated by X-ray diffraction.
Selective or non-selective binding to calcium (choice B) is common in
proteins.
Heme moieties containing iron (choice C) are a part of myoglobin and
hemoglobin.
Alpha helices (choice D) are a common secondary structure in proteins, but
do not contribute to the protein forming amyloid.
The presence of multiple subunits (choice E) is also common in proteins, but
does not contribute to a protein forming amyloid.
Question 6 of 6
Which of the following would most likely be found in the Congo red-stained
extracellular deposits with the bright green appearance under
polarized light?
/ A. Amyloid AA
/ B. Beta-2-microglobulin
/ C. Beta protein precursor
/ D. Immunoglobulin light chains
/ E. Transthyretin

Explanation - Q: 3.6 Close

The correct answer is D. Amyloidosis occurs in a large variety of forms.
Primary amyloidosis is one of the more common forms of systemic
amyloidosis, and can affect a variety of organs, including the heart, kidney,
peripheral nerve, gastrointestinal tract, and respiratory tract. In primary
amyloidosis, the amyloid is composed of immunoglobulin light chains, and
the disease is now interpreted as a plasma cell disorder closely related to
multiple myeloma. This interpretation is clinically significant, as it has led to
modern treatments of primary amyloidosis (which formerly had a dismal
prognosis) with the chemotherapies designed for multiple myeloma. The
treatments are affective only if the disease is recognized and passed to the
appropriate specialists as early as possible in the clinical course.
Amyloid AA (choice A) is seen in inflammation-associated amyloidosis and
familial Mediterranean fever.
Beta-2-microglobulin (choice B) comprises the amyloid of dialysis-
associated amyloidosis.
Beta protein precursor (choice C) comprises the amyloid seen in the brains
of patients with Alzheimer's disease and Down syndrome.
Transthyretin (choice E) comprises the amyloid seen in familial amyloidosis
and senile cardiac amyloidosis.

A 40-year-old man presents to the emergency department complaining of severe
shortness of breath. The breathlessness has been
worsening over the past few years, and the patient reports growing tachypneic
with mild exertion, and sometimes even at night. On
examination, he has generalized edema, jugular venous distention, and hepatic
distention. Cardiac examination shows a right ventricular
heave, a right-sided S3, and S4 with a pulmonary ejection click. A chest x-ray
film shows cardiomegaly and widening of the hilar vessels,
including the pulmonary arteries. An electrocardiogram shows talI, peaked P
waves in leads lI, III, and aVF, right axis deviation, and right
ventricular hypertrophy.
Question 1 of 5
Which of the following is the most likely diagnosis?
/ A. Cor pulmonale
/ B. Hypertrophic cardiomyopathy
/ C. Left ventricular failure
/ D. Myocardial infarction
/ E. Pulmonary embolus (acute)
Explanation - Q: 4.1 Close

The correct answer is A. This patient has cor pulmonale, which is defined
as enlargement of the right ventricle secondary to diseases of the lung,
thorax, or pulmonary circulation. In this case, it is chronic, given the duration
of the patient's symptoms and the presence of many clinical sequelae of the
condition: edema, jugular venous distention, hepatic distention, and right
ventricular heave. The electrocardiogram also supports the diagnosis of
enlargement of the right ventricle showing right axis deviation due to the
increase in the mass of the right heart. Evidence of right atrial enlargement is
also present, i.e., the tall peaked P waves in leads II, III, and aVF (P
pulmonale).
Hypertrophic cardiomyopathy (choice B) is an anomaly in which the
myocardium hypertrophies. The fibers are erratic and conduction
abnormalities and outflow obstruction may result. Typically, this disorder
presents in the second decade of life, and will manifest as dysrhythmia
and/or shortness of breath. In addition, a right axis deviation would be
inconsistent with this cardiomyopathy because the left ventricle
hypertrophies, as well as the right. Thus, this diagnosis is unlikely.
Left ventricular failure (choice C) often accompanies right ventricular failure,
but in this case, the right-sided symptoms, such as systemic edema, jugular
venous distention, and hepatic congestion, are more pronounced. Left-sided
failure shows engorgement of the entire pulmonary tree in conjunction with
pulmonary edema.
Myocardial infarction (choice D) is unlikely. The ECG findings are not
consistent with the pattern typically seen in MI. In addition, this patient does
not suffer from the symptoms of myocardial infarction, such as chest pain,
pressure, jaw numbness, and diaphoresis.
Pulmonary embolus (choice E) may cause acute right heart strain and
failure, but this patient has a chronic condition. Chronic emboli may produce
increased resistance in the pulmonary tree and a picture similar to this.



Question 2 of 5
Pulmonary hypertension is suspected in the patient, and a Swan-Ganz catheter
is placed. Which of the following denotes the correct
anatomic sequence of vessels that would be traversed by the catheter if it was
introduced into the left subclavian vein?
/ A. Left subclavian vein, Ieft brachiocephalic vein, superior vena cava, right
atrium, right ventricle, pulmonary artery
/ B. Left subclavian vein, Ieft common carotid, superior vena cava, right atrium,
right ventricle, pulmonary artery
/ C. Left subclavian vein, Ieft jugular vein, Ieft atrium, Ieft ventricle, aorta
/ D. Left subclavian vein, Ieft jugular vein, superior vena cava, right atrium, right
ventricle, pulmonary artery
/ E. Left subclavian vein, superior vena cava, right atrium, right ventricle,
pulmonary artery

Explanation - Q: 4.2 Close

The correct answer is A. The correct sequence for a catheter inserted into
the left subclavian vein is as follows: left subclavian vein, left brachiocephalic
vein, superior vena cava, right atrium, right ventricle, pulmonary artery. With
this catheter in place, a variety of cardiac parameters can be measured,
including pressures in the pulmonary artery. Thus, this catheter can aid in
establishing the diagnosis of pulmonary hypertension.



Calcium channel blockers can be used in this setting to decrease pulmonary
vascular resistance. Which of the following is the calcium
channel blocker that will have the most predominant effect on vascular smooth
muscle?
/ A. Diltiazem
/ B. Hydrochlorothiazide
/ C. Nifedipine
/ D. Pseudoephedrine
/ E. Verapamil

Explanation - Q: 4.3 Close

The correct answer is C. The calcium channel blockers vary in the
propensity to affect vascular smooth muscle versus their effect on cardiac
muscle. Thus, in this case, it is important to select an agent that has
maximum ability to relax the smooth muscle in the pulmonary vessels. The
effect on smooth muscle is as follows: nifedipine>diltiazem (choice A)
>verapamil (choice E). The effect on cardiac muscle is as follows:
verapamil>diltiazem>nifedipine. Thus nifedipine is the agent of choice.
Hydrochlorothiazide (choice B) is a diuretic, and thus would have no effect
on the vascular smooth muscle.
Pseudoephedrine (choice D) is an alpha agonist, and therefore would cause
vasoconstriction.

Question 4 of 5
Which of the following physiologic stimuli will result in decreased pulmonary
vascular resistance?
/ A. Decreased cardiac output
/ B. Increased cardiac output
/ C. Low O2 tension
/ D. Lung volumes near residual volume (RV)
/ E. Lung volumes near total lung capacity (TLC)


Explanation - Q: 4.4 Close

The correct answer is B. A unique feature of the pulmonary circulation is
that it maintains itself as a low-pressure system. Many of the mechanisms
that control pulmonary vasculature differ from those of the systemic
circulation. One of these features is that pulmonary vasculature resistance is
decreased in response to increased cardiac output. This is accomplished
through distention of open capillaries and the recruitment of collapsed
capillaries. Thus, the resistance in the pulmonary tree decreases in response
to increased right ventricular output. In the pathologic state of pulmonary
hypertension, in which the resistance is elevated and the ventricle fails, this
decreased cardiac output (choice A) may compound the problem and trigger
increased resistance in spite of the primary elevation.
Low O
2
tension (choice C) in the pulmonary vessels initiates
vasoconstriction. In the systemic circulation, low O
2
tension initiates
vasodilation.
Lung volume also affects pulmonary vascular resistance. The curve of lung
volume versus pulmonary vascular resistance is U-shaped. This effect is due
to the fact alveolar and extra-alveolar vessels act as resistors in series
(additive), and these vessels have little intrinsic support. Thus, resistance in
these vessels is affected by pleural pressures. At low lung volumes (choice
D), the alveolar vessels are open, but extra-alveolar vessels are
compressed. At high lung volumes (choice E), the alveolar vessels are
compressed by distended alveoli, but the extra-alveolar vessels become
distended due to the increase in transmural pressure. Thus a U-shaped
curve describes this relationship.
Question 5 of 5
Some of the examination findings indicate hepatic congestion. Which of the
following terms is commonly used to identify the macroscopic
pattern of red, depressed hepatic nodules with pale periphery that accompanies
the chronic hepatic congestion seen in this condition?
/ A. Centrilobular hemorrhage
/ B. Cirrhosis
/ C. Fatty change
/ D. Nutmeg liver
/ E. Piecemeal necrosis


Explanation - Q: 4.5 Close

The correct answer is D. Chronic passive congestion of the liver leads to a
macroscopic pattern known as nutmeg liver. This is due to the congestion of
blood in the centrilobular region (dark) with hypoxia and fatty change in the
more peripheral hepatocytes. When viewed macroscopically, this pattern
resembles that seen in a cross section of a nutmeg, hence the name.
In this condition, centrilobular hemorrhage (choice A) usually only occurs in
severe acute ischemia. This patient has a chronic condition, and thus most
likely will have nutmeg liver instead.
Cirrhosis (choice B) of the liver may result from chronic damage caused by
chronic congestion. It however produces a scarred, whitish, shrunken liver,
and not the pattern seen here.
Fatty liver (choice C) would produce a large, smooth yellow liver and would
not resemble the pattern seen here.
Piecemeal necrosis (choice E) is a microscopic finding of scattered
hepatocellular necrosis. This diagnosis cannot be made macroscopically.


A 45-year-old man presents to the emergency department complaining of chest
pain, which began twenty minutes before while he was filling
up his car with gasoline. He describes the pain as substernaI, intense, dulI, and
squeezing. It does not change with respiration. He also
complains that he is nauseated. He has never experienced anything like this
before. His temperature is 37.5 C (99.5 F), blood pressure is
124/76 mm Hg, pulse is 80/min, respiratory rate is 22/min, and oxygen saturation
is 95% on room air. On physical examination, he is
diaphoretic. His lungs are clear, his heart rate is regular, and he has a normaI S1
and S2 without murmur, rub, or gallop. The examiner
estimates that his jugular venous pressure is elevated to the angle of the jaw. His
abdomen is nontender, with normal bowel sounds. An
electrocardiogram is performed, which reveals sinus rhythm, normal axis, normal
intervals, and ST elevation in leads lI, III, and aVF. A chest x-
ray film reveals no apparent cardiac or pulmonary abnormalities.

Question 1 of 7

Which of the following is the most likely diagnosis?
/ A. Acute myocardial infarction
/ B. Aortic dissection
/ C. Gastroesophageal reflux
/ D. Pericarditis
/ E. Pulmonary embolus

Explanation - Q: 1.1 Close

The correct answer is A. The differential diagnosis of chest pain is broad
and includes all the answers on this list, all of which can present, as in this
patient, with a relatively normal examination of the heart and lungs. Acute
myocardial infarction is the only one of these findings that is associated with
ST segment elevation in an anatomical distribution (in this case, the "inferior"
leads). His elevated jugular venous pressure is also a clue to abnormal
cardiac function; however, this might be present in severe constrictive
pericarditis or pulmonary embolism as well.
Aortic dissection (choice B) often presents with chest pain or pain radiating
to the back. It is not, however, typically associated with ST segment changes
on the EKG, unless the dissection extends proximally into the ostia of the
coronary arteries, obstructing flow, and resulting in secondary acute
myocardial infarction, in which case a patient could present like this.
However, this presentation would be an uncommon presentation of a
relatively uncommon disease. The risk for aortic dissection is increased with
long-standing essential hypertension, other peripheral vascular disease,
hyperlipidemia, and advanced age, as well as connective tissue disorders
such as Marfan syndrome or Ehlers-Danlos syndrome.
Patients with gastroesophageal reflux (choice C) may complain of intense
substernal chest pain that is difficult to distinguish from the pain of
myocardial infarction. However, the ST elevations on the EKG suggest
transmural ischemia of the myocardium and do not occur with isolated
gastroesophageal reflux. Do not let relatively normal vital signs and
gastrointestinal symptoms such as nausea fool you!
Pericarditis (choice D), an inflammatory disease of the pericardium, presents
with chest pain and is often associated with ST elevation on the EKG, as well
as PR interval depression. However, the ST elevation usually involves
multiple leads of the EKG, and is not in a strictly anatomic distribution.
Pericarditis is often associated with a friction rub.
Pulmonary thromboembolism (choice E) can also present with chest pain. A
patient who has had a large PE, however, typically will not have normal vital
signs, and is likely to have tachycardia, tachypnea, and possibly hypoxemia.
Hemodynamically significant pulmonary embolism can present with elevated
JVP due to right heart strain. The patient might have ST depression in the
inferior leads, but will not have ST elevation. The pain associated with
pulmonary embolus is generally pleuritic in nature, that is, lateralizing, and
changing with inspiration. Risk factors for pulmonary embolism include
hypercoagulable states, immobilization, and vascular injury (Virchow's triad).
Question 2 of 7
What is the pathophysiologic process most likely to be responsible for this
patient's presentation?
/ A. Atherosclerotic plaque rupture resulting in thrombus formation
/ B. Buildup of atherosclerotic stenosis to produce high-grade obstruction of the
artery
/ C. Dissection of the vessel
/ D. Embolization of blood clot, air, or foreign material
/ E. Myocardial hypertrophy resulting in vessel narrowing


Explanation - Q: 1.2 Close

The correct answer is A. Acute coronary syndromes are thought to be the
result of rupture of a pre-existing atherosclerotic plaque, often one that is not
producing high-grade stenosis. When the endothelial surface covering the
lipid core of an unstable plaque ruptures, a platelet plug forms and the
clotting cascade is activated, rapidly propagating thrombus formation and
suddenly occluding the vessel. This results in transmural ischemia, which
becomes infarction, should the clot fail to recanalize quickly.
Atherosclerotic stenosis (choice B) is an important pathologic component of
coronary artery disease. However, high-grade coronary stenoses most
frequently cause stable angina, that is, chest pain and myocardial ischemia
induced when an increase in myocardial oxygen demand exceeds the fixed
supply that can be obtained through a severely stenotic vessel. As vessels
progressively narrow over time, they produce ischemia, but not sudden
infarction. Often distal to a narrowed vessel, collaterals will form from less
diseased vessels, compensating for the reduced flow.
Coronary artery dissection (choice C) is a rare phenomenon that can
produce transmural ischemia and infarction, but most commonly occurs in
the setting of instrumentation of the vessel.
Embolization (choice D) is a relatively rare phenomenon in the coronary
circulation under normal circumstances. Foreign material can only enter the
left side of the heart via right-to-left intracardiac shunt, pulmonary vein
interruption, or surgical opening of the left heart. Thrombus that forms in the
left atrium (usually the result of low-flow states such as mitral stenosis or
atrial fibrillation) can also embolize; patients with atrial fibrillation are
anticoagulated to reduce the risk of cerebral embolization of left atrial clot.
Myocardial hypertrophy (choice E) can restrict subendocardial oxygen
supply by creating high capillary pressure relative to arteriolar pressure. This
does not occur in large coronary vessels, but can produce subendocardial
ischemia in disease states that are associated with hypertrophic
myocardium, such as aortic stenosis, long-standing essential hypertension,
and idiopathic hypertrophic subaortic stenosis (IHSS).
Question 3 of 7
Which of the following vessels is most likely to be diseased in this patient?
/ A. Coronary sinus
/ B. Left anterior descending coronary artery
/ C. Left circumflex coronary artery
/ D. Left main coronary artery
/ E. Right coronary artery


Explanation - Q: 1.3 Close

The correct answer is E. Acute myocardial infarction is usually due to
obstruction of one coronary vessel. The right coronary artery exits the right
sinus of Valsalva of the aorta and gives off branches to the right ventricle, the
SA nodal artery (in 70% of patients), the AV nodal artery, and, in the 85% of
patients whose circulations are said to be "right-dominant," the posterior
descending artery, which supplies the inferior wall of the RV and the LV as
well as the posterior 1/3 of the interventricular septum. This patient has EKG
signs of an inferior myocardial infarction, with ST elevation in the inferior
leads, II, III, and aVF. He also has a physical sign of right ventricular
dysfunction (elevated jugular venous pressure).
The coronary sinus (choice A) is the principal vein draining the left ventricle
and runs alongside the circumflex artery in the posterior AV groove. It is not
a common site for atherosclerotic disease or for obstruction.
The left anterior descending artery (choice B) supplies the anterior and
anteroseptal portions of the left ventricle. Obstruction would produce ST
elevation in the anterior (V2-V6) and occasionally the lateral (I, aVF) leads of
the EKG, with possible "reciprocal" ST depression in the inferior leads.
The left circumflex artery (choice C) supplies the lateral wall of the left
ventricle. 85% of patients have a "right-dominant" coronary anatomy. That is,
the right coronary artery gives off the posterior descending artery (PDA). In
the "left-dominant" remaining 15%, the PDA comes off the circumflex.
Isolated inferior EKG lead changes are, therefore, most likely to be due to
RCA obstruction; circumflex obstruction typically produces EKG lead
changes in the lateral (I, aVL, V5, V6) leads.
The left main coronary artery (choice D) exits the aorta at the left sinus of
Valsalva and divides into the left anterior descending and left circumflex
arteries. Obstruction of the left main makes the entire left ventricle ischemic,
often resulting in cardiogenic shock. This would produce ST segment
elevation in leads I, aVL, and V2-V6.


Question 4 of 7

This patient is given aspirin in the emergency department. Decreased production
of which of the following mediators is responsible for the
beneficial effects of aspirin in this disorder?
/ A. cAMP
/ B. PIatelet glycoprotein lIB/IIIA
/ C. Prostacyclin
/ D. Thromboxane A2
/ E. Ubiquinone (coenzyme Q)

Explanation - Q: 1.4 Close

The correct answer is D. Aspirin irreversibly inhibits the enzyme
cyclooxygenase, which produces all the prostaglandin mediators from
arachidonic acid. Cyclooxygenase in platelets produces thromboxane A2,
which is a potent promoter of platelet aggregation and vasoconstriction. By
blocking this, aspirin irreversibly inhibits platelet function, preventing
aggregation at the site of plaque rupture. Platelets, having no nuclei, are
unable to synthesize more cyclooxygenase, and therefore thromboxane
production is inhibited for the life of the platelet, approximately 10 days.
Cyclic AMP (choice A) is an intracellular small molecule responsible for
multiple signal transduction pathways. In cardiac myocytes, it activates
protein kinases responsible for the phosphorylation of calcium channels,
promoting entry of calcium into the cell. cAMP is broken down by
phosphodiesterase, the inhibition of which is responsible for the beneficial
effects of inotropic phosphodiesterase inhibitors such as milrinone.
Platelet surface glycoprotein IIb/IIIA (choice B) binds fibrinogen and von
Willebrand factor, promoting aggregation and clot formation. It is inhibited by
drugs like eptifibatide and tirofiban, which are used in acute coronary
syndromes to further inhibit platelet aggregation and thrombus formation.
Prostacyclin (choice C) is produced by cyclooxygenase in endothelial cells,
where it promotes vasodilation and inhibits platelet aggregation. Prostacyclin
should therefore be a beneficial mediator. Aspirin inhibits prostacyclin
formation, however, endothelial cells can produce more cyclooxygenase and
are able to continue to synthesize adequate amounts of prostacyclin.
Ubiquinone (choice E), or coenzyme Q, is a naturally occurring coenzyme
that plays a vital role in the mitochondrial electron transport chain. Studies
have shown an association between decreased levels of coenzyme Q and
heart disease, so inhibition of ubiquinone production would not likely be
beneficial.

Question 5 of 7
EIevation of which of the following serum proteins is the most specific
biochemical marker for this patient's condition?
/ A. AIanine aminotransferase
/ B. Creatine phosphokinase
/ C. Lactate dehydrogenase
/ D. Transferrin
/ E. Troponin


Explanation - Q: 1.5 Close

The correct answer is E. Troponins (in isoforms troponin C, troponin I, and
troponin T) are required for actin-myosin cross linking in cardiac muscle.
Small elevations in serum troponin levels are currently the most sensitive
clinical serum markers for myocardial injury, elevating within 3-12 hours of
infarction. Levels remain elevated for 5-14 days.
Alanine aminotransferase (choice A) occurs in both cardiac muscle and in
the liver, and has been used in the past as a marker of cardiac injury.
However, currently, its more common clinical use is as a marker of
hepatocyte injury. Its time course of elevation in MI is intermediate between
CPK and LDH (see below).
Creatine phosphokinase (choice B) has been the mainstay of diagnosis of
myocardial injury for many years. CPK has several isoforms, of which the MB
isoform is specific for cardiac muscle. The fraction of the total CPK that is the
MB isoform has been used to differentiate myocardial injury from other injury
processes elevating CPK. CPK is also elevated with skeletal muscle and with
brain injury, but neither of these tissues contains significant amounts of MB
isoform. In MI, CPK levels usually rise within 8 to 24 hours and return to
normal after 48 to 72 hours.
Lactate dehydrogenase (choice C) is, like alanine aminotransferase, an
enzyme that is released both with cardiac injury and with hepatocellular
injury. It can also be elevated in hemolysis and with some neoplasms. In MI,
it generally rises within 12 hours and peaks after 24-48 hours, remaining
elevated for 10-14 days. These properties made LDH, prior to the
introduction of troponin assays, the test used to detect MI occurring more
than a day previously.
Transferrin (choice D) is a plasma protein responsible for the uptake of iron
after absorption in the small intestine, and is responsible for iron-binding
capacity in the blood. It is measured (usually as "total iron-binding capacity")
in the differential diagnosis of the anemias.
Question 6 of 7
Three days after hospital admission, the patient suddenly develops shortness of
breath and becomes hypotensive. His heart rate is 100/min,
with a normaI PR and QRS intervaI. His blood pressure is 75/50 mm Hg. His
respiratory rate is 38/min and his oxygen saturation on 2 Iiters
via nasal cannula drops to 60%. A chest x-ray reveals bilateral fluffy infiltrates in
the lung fields. Which of the following complications of his
condition has most likely occurred?
/ A. Dilation of the left ventricle
/ B. Dressler syndrome
/ C. Rupture of the left ventricular free wall
/ D. Rupture of the posteromedial papillary muscle
/ E. Ventricular tachycardia

Explanation - Q: 1.6 Close

The correct answer is D. This patient is suddenly in cardiogenic shock with
severe pulmonary edema. This could be the result of arrhythmia, cardiac
tamponade, or left ventricular valvular dysfunction. He had an inferior MI,
which is most likely due to thrombosis of the right coronary artery. The
posteromedial papillary muscle is supplied by the RCA alone in most patients
and is prone to rupture in inferoposterior MI. Rupture leads to acute and
severe mitral valve dysfunction, resulting in pulmonary edema and poor
forward ejection.
Dilation of the left ventricle (choice A) typically occurs after extensive
damage occurs, which would have appeared on this patient's acute EKG as
ST elevation in the anterior leads. Dilation can result in mitral regurgitation,
but typically of an insidious onset.
Dressler syndrome (choice B) is a late complication of MI that may occur
weeks to months later, characterized by symptoms of pericarditis including
pleuritic chest pain, fever, friction rub, and elevated white blood cell count.
Patients can also develop early postinfarction pericarditis in the days to
weeks following MI with friction rub and pericardial effusion. This is seldom
associated with cardiac tamponade.
Rupture of the LV free wall (choice C) is a complication more likely to occur
with more extensive damage to the LV than is produced in an inferior MI
such as this patient had. However, LV free wall rupture would produce
cardiac tamponade, which could produce this clinical picture.
Ventricular tachycardia (VT) (choice E) is a complication of MI (though risk is
highest early in the course of infarction) and could also produce this clinical
picture. However, it is excluded by the EKG, which reveals an atrial-
ventricular conducted rhythm (VT displays no P waves) and a narrow-
complex QRS (VT typically has a QRS much greater than 0.12 s).
Question 7 of 7
The patient is taken emergently to the operating room. During surgery, a sample
of affected myocardial tissue is sent to the pathology
Iaboratory for examination. Which of the following would be the likely pathologic
finding(s)?
/ A. Acellular fibrosis
/ B. Monocyte infiltration, absent nuclei and striations
/ C. Myocyte disarray
/ D. Myocyte edema, hemorrhage, and dense neutrophil infiltration
/ E. Wavy myofibers with eosinophilic contraction bands

Explanation - Q: 1.7 Close

The correct answer is B. Three days after infarction, coagulation necrosis is
complete, with complete loss of cellular structure (hence the high risk of
mechanical complications such as rupture) and infiltration of monocytes to
phagocytize debris.
Acellular fibrosis (choice A) replaces necrosis after many weeks when
debris is removed and fibroblasts have invaded the dead tissue and replaced
it with collagen.
Myocyte disarray (choice C) is associated with hypertrophic subaortic
stenosis (IHSS), rather than myocardial infarction.
Myocyte edema, with hemorrhage and neutrophil infiltration (choice D)
occurs within 4-12 hours after infarction.
Wavy myofibers and contraction bands (choice E) are the first light
microscopic pathologic changes to occur after MI, and appear within 1-3
hours after infarction.


A 52-year-old man presents to the emergency department because of severe
chest pain. The excruciating pain began abruptly, 30 minutes
previously, and feels to the patient as if something were "ripping." When asked to
point to where the pain is worst, the patient points to the
precordium. The man additionally reports that the pain seems to be changing in
position slowly.
Question 1 of 6
Which of the following is most likely causing the patient's severe pain?
/ A. Aortic dissection
/ B. Atherosclerotic aortic aneurysm
/ C. Esophageal reflux
/ D. Myocardial infarction
/ E. Peptic ulcer

Explanation - Q: 2.1 Close

The correct answer is A. This patient has a classic presentation of aortic
dissection. Any time a patient in excruciating chest pain describes the pain
as "tearing" or "ripping," you should strongly consider the diagnosis of aortic
dissection. The pain may move with time as the dissection progresses. Aortic
dissection is a highly lethal condition that may lead to aortic rupture, most
often into the pericardial cavity or left pleural space. The two most common
sites of origin of the dissection are in the proximal aorta within 5 cm of the
aortic valve and in the descending thoracic aorta just distal to the origin of
the left subclavian artery. CT scan with contrast is often used to confirm the
diagnosis suspected clinically. Therapy is promptly initiated with medications
that lower the blood pressure to try to prevent extension of the dissection.
Surgery is usually then performed in patients in which the dissection begins
in the proximal aorta near the aortic root; medical therapy alone can
sometimes be used for those with distal aortic dissection that has not
compromised blood flow to limbs or organs.
Atherosclerotic aortic aneurysm (choice B) more commonly involves the
abdominal aorta, and, when rupturing, may produce excruciating pain that is
usually referred to the lower abdomen and back.
The pain of esophageal reflux (choice C) is rarely excruciating, and usually
does not produce a ripping or tearing sensation.
Myocardial infarction (choice D) can produce severe precordial chest pain,
but the pain usually does not move with time and does not have a tearing or
ripping character.
Peptic ulcer pain (choice E) may be severe and referred to the chest, but
patients are more likely to use terms like "burning" than ripping or tearing,
and the pain does not slowly change position.
Question 2 of 6
Extension of this patient's disease process would be most likely to produce which
of the following?
/ A. Aortic insufficiency
/ B. Aortic stenosis
/ C. Mitral insufficiency
/ D. Mitral stenosis
/ E. Tricuspid stenosis

Explanation - Q: 2.2 Close

The correct answer is A. Dissecting aneurysms tend to start near the root
of the aorta, and aortic insufficiency is a common complication. This can be
very helpful in the initial evaluation of the patient, since up to 2/3 of the
patients with proximal aortic dissection demonstrate, on auscultation, the
characteristic murmur of aortic insufficiency, which is a pandiastolic
decrescendo murmur that is loudest over the sternum and left lower sternal
border. Aortic stenosis (choice B) usually does not occur.
Involvement of the mitral (choices C and D) and tricuspid valves (choice E)
would be very rare, and probably only seen if the aortic dissection interrupted
the orifices of the coronary arteries and induced a secondary myocardial
infarction.






Question 3 of 6
If enzyme chemistries were sent, which would be the most likely results?
/ A. Decreased AST, elevated CK, decreased LDH
/ B. EIevated AST, elevated CK, normal to decreased LDH
/ C. EIevated AST, normaI CK, normaI LDH
/ D. NormaI AST, elevated CK, elevated LDH
/ E. NormaI AST, normaI CK, normal to elevated LDH
Explanation - Q: 2.3 Close

The correct answer is E. Unless aortic dissection secondarily causes a
myocardial infarction secondary to occlusion of the coronary arteries,
aspartate aminotransferase (AST) and creatine kinase (CK) levels should be
normal. Lactic dehydrogenase (LDH) levels may be normal, or elevated if
some hemolysis is occurring within the area of dissection.
In general, AST can be elevated (choices B and C) in a variety of cardiac
diseases (e.g., myocardial infarction, heart failure, myocarditis, pericarditis),
muscle damage (e.g., myositis, muscular dystrophy, trauma), and damage to
liver, pancreas, kidney, or brain. AST is decreased (choice A) in pyridoxine
(vitamin B6) deficiency and in the terminal stages of liver disease. In general,
CK can be elevated (choices A, B, and D) in disease or damage involving
heart, muscle, or brain. Decreased CK has no medical significance. Lactic
dehydrogenase (LDH) can be elevated (choice D) in myocardial infarction,
pulmonary infarct, hemolytic and pernicious anemia, hematologic
malignancies, and disease of liver, kidney, or brain. Decreases in LDH
(choices A and B) are not medically significant.

Question 4 of 6
If surgery is necessary to repair this problem, the surgeon will be required to
understand the anatomic relationship of the aorta to the
surrounding structures. Which of the following most accurately describes the
descending portion of the thoracic aorta?
/ A. It descends on the right side of the thoracic vertebrae
/ B. It flattens the posterior aspect of the trachea
/ C. It is to the left of the esophagus at the hiatus
/ D. It is to the left of the thoracic duct at the T10 Ievel
/ E. It is to the right of the inferior vena cava

Explanation - Q: 2.4 Close

The correct answer is D. The thoracic duct is the main lymphatic duct and it
lies on the bodies of the inferior seven thoracic vertebrae. It conveys most of
the lymph of the body to the venous system. It passes superiorly from the
cisterna chyli (the expanded inferior end of the thoracic duct) through the
aortic hiatus in the diaphragm. The thoracic duct ascends in the posterior
mediastinum, on the right of the thoracic aorta and to the left of the azygos
vein. At the level of T4, T5, or T6, the thoracic duct crosses to the left,
posterior to the esophagus and ascends to the superior mediastinum. The
thoracic duct empties into the venous system near the union of the left
internal jugular and subclavian veins.
As a continuation of the aortic arch, the descending aorta begins on the left
side of the inferior border of the body of the T4 vertebra and descends in the
posterior mediastinum on the left sides of T5 to T12 (choice A).
The trachea travels in the superior mediastinum and does not have direct
contact with the descending thoracic aorta. The trachea is kept patent by a
series of C-shaped tracheal cartilages. The posterior aspect is flat where it is
applied to the esophagus, not the aorta (choice B).
At the level of the esophageal hiatus (choice C), the esophagus lies anterior
to the descending thoracic aorta.
The inferior vena cava (choice E) is located to the right of the abdominal
aorta, not the thoracic aorta. The IVC returns blood from the lower limbs,
most of the abdominal wall, and the abdominopelvic viscera. This vessel
begins anterior to L5 vertebra by union of the common iliac veins. It then
ascends on the right psoas major muscle to the right of the median plane
and aorta. It passes through the vena caval foramen in the diaphragm at the
level of T8 to enter the right atrium.

Question 5 of 6
Which of the following would be most likely to be seen on pathological
examination of a specimen removed from this patient at surgery?
/ A. Bacterial vegetations
/ B. Cystic medial degeneration
/ C. Multiple small granulomas
/ D. Parasitic organisms
/ E. Polyarteritis nodosa

Explanation - Q: 2.5 Close

The correct answer is B. Cystic medial degeneration is a disruption and
fragmentation of the elastic tissue in aortic media, with formation of areas
devoid of elastin. These changes weaken the aortic wall, predispose for
dissection, and are seen in the majority of cases of aortic dissection.
Bacterial vegetations (choice A) are a feature of endocarditis.
Multiple small granulomas (choice C) can be seen in temporal arteritis and
Takayasu arteritis.
Parasitic organisms (choice D) do not usually affect the aorta; the organisms
of trichinosis and Chagas disease can affect the heart.
Polyarteritis nodosa (choice E) is a focal inflammation that usually involves
smaller blood vessels than the aorta.
Question 6 of 6
Which of the following conditions has been associated with this patient's
disease?
/ A. Cushing syndrome
/ B. Dandy-Walker syndrome
/ C. Kawasaki syndrome
/ D. Marfan syndrome
/ E. Tourette syndrome

Explanation - Q: 2.6 Close

The correct answer is D. Marfan syndrome is an autosomal dominant
connective tissue disease characterized by skeletal changes (e.g., tall
stature, long limbs, long fingers, lax joints), a tendency to develop
dislocations of the lens of the eye, and a tendency to develop aortic
dissection secondary to prominent cystic medial degeneration in the aortic
media. A similar condition, Ehlers-Danlos syndrome, also predisposes for
dissecting aneurysm. Other predisposing factors include congenital
cardiovascular abnormalities (e.g., coarctation of the aorta, patent ductus
arteriosus, bicuspid aortic valve) that increase the turbulence of blood flow in
the aorta, atherosclerosis, and trauma (including iatrogenic trauma during
arterial catheterization and cardiovascular surgical procedures). The other
conditions listed in the choices are unrelated to aortic dissection.
Cushing syndrome (choice A) is a characteristic pattern of physical changes
(truncal obesity, moon face, buffalo hump), biochemical/hormonal changes
(hypertension, altered carbohydrate and protein metabolism, amenorrhea),
and sometimes psychiatric disturbances that are seen in patients with
increased levels of adrenocortical hormones.
Dandy-Walker syndrome (choice B) is a congenital abnormality of the
cerebellum and fourth ventricle.
Kawasaki syndrome (choice C) is a febrile childhood disease that
predisposes for the formation of tiny aneurysms of the coronary arteries.
Tourette syndrome (choice E) is a motor and vocal tic disorder.




A 35-year-old man with no significant past medical history presents to clinic with
a six week history of worsening chest discomfort and pain.
He describes the pain as a substernal burning sensation that occasionally wakes
him up at night and is often worse after he eats. He
sometimes notices a sour taste in his mouth when he wakes up in the morning.
He has no dysphagia or odynophagia. The pain is unrelated
to exertion, and he jogs 3 miles every other day without difficulty or chest pain.
His vital signs and physical examination are normaI.
Question 1 of 6
Which of the following is the most likely diagnosis?
/ A. Acute viral pericarditis
/ B. Aortic dissection
/ C. Candida esophagitis
/ D. Gastroesophageal reflux disease (GERD)
/ E. Stable angina

Explanation - Q: 3.1 Close

The correct answer is D. A patient who presents with chest discomfort that
is burning in nature, and worsened after eating without symptoms of
dysphagia or odynophagia, most likely has gastroesophageal reflux disease
(GERD). GERD occurs when there is reflux of gastric contents into the
esophagus. This may occur with or without inflammation. It is often caused
by inappropriate relaxation of the lower esophageal sphincter. Certain foods
such as peppermint, caffeine, and high-fat and spicy foods are often
associated with GERD.
Acute viral pericarditis (choice A) would present with more severe and
sudden onset of chest pain that is relieved with leaning forward or sitting up.
Acute viral pericarditis is often associated with a prodrome and usually
presents with a fever. Occasionally, a pericardial friction rub can be heard on
exam.
Aortic dissection (choice B) would also present as sudden onset of severe
chest pain, which often radiates to the back. Patients can have hypotension,
depending on the severity of the dissection. Patients can also have unequal
pulses in their extremities if the dissection affects one of the major arteries
branching off the aortic arch.
Candida esophagitis (choice C) would present with dysphagia and
odynophagia. Patients also have oral thrush, and generally are
immunocompromised. These patients usually have a fever.
Stable angina (choice E) should present with typical chest pain that is
worsened after exertion. The fact that this patient can jog 3 miles without
difficulty goes against stable angina. Furthermore, he is young and does not
have any risk factors for cardiac disease such as hypertension, diabetes, or
hypercholesterolemia.
Question 2 of 6
Which of the following tests would be most likely to confirm the probable
diagnosis?
/ A. 24-hour ambulatory esophageal luminal pH monitoring
/ B. Cardiac angiogram
/ C. Chest radiograph
/ D. Exercise treadmill test
/ E. Serologic blood tests for H. Pylori infection

Explanation - Q: 3.2 Close

The correct answer is A. Twenty-four hour ambulatory esophageal luminal
pH monitoring is one of the most sensitive tests for GERD. In most cases,
the disease is diagnosed clinically by history, but pH monitoring would help
confirm the diagnosis.
Cardiac angiograms (choice B) are used to evaluate the coronary arteries
for signs of blockage, to evaluate heart function, or to evaluate cardiac valve
function.
A chest radiograph (choice C) can be used to evaluate the structures in the
thorax, but will not help confirm the diagnosis because GERD patients
generally have normal chest radiographs.
An exercise treadmill test (choice D) is used to evaluate patients who are
believed to have underlying coronary heart disease or to rule out heart
disease.
Serologic blood testing for H. pylori infection (choice E) only documents the
presence of a current infection or the history of an H. pylori infection. A past
or present infection does not confirm a diagnosis, because GERD can occur
in the setting with or without H. pylori. Furthermore, the role of H. pylori in
GERD is still unclear.
Question 3 of 6
The patient is treated with cimetidine, which completely relieves his symptoms.
Which of the following is the mechanism of action of this
medication?
/ A. Beta-1 adrenergic blockade
/ B. Histamine H2 receptor blockade
/ C. Inhibition of cell wall synthesis
/ D. Inhibition of cyclooxygenase
/ E. Smooth muscle relaxation
Explanation - Q: 3.3 Close

The correct answer is B. Cimetidine and other histamine H2 receptor
blockers such as ranitidine block the action of histamine on H2 receptors,
resulting in a decrease in gastric acid production, thus decreasing the
symptoms of GERD.
Beta-1 adrenergic blockade (choice A) (e.g., atenolol, metoprolol) is used to
lower blood pressure, which is not related to GERD.
Inhibition of cell wall synthesis (choice C) (e.g., amoxicillin) is a mechanism
that is used by many antibiotics. GERD can be associated with the presence
of H. pylori, but the treatment of H. pylori with antibiotics in GERD patients
remains controversial, and its benefit remains questionable.
Inhibition of cyclooxygenase (choice D) (e.g., ibuprofen, naproxen) does not
play a role in the treatment of GERD. GERD may or may not be associated
with inflammation of the esophagus, but anti-inflammatory agents may
actually worsen symptoms.
Smooth muscle relaxation (choice E) (e.g., nitroglycerin) does play a role in
the relief of esophageal spasm, but this patient does not complain of
dysphagia or odynophagia.
Question 4 of 6

The physician cautions the patient about cimetidine because of which of the
following potential side effects?
/ A. CNS depression
/ B. Hypertensive crisis
/ C. Inhibition of hepatic metabolism
/ D. Masking symptoms of hypoglycemia
/ E. Ototoxicity

Explanation - Q: 3.4 Close

The correct answer is C. Many drugs can lead to clinically significant drug
interactions via inhibition of the hepatic drug-metabolizing enzymes,
particularly the cytochrome P450 isozymes. This can lead to unwanted
elevations of plasma drug levels. Cimetidine is a classic example of one of
these drugs. Other examples include erythromycin, ketoconazole,
sulfonamides, quinidine, and disulfiram.
Benzodiazepines and barbiturates are examples of drugs that can cause
CNS depression (choice A).
MAO inhibitors prior to the ingestion of tyramine-containing foods can cause
a hypertensive crisis (choice B).
Beta blockers can mask the symptoms of hypoglycemia (choice D).
Aminoglycosides can produce ototoxicity (choice E). The risk of ototoxicity
may be further increased if the patient is also taking loop diuretics.

Question 5 of 6

The patient's symptoms are initially controlled on cimetidine. After 10 years, he
develops refractory symptoms, and the physician places him
on a proton pump inhibitor. Which of the following medications was most likely
prescribed?
/ A. Lansoprazole
/ B. Loperamide
/ C. Metoclopramide
/ D. Ondansetron
/ E. Ranitidine

Explanation - Q: 3.5 Close

The correct answer is A. Lansoprazole is a proton pump inhibitor and acts
directly to inhibit the gastric parietal cell hydrogen-potassium ATPase. It can
be used as the initial treatment for GERD, or for refractory cases.
Loperamide (choice B) is an anti-diarrheal agent, which inhibits peristalsis.
Using it in this setting may worsen the symptoms of GERD.
Metoclopramide (choice C) stimulates upper gastrointestinal motility. It can
be used in refractory cases of GERD, but it is not a proton pump inhibitor.
Ondansetron (choice D) is an antiemetic and acts by selectively
antagonizing serotonin 5-HT
3
receptors. It is primarily used is severe cases
of nausea, such as in patients receiving chemotherapy for cancer treatment.
Ranitidine (choice E) is also an histamine H2 receptor blocker, like
cimetidine. Some patients who do not respond to one histamine H2 receptor
blocker, may respond to another, but ranitidine blocks the action of histamine
on H2 receptors, resulting in a decrease in gastric acid production. It is not a
proton pump inhibitor.

Question 6 of 6
Histologic examination of the affected tissue shows Barrett's esophagus. This is
most correctly described as which of the following?
/ A. Adenocarcinoma
/ B. Esophageal stricture
/ C. H.Pylori infection
/ D. Localized outpouching of the esophageal wall
/ E. Metaplasia of the squamous epithelium

Explanation - Q: 3.6 Close

The correct answer is E. Patients who have long-standing GERD are at risk
for development of Barrett's esophagus, which is the replacement of the
normal esophageal squamous epithelium with columnar epithelium
(metaplasia). This is a premalignant lesion that needs to be monitored
regularly for the development of adenocarcinoma.
Adenocarcinoma (choice A) is a malignant lesion that can result from
cellular metaplasia, but Barrett's esophagus is the premalignant lesion that
occurs before the development of adenocarcinoma of the esophagus.
Esophageal strictures (choice B) can occur in patients with long-standing
GERD, but the presence of a stricture does not mean that there is cellular
dysplasia, or Barrett's esophagus.
H. pylori infection (choice C) can occur in the setting of GERD, but it is not
synonymous with Barrett's esophagus.
An esophageal diverticulum is a localized outpouching of the esophageal
wall (choice D). This is unrelated to Barrett's esophagus.


A 45-year-old man presents with a 3-day history of persistent, severe chest pain.
Prior to this, he had flu-Iike symptoms for 2 weeks, including
fever, cough, myalgias, and arthralgias. His pain is worse when he takes a deep
breath and is improved when he sits up. On physical
examination, he is febrile, and his pulse is 110/min. His oxygen saturation is
normaI, and his breath sounds are equal and clear to
auscultation over all lung fields. There is a scratching and scraping, high-pitched
sound on auscultation of the heart over the left third
intercostal space, which is increased when the patient is sitting forward.







Question 1 of 4

Which of the following is the most likely diagnosis?
/ A. Acute pericarditis
/ B. Aortic dissection
/ C. Pneumonia
/ D. Pulmonary embolus
/ E. Tension pneumothorax

Explanation - Q: 4.1 Close

The correct answer is A. This patient has symptoms that are typical of
inflammation of the pericardial sac. In addition, the sound that is heard over
his heart is a pericardial friction rub. Acute pericarditis is often associated
with viral syndromes, connective tissue diseases, renal failure, myocardial
infarction, and tumor invasion of the pericardium.
Aortic dissection (choice B) will also present with severe chest pain, but it is
tearing in quality, and is not positional, nor pleuritic in nature.
Pneumonia (choice C) may present similarly, but auscultation of the lung
fields should reveal abnormal breath sounds, and a pericardial friction rub
should not be heard.
Pulmonary embolus (choice D) can present with chest pain that is worse
with deep breaths. However, it is not positional in nature, and it is not
associated with a pericardial friction rub. Depending on the size of the
embolus, the oxygen saturation may be abnormal.
Tension pneumothorax (choice E) is not associated with flu-like symptoms
nor a pericardial friction rub. Furthermore, there should be an absence of
breath sounds over the affected part of the lung.
Question 2 of 4
Which of the following would help confirm the diagnosis?
/ A. Angiogram showing a clot in one of the coronary arteries
/ B. Chest radiograph showing multiple emphysematous bullae
/ C. CT scan of the chest showing a widened mediastinum
/ D. EIectrocardiogram showing diffuse ST elevation
/ E. Endoscopy revealing esophageal varices


Explanation - Q: 4.2 Close

The correct answer is D. Acute pericarditis often presents with diffuse ST
elevation on an electrocardiogram.
An angiogram showing a clot in one of the coronary arteries (choice A)
would be seen in a patient having an acute myocardial infarction.
A chest radiograph showing multiple emphysematous bullae (choice B)
would be seen in a chronic smoker, and would be a possible cause of
spontaneous pneumothorax.
CT scan of the chest showing a widened mediastinum (choice C) would be
seen in a patient with an acute aortic dissection.
Endoscopy revealing esophageal varices (choice E) is seen in patients with
chronic liver disease or portal hypertension.

Question 3 of 4
Other than an antecedent viral syndrome, which of the following conditions can
predispose a patient to this problem?
/ A. AIcohol abuse
/ B. Liver failure
/ C. Peptic ulcer disease
/ D. Recent total hip replacement
/ E. Renal failure

Explanation - Q: 4.3 Close

The correct answer is E. Patients with renal failure or uremia can often
present with a fibrinous or serofibrinous pericarditis.
Alcohol abuse (choice A) and liver failure (choice B) do not necessarily
predispose a patient to pericarditis, nor are they associated with pericarditis.
Peptic ulcer disease (choice C) may cause epigastric pain that can be
confused with chest pain, and patients may have recurrent bleeding, but this
disorder is not associated with pericarditis.
A recent total hip replacement (choice D) predisposes patients to the
development of a deep venous thrombosis, which can embolize and cause a
pulmonary embolus. This is not associated with pericarditis.

Question 4 of 4
The patient is treated with a nonsteroidal anti-inflammatory agent. Which of the
following was prescribed?
/ A. AIIopurinol
/ B. Gemfibrozil
/ C. Indomethacin
/ D. Labetalol
/ E. Methocarbamol

Explanation - Q: 4.4 Close

The correct answer is C. Indomethacin or any of the other nonsteroidal
anti-inflammatory agents can be used in the treatment of acute pericarditis.
Allopurinol (choice A) is used for the treatment of gout. It inhibits xanthine
oxidase, which decreases the production of uric acid.
Gemfibrozil (choice B) is a lipid lowering agent used in patients with
hypercholesterolemia.
Labetalol (choice D) is a beta blocker and is used in hypertensive
emergencies.
Methocarbamol (choice E) is a muscle relaxant, which helps to relieve pain
associated with muscle spasms.


A 62-year-old white man is brought to the emergency department after
experiencing twenty minutes of sudden onset, crushing, substernal
chest pain radiating to his neck and left shoulder. The pain started when the
patient was lifting a heavy trolley. He denies shortness of breath,
back pain, or loss of consciousness. In the emergency department, he is given
sublingual nitroglycerin tablets, which relieve the pain almost
immediately. The patient has a history of hypertension, drinks beer several times
a week, smokes one pack of cigarettes per day, and does
not exercise. He is not on any medications. On physical examination, he is a
diaphoretic patient with a temperature of 36.6 C (98 F), blood
pressure of 150/80 mm Hg, pulse of 84/minute, and respirations of 16/minute.
His heart and breath sounds are normaI, his abdomen is soft
and non-tender, and no bruits were heard. A chest radiograph is normaI. An
electrocardiogram confirms T-wave inversion in leads V2 and
V3, with an otherwise normal sinus rhythm. The patient is started on atenoloI, a
nitroglycerin paste patch, and aspirin. He is admitted into the
hospitaI, where he remains pain free. Troponin levels taken every eight hours for
the next 24 hours are all within normal limits.

Question 1 of 5

Which of the following is the most likely diagnosis?
/ A. Angina
/ B. Gastroesophageal reflux disease
/ C. Myocardial infarction
/ D. Pulmonary embolism
/ E. Thoracic aortic aneurysm rupture

Explanation - Q: 5.1 Close

The correct answer is A. The patient has four independent cardiac risk
factors (male sex, smoker, hypertensive, inactivity). He presented with typical
cardiac chest pain on exertion, accompanied by diaphoresis, which was
relieved with nitroglycerin. This is typical of angina. T-wave inversion is also
consistent with ischemia. However, this is unlikely to be a myocardial
infarction (choice C) because there were no raised ST segments on the
electrocardiogram and his cardiac enzymes (e.g., troponin) were not
elevated. He would probably require a cardiac stress test to see if the EKG
changes could be reproduced in a controlled stressful environment (e.g.,
treadmill). On this visit, his symptoms are better described as being anginal
with ischemic EKG findings on the anterior cardiac wall.
Gastroesophageal reflux disease (choice B) would present with a
longitudinal burning sensation in the epigastric area, usually after the
consumption of large meals, caffeinated products, exercising, or lying flat
after eating. It is treated with H2-blockers and proton pump inhibitors, along
with eating smaller meals and the avoidance of caffeine. It is an important
part of the differential diagnosis of chest pain.
The patient has risks for thromboembolism, including smoking and inactivity,
which could lead to pulmonary embolism. Pulmonary embolism (choice D)
would be suggested if the patient had a sudden onset of shortness of breath
and chest pain. In addition, on examination, one would look for tachycardia,
tachypnea, and diaphoresis. The work-up includes D-dimers, a lower leg
Doppler, prothrombin time (PT), partial thromboplastin time (PTT), and a
ventilation/perfusion scan. Treatment would be anticoagulation with heparin,
and then eventually with Coumadin.
Rupture of a thoracic aortic aneurysm (choice E) would present as severe
chest pain radiating to the back. The patient would typically be elderly. The
blood pressure may drop and there will sometimes be a discrepancy in arm-
to-arm blood pressure readings.
Question 2 of 5
Which of the following vessels is most likely affected in this patient?
/ A. Circumflex coronary artery
/ B. Left anterior descending artery
/ C. Marginal artery
/ D. Posterior descending artery
/ E. Right coronary artery

Explanation - Q: 5.2 Close

The correct answer is B. The electrocardiogram revealed inverted T-waves
(suggestive of ischemia) in leads V2 and V3. These leads correspond mainly
to the anterior wall of the left ventricle, where the ischemia is likely to have
taken place. The left anterior descending artery is a terminal branch of the
left coronary artery (the other branch being the circumflex artery). It passes
posterior to the pulmonary artery, then anteriorly between this vessel and the
left auricle to reach the anterior longitudinal sulcus. Since it supplies blood to
the anterior walls of both ventricles, ischemia in this location would manifest
as T-wave inversion in leads V1 to V4.
The circumflex artery (choice A) supplies the lateral wall of the heart (atrium
and ventricle). Ischemia with this artery would manifest as T-wave inversion
of leads I, aVL, and leads V5 and V6. The circumflex artery branches off the
left coronary artery and follows the left part of the coronary sulcus giving
branches to the left atrium and ventricle.
The marginal artery (choice C) is a branch of the right coronary artery, and it
supplies branches to both surfaces of the right ventricle. Disease in this
vessel is not associated with specific EKG changes.
Inverted T-waves in leads II, III, and aVF would correspond mainly to
ischemia of the inferior wall of the left ventricle. The main artery supply to this
area is the right coronary artery (choice E). It arises from the anterior aortic
sinus, then passes between the conus arteriosus and the right auricle, and
then runs in the right portion of the coronary sulcus. From there it continues
on the diaphragmatic surface of the heart from the right to left, as far as the
posterior longitudinal sulcus. It eventually arrives at the apex of the heart in
the form of the posterior descending artery (choice D).



Question 3 of 5

Which of the following is most likely responsible for this patient's symptoms?
/ A. Atheromatous plaque rupture
/ B. Hyaline arteriosclerosis
/ C. Hyperplastic arteriosclerosis
/ D. Thrombus embolization
/ E. Vasospasm of the coronary vessels.

Explanation - Q: 5.3 Close

The correct answer is A. Several hypotheses have been formulated about
atheromatous plaque formation. One hypothesis involves endothelial wall
damage, resulting in monocyte and platelet adhesion and migration of
monocytes into the intima from the lumen and smooth muscle media.
Another hypothesis involves hyperlipidemia causing an increased rate of LDL
(low density lipoprotein) penetration into the artery wall, causing endothelial
injury and the promotion of foam cells. Once the atheromatous plaque is
formed, any rupture (e.g., with vasospasm) will expose subendothelial
collagen, promoting platelet adherence, and the eventual aggregation of
platelets. This may cause a total occlusion of the coronary vessel (as in the
case of an infarction) or a transient subocclusion (that leads to angina due to
ischemia).
Arteriosclerosis typically produces less acute effects than the occlusion seen
with atheromatous plaque formation. Hyaline arteriosclerosis (choice B) is
microangiopathy seen in hypertensive and diabetic patients. It is due to
leakage of plasma components across the vascular endothelium and
increasing extracellular matrix production by smooth muscle walls. This
eventually narrows the arteriolar lumina causing reduced blood flow for that
organ e.g., the kidney.
Hyperplastic arteriosclerosis (choice C) is usually seen in "malignant" or
severe hypertension and is characterized by laminated thickenings of the
wall of the lumina. The most commonly affected sites are the kidney,
gallbladder, pancreas, and intestines.
An embolus is a detached intravascular solid, liquid, or gaseous mass
carried by the blood to a site distal to the point of origin. Thrombus
embolization (choice D) represents 99% of all emboli. An example is a
pulmonary (thrombo)embolism. Although atherosclerosis may provide a site
for thrombus formation and embolization, this is not the most common
mechanism for angina.
Vasospasm (choice E) may cause angina more rarely; this is called
Prinzmetal's angina. There may be essentially no major atheromatous
changes in the coronary vessels. Prinzmetal's angina is often treated with
calcium channel blockers.

Question 4 of 5
The cardiac enzyme marker troponin is normally found predominantly in which of
the following sites?
/ A. Cell membranes of myocardial cells
/ B. Endoplasmic reticulum
/ C. Mitochondria
/ D. Myofibrils
/ E. Nuclei
/ F. Ribosomes

Explanation - Q: 5.4 Close

The correct answer is D. Troponins are tightly bound structural proteins that
regulate the calcium-mediated interaction of actin and myosin in striated
muscle (myofibrils). Troponin release would indicate cell death, such as that
seen in infarction. The rise of this marker after cardiac injury parallels that of
creatine kinase (CK) (another cardiac enzyme marker). However, in contrast
to CK, baseline levels of troponin are undetectable in normal volunteers.
Neither troponin nor creatine kinase are found in cell membranes (choice A)
of the myocardium.
Creatine kinase is a cardiac marker that resides in the cytoplasm and
mitochondria (choice C). During cardiac injury, destruction of the cell walls
and sarcolemma of the myocardial cells causes rapid release of the CK into
the bloodstream. It is also released in noninfarction cardiac injuries (e.g.,
blunt chest trauma).
The nucleus (choice E), ribosomes (choice F), and endoplasmic reticulum
(choice B) are sites of transcription, translation and post-translational
modification of troponin. Troponin is then incorporated into the myofibril
structure via covalent attachment.

Question 5 of 5

Which of the following is the mechanism of action of nitroglycerin?
/ A. Increases stroke-work index of the heart
/ B. Maintains coronary perfusion despite fluctuations in blood pressure
/ C. Relaxes bronchial smooth muscle
/ D. Relaxes vascular smooth muscle
/ E. Unblocks coronary vessels by fibrinolysis

Explanation - Q: 5.5 Close

The correct answer is D. Relaxation of vascular smooth muscle is the
principal pharmacologic action of nitroglycerin. Nitroglycerin (glyceryl
trinitrate) is a therapeutically active member of the nitrate group of drugs.
Nitrates improve oxygen supply to the myocardium (dilation of the coronary
artery, reduction of the end-diastolic pressure in the left ventricle) and
reduces myocardial oxygen requirements (dilation of the peripheral arteries,
lowering of the systemic blood pressure). Reduction of venous return is a
particular benefit (reduction of preload) in the event of heart failure. In
smooth muscle cells, nitrates increase the intracellular concentration of nitric
oxide (NO). NO stimulates guanylate cyclase, forming cGMP, which probably
dephosphorylates the phosphorylated form of myosin light chain, leading to
smooth muscle relaxation.
Myocardial oxygen consumption or demand (as measured by the pressure-
rate product, tension-time index, and stroke-work index, choice A) is
decreased by both the arterial and venous effects of nitroglycerin, and a
more favorable supply-demand ratio can be achieved. Therapeutic doses of
intravenous nitroglycerin reduce systolic, diastolic, and mean arterial blood
pressures.
Effective coronary perfusion pressure (choice B) is usually maintained, but
can be compromised if blood pressure falls excessively or increased heart
rate decreases diastolic filling time.
The bronchial smooth muscle is unaffected by nitrates. Bronchodilation
(choice C) can be achieved by stimulation of beta-2 adrenergic receptors.
Inhalation of beta-agonists, such as albuterol, can help reduce wheezing
during asthmatic episodes.
Reperfusion of coronary vessels through fibrinolysis (choice E) is the action
of fibrinolytic agents like alteplase or streptokinase. They are used primarily
in myocardial infarctions. If the agent is administered done quickly enough,
the infarct size is reduced and left ventricular function is preserved, thereby
reducing mortality.


A 19-year-old college student is referred to a family medicine physician for an
abnormal heart sound heard on a routine school physical
examination. On questioning, he denies chest pain or shortness of breath, but
says he does occasionally experience palpitations. He is
adopted and does not know his family history. On physical examination, he is
201 cm (79 inches) talI, weighs 73 kg (160 Ib), and has long
arms and legs. On examination, there is a mid-systolic click, heard best at the
apex. The click also occurs earlier when the patient stands or
performs a Valsalva maneuver.
Question 1 of 6
Which of the following cardiac abnormalities does this patient most likely have?
/ A. Aortic regurgitation
/ B. Aortic stenosis
/ C. Mitral stenosis
/ D. Mitral valve prolapse
/ E. Tricuspid regurgitation

Explanation - Q: 6.1 Close

The correct answer is D. Mitral valve prolapse is a common valvular
abnormality that can be benign and asymptomatic, but it can also cause
palpitations, chest pain, and shortness of breath. It is due to stretching of the
posterior mitral valve leaflets, resulting in prolapse of the valve. This change
produces the classic midsystolic click, that is sometimes is followed by a
murmur.
Aortic regurgitation (choice A) would result in a blowing, diastolic murmur
heard best at the left second intercostal space.
Aortic stenosis (choice B) would result in a systolic ejection murmur heard
best at the right second intercostal space.
Mitral stenosis (choice C) produces a low-pitched diastolic murmur heard
best over the apex. It is often preceded by an opening snap.
Tricuspid regurgitation (choice E) produces a blowing systolic murmur heard
best at the left lower sternal border.
Question 2 of 6
An echocardiogram is performed. Which of the following would likely be observed
during the study?
/ A. Ballooning of the aortic valve into the ventricle during diastole
/ B. Ballooning of the mitral valve into the atrium during diastole
/ C. Rupture of the aortic valve
/ D. Rupture of the tricuspid valve
/ E. Stenotic mitral valve

Explanation - Q: 6.2 Close

The correct answer is B. Ballooning of the mitral valve into the atrium
during diastole is diagnostic of mitral valve prolapse.
Ballooning of the aortic valve into the ventricle during diastole (choice A)
does not occur in mitral valve prolapse. In addition, this would not result in a
mid-systolic click.
Rupture of the aortic valve (choice C) could result in severe hemodynamic
compromise, especially if it were acute, due to a large regurgitant flow of
blood. A harsh diastolic murmur would be heard on examination.
Rupture of the tricuspid valve (choice D) would cause a blowing systolic
murmur heard over the left lower sternal border.
A stenotic mitral valve (choice E) is not associated with mitral valve
prolapse. In mitral stenosis, the valve is thick and stiff. There is a low-pitched
diastolic murmur, which is often preceded by an opening snap.

Question 3 of 6
Which of the following genetic disorders can be associated with this abnormality?
/ A. KIinefelter syndrome
/ B. Marfan syndrome
/ C. Osler-Weber-Rendu syndrome
/ D. Tay-Sachs disease
/ E. Wilson disease

Explanation - Q: 6.3 Close

The correct answer is B. Marfan syndrome is a connective tissue defect
due to a deficiency in fibrillin. This results in defects in skeletal, visual, and
cardiovascular structures, such as mitral valve prolapse and/or aortic
aneurysms.
Klinefelter syndrome (choice A) is chromosomal disorder, most often
characterized by the karyotype 47,XXY. It results in hypogonadism, and in
some cases, mild mental retardation.
Osler-Weber-Rendu syndrome (choice C) is also known as hereditary
hemorrhagic telangiectasia. It is characterized by telangiectasias of the skin
and mucous membranes and hemorrhage at these sites.
Tay-Sachs disease (choice D) is a lysosomal storage disease caused by a
deficiency of hexosaminidase A. It results in central nervous system
degeneration, mental retardation, motor deterioration, and blindness.
Wilson disease (choice E) is a disorder of copper metabolism. It is
characterized by decreased serum ceruloplasmin and results in
accumulation of copper in the liver, kidney, brain, and cornea.

Question 4 of 6
This patient is diagnosed with a genetic disorder associated with the valvular
abnormality. Which of the following modes of inheritance does
this disease follow?
/ A. Autosomal dominant inheritance
/ B. Autosomal recessive inheritance
/ C. Mitochondrial inheritance
/ D. X-Iinked dominant inheritance
/ E. X-Iinked recessive inheritance

Explanation - Q: 6.4 Close

The correct answer is A. Marfan syndrome is inherited in an autosomal
dominant manner. Usually, in diseases with this pattern of inheritance, one
heterozygous parent carries a gene with the phenotypic expression of the
disorder, and the other parent is normal.
Autosomal recessive inheritance (choice B) usually occurs when both
parents are heterozygotes and do not phenotypically manifest the disorder.
Cystic fibrosis is an example of an autosomal recessive disease.
Mitochondrial inheritance (choice C) is due to the maternal transmission of
mitochondrial genes. Mitochondrial myopathies are inherited in this pattern.
X-linked dominant inheritance (choice D) is a rarity. The heterozygous
female and the hemizygous male will manifest the phenotype.
X-linked recessive inheritance (choice E) is most often the result of a
heterozygous female parent and a genotypically and phenotypically normal
male parent. Male children who inherit the affected X chromosome will
manifest the disorder, but the female children will only be carriers.
Question 5 of 6
Over the next year the patient develops chest pain, shortness of breath, and
progressive fatigue. On auscultation, he has a midsystolic click,
which is now followed by a high-pitched, blowing systolic murmur. Which of the
following is the most likely cause of this new development?
/ A. Aortic aneurysm
/ B. Aortic stenosis
/ C. Mitral regurgitation
/ D. Mitral stenosis
/ E. Myocardial infarction

Explanation - Q: 6.5 Close

The correct answer is C. In severe cases of mitral valve prolapse, patients
can develop mitral regurgitation, which may require surgical treatment if the
symptoms are severe.
Aortic aneurysm (choice A) can occur in Marfan disease, but it is not
associated with mitral prolapse or a systolic murmur.
Aortic stenosis (choice B) causes a systolic ejection murmur heard best at
the right second intercostal space, which often radiates to the carotid
arteries. It is not associated with extra heart sounds.
Mitral stenosis (choice D) is not a complication of mitral valve prolapse.
Furthermore, it causes a diastolic murmur, which can be preceded by an
opening snap.
Myocardial infarction (choice E) is not associated with mitral valve prolapse.
Question 6 of 6
The patient is treated with propanoloI. What is the mechanism of action of this
medication?
/ A. AIpha-1 selective adrenergic blockade
/ B. Beta-1 selective adrenergic blockade
/ C. Calcium channel blockade
/ D. Nonselective beta adrenergic blockade
/ E. Nonselective alpha adrenergic blockade

Explanation - Q: 6.6 Close

The correct answer is D. Propanolol, as well as timolol, pindolol, nadolol,
and labetalol, all exhibit nonselective beta-adrenergic blockade. They are
generally used in the treatment of hypertension, angina, and arrhythmias.
Alpha-1 selective adrenergic blockade (choice A) is a mechanism used by
prazosin, terazosin, and doxazosin. These agents can be used to treat
hypertension or urinary retention due to benign prostatic hypertrophy.
Beta-1 selective adrenergic blockade (choice B) is a mechanism used by
metoprolol, atenolol, and esmolol. Unlike the nonselective beta-adrenergic
blockers, these medications selectively block beta 1 receptors over beta 2
receptors.
Calcium channel blockade (choice C) is a mechanism used by nifedipine,
diltiazem, and verapamil. They can also be used to treat hypertension and
angina. Verapamil and diltiazem can also be effective in the treatment of
arrhythmias.
Nonselective alpha-adrenergic blockade (choice E) is a mechanism used by
phenoxybenzamine and phentolamine. They can be used in the treatment of
pheochromocytoma.


A 21-year-old woman presents to an urgent care clinic with complaints of sharp,
knife-Iike chest pain and shortness of breath for the past 4
days. She states that the pain is worse with inspiration, coughing, and
movement. However, it is not related to position. She also complains
of fever, chills, and a dry cough. She has a history of some type of rheumatologic
disease, but she does not know which one. She was
previously seen by a rheumatologist, but was lost to follow up, and stopped
taking her medications 6 months ago. Her physical examination is
notable for a temperature of 37.8 C (100 F), tachycardia, decreased breath
sounds at both lung bases, and a friction rub. There is no pinpoint
chest wall tenderness.
Question 1 of 6
Which of the following is the most likely cause of this patient's chest pain?
/ A. Angina pectoris
/ B. Anxiety
/ C. Esophageal spasm
/ D. PIeuritis
/ E. Rib fracture

Explanation - Q: 7.1 Close

The correct answer is D. Pleuritis is inflammation of the parietal pleura of
the lung, which can often result in a friction rub heard on auscultation.
Patients can also have pleural effusions. Pleuritis is associated with many
different disease processes, including collagen vascular diseases and
infections.
Angina pectoris (choice A) is chest pain related to underlying coronary artery
disease. The pain is usually substernal, worse with exertion, and relieved by
rest. It is not associated with fever or chills, nor is it pleuritic in nature.
Anxiety (choice B) can often be a cause of chest pain. It is a clinical
diagnosis and should be associated with other symptoms of anxiety. It is not
pleuritic in nature, and is not associated with a cough, fever, or chills.
Esophageal spasm (choice C) is due to uncoordinated contractions of the
esophagus. It is generally associated with dysphagia.
Rib fracture (choice E) should result in well-localized chest pain. There is
pinpoint chest wall tenderness, and bone crepitus can be noted on palpation.
Question 2 of 6
On a chest radiograph, the patient is noted to have bilateral pleural effusions.
Evaluation of the fluid would most likely reveal which of the
following?
/ A. Exudative effusion
/ B. Low pleural fluid LDH
/ C. PIeural fluid to serum lactate dehydrogenase (LDH) ratio <0.6
/ D. PIeural fluid to serum protein ratio <0.5
/ E. Transudative effusion

Explanation - Q: 7.2 Close

The correct answer is A. This patient has an exudative pleural effusion
associated with an underlying rheumatologic condition.
Pleural fluid to serum protein ratio <0.5 (choice D), pleural fluid to serum
lactate dehydrogenase (LDH) ratio <0.6 (choice C), and low pleural fluid
LDH (choice B) are all diagnostic of a transudative effusion.
A transudative effusion (choice E) occurs when systemic factors that affect
the formation of pleural fluid are altered. It is associated with heart failure,
cirrhosis, and nephrotic syndrome.
Question 3 of 6
The patient also gives a history of symmetric joint pain and swelling as well as a
malar rash and photosensitivity. Which of the following is the
most likely diagnosis?
/ A. Ankylosing spondylitis
/ B. Polyarteritis nodosa
/ C. Psoriatic arthritis
/ D. Rheumatoid arthritis
/ E. Systemic lupus erythematous (SLE)

Explanation - Q: 7.3 Close

The correct answer is E. Systemic lupus erythematous (SLE) is an
autoimmune disorder that can affect multiple organ systems, especially
joints, serous membranes, lungs, skin, and kidneys. It is characterized by the
presence of pathogenic autoantibodies and immune complexes, which cause
cellular and tissue damage.
Ankylosing spondylitis (choice A) is a chronic disease in which inflammatory
changes and new bone formation occur at the attachment of tendons and
ligaments to bone. It often affects the spine and sacroiliac joints, and may
result in rigidity and fixation of the spine.
Polyarteritis nodosa (choice B) is an immune complex vasculitis, which
involves small and medium sized arteries. It can affect any organ system,
and predominantly occurs in men.
Psoriatic arthritis (choice C) is an arthritis associated with psoriasis. It can
present in many different ways, including a monoarthritis or a polyarthritis,
which can be symmetrical or asymmetrical.
Rheumatoid arthritis (choice D) is a chronic inflammatory disorder that
primarily affects the joints. It can produce extra-articular manifestations
including arteritis, scleritis, and pleural involvement, but it is not associated
with a malar rash or photosensitivity.
Question 4 of 6
Evaluation of the malar rash would most likely reveal which of the following?
/ A. Coral red fluorescence under Wood's lamp
/ B. Hyperplasia of all components of the epidermis
/ C. IgG autoantibodies directed against the epidermal basement membrane
/ D. Immune complex deposits at the dermaI-epidermal junction
/ E. Proliferation of the epidermis and parakeratosis


Explanation - Q: 7.4 Close

The correct answer is D. Immune complex deposits at the dermal-
epidermal junction are classically associated with the malar rash of SLE.
Coral red fluorescence under Wood's lamp (choice A) evaluation occurs in
erythrasma, which is a chronic bacterial infection caused by
Corynebacterium minutissimum.
Hyperplasia of all components of the epidermis (choice B) occurs in lichen
simplex chronicus, which is a well-circumscribed area of lichenification due to
repeated physical trauma such as scratching or rubbing.
IgG autoantibodies directed against the epidermal basement membrane
(choice C) cause a disease known as bullous pemphigoid. It is an
autoimmune disorder, which presents as chronic bullous eruptions.
Proliferation of the epidermis and parakeratosis (choice E) are associated
with psoriasis, which is a chronic inflammatory process characterized by
erythematous papules and plaques with silvery scaling.


Question 5 of 6
Which of the following laboratory tests would help confirm the diagnosis of the
underlying rheumatologic disorder?
/ A. Antibodies to single-stranded DNA
/ B. Antibodies to Sm antigen
/ C. EIevated C reactive protein
/ D. EIevated sedimentation rate
/ E. Rheumatoid factor

Explanation - Q: 7.5 Close

The correct answer is B. Antibodies to Sm antigen as well as antibodies to
double-stranded DNA are highly specific for systemic lupus erythematous.
Antibodies to single-stranded DNA (choice A) can be found in drug drug-
induced lupus, but not in idiopathic SLE.
Elevated C reactive protein (choice C) and elevated sedimentation rate
(choice D) often occur in SLE, especially when it is active. However, these
tests are nonspecific and can be elevated whenever there is inflammation or
infection. They do not confirm the diagnosis of SLE.
Rheumatoid factor (choice E) is often positive in patients with rheumatoid
arthritis. It is not associated with SLE.
Question 6 of 6
The patient is treated empirically with levofloxacin for a possible pneumonia.
Which of the following is the mechanism of action of this drug?
/ A. Inhibition of cell wall synthesis
/ B. Inhibition of DNA gyrase and topoisomerase IV
/ C. Inhibition of ergosterol synthesis
/ D. Inhibition of protein synthesis by blocking translocation
/ E. Inhibition of squalene epoxidase

Explanation - Q: 7.6 Close

The correct answer is B. Levofloxacin is a bactericidal antibiotic that inhibits
DNA gyrase and topoisomerase IV. It belongs to the group of antibiotics
known as fluoroquinolones, which also includes ciprofloxacin and
norfloxacin.
Penicillin is an example of an antibiotic that inhibits cell wall synthesis
(choice A).
Fluconazole is an example of an antifungal agent that inhibits ergosterol
synthesis (choice C).
Macrolides inhibit protein synthesis by blocking translocation (choice D). The
macrolides include erythromycin, clarithromycin, and azithromycin.
Inhibition of squalene epoxidase (choice E) results in decreased cell
membrane ergosterol synthesis. This is a mechanism of action used by
antifungal agents, including terbinafine.

A 55-year-old man consults a physician because he has become increasingly
constipated over the last 2 months. This has occurred despite
attempts to increase fiber in his diet and to markedly increase his physical
activity. Over-the-counter laxatives have been mostly ineffective. On
further questioning, the man reports that he has twice seen fresh blood on his
stools, which he attributed to hemorrhoids. He has also noticed a
sense of fullness and discomfort in his lower left abdominal quadrant, but
attributed these findings to his constipation. Digital examination is
negative. Colonoscopy demonstrates an area of stricture with surface ulceration
10 cm above the anal verge. The distal colon is surgically
resected. Pathologic examination demonstrates a napkin-ring lesion with marked
thickening of the mucosal layer of the rectum. On cross-
section, the lesion is seen to extend through the rectal muscle and into, but not
through, the adjacent serosal adipose tissue
Question 1 of 6
Which of the following is the most likely diagnosis?
/ A. Adenocarcinoma
/ B. Hyperplastic polyp
/ C. Squamous cell carcinoma
/ D. Tubular adenoma
/ E. Villous adenoma

Question 2 of 6
Further evaluation fails to demonstrate the patient's disease in either lymph
nodes or distant sites. This patient's disease would be staged as
which of the following?
/ A. Duke's classification A
/ B. Duke's classification B1
/ C. Duke's classification B2
/ D. Duke's classification C1
/ E. Duke's classification C2
/ F. Duke's classification D

Explanation - Q: 1.2 Close

The correct answer is C. The Duke's classification is commonly used for
staging of colorectal carcinoma. This patient has stage B2, characterized by
invasion through the muscularis propria and without lymph node involvement
or distant metastases. Duke's classification B2 has a 55-65% 5-year survival
rate.
Duke's classification A (choice A) has tumor limited to the mucosa with no
metastatic disease, and has a 5-year survival of more than 90%.
Duke's classification B1 (choice B) has invasion into the muscularis propria,
but no lymph node or distant metastases; this stage has a 5-year survival of
70-85%.
Duke's classification C1 (choice D) has invasion into the muscularis propria
and lymph node but not distant metastatic disease; the 5-year survival is 45-
55%.
Duke's classification C2 (choice E) has invasion through the muscularis
propria and lymph node but not distant metastases; the 5-year survival is 20-
30%.
Duke's classification D (choice F) is given whenever distant metastatic
disease is present, and has a 5-year survival of less than 1%.
Question 3 of 6
Which of the following lesions would yield the poorest prognosis in this patient?
/ A. Hyperplastic polyp
/ B. Juvenile polyp
/ C. Peutz-Jeghers polyp
/ D. Tubular adenoma
/ E. Villous adenoma

Explanation - Q: 1.3 Close

The correct answer is E. The adenomatous polyps, which are considered to
have some potential for undergoing malignant transformation, include both
villous adenomas and tubular adenomas (choice D). Villous adenomas tend
to be larger and have more severe atypia than tubular adenomas, and
consequently have a greater risk of progression to frank carcinoma.
Hyperplastic polyps (choice A), juvenile polyps (choice B), and Peutz-
Jeghers polyps (choice C) are not considered to be premalignant lesions.
Question 4 of 6
One of the acquired genetic changes that can occur during the progression of
this disease is the loss of the p53 site on chromosome 17. The
gene product of this site is thought to function as which of the following?
/ A. Apoptosis regulator
/ B. DNA repair regulator
/ C. Growth factor
/ D. Growth factor receptor
/ E. Signal transduction inhibitor

Explanation - Q: 1.4 Close

The correct answer is A. The biochemistry of tumor cells has been an
active topic of research over the last few decades, and many molecular
changes have been elucidated. p53 acts as a cell cycle and apoptosis
(programmed cell death) regulator, and a loss of p53 activity has been linked
to progression of a late adenomatous polyp to adenocarcinoma of the colon.
BRCA-1 and BRCA-2, which are important in breast cancer, are examples of
gene products that act as DNA repair regulators (choice B).
Examples of growth factors (choice C) important in tumor growth include
PDGF (platelet-derived growth factor) and FGF (fibroblast growth factor).
An example of a growth factor receptor (choice D) important in tumor growth
is the EGF (epidermal growth factor) receptor.
The APC gene product, discussed in the previous question, acts as an
inhibitor of signal transduction (choice E).
Question 5 of 6
If the patient was unable to tolerate surgery, the treatment of choice would be
which of the following?
/ A. Cisplatin
/ B. Cyclophosphamide
/ C. FIuorouracil
/ D. Methotrexate
/ E. Sulfasalazine

Explanation - Q: 1.5 Close

The correct answer is C. In the treatment of colorectal cancer, surgical
resection is the treatment of choice for virtually all patients who have
resectable lesions and can tolerate general anesthesia. Regional lymph node
dissection is generally recommended to determine staging, which
subsequently guides decisions for pharmacological therapy. Adjuvant
chemotherapy generally includes treatment with fluorouracil and either
levamisole or leucovorin. This therapy has been shown to reduce mortality
by about one-third. Fluorouracil, or 5-FU, is an antimetabolite that blocks the
methylation reaction of deoxyuridylic acid to thymidylic acid. This interferes
with DNA synthesis, and to a lesser extent, RNA synthesis. 5-FU is indicated
for the palliative management of colorectal, breast, stomach, and pancreas
carcinoma. This agent is commonly associated with the development of
nausea, vomiting, alopecia, severe ulceration of the oral and GI mucosa, as
well as dermopathy (erythematous desquamation of the palms and soles).
Cisplatin (choice A) is an antineoplastic agent that exerts its antineoplastic
activity by binding to DNA, producing intrastrand cross-links, and forming
DNA adducts. Cross-linkage of DNA strands blocks the synthesis of DNA,
RNA, and protein. This agent is commonly used to treat solid tumors, such
as metastatic testicular carcinoma, ovarian carcinoma, as well as bladder
carcinoma. Aside from immunosuppression, the most common side effect of
this agent is severe persistent emesis.
Cyclophosphamide (choice B) is an antineoplastic commonly used to treat a
variety of malignant diseases, such as Hodgkin disease, as well as several
types of leukemia. It is associated with the development of hemorrhagic
cystitis in approximately 7 -12% of all patients receiving this medication. This
medication is an alkylating agent related to the nitrogen mustards.
Methotrexate (choice D) competitively inhibits dihydrofolic acid reductase. It
is indicated for the treatment of severe, active, rheumatoid arthritis in adults
who have an insufficient response with conventional therapies. It is also used
in antineoplastic therapeutic regimens, such as those for ALL, cancers of the
head, neck, and breast. It is contraindicated in pregnancy, nursing mothers
and hepatic insufficiency, as well as in patients with AIDS, blood dyscrasias,
bone marrow hypoplasia, leukopenia, and severe anemia.
Sulfasalazine (choice E) is a competitive antagonist of para-aminobenzoic
acid (PABA). It is indicated for the treatment of ulcerative colitis. Non-labeled
indications include use in patients with rheumatoid arthritis, Crohn disease,
and ankylosing spondylitis. Warnings and precautions include use in patients
with porphyria, since it can precipitate an attack; the drug causes
phototoxicity as well as the hematologic problems.
Question 6 of 6
A close relative of the patient is diagnosed with a familial form of colon cancer, in
which cancer develops in the setting of thousands of
adenomatous polyps in the boweI. A gene at which of the following chromosomal
locations has been linked to this cancer?
/ A. 3p25
/ B. 5q12
/ C. 11p13
/ D. 13q14
/ E. 22q

Explanation - Q: 1.6 Close

The correct answer is B. Acquired and inherited genetic changes seen in
colon cancer include loss of APC on chromosome 5q, activation of k-ras on
12q, loss of DCC on 18q, and loss of p53 on 17p. Colon cancers presenting
at less than 40 years of age are more likely to have a familial component.
3p25 (choice A) is the location of the VHL gene, which is important in von
Hippel-Lindau disease, renal cell carcinoma, and pheochromocytoma.
11p13 (choice C) is the location of the WT1 gene, which has been linked to
Wilms tumor.
13q14 (choice D) is the location of the Rb gene, which is important in
retinoblastoma, osteosarcoma, and to lesser degrees, cancers of the breast,
lung, prostate, and bladder.
22q (choice E) is the location of the NF2 gene, which has been linked to
neurofibromatosis type 2, central schwannomas, and meningiomas.


A 26-year-old woman gives birth, at term, to a 3200 g (7 Ib 1 oz) baby boy.
Physical examination of the neonate is unremarkable. However, the
baby still has not passed stool by the third day of life, and develops intestinal
obstruction with vomiting. The small amount of stool that is
eventually passed consists of particularly thick, tenacious meconium.

Question 1 of 8
The baby undergoes a laboratory test in which a small electrical current is
passed through his skin and the sweat produced by this stimulus is
collected. Which of the following would be an expected result in the sample
obtained?
/ A. Decreased Mg2+
/ B. Decreased Na+
/ C. Decreased phosphate
/ D. Increased Mg2+
/ E. Increased Na+
/ F. Increased phosphate

Explanation - Q: 2.1 Close

The correct answer is E. The underlying biochemical defect involves the
cystic fibrosis transmembrane conductance regulator (CFTR) protein, which
is coded for by a gene on the long arm of chromosome 7. The CFTR protein
forms a channel across the plasmalemma that regulates the flux of negative
ions, e.g., chloride and bicarbonate. Alterations in transport of these ions
secondarily impair water and sodium ion transport, leading to very viscous
mucous secretions and abnormal sweat electrolytes. If asked, some mothers
will report that their babies taste "salty" when they kiss them. Once cystic
fibrosis is suspected, the diagnosis can be confirmed with measurements of
sodium secretion in the sweat. Sweat production can be stimulated with the
drug pilocarpine, which is driven into the skin with a small electrical current
(iontophoresis). Sweat sodium levels must be elevated above 70 mmol/L on
two separate occasions for the diagnosis of cystic fibrosis to be made.
Although the actual ion transported by the channel is chloride, sodium ion,
which is more easily measured, will follow the chloride ion and can be
monitored in the sweat.
Mg
2+
(choices A and D) and phosphate (choices C and F) are not altered by
the abnormal CFTR protein.
Question 2 of 8
The underlying biochemical defect in the neonate's disease most likely involves
which of the following?
/ A. Amino acid transporter
/ B. Enzyme in glycolysis
/ C. Enzyme in tyrosine metabolism
/ D. Hemoglobin
/ E. Transmembrane ion channel

Explanation - Q: 2.2 Close

The correct answer is E. The neonate likely has cystic fibrosis. The earliest
and most distinctive presentation of this disease, which is seen in 10-15% of
cases, is meconium ileus, due to tenacious pancreatic secretions that stick to
the walls of the intestine, and cannot be passed through. Other children
present at a somewhat older age with failure to gain weight despite good
food intake. Cystic fibrosis is the most common genetic recessive disease in
the Caucasian population. It is a multisystem disease that can cause
problems through life. The disease is characterized by thick, tenacious bodily
secretions that eventually can lead to the destruction of the exocrine
pancreas and lungs. The metabolic defect involves a transmembrane
conductance regulator (CFTR) protein that regulates the transport of
negative ions such as chloride and bicarbonate. The other choices are
distracters unrelated to this case.


Question 3 of 8





In order to confirm the diagnosis, genetic testing was done on the patient (III-1)
and other available family members referenced in the pedigree
shown above. DNA from each individual was amplified using a polymerase chain
reaction (PCR) and tested using a panel of allele-specific
oligonucleotide (ASO) probes for the more common mutations in the gene
associated with this disease. A probe for the normal allele was
included next to each probe for a disease-producing mutation. The results
appear below.




Which of the following conclusions can be made about the baby?
/ A. He is a carrier of the DF508 allele
/ B. He is a carrier of the W1282X allele
/ C. He is a compound heterozygote for two disease-producing alleles
/ D. He is a homozygote for the DF508 mutation
/ E. He is a homozygote for the normal allele


Explanation - Q: 2.3 Close

The correct answer at C. The proband (III-1) is a compound heterozygote
for two disease-producing alleles (DF508 from the mother, and W1282X from
the father). Both mutations, associated with cystic fibrosis, occur in the CFTR
gene at 7q31. A compound heterozygote is an individual carrying two
different disease-causing mutations at a locus. In recessive diseases, such
as cystic fibrosis, compound heterozygotes are almost always affected.
DF508 (deletion of phenylalanine at position 508 in the CFTR protein) is the
most common mutation associated with cystic fibrosis in individuals of
European ancestry. An exception (do not memorize for the USMLE) is
Ashkenazi Jews in whom the W1282X (a nonsense mutation in which the
codon for tryptophan at position 1282 in the CFTR protein is converted to a
stop codon) is more common. The resultant CFTR protein ends with the
amino acid at position 1281. Both the CFTR proteins encoded by the DF508
allele and the W1282X allele are non-functional.
Choices A and B are not correct since the proband is an affected individual
and would not be best described as a carrier.
Choices D and E specify homozygous individuals in which both alleles of the
gene are identical. This interpretation is inconsistent with the blot shown in
which the proband's amplified DNA reacts with two different probes, making
him a heterozygote.

Question 4 of 8




The patient's uncle (II-4) and his wife (II-5) are concerned that their expected
child (III-2) may be affected with the disease but are not
immediately available for genetic testing. The geneticist knows that for this
disease, the carrier frequency in the general population is 1 in 25.
Based on this, what is the risk that the expected child (III-2) will be affected?
/ A. 1/25
/ B. 1/50
/ C. 1/100
/ D. 1/200
/ E. 1/625

Explanation - Q: 2.4 Close

The correct answer is D. Because individual I-1 is homozygous for the
normal allele (from the dot blot) and individual I-2 is a carrier of the W1282X
mutation (from the dot blot), the uncle (II-4) has a 1/2 risk for carrying a
mutation, and a 1/2 chance of passing it along if he does. Therefore, from
this father, the risk to the expected child (III-2) is 1/2 x 1/2 = 1/4.
The mother (II-5), an unrelated individual from the general population has a
1/25 chance of carrying a mutation, and a 1/2 chance of passing it along if
she does. The risk from the mother is 1/25 x 1/2 = 1/50.
The risk of these parents having an affected child is 1/4 x 1/50 = 1/200.
Question 5 of 8
The infant survives the neonatal period. Later, as the infant is started on solid
foods, he develops malabsorption and steatorrhea and it
becomes necessary to replace the pancreatic enzymes that are not being
released into the gut because of the viscosity of the pancreatic
secretions. Which of the following should be replaced?
/ A. Intrinsic factor
/ B. Lactase
/ C. Lipase
/ D. Maltase
/ E. Sucrase

Explanation - Q: 2.5 Close

The correct answer is C. The therapy of cystic fibrosis, while still far from
perfect, is one of the great success stories of modern medicine. Formerly,
most patients died in early childhood. Damage to the pancreas by tenacious
secretions entrapped in the ducts eventually leads to a deficient production
of pancreatic enzymes including amylase, lipase, and protease. The use of
(extremely expensive - up to $1000/month) oral pancreatic enzyme
(amylase+lipase+protease) replacement therapy throughout life means that
these children are no longer dying of malnutrition.
Intrinsic factor (choice A) is deficient in pernicious anemia.
Lactase (choice B), maltase (choice D), and sucrase (choice E) are
enzymes on the intestinal mucosa that help to process sugars, and can be
genetically deficient.


Question 6 of 8

The child has problems throughout childhood with recurrent pneumonia. At age
eight, he develops a particularly serious case of pneumonia,
which is characterized by production of sputum with an unusual bluish-green
coloration. This finding should specifically suggest which of the
following microorganisms as the cause of the pneumonia?



Explanation - Q: 2.6 Close

The correct answer is C. Pseudomonas aeruginosa is an important cause
of pneumonia in these children and can be suspected clinically because the
organism produces a blue-green pigment that may stain sputum. Therapy
often requires intravenous antibiotics, as the organism is both very tissue-
destructive and, often, very antibiotic-resistant.
The other organisms listed (choices A, B, D, and E) can also cause
pneumonia in cystic fibrosis patients, but would not produce the unusual
sputum discoloration.



Question 7 of 8

By age 15, a chest x-ray film shows visible bronchial shadows out to the lung
periphery. This finding suggests which of the following?
/ A. Asthma
/ B. Bronchiectasis
/ C. Chronic bronchitis
/ D. Emphysema
/ E. Pneumonia

Explanation - Q: 2.7 Close

The correct answer is B. Repeated episodes of bronchopneumonia can
cause permanent bronchial dilation known as bronchiectasis, which is visible
radiologically. Physiotherapy with postural therapy supplemented by
percussion of the chest is now used routinely as a prophylactic measure to
remove the tenacious bronchial secretions that predispose for pneumonia.
Pulmonary infections are treated aggressively. The result of these
improvements in management is that many of these children are now
surviving to their late teens or early twenties. By that point, the lung damage,
which typically includes bronchiectasis, has usually been sufficient to also
cause right heart damage, leading to cor pulmonale. Combined heart-lung
transplants are now being attempted in some of these young people, and
offer the potential of another decade of survival.
Asthma (choice A) and chronic bronchitis (choice C) cannot be diagnosed
radiologically.
Emphysema (choice D) will produce overly dark lung fields (lucencies) on x-
ray films.
Pneumonia (choice E) is associated with infiltrates that are visible as areas
of increased density on chest x-ray films.
*** Kleptomania FAQ is at http://www.structurise.com/kleptomania/faq.htm ***

Question 8 of 8

At age 19, the patient develops persistent severe ankle edema. This finding
suggests which of the following?
/ A. Cor pulmonale
/ B. Endocarditis
/ C. Myocardial infarction
/ D. Pericarditis
/ E. Pulmonary valvular stenosis

Explanation - Q: 2.8 Close

The correct answer is A. By young adulthood, the lung damage caused by
cystic fibrosis has usually been sufficient to also cause right heart damage,
leading to cor pulmonale. The other cardiac lesions listed (choices B, C, D,
and E) are not seen with increased frequency in these patients.

A 3-month-old child is taken to the emergency department because his bowel
movements have completely stopped, and he has developed a
fever and an expanded abdomen. The baby has a history of a several day delay
before passing meconium after birth. Since that time, the
baby had been willing to take some nourishment, but fed poorly and seemed to
be constipated all of the time. A sweat chloride test performed
at 6 weeks of age was normaI.
Question 1 of 5
Which of the following is the most likely diagnosis?
/ A. Cystic fibrosis
/ B. Diaphragmatic hernia
/ C. Esophageal atresia
/ D. Hirschsprung disease
/ E. Hypertrophic pyloric stenosis

Explanation - Q: 3.1 Close

The correct answer is D. This child most likely has Hirschsprung disease. In
this condition, a congenital failure of adequate innervation of the distal bowel
causes a functional obstruction secondary to failed peristalsis.
Characteristically, the involved bowel segment is narrowed, and there is a
dilatation proximal to the obstruction in the "normal" bowel. Depending upon
the length of the bowel segment that is involved, affected individuals may be
diagnosed in infancy (delayed passage of meconium with normal anal tone
and empty rectal ampulla), during the first year of life (chronic constipation,
large fecal mass on abdominal palpation), or even at later ages, including
adulthood (particularly in mild cases) with chronic constipation. Most
individuals with Hirschsprung disease have normal life spans, although in
rare cases when very large segments of bowel are involved, patients may
suffer from chronic malnutrition, with all of its complications.
Cystic fibrosis (choice A) is one cause of delayed passage of meconium, but
the results of the sweat chloride test argue against this diagnosis.
Diaphragmatic hernia (choice B) is a neonatal emergency requiring
immediate surgery because loops of bowel have herniated through the
diaphragm and are compressing the lungs, thereby preventing the lungs from
inflating.
Esophageal atresia (choice C) and hypertrophic pyloric stenosis (choice E)
typically cause food regurgitation and vomiting.

Question 2 of 5

A fulI-thickness biopsy of an involved area of the gastrointestinal react would
most likely show a lack of which of the following cell types?
/ A. Columnar epithelial cells
/ B. Endothelial cells
/ C. Fibrocytes
/ D. Ganglion cells
/ E. Smooth muscle cells



Explanation - Q: 3.2 Close

The correct answer is D. The histologic basis of Hirschsprung disease is a
lack of ganglion cells in both Meissner's submucosal plexus and Auerbach's
myenteric plexus. This lack of ganglion cells may be accompanied by
hypertrophied nerve bundles with a high acetylcholinesterase content. The
origins of the ganglion cell deficit in neuronal cells is still not completely
worked out, but may involve a failure of the cells to migrate during
development from the proximal bowel to the distal bowel. Uninvolved
portions of bowel have a normal distribution of ganglion cells and nerve
fibers. The other cell types listed are found in normal numbers.
Columnar epithelial cells (choice A) line the large intestinal lumen.
Endothelial cells (choice B) line blood vessels.
Fibrocytes (choice C) are found in the submucosa and between muscle
layers.
Smooth muscle cells (choice E) are found in the muscular wall of the gut.

Question 3 of 5
Which of the following structures is invariably involved in this patient's condition?
/ A. Appendix
/ B. Ascending colon
/ C. Cecum
/ D. IIeum
/ E. Rectum

Explanation - Q: 3.3 Close

The correct answer is E. The rectum is invariably involved in Hirschsprung
disease, and the absence of ganglion cells may also be present proximally to
varying degrees. The defective innervation is usually for all ganglion cells
past a point that varies from individual to individual, and no skip lesions are
seen. Involvement of the other structures listed in the choices can occur, but
is not invariable.

Question 4 of 5

This child is at particular risk of developing which of the following complications?
/ A. Amebiasis
/ B. Crohn disease
/ C. Diverticulitis
/ D. Toxic enterocolitis
/ E. UIcerative colitis

Explanation - Q: 3.4 Close

The correct answer is D. Toxic enterocolitis, or toxic megacolon, is a feared
and potentially fatal complication of Hirschsprung disease. The condition can
develop when chronic obstipation induces bacterial overgrowth. Production
of bacterial toxins then induces a fulminant water loss into the bowel lumen,
or if the colon is not completely blocked by stool, a profuse watery diarrhea.
The other conditions listed are not associated with Hirschsprung disease.
Amebiasis (choice A) is a gastrointestinal infection with amebae (usually
Entamoeba histolytica) that is typically acquired by drinking contaminated
water, and may be asymptomatic, or cause a dysentery-like illness.
Crohn disease (choice B) and ulcerative colitis (choice E) are chronic
inflammatory bowel diseases that appear to have an autoimmune
component, and these diseases may be associated with other autoimmune
diseases.
Diverticulitis (choice C) typically affects older individuals and is most strongly
associated with a low fiber diet.




Question 5 of 5
The child is treated with a diverting colostomy that is pulled through a transverse
left lower quadrant abdominal incision. Which of the following
muscles would be most likely to be affected by the incision?
/ A. IIiacus
/ B. Psoas
/ C. Quadratus lumborum
/ D. Rectus abdominis
/ E. Transversus abdominis

Explanation - Q: 3.5 Close

The correct answer is D. Of the muscles listed, only the rectus abdominis,
which is the largest of the abdominal muscles, would be likely to be
damaged. The rectus abdominis muscle is a long, straight muscle that is
oriented longitudinally, arises from the pubic bone and symphysis pubis, and
inserts in the anterior surface of the costal cartilages and xiphoid process.
The muscle is wide superiorly and narrower inferiorly, which helps to protect
it from damage during the diverting colostomy procedure. A diverting
colostomy is commonly used in (tiny) newborn infants, and then later
switched to a "pull-through" colostomy (through the anus) when the child is
larger.
The iliacus (choice A), the closely related psoas (choice B), and quadratus
lumborum (choice C) are all muscles of the posterior abdominal wall.
The transversus abdominis (choice E) runs horizontally in the upper half of
the abdominal wall.

A patient complains to his physician of chronic constipation. On the instruction of
his physician, the patient increases his dietary fiber, but his
constipation persists. The physician refers the patient to a gastroenterologist.
Colonoscopy reveals over fifty 2-3 mm openings into the bowel
mucosa. No bleeding is seen, and the intervening mucosa appears normaI.
Question 1 of 5
Which of the following is the most likely diagnosis?
/ A. Amebiasis
/ B. Crohn disease
/ C. Diverticulosis
/ D. Pseudomembranous colitis
/ E. UIcerative colitis

Explanation - Q: 4.1 Close

The correct answer is C. The appearance described is typical for
diverticulosis. The openings seen are into diverticula, which are small,
acquired outpouchings of the mucosa through the muscle of the bowel.
Milder cases of diverticulosis are usually asymptomatic; patients with
extensive diverticula may experience mild cramps, bloating, or constipation.
In amebiasis (choice A), the patient would have much more severe
symptoms and bleeding would probably be present.
In Crohn disease (choice B), pseudomembranous colitis (choice D), and
ulcerative colitis (choice E), the mucosa would appear obviously diseased.

Question 2 of 5
When population studies are done, which of the following sites has the largest
number of the lesions described in the question stem?
/ A. Cecum
/ B. Descending colon
/ C. Rectum
/ D. Sigmoid colon
/ E. Transverse colon

Explanation - Q: 4.2 Close

The correct answer is D. While diverticula can be found throughout the
colon (choices A, B, C, and E), most people have a predominance of
diverticula in the left side of the colon, and the sigmoid colon is the most
frequent site of involvement on the left side.

Question 3 of 5
The patient would be most likely to be which of the following ages?
/ A. 15 years old
/ B. 20 years old
/ C. 30 years old
/ D. 40 years old
/ E. 65 years old

Explanation - Q: 4.3 Close

The correct answer is E. Diverticulosis is primarily a disease of the elderly,
and the more extensive the disease, the older the individuals tend to be.
Diverticulosis is rare below the age of 40, occurs in about half of Americans
between the ages of 60 and 80, and is nearly universal in individuals older
than 80 years.

Question 4 of 5
Which of the following would be most effective in preventing this patient's
disorder?
/ A. High fat diet
/ B. High fiber diet
/ C. High meat diet
/ D. High sugar diet
/ E. High vitamin C diet

Explanation - Q: 4.4 Close

The correct answer is B. A high fiber diet tends to soften stool, and reduces
the chance of fecal impaction within a diverticulum, with resultant increased
risk of developing diverticulitis (inflamed diverticula). It is also thought that a
high fiber diet may reduce the rate at which diverticula develop, although
evidence for this is not completely conclusive.
The typical American diet with high fat (choice A), high meat (choice C),
and high sugar (choice D) is thought to predispose for diverticulosis.
While vitamin C (choice E) is good for general health, it does not, per se,
have a role in preventing diverticulosis or its complications.

Question 5 of 5
If an inflammatory/infectious complication did occur because the patient failed to
modify his diet, this would most likely clinically resemble
which of the following diseases?
/ A. Acute hepatitis
/ B. Appendicitis
/ C. Chronic hepatitis
/ D. Peptic ulcer disease
/ E. Renal calculus

Explanation - Q: 4.5 Close

The correct answer is B. Diverticula are similar structures to the appendix,
and acute diverticulitis can share many clinical features with acute
appendicitis, including abdominal pain, lower abdominal tenderness (usually
left-sided), fever, nausea, vomiting, chills, and cramping. In some older
patients, these symptoms may be blunted, and the patient may only appear
to be fairly severely ill without obvious localizing signs. Diverticulitis should
be considered a serious complication, since death can occur if the inflamed
diverticula rupture and seed the abdomen with fecal bacteria, leading to
peritonitis, and potentially, sepsis. Diverticular disease can also evolve to a
chronic diverticulitis that predisposes for stricture, abscess formation, fistula
formation, and intestinal obstruction. Severe cases may require resection of
much of the colon to prevent further complications from developing.
Diverticula can also be a significant source of GI bleeding, which can be
severe because the bleeding is often from a small artery that has been
distorted by a diverticulum and then ruptures.
Jaundice or other symptoms that would suggest hepatic involvement
(choices A and C) are not part of the clinical picture of diverticulitis.
The gnawing pain of peptic ulcer (choice D) and the sharp, intense pain of
renal calculus (choice E) are different from the diffuse abdominal pain with
focal tenderness seen in diverticulitis.



A 32-year-old woman consults a physician because she has been having diffuse
abdominal pain accompanied by chronic constipation with
narrow caliber, hard stools that are painful to defecate. She has tried laxatives,
but they do not seem to work. Sometimes the hard stools
alternate with periods of diarrhea with small volumes of loose stooI, with
evacuation preceded by urgency. At times, her stool is coated with
white mucus. She also has problems with bloating and gas. The patient is sent to
a gastroenterologist, who performs a variety of diagnostic
tests including a lactose tolerance test, barium enema, and colonoscopy. There
are no abnormal results on these studies.
Question 1 of 5
Which of the following is the most likely diagnosis?
/ A. Crohn disease
/ B. Diverticulitis
/ C. Irritable bowel syndrome
/ D. UIcerative colitis
/ E. Viral gastroenteritis
Explanation - Q: 5.1 Close

The correct answer is C. Irritable bowel syndrome is an often chronic
functional bowel disorder that may cause either constipation or diarrhea,
usually accompanied by abdominal pain. Patients vary in whether
constipation or diarrhea is the predominant symptom. In most patients, the
symptoms start before age 35, and there is a female predominance, at least
in those patients seeking medical care. Characteristically, the usual
screening diagnostic tests for gastrointestinal disease are unrevealing.
Crohn disease (choice A), diverticulitis (choice B), and ulcerative colitis
(choice D) would all produce visible lesions on barium enema or
colonoscopy.
Viral gastroenteritis (choice E) rarely lasts longer than one or two weeks.

Question 2 of 5
Population studies have estimated the prevalence of this patient's condition in the
United States to be which of the following?
/ A. Less than 1%
/ B. 2-6%
/ C. 10-20%
/ D. 30-50%
/ E. 75-90%

Explanation - Q: 5.1 Close

The correct answer is C. Irritable bowel syndrome is an often chronic
functional bowel disorder that may cause either constipation or diarrhea,
usually accompanied by abdominal pain. Patients vary in whether
constipation or diarrhea is the predominant symptom. In most patients, the
symptoms start before age 35, and there is a female predominance, at least
in those patients seeking medical care. Characteristically, the usual
screening diagnostic tests for gastrointestinal disease are unrevealing.
Crohn disease (choice A), diverticulitis (choice B), and ulcerative colitis
(choice D) would all produce visible lesions on barium enema or
colonoscopy.
Viral gastroenteritis (choice E) rarely lasts longer than one or two weeks.

Question 2 of 5
Population studies have estimated the prevalence of this patient's condition in the
United States to be which of the following?
/ A. Less than 1%
/ B. 2-6%
/ C. 10-20%
/ D. 30-50%
/ E. 75-90%

Explanation - Q: 5.2 Close

The correct answer is C. The incidence (new diagnosis rate) of irritable
bowel syndrome is 1-2% a year, but because the condition is so often long-
standing, the prevalence seen in screening population studies is much
higher, at 10-20%. Many of these patients only rarely or never seek medical
attention for their bowel complaints. 20-50% of gastrointestinal referrals are
for what is eventually diagnosed as irritable bowel syndrome.

Question 3 of 5
Which of the following findings would suggest an alternative diagnosis in this
patient?
/ A. Abnormal stool frequency
/ B. BIood in the stool
/ C. Feeling of abdominal distension
/ D. Passage of mucus
/ E. Straining at stool

Explanation - Q: 5.3 Close

The correct answer is B. One of the problems with the medical handling of
irritable bowel syndrome is that nongastroenterologists sometimes jump to
the diagnosis as an explanation for the patient's symptoms when actually
other, either more treatable or more serious, disease is present. It is
therefore important to be aware of the types of features that make the
diagnosis of irritable bowel syndrome less likely. These include blood in the
stool, pain that awakens or interferes with sleep, diarrhea that awakens or
interferes with sleep, weight loss, fever, and an abnormal physical
examination. The features listed in the other choices are common in irritable
bowel syndrome.

Question 4 of 5
While formerly, many physicians regarded this disease as a nearly purely
psychopathologic phenomenon, evidence now suggests that which
of the following physiologic abnormalities are often present in these individuals?
/ A. Decreased visceral pain sensation
/ B. Generalized smooth muscle hyporesponsiveness
/ C. Longer intervals between small intestinal migratory motor complexes
/ D. Narrowed dermatomal distributions of referred pain
/ E. Variations in colonic slow wave frequency


Explanation - Q: 5.4 Close

The correct answer is E. Like achalasia, which was formerly thought to be a
nearly purely psychologic problem, but is now accepted as having a clear
physiologic basis, the evidence supporting the presence of altered
physiology in irritable bowel syndrome is becoming increasingly compelling.
Current thinking suggests that irritable bowel syndrome may be best
visualized as a combination of altered GI motility, visceral hyperalgesia, and
psychopathology. With respect to altered GI motility, the colon in patients
with irritable bowel syndrome is now known to have variations in slow wave
frequency. Additionally, there is a blunted, late response of the colonic spike
potential following eating. These colonic alterations tend to be more marked
in patients prone to diarrhea. The transit time of a meal through the small
bowel tends to be increased in patients with predominately constipation, and
decreased in those with predominately diarrhea. The intervals between
migratory motor complexes are also often shorter (rather than longer as in
choice C) than in asymptomatic individuals. It is thought that all of these
alterations may be related to a generalized smooth muscle
hyperresponsiveness (not hyporesponsiveness as in choice B), that also
often affects the bladder and produces symptoms including frequency,
urgency, nocturia, and hyperresponsiveness to methacholine challenge. In
addition to the altered smooth muscle response, visceral hyperalgesia (rather
than decreased pain as in choice A) is also frequently present. This can be
demonstrated by studies in which balloons are inflated in the colon and small
intestine, and produce pain at lower volumes than in controls. These patients
also frequently report widened (rather than narrowed as in choice D)
dermatomal distributions of referred pain. It is thought that there may be an
increased sensitization of the intestinal nociceptive pathways.
The role of psychiatric disturbance in these patients is unclear. On the one
hand, psychological disturbances among patients seen in clinic are strikingly
frequent (up to 77% of patients). However, on the other hand, the large
numbers of individuals identified in screening studies who have irritable
bowel symptoms, but do not aggressively seek medical attention for them,
appear to be, on average, no different from the general population. It may be
that the emotional disturbances experienced by severely psychiatrically
disturbed individuals tend to exacerbate the irritable bowel symptoms, and
also that these individuals often tend to have lowered pain thresholds and
lowered ability to ignore discomfort than other individuals. Treatment of
irritable bowel disease remains primarily supportive (good doctor-patient
relationship, psychiatric help if needed, avoidance of any identified triggers,
caffeine avoidance, increasing fiber in diet) at the moment. This may change
rapidly in the future as the recent increases in understanding the underlying
pathophysiology are suggesting a variety of experimental pharmacologic
approaches including peripheral opiate narcotic antagonists to modulate
visceral nociception without central effect, serotonin agonists and
somatostatin agonists to stimulate colonic transit, muscarinic receptor
antagonists to inhibit intestinal motility, cholecystokinin receptor antagonists
to stimulate intestinal mobility, and gut-selective calcium channel blockers to
decrease postprandial motility.

Question 5 of 5

This patient is initially prescribed a course of dietary interventions and patient
education, however this approach is largely unsuccessfuI. The
most appropriate next step in the therapy of this patient would be which of the
following?
/ A. Hyoscyamine
/ B. Metronidazole
/ C. Prednisone
/ D. Sulfasalazine
/ E. Surgery

Explanation - Q: 5.5 Close

The correct answer is A. Dietary therapy is effective in almost 2/3 of
patients with irritable bowel syndrome. Pharmacological therapy is often
reserved for patients with refractive/severe signs and symptoms of this
condition. The antispasmodic agents are the most commonly used agents in
the treatment of this condition. They can alleviate postprandial abdominal
pain in these patients when administered 30-60 minutes before a meal.
Commonly used agents are dicyclomine and hyoscyamine. Antidiarrheals
and opioids may be useful in patients with frequent loose stools. Furthermore
they may be used prophylactically when diarrhea is anticipated, or would be
inconvenient. Psychotropic agents may also be used in patients with chronic,
unremitting abdominal pain, since some symptoms may be due to psychiatric
disturbances, and hence, may respond to antidepressants such as
desipramine or sertraline.
Metronidazole (choice B) is an antibiotic that is considered by many to be
the treatment of choice for antibiotic-induced colitis, since it is less expensive
than vancomycin and does not encourage the emergence of vancomycin-
resistant bacteria.
Corticosteroids such as prednisone (choice C) are commonly used in the
treatment of ulcerative colitis, but not irritable bowel syndrome.
Sulfasalazine (choice D) is a competitive antagonist of PABA. It is indicated
for the treatment of ulcerative colitis. Unlabeled indications include the
treatment of rheumatoid arthritis, Crohn disease, collagenous colitis and
ankylosing spondylitis.
Surgery (choice E) may be used in the treatment of severe ulcerative colitis
unresponsive to traditional therapies. However, it is not used in the treatment
of irritable bowel syndrome.



A 50-year-old man consults a physician because he has developed a chronic,
non-productive cough and is experiencing a reduced ability to
do strenuous work. His symptoms have developed insidiously. On questioning,
he states that he is a smoker and has also worked as a
contractor for all of his adult life. Physical examination is notable for the presence
of repetitive end-inspiratory basal crackles and finger
clubbing. A chest x-ray film shows diffusely distributed, small irregular opacities
that are most prominent in the lower lung zones. Localized
areas of pleural thickening are also noted. No large masses are seen.

Question 1 of 4

The chest x-ray film is most consistent with which of the following?
/ A. Emphysema
/ B. Interstitial disease
/ C. Lobar pneumonia
/ D. Lung cancer
/ E. PIeural effusion

Explanation - Q: 1.1 Close

The correct answer is B. Diffusely distributed small irregular opacities
suggest the presence of interstitial lung disease.
Emphysema (choice A) would produce unusually dark lung fields.
Lobar pneumonia (choice C) would produce a "white out" of one or more
lung lobes.
Lung cancer (choice D), if large, would produce a mass lesion (often
involving a bronchus), or, if very small, might not be recognized on chest x-
ray.
Pleural effusion (choice E) would cause a whitened area due to fluid below
the lung

Question 2 of 4

The patient is sent for spirometry for further evaluation. FEV1 and FVC are both
shown to be about 60% of the expected values, and the ratio
of FEV1/FVC is 90%. These findings are most consistent with which of the
following?
/ A. Asthma
/ B. Bronchiectasis
/ C. Chronic bronchitis
/ D. Emphysema
/ E. Restrictive lung disease

Explanation - Q: 1.2 Close

The correct answer is E. Spirometry is commonly used to subdivide non-
tumorous lung diseases into diseases that are predominately obstructive in
nature and those that are predominately restrictive. The process of
spirometry involves controlled breathing in and out while airflow is plotted
against volume to obtain a continuous loop. FVC (forced vital capacity) is the
maximum volume of air that can be forcibly and rapidly exhaled following a
maximum inspiration. FEV1 (forced expiratory volume in the first second) is
the volume of air expelled in the first second of a forced expiration starting
from full inspiration. Restrictive lung disease usually shows a reduction in
both FVC and FEV1, and the ratio of FEV1/FVC is greater than 80%. If these
readings are obtained in a patient, further studies to measure lung volumes
are usually performed to confirm the finding.
Asthma (choice A), bronchiectasis (choice B), chronic bronchitis (choice
C), and emphysema (choice D) all tend to produce obstructive patterns, with
FEV1 disproportionately decreased when compared to FVC.


Question 3 of 4

The patient's work history is most suggestive of exposure to which of the
following?
/ A. Asbestos
/ B. Beryllium
/ C. Coal
/ D. Kaolin
/ E. Silica

Explanation - Q: 1.3 Close

The correct answer is A. Working history often offers helpful clues about
possible toxic exposures that may have contributed to lung disease.
Asbestos was formerly a common constituent of insulating material in
buildings because of both its insulating properties and its fire-resistant
properties. People working on old buildings, particularly when removing the
old insulation, are consequently vulnerable to high exposures unless they
take care to minimize exposure with respirators. The risk to individuals living
and working in old buildings is usually markedly less, since the asbestos is
typically found behind walls. Individuals who work in asbestos mines
(principally in Canada, South Africa, and the former USSR) may also have
high exposures.
Beryllium (choice B) is used in the nuclear industry and in x-ray tubes, and
was formerly used in ceramics, metallic alloys, and fluorescent lights.
Most significant coal (choice C) exposures occur in coal miners.
Kaolin (choice D) is a component of clay dust.
Silica (choice E) is found in sand and glass, and significant exposures can
be seen in individuals working in environments where small particles of these
materials may become aerosolized.

Question 4 of 4

In addition to predisposing for pulmonary fibrosis and bronchogenic carcinoma,
this patient's disease is associated which of the following?
/ A. Basal cell carcinoma
/ B. Germ cell tumor
/ C. Hemangioma
/ D. Mesothelioma
/ E. PIeomorphic adenoma


Explanation - Q: 1.4 Close

The correct answer is D. Asbestos exposure is also linked to late (often
after 20 years) development of the rare tumor, malignant mesothelioma. The
amphibole forms of asbestos appear to be much more likely to induce
mesothelioma than does chrysotile, and some authors have even speculated
that the rare cases of mesothelioma in persons with predominant exposure
to the serpentine form of asbestos may have been actually related to trace
exposures to the amphibole forms. In contrast, all types of asbestos can
cause pleural plaques, pulmonary fibrosis, and lung cancer. None of the
other lesions listed in the choices have ties to asbestos.
Basal cell carcinoma (choice A) is a form of skin cancer.
Germ cell tumors (choice B) usually occur in testes and ovary.
Hemangioma (choice C) is a benign tumor of blood vessels.
Pleomorphic adenoma (choice E) is a salivary gland tumor.






A 70-year-old man is seen by his family practice physician during a routine office
visit. The man complains of not feeling well for the last three
months. Further questioning reveals that the patient has a chronic, unproductive
cough that he attributes to an old smoking history. Physical
examination is notable for a 15-pound weight loss since the last office visit three
months previously. A multinodular infiltrate is seen in the lung
field behind and above the right clavicle.

Question 1 of 5

The patient is injected intradermally with PPD. 3 days after the injection, there is
a 13-mm diameter area of induration at the injection site. This
reaction is an example of which of the following types of immune response?
/ A. Type l hypersensitivity
/ B. Type ll hypersensitivity, cytotoxic subtype
/ C. Type ll hypersensitivity, noncytotoxic subtype
/ D. Type lll hypersensitivity
/ E. Type IV hypersensitivity

Explanation - Q: 2.1 Close

The correct answer is E. This patient has a positive PPD test, as indicated
by an area of induration greater than or equal to 10 mm. PPD is a purified
protein derivative of tuberculin, so this finding indicates that the patient has
tuberculosis. The PPD reaction is an example of type IV hypersensitivity,
also known as delayed-type hypersensitivity.
Type I hypersensitivity (choice A) is immediate hypersensitivity, and can be
seen for example, in hay fever and atopic dermatitis.
Cytotoxic type II hypersensitivity (choice B) can be seen in autoimmune
hemolytic anemia and transfusion reactions.
Noncytotoxic type II hypersensitivity (choice C) can be seen in myasthenia
gravis and Graves disease.
Type III hypersensitivity (choice D) is due to immune complex deposition,
and can be seen in systemic lupus erythematosus and rheumatoid arthritis.
Question 2 of 5
Which of the following principally mediates this form of hypersensitivity?
/ A. Cytotoxic T cells, TH1 cells, and macrophages
/ B. IgE, basophils, and mast cells
/ C. IgG and complement
/ D. IgG, IgM, neutrophils, and macrophages
/ E. IgG, IgM, neutrophils, macrophages, and natural killer cells

Explanation - Q: 2.2 Close

The correct answer is A. Cytotoxic T lymphocytes, T helper 1 lymphocytes,
and macrophages mediate the delayed-type hypersensitivity reaction, which
develops in response to viral, fungal, and intracellular bacterial antigens.
Antibodies do not play a significant role in this type of response.
IgE, basophils, and mast cells (choice B) are involved in the production of
type I hypersensitivity.
IgG and complement (choice C) is a mechanism of toxicity in type II
cytotoxic hypersensitivity.
IgG, IgM, neutrophils, and macrophages (choice D) may be involved in types
II or III hypersensitivities.
IgG, IgM, neutrophils, macrophages, and natural killers (choice E) may be
involved in types II or III hypersensitivities.

Question 3 of 5
The strongest definitive identification of the pathogen responsible for this
patient's disease would be provided by a positive result on which of
the following biochemical tests?
/ A. Arylsulfatase
/ B. Heat-stable catalase
/ C. Niacin
/ D. Nitrate reductase
/ E. Urease

Explanation - Q: 2.3 Close

The correct answer is C. Mycobacterium tuberculosis is the only member of
the genus Mycobacterium that is a producer of niacin.
Mycobacterium fortuitum and M. chelonae produce arylsulfatase (choice A),
but M. tuberculosis does not.
Heat-stable catalase (choice B) is produced by Mycobacterium kansasii, M.
avium intracellulare, and M. fortuitum, but not by M. tuberculosis.
Mycobacterium tuberculosis, M. kansasii, and M. fortuitum produce nitrate
reductase (choice D), however niacin is more definitive for the identification
of M. tuberculosis.



Question 4 of 5
The mass lesion in the patient's lung is evaluated with fiberoptic bronchoscopy
with transbronchial biopsy. Sputum collected after the
procedure demonstrates acid-fast bacteria with a "beaded" appearance. Giant
cells found in the biopsy material would be likely to express
which of the following membrane markers?
/ A. CD4
/ B. CD8
/ C. CD14
/ D. CD16
/ E. CD19

Explanation - Q: 2.4 Close

The correct answer is C. CD14, the endotoxin receptor, is a standard
marker for macrophages and cells of their lineage. The giant cells in a TB
granuloma are histiocytes, which are modified macrophages, so they would
bear macrophage cell markers.
CD4 (choice A) is a cell marker for helper T lymphocytes that would be
found in the halo of lymphocytes surrounding the Langerhans giant cells, but
would not be on the giant cells themselves.
CD8 (choice B) is a cell marker for cytotoxic T lymphocytes that would be
present in the halo of lymphocytes surrounding the giant cells, but would not
be on the giant cells themselves.
CD16 (choice D) is a cell marker for NK cells, which would not be on the
giant cells in a TB granuloma.
CD19 (choice E) is a cell marker for B-lymphocytes, which are not likely to
be present in a TB granuloma, a reaction mediated exclusively by cell-
mediated immunity.


Question 5 of 5

The principal drug recommended for treatment of this patient's disease targets
which of the following molecules?
/ A. Arabinogalactan
/ B. Dihydrofolate reductase
/ C. Dihydropteroate synthetase
/ D. Mycolic acid
/ E. Peptidoglycan


Explanation - Q: 2.5 Close

The correct answer is D. The mainstay of therapy for tuberculosis is
treatment with drugs such as isoniazid, rifampin, ethambutol, ethionamide,
pyrazinamide, and streptomycin. Both isoniazid and ethionamide target the
mycolic acid molecules in the mycobacterial cell wall. A single drug, usually
isoniazid, can be used for chemoprophylaxis and when clinical disease is
absent. Multiple drugs are usually used in other settings. Mycobacterium
tuberculosis resistant to all first-line drugs is being seen with increasing
frequency among AIDS patients.
Arabinogalactan (choice A) is the molecule targeted by ethambutol, which is
a drug added to the anti-mycobacterial regimen only when drug-resistant
strains are implicated.
Dihydrofolate reductase (choice B) is the molecule targeted by trimethoprim,
which is not the principal drug recommended for treatment of tuberculosis.
Dihydropteroate synthetase (choice C) is the molecule targeted by dapsone,
which is the drug of choice for Mycobacterium leprae, not M. tuberculosis.
Peptidoglycan (choice E) is the molecule targeted by the penicillins,
vancomycin, and cycloserine.

A 60-year-old man presents to the emergency department complaining of
shortness of breath, cough, and copious sputum production. He
states that he has been coughing for years, and has had increased sputum
production for several months each year. On examination, he is
obese, afebrile, cyanotic, and in acute distress. Coarse rales are auscultated
bilaterally at the lung bases. He smokes two packs of cigarettes
a day and has a seventy-five pack-year smoking history. A chest x-ray film
appears normaI, except for slightly enlarged lung fields.

Question 1 of 5

Which of the following is the most likely diagnosis?
/ A. Chronic bronchitis
/ B. Emphysema
/ C. Myocardial infarction
/ D. Pneumonia
/ E. Pulmonary embolus

The correct answer is A. This patient has findings classic for the "blue bloater"
of chronic bronchitis. Patients with chronic bronchitis have excessive
tracheobronchial mucus production sufficient to cause cough with expectoration
for at least three months of the year for more than two consecutive years. "Blue
bloaters" are named for their obese body habitus, copious sputum production,
and cyanotic episodes. This condition may occur initially without airway
obstruction, but eventually, most patients progress to obstructive disease.
Patients with emphysema (choice B) represent another form of COPD. They are
known as "pink puffers" because they do not become cyanotic until they
decompensate. They display a thin body habitus and belabored breathing. This
patient is not consistent with the pink puffer of emphysema.
This patient does not have the classic findings for myocardial infarction (choice
C), which include: chest pressure or pain, shortness of breath, and/or pain that
radiates to the jaw or left arm.
It is unlikely that this patient has pneumonia (choice D). Patients with pneumonia
have cough with purulent sputum production, but they are usually febrile and
have chest x-ray opacities.
While the diagnosis of pulmonary embolus (choice E) is elusive due to its varied
presentations, it is unlikely that this patient has a pulmonary embolus. Findings
for PE include pleuritic chest pain, shortness of breath, hemoptysis, and a history
of calf pain indicative of deep vein thrombosis.
Question 2 of 5
As this patient waits in the emergency department, his condition begins to
deteriorate. He turns increasingly blue and an arterial blood gas is
drawn. His PO2 is 45 mm Hg, which under normal conditions means that his
hemoglobin would be 75% saturated. Which of the following
mechanisms could cause a hemoglobin saturation of less than 75% at this pO2?
/ A. Decreased 2,3-DPG Ievels
/ B. Decreased hemoglobin
/ C. Decrease in body temperature
/ D. Decreased PCO2
/ E. Decreased serum pH

Explanation - Q: 3.2 Close

The correct answer is E. A decrease in pH (an increase in H
+

concentration) decreases the affinity of hemoglobin for O
2
. This facilitates
unloading of oxygen from hemoglobin to the tissues.
Choices A, C, and D increase the affinity of hemoglobin for O
2
, and thus
cause the Hb to retain the O
2
.
Choice B is a distracter.

Question 3 of 5
Why must care be exercised when administering O2 to this patient?
/ A. Administering O2 washes out alveolar CO2 and inhibits respiration
/ B. Chronic hypoxia alters the blood-brain barrier such that CO2 cannot diffuse
into the medullary apneustic center
/ C. Chronic hypoxia induces atrophy in the dorsal respiratory group in the
medulla
/ D. Increased PO2 worsens CO2 retention by decreasing respiratory drive
/ E. O2 is acutely toxic to the chronically hypoxic alveolar epithelium

Explanation - Q: 3.3 Close

The correct answer is D. In a patient with normal respiratory function, the
PCO
2
and pH of cerebrospinal fluid drives the respiratory center in the
medulla oblongata. As serum PCO
2
rises, increased CO
2
diffuses across the
blood-brain barrier. When this CO
2
is buffered with the high HCO
3
-
of
cerebral spinal fluid, the concentration of H
+
rises accordingly. The
chemosensitive cells in the medulla's respiratory center respond to this
localized decrease in pH by stimulating ventilation. The pH in the CSF
returns to nearly normal, more quickly than the renal compensation of the
arterial pH, which takes 2-3 days. Patients with chronic CO
2
retention will
have an abnormally low ventilation for their PCO
2
because the pH of their
CSF is nearly normal. Therefore, in patients with chronic CO
2
retention,
arterial hypoxemia becomes their primary ventilatory stimulus. When the
patient is given supplemental O
2
, the PO
2
rises, and the hypoxic stimulation
disappears, and respiration can become markedly depressed. The PCO
2
-
dependent respiratory drive does not revert immediately, and thus the patient
hypoventilates and retains CO
2
, which may precipitate coma, stupor, or
death.
Administering O
2
at high flow rates (choice A) may wash out alveolar CO
2
,
but this is not the mechanism for hypoventilation of the chronically hypoxic
patient.
Chronic hypoxia does not alter the diffusing capacity of the BBB (choice B).
Chronic hypoxia does not cause the respiratory center to atrophy (choice C).
Chronically high O
2
concentrations can damage the alveolar epithelium, but
in the acute setting, it does not alter its diffusion capabilities (choice E).


Question 4 of 5
The mucus seen in this patient is derived from which of the following cell types?
/ A. AIveolar macrophages
/ B. Goblet cells
/ C. Neuroendocrine cells
/ D. Type l pneumocyte
/ E. Type ll pneumocyte

Explanation - Q: 3.4 Close

The correct answer is B. In chronic bronchitis, goblet cell hyperplasia is
seen in the airways. This hyperplasia, caused by chronic irritation (usually by
tobacco smoke), results in increased mucus secretion and formation of
mucus plugs. These mucus plugs obstruct the airways, and are responsible
for part of the obstructive component of chronic bronchitis. Some degree of
loss of elastic recoil of the airways is also seen and further adds to the
obstructive component of the disease.
Alveolar macrophages (choice A) do not secrete mucus. They have a
phagocytic function.
Neuroendocrine cells (choice C) are present in the respiratory tract. They
are the cell of origin for oat cell carcinomas, and the neuroendocrine
capabilities of these cells become evident with the paraneoplastic syndromes
seen in this high-grade carcinoma.
Type I pneumocytes (choice D) comprise the majority of alveolar epithelium.
They provide the majority of the surface area on which gas exchange occurs.
Type II pneumocytes (choice E) secrete the alveolar surfactant that allows
alveoli of different diameters to inflate at the same pressure.

Question 5 of 5
Which of the following spirometry profiles would most likely be seen in this
patient?
/ A. Decreased TLC, decreased FEV1
/ B. Decreased TLC, decreased RV
/ C. Decreased TLC, increased FEV1
/ D. EIevated TLC, decreased FEV1
/ E. NormaI TLC, decreased FEV1

Explanation - Q: 3.5 Close

The correct answer is E. Patients with chronic bronchitis tend to have
normal TLC and decreased FEV1. Patients with chronic bronchitis do not
typically have the increased TLC that their counterparts with emphysema
have. They may however, have a modestly increased residual volume due to
air trapping distal to mucus plugs. Similarly these mucus plugs obstruct the
airways, creating the obstructive component of chronic bronchitis. Hence a
decreased FEV1 is noted.
Choices A and B are not typical of any common pulmonary disorder.
Choice C is consistent with restrictive lung disease. The increased
fibroelastic elements in the lung parenchyma decrease the lung capacity,
while allowing the airways to remain open at increasingly lower pressures.
Thus, a decreased TLC is seen with an increased FEV1.
Choice D is consistent with emphysema. These patients have destruction of
the fibroelastic elements of the lung. As a result, the TLC increases. With
decreased elastic tissue, the airways collapse at higher airway pressures,
and a decreased FEV1 is seen.


A 72-year-old woman presents to the emergency department with cough, fever,
and shortness of breath. The woman lives alone at home, but
spends part of each day shopping and riding public buses. Approximately 4 days
previously she had developed an upper respiratory infection.
Approximately 2 days ago, she abruptly became much more ilI, and her
symptoms started worsening, beginning with a single, Iong, shaking
chilI. Since that time, she has had fever, pain with breathing, cough, and
dyspnea. She decided to come to the emergency department when
her temperature at home was 103.4 F. In the emergency department, her
temperature is 39.9 C (103.8 F), blood pressure is 90/50 mm Hg,
pulse is 120/min, and respirations are 30/min. No breath sounds are heard over
her lower left lung field, but they can be heard at other sites.

Question 1 of 7

A chest x-ray film would be most likely to demonstrate which of the following:
/ A. A single roughly ovoid white area
/ B. Complete whitening over one lobe of her lungs
/ C. Marked dilation and elongation of bronchial spaces
/ D. Multiple small spotty areas of white found primarily near the bronchi
/ E. No obvious radiologic changes

Explanation - Q: 4.1 Close

The correct answer is B. This patient has lobar pneumonia, which is seen
radiologically as a complete whitening of one or more lobes of the lungs.
Choice A describes the appearance of a mass lesion of the lung, such as
tumor or tuberculosis
Choice C describes the radiologic changes accompanying bronchiectasis.
Choice D describes the radiologic appearance of bronchopneumonia, rather
than lobar pneumonia.
While very early in lobar pneumonia, no obvious radiologic changes may be
seen (choice E), this patient's lack of breath sounds over the lower left lung
field indicates that her pneumonia is past this very early stage.

Question 2 of 7
Gram's stain of a smear from a sputum sample demonstrates gram-positive
lancet-shaped diplococci in short chains. Which of the following
would most likely be identified after culturing?


Explanation - Q: 4.2 Close

The correct answer is E. Streptococcus pneumoniae is the most commonly
identified causative organism for bacterial pneumonia. Up to two-thirds of
bacteremic community-acquired pneumonias are due to this organism. 5-
25% of healthy individuals carry S. pneumoniae in their pharynx. The classic
description of S. pneumoniae on Gram's stain is that given in the question
stem. S. pneumoniae can be verified by the Quellung reaction, or
counterimmunoelectrophoresis to determine serotypes of isolated strains or
for case detection using sputum specimens (there are more than 80 distinct
serotypes based on studies of capsular antigens).
Chlamydia pneumoniae(choice A) is not identified on the basis of Gram's
stain, but rather, can be seen with Giemsa or immunofluorescence.
Haemophilus influenzae(choice B) and Legionella pneumophila(choice C)
are gram-negative rods.
Staphylococcus aureus(choice D) is also a gram-positive coccus, but the
classic description of this organism typically includes a reference to "grape-
like clusters."



Question 3 of 7
Which of the following is thought to contribute to the ability of gram-positive
organisms to retain the Gram's stain during the decolorization
process?
/ A. Large periplasmic space
/ B. Presence of capsule
/ C. Presence of outer membrane
/ D. Presence of pili
/ E. Thick peptidoglycan layer
Explanation - Q: 4.3 Close

The correct answer is E. The peptidoglycan layer in the cell wall of gram-
positive organisms is much thicker than that in gram-negative organisms,
and is thought to contribute to the gram-positive staining reaction.
A large periplasmic space (choice A) and an outer membrane (choice C)
are characteristics of gram-negative cell walls.
Capsules (choice B) can be seen in both gram-positive and gram-negative
species.
Pili (choice D) are small hair-like structures that are most often seen in
gram-negative organisms.


Question 4 of 7
Which of the following is a characteristic feature on culture of this patient's
organism?
/ A. AIpha-hemolytic colonies inhibited by optochin on blood agar and lysed by
bile
/ B. Beta-hemolytic colonies that are bacitracin-resistant on blood agar
/ C. Beta-hemolytic colonies that are inhibited by bacitracin on blood agar
/ D. Catalase-negative organisms that hydrolyze esculin in 40% bile and 6.5%
NaCI
/ E. Catalase-positive, coagulase-positive organisms that cause beta-hemolytic,
yellow colonies on blood agar

Explanation - Q: 4.4 Close

The correct answer is A. These findings are used to identify Streptococcus
pneumoniae. Optochin sensitivity is used to differentiate the viridans
streptococci (resistant) from S. pneumoniae (sensitive). Another test used to
identify S. pneumoniae is the Quellung reaction. The only medically
important optochin-sensitive organism that gives a positive Quellung reaction
is S. pneumoniae.
Choice B is seen with Streptococcus agalactiae.
Choice C is seen with Streptococcus pyogenes.
Choice D is seen with Enterococcus faecalis.
Choice E is seen with Staphylococcus aureus.
Question 5 of 7
At this stage of the disease (two days following onset of first symptoms), and
before treatment is started, what are the expected
histopathologic findings in the affected lung parenchyma?
/ A. Advanced organization with macrophages and fibroblasts
/ B. Interstitial lymphomonocytic inflammatory infiltration
/ C. Intra-alveolar purulent exudate admixed with erythrocytes
/ D. Resolution of inflammatory exudate with semifluid debris
/ E. Vascular congestion without intra-alveolar exudation

Explanation - Q: 4.5 Close

The correct answer is C. Acute lobar pneumonia has classically been
subdivided into four pathologic stages. The initial stage of congestion(choice
E) lasts less than 24 hours and is characterized by engorgement of vessels,
with intra-alveolar fluid containing few cells, but often, numerous bacteria.
Over the following few days, an exuberant intra-alveolar exudate of red cells,
neutrophils, and fibrin develops, heralding the stage of red hepatization
(choice C; so-called because the lung resembles liver at this stage). During
the last half of the first week of illness, the red cells begin to break down, but
a fibrinous exudate remains in the alveoli; this is the stage of gray
hepatization. If death does not supervene, resolution (choice D) occurs in
the second week in untreated cases, with digestion of the exudate to leave
semifluid debris that are phagocytized, or coughed up. In some cases, the
exudate, rather than resolving in this manner, undergoes further organization
(choice A).
An interstitial lymphomonocytic infiltrate (choice B) is characteristic of
primary atypical pneumonia, caused by Mycoplasma or viruses.

Question 6 of 7
The patient's infection is treated with parenteral penicillin, to which she promptly
responds. This drug acts by which of the following
mechanisms?
/ A. Inhibits bacterial protein synthesis
/ B. Inhibits growth of cell walls
/ C. Interferes with bacteriaI DNA synthesis
/ D. Interferes with folate metabolism
/ E. Punches holes in cell membranes
Explanation - Q: 4.6 Close

The correct answer is B. The penicillins and cephalosporins both inhibit cell
wall synthesis. Penicillin G is the preferred antibiotic for sensitive strains;
25% of strains are resistant and can be treated with cephalosporins,
erythromycin, and clindamycin. Pneumococcal pneumonia can be prevented
in a number of cases; the pneumococcal vaccine contains 23 specific
polysaccharide antigens found in 85-90% of the serious pneumococcal
infections.
Inhibitors of bacterial protein synthesis (choice A) include aminoglycosides,
chloramphenicol, macrolides, and tetracycline.
Inhibitors of nucleic acid synthesis (choice C) include fluoroquinolones and
rifampin.
Inhibitors of folic acid synthesis (choice D) include sulfonamides,
trimethoprim, and pyrimethamine.
Agents that disrupt cell membranes (choice E) include azole and polyene
antifungal agents.

Question 7 of 7
A few minutes after the patient receives the antibiotic therapy, she develops an
adverse reaction characterized by an itchy skin eruption and
acute respiratory distress. This reaction is most likely attributable to which of the
following mechanisms?
/ A. Antibody-mediated cellular dysfunction
/ B. Complement-dependent reaction
/ C. Delayed-type hypersensitivity
/ D. IgE-mediated mast cell degranulation
/ E. Immune-complex deposition

Explanation - Q: 4.7 Close

The correct answer is D. This patient has had an acute allergic reaction to
the antibiotic, which can be further classified as an immediate
hypersensitivity reaction. These reactions are mediated in a way similar to
hay fever, with preformed IgE binding to the antibiotic antigen, and then the
antigen-antibody complex triggering degranulation of mast cells with release
of histamine and other active substances. These substances then cause
both the itchiness of the skin and the bronchospasm that caused the
respiratory distress.
An example of a disease caused by antibody-mediated cellular dysfunction
(choice A) is Graves disease, in which antibodies to the TSH receptor cause
a non-physiologic chronic stimulation of thyroid epithelial cells.
An example of a complement-dependent reaction (choice B) is hemolytic
disease of the newborn.
An example of delayed-type hypersensitivity (choice C) is the tuberculin
reaction.
An example of an immune complex deposition disease (choice E) is the
vasculitis seen in systemic lupus erythematosus.


A 14-year-old girl receives a bone marrow transplant as part of her treatment for
acute lymphoblastic lymphoma. During the period of profound
immunosuppression before the marrow engrafts, she develops nonproductive
cough, fever, mild hemoptysis, and pleuritic chest pain. A plain
chest x-ray film shows a pleuraI-based wedge-shaped lesion with focal
cavitation. Open chest lung biopsy reveals necrosis and hemorrhage.
Septate fungal forms with dichotomous 45-degree branching are seen in the
necrotic areas and involving the walls of several blood vessels.
When the fungus is cultured, it is found to be a monomorphic fungus.

Question 1 of 6

Which of the following is a monomorphic filamentous fungus?


Explanation - Q: 5.1 Close

The correct answer is A. The fungi that cause "deep infections" in humans
are subdivided into the dimorphic forms (which, depending upon
temperature, can be either yeast forms or hyphal forms) and the
monomorphic forms (which grow in the same general form at different
temperatures). Of the fungi listed, only Aspergillus is monomorphic.
Aspergillus is a common saprophytic mold found on decaying material in the
environment throughout the world. It can cause human diseases, including
allergic bronchopulmonary aspergillosis (which is essentially an allergic
reaction to inhaled Aspergillus conidia or spores), fungus ball (in which the
Aspergillus grows without invading in a preexisting cavitary lesion of the
lung), invasive aspergillosis (including pneumonia, meningitis, and other
systemic infections), and cellulitis. Invasive aspergillosis is most often seen in
severely immunocompromised patients with severe neutropenia, notably
including those with a history of transplantation, chronic granulomatous
disease, and leukemia. This patient's presentation is typical for invasive
pulmonary aspergillosis. The other fungi listed (choices B, C, D, and E) are
all dimorphic.

Question 2 of 6
Two commonly encountered invasive fungi are Aspergillus and Candidia. In
tissue specimens, which of the following features is often helpful in
distinguishing these organisms?

/ A. Aspergillus has both hyphae and pseudohyphae
/ B. Aspergillus has budding yeasts
/ C. Aspergillus has germ tubes
/ D. Aspergillus shows dichotomous branching
/ E. Aspergillus shows generally obtuse angles of branching

Explanation - Q: 5.2 Close

The correct answer is D. Aspergillus has hyphae, but not pseudohyphae,
budding yeasts, or germ tubes. The branching is dichotomous and at an
acute (often about 45 degrees) angle (opposite of choice E).
Characteristics to look for with Candida include both pseudohyphae and true
hyphae (choice A), budding yeasts (choice B), and occasionally germ tubes
(choice C, better seen with some culture methods).


Question 3 of 6

Involvement by this patient's infection of which of the following is considered to
have the worst prognosis?
/ A. Brain
/ B. Lung
/ C. Middle ear
/ D. Sinus
/ E. Skin

Explanation - Q: 5.3 Close

The correct answer is A. Cerebral aspergillosis is a feared complication of
other forms of aspergillosis because most patients die despite appropriate
antifungal therapy. Of the other sites listed in the question choices, true
invasive infection of the lung (choice B), as opposed to a fungus ball, is
considered the most serious site of infection, though less so than infection of
the brain.
Middle ear and sinus involvement (choices C and D) become clinically
worrisome when dissemination or extension to the brain occurs.
Skin involvement (choice E) is worrisome if dissemination occurs.

Question 4 of 6
Which of the following is the most appropriate pharmacotherapy for this patient?
/ A. Amphotericin B
/ B. CIotrimazole
/ C. FIuconazole
/ D. FIucytosine
/ E. Griseofulvin

Explanation - Q: 5.4 Close

The correct answer is A. Amphotericin B is the mainstay of therapy of
invasive aspergillosis. This medication has a wide fungicidal spectrum and
remains either the drug of choice or the co-drug of choice for severe
infections caused by Aspergillus, Candida, Cryptococcus, Histoplasma,
Mucor, and Sporothrix. Alternative antifungal agents that are not the
preferred therapy but do have some activity against aspergillus include
itraconazole, flucytosine (choice D), and voriconazole.
Clotrimazole (choice B) is used topically for candidal and dermatophytic
infections.
Fluconazole (choice C) is the drug of choice for esophageal and invasive
candidiasis and coccidioidomycosis, and is used for prophylaxis and
suppression in cryptococcal meningitis.
Flucytosine (choice D) is also synergistic with amphotericin B in candidiasis
and cryptococcosis.
Griseofulvin (choice E) is given orally, but is active only against
dermatophytes.

Question 5 of 6

Which of the following best describes the mechanism of action of the most
appropriate medication for this patient's disease?
/ A. Forms pores in fungal membranes
/ B. Inhibits the demethylation of lanosterol
/ C. Inhibits squalene epoxidase
/ D. Inhibits thymidylate synthase
/ E. Interferes with the synthesis of ergosterol

Explanation - Q: 5.5 Close

The correct answer is A. The polyene anti-fungal amphotericin B is an
amphoteric compound, with both polar and non-polar structural elements,
that interacts with ergosterol in fungal membranes to form artificial "pores,"
which disrupt membrane permeability. Resistant fungal strains are those that
appear to have low ergosterol content in their cell membranes. Amphotericin
B is given by slow IV infusion and penetrates poorly into the central nervous
system (intrathecal injections can be used). It has a half-life of more than 2
weeks and is removed by both metabolism and renal elimination.
Antifungal agents in the azole class (including ketoconazole, fluconazole,
and itraconazole) interfere with the synthesis of ergosterol (choice E) by
inhibiting the P450-dependent 14-alpha-demethylation of its precursor
molecule, lanosterol (choice B).
Flucytosine is activated by fungal cytosine deaminase to 5-fluorouracil (5-
FU), which can be incorporated into fungal RNA and can be also used to
form 5-fluorodeoxyuridine monophosphate, which in turn inhibits thymidylate
synthase (choice D) and the synthesis of thymine.
The anti-dermatophytic drug terbinafine inhibits squalene epoxidase (choice
C) and thus decreases ergosterol synthesis (choice E).
Question 6 of 6

Toxicity to which of the following organs is most likely to limit the administration
of the most appropriate medication for this patient's disease?
/ A. Brain
/ B. Heart
/ C. Kidney
/ D. Liver
/ E. Lung







Explanation - Q: 5.6 Close

The correct answer is C. Nephrotoxicity is most likely to be dose-limiting,
and may force amphotericin treatment to end prematurely. This is an
important problem, since we have so few drugs with wide spectrum activity
against invasive fungal organisms. An amphotericin B lipid complex (ABLC,
Abelcet) that may be less nephrotoxic in those patients who cannot tolerate
conventional amphotericin is available. Amphotericin B also causes infusion-
related reactions including fever, chills, nausea, vomiting, headache,
generalized malaise, hypotension, and arrhythmias.
Major problems encountered less commonly than renal toxicity include
anaphylaxis, generalized pain, bone marrow toxicity, rash, a variety of
cardiac problems including cardiac arrest (choice B), liver failure (choice D),
pulmonary edema (choice E), and convulsions (choice A).


A 45-year-old man is admitted to the hospital for the evaluation of diplopia,
weakness of his lower extremities, and gait difficulties. During the
interview, the patient reveals that has been forcing himself to vomit after almost
every meal over the last 6 weeks. He denies associated eye
pain and discomfort, headache, or dysphagia. He has never experienced such
symptoms in the past. Examination of the patient demonstrates
slight disorientation, vertical nystagmus worse on downgaze, diffuse weakness of
the lower extremities, bilateral dysmetria, and hypothermia.
Laboratory analyses reveal mild dehydration and hypokalemia.

Question 1 of 5

Which of the following is the most likely diagnosis?
/ A. Anticonvulsant intoxication
/ B. Benedict syndrome
/ C. Delirium tremens
/ D. Wernicke encephalopathy
/ E. Wernicke-Korsakoff syndrome
Explanation - Q: 1.1 Close

The correct answer is D. Wernicke encephalopathy is caused by a
nutritional deficiency of thiamine. Classic symptoms and signs include
"Wernicke's triad": acute mental confusion, ataxia, and ophthalmoplegia,
although not all the patients present with all of these. A common
misconception about Wernicke encephalopathy is that it is seen exclusively
in alcoholics. Prolonged vomiting and malnutrition, eating disorders, hunger
strikes, prolonged intravenous feeding, and malabsorption syndrome can
also be a potential cause of thiamine deficiency. Mental confusion is
characterized by impaired awareness, spatial disorientation, and inability to
concentrate. Ocular abnormalities are the hallmarks of this disease.
Horizontal or vertical nystagmus and paralysis of lateral rectus muscles are
common. The wide-based ataxic gait results from cerebellar dysfunction,
either alone or in combination with vestibular dysfunction.
Lateral nystagmus, ataxia, and drowsiness, and a history of drug abuse may
be associated with phenytoin intoxication (choice A).
Benedict syndrome (choice B) is a brain stem syndrome of crossed
oculomotor paralysis, with tremor and hemiparesis.
Tremors, hallucinations, severe agitation, and seizures are the features of
delirium tremens (choice C).
If persistent memory and learning deficits are present in addition to
Wernicke's triad, the complex disorder is termed Wernicke-Korsakoff
syndrome (choice E).


Question 2 of 5
The patient's status has improved after an administration of a vitamin. This
vitamin plays a central role in the metabolism of which of the
following compounds?
/ A. Amino acids
/ B. Carbohydrates
/ C. Cholesterol
/ D. DNA
/ E. Fatty acids

Explanation - Q: 1.2 Close

The correct answer is B. Thiamine (vitamin B
1
) is converted intracellularly
to its active form, thiamine pyrophosphate, which is an essential cofactor in
intermediate carbohydrate metabolism. Thiamine acts as a coenzyme for
enzymes involved in the tricarboxylic acid cycle, which plays a critical role in
the production of energy from food, and the pentose-phosphate pathway,
which produces an important intermediate, ribose-5-phosphate, required for
the synthesis of ATP, GTP, DNA, RNA, and NADPH. The brain is particularly
vulnerable to thiamine deficiency because it relies exclusively on
carbohydrates for immediate energy demands. The selective vulnerability of
certain structures accounts for the specific clinical manifestations of
Wernicke encephalopathy.
The active form of vitamin B6, pyridoxal phosphate, is a coenzyme in several
types of reactions, mostly involving amino acid metabolism (choice A).
Pantothenic acid is a component of coenzyme A (CoA), and thus is essential
in the synthesis of cholesterol (choice C).
The synthesis of DNA (choice D) from its precursors is dependent on folate
coenzymes.
Biotin is important in the synthesis of fatty acids (choice E).


Question 3 of 5

The physiologically active form of this vitamin acts as a coenzyme for which of
the following enzymes?
/ A. GIycogen phosphorylase
/ B. L-methylmalonyI-CoA mutase
/ C. Methionine synthase
/ D. Pyruvate carboxylase
/ E. Pyruvate dehydrogenase
Explanation - Q: 1.3 Close

The correct answer is E. Thiamine pyrophosphate (TPP), the
physiologically active form of thiamine, is required for the activity of pyruvate
dehydrogenase (PDH). PDH catalyzes oxidative decarboxylation of pyruvate
to acetyl-CoA, regulating entry into the citric acid cycle for metabolites
leaving glycolysis. The PDH complex is comprised of 3 separate enzymes:
pyruvate dehydrogenase (decarboxylase), dihydrolipoamide transacetylase
and dihydrolipoamide dehydrogenase. Besides TPP, the complex also
requires CoA, NAD, FAD, and lipoic acid as coenzymes. The mechanism of
TPP action includes formation of a carbanion that can attack the electron-
deficient keto carbon of pyruvate. In addition, TPP functions as a coenzyme
for alpha-ketoglutarate dehydrogenase and transketolase.
Pyridoxal phosphate serves as a coenzyme for glycogen phosphorylase
(choice A).
Vitamin B12 acts as a cofactor of L-methylmalonyl-CoA mutase (choice B),
which catalyzes conversion of L-methylmalonyl-CoA to succinyl-CoA.
Methionine syntase (choice C) activation leads to synthesis of methionine
from homocysteine with folic acid as a coenzyme.
Biotin is required for the activation of pyruvate carboxylase (choice D), a
crtical enzyme in the pathway of gluconeogenesis.
Question 4 of 5
Which of the following would be the most appropriate initial therapy for this
patient?
/ A. Chlordiazepoxide
/ B. Cyanocobalamin
/ C. GIucose
/ D. Niacin
/ E. Thiamine

Explanation - Q: 1.4 Close

The correct answer is E. 50-100 mg of thiamine IV should be administered
promptly. Objective of thiamine therapy is to replenish vitamin stores. Thus,
50 mg IM qd of thiamine should be given until the patient resumes a normal
diet. Magnesium is also required for normal function of thiamine
pyrophosphate, so correction of magnesium deficiency should be the final
step in treating this patient, after thiamine replenishment is well underway.
10-30 mEq of magnesium per day and 100-200 mEq of potassium (or more,
depending of the severity of hypokalemia) would be necessary to achieve
optimum metabolic balance. Patient will need IV glucose solutions, but
thiamine administration should be begun prior to treatment with IV glucose.
Administration of glucose prior to thiamine will increase the metabolic needs
for thiamine, and hence will drastically worsen the thiamine deficiency.
Correction of dehydration and metabolic alkalosis, if any, would represent
additional steps in therapy of this patient.
Chlordiazepoxide (choice A) is a benzodiazepine that would be indicated for
treatment of delirium tremens, rather than Wernicke syndrome.
Pernicious anemia is caused by impaired absorption of cyanocobalamin
(choice B) because of the lack of intrinsic factor.
Glucose (choice C) infusion before thiamine administration may precipitate
Wernicke disease.
Pellagra is the systemic disease resulting from niacin deficiency (choice D).
Question 5 of 5
Pathologic examination of this patient's brain would most likely reveal damage to
which of the following structures?
/ A. Caudate nucleus and putamen
/ B. Midline brain structures
/ C. Motoneurons in the anterior horns and cranial motor nuclei
/ D. Posterior columns and posterior roots
/ E. Substantia nigra

Explanation - Q: 1.5 Close

The correct answer is B. Midline brain structures show significant damage
in patients with Wernicke encephalopathy. The medial thalamic and
hypothalamic structures, periaqueductal and oculomotor regions of the
midbrain, and the superior cerebellar vermis are commonly damaged in this
disease. Wernicke originally detected punctate hemorrhages in the gray
matter surrounding the third and fourth ventricles and aqueduct of Sylvius.
The lesions in the oculomotor areas do not show significant neuronal
destruction, which may be the explanation for the rapid improvement of the
symptoms with thiamine repletion. Pathologic findings in the superior vermis
involve degeneration of all layers of the cerebellar cortex.
Gross atrophy of the caudate nucleus and putamen (choice A), with
selective neuronal loss and astrogliosis is seen in Huntington disease.
Amyotrophic lateral sclerosis is a disorder of the anterior horn cells of the
spinal cord and the cranial motor nuclei (choice C).
Demyelination involving the posterior columns and inflammatory changes
with fibrosis of posterior roots (choice D) are typical findings in tabes
dorsalis.
Loss of pigmented dopaminergic neurons in the substantia nigra (choice E)
and the presence of Lewy bodies is characteristic of Parkinson disease.
A 57-year-old man is evaluated because of progressive memory problems and
language disturbance. Several months ago, he started noticing
increasing difficulty with driving, turning left instead of right and getting lost in his
own neighborhood. His family states that he would repeatedly
ask the same question and started forgetting easily, particularly recent events.
They also noted waxing and waning difficulties with speech, an
ataxic gait, uncontrollable trembling, and a tendency to doze off easily. There is
no family history of psychiatric illness, epilepsy, or neurological
or neurodegenerative disorders.
Question 1 of 6
Which of the following is the most likely diagnosis?
/ A. Creutzfeldt-Jakob disease (CJD)
/ B. Fatal familial insomnia
/ C. Gerstmann-Straussler-Scheinker syndrome
/ D. Kuru
/ E. Variant Creutzfeldt-Jakob disease (vCJD)

Explanation - Q: 2.1 Close

The correct answer is A. CJD is a degenerative disorder of the central
nervous system that is caused by accumulation of abnormally folded protein
(PrPsc) particles termed prions. Normal prion protein is termed PrPc
(cellular), while an abnormal, pathogenic isoform of the prion protein is
designated PrPsc. This accumulation may be initiated by germline mutations
in the PRNP gene (familial), by abnormal prion proteins from infected
sources (mostly, iatrogenic), or somatic mutation or spontaneous conversion
of PrPc to PrPsc (sporadic form). The sporadic form is the prevalent form,
accounting for more than 85% of human prion disease cases. It usually
presents in late middle age (50-75 yr), with rapidly progressive dementia,
ataxia, dysarthria, myoclonic fasciculations, somnolence, and eventually
death, usually following pneumonia, within a year of onset. MRI typically
shows bilateral areas of increased intensity, predominantly in the caudate
and putamen. On light microscopy, the pathologic hallmarks of CJD are
spongiform degeneration, astrogliosis and the lack of an inflammatory
response. Spongiform changes occur in the putamen, caudate nucleus,
cerebral cortex, thalamus, and cerebellum. The amyloid plaques that are
seen in about 10% of cases are histologically different from those seen in
scrapie or Kuru. There is no known effective therapy for treating or
preventing CJD, with the exception of the prevention of iatrogenic cases.
Fatal familial insomnia (choice B) is an autosomal dominant disease,
characterized by progressive insomnia, dysautonomia, and motor signs. The
mutation of the PrP gene occurs at codons 178 and 129 that code for
methionine. The pathology shows severe selective atrophy of the thalamus.
Gerstmann-Straussler-Scheinker syndrome (GSS, choice C) typically occurs
in the 4th-5th decade. It is characterized by cerebellar ataxia, and less
commonly, dementia. Pathological examination reveals widespread plaque
formation.
Kuru (choice D) is an epidemic transmissible spongiform encephalopathy
(TSE) that occurred in certain Fore tribes in Papua, New Guinea. Cerebellar
ataxia, shivering-like tremor, dysarthria, and death usually within the year are
the features of this disease. It was a result of a ritualistic consumption of the
brains of dead relatives (form of cannibalism). It was prevalent in women and
children, since adult men seldom ate ritually prepared brains. Since the
authorities banned this endocannibalism, the disease is slowly disappearing.
Variant CJD (choice E) differs from classic CJD in several ways. The
patients are younger (16-41 yr), the presenting features are often behavioral
changes, ataxia, and peripheral sensory disturbances (dementia develops
later). The time between the onset of symptoms and death is longer, and the
EEG pattern is not characteristic for CJD. Pathologically, characteristic
features are "florid" plaques, with the central core of PrP amyloid surrounded
by vacuoles in a pattern resembling a flower.
Question 2 of 6
Which of the following electroencephalographic (EEG) findings would most likely
be recorded in this patient?
/ A. AIpha rhythm
/ B. Paroxysmal diffuse outbursts of high voltage, fast rhythm with many spikes
/ C. Periodic sharp waves
/ D. Polyphasic sharp and slow complexes
/ E. Theta rhythm, K-complexes

Explanation - Q: 2.2 Close

The correct answer is C. The EEG in Creutzfeldt-Jakob disease is
characterized initially by biphasic or triphasic discharges, approximately 1-2
seconds apart. They are present during wakefulness and disappear during
sleep. Sometimes, a normal EEG pattern may be seen in these patients at
this stage. As the disease advances, the pattern becomes generalized and
synchronous with periodic, stereotypic 200-400 msec sharp waves that
occurs at intervals of 0.5-1.0 second, representing the EEG hallmarks of this
disease. The sharp waves typically react to external stimuli. Certain drugs,
e.g., barbiturates and benzodiazepines, may temporarily eliminate periodic
patterns.
An EEG rhythm with the frequency of 8-13 Hz and low amplitude (below
50V), that is most prominent over the parieto-occipital cortex with the eyes
closed is called alpha rhythm (choice A). It is indicative of relaxed
wakefulness in most normal individuals.
A characteristic fast rhythm with many spikes and paroxysmal diffuse
outbursts of high voltage (choice B) is seen in generalized tonic clonic
seizures (grand mal seizures).
Polyphasic, repetitive, sharp and slow complexes (choice D) of high voltage
that recur every 5-15 seconds are characteristic of subacute sclerosing
panencephalitis (SSPE).
Theta activity, sleep spindles and K complexes (choice E) are characteristic
of stage 2 of slow wave sleep.


Question 3 of 6
Which of the following would most likely inactivate the causative agent of this
disease?
/ A. Ethylene oxide
/ B. Formalin
/ C. 1M sodium hydroxide
/ D. Proteinase K
/ E. UItraviolet irradiation

Explanation - Q: 2.3 Close

The correct answer is C. Appropriate decontamination procedures minimize
the risk of CJD transmission. Since prions are resistant to most commonly
used routine procedures, it is necessary to use specific methods to inactivate
them. Prions resist inactivation by nucleases, treatment with psoralens,
divalent cations, acids, hydroxylamine, boiling, and metal ion chelators. The
methods of choice include 1M NaOH, 4.0 M guanidinium hydrochloride, or
sodium hypochlorite (2% free chlorine concentration). However, the latter two
can damage fabric and metal, and irritate the airways of laboratory workers.
Therefore, sodium hydroxide (for at least 1 hour contact time) has been
recommended. Using this method, over 99% of infectivity will be eliminated.
Because paraformaldehyde vaporization does not affect prion titers,
decontamination of biosafety cabinets must be performed using 1M NaOH
followed by 1N HCl, and rinsing with water.
Low temperature sterilization with ethylene oxide (choice A) will not
eliminate prions. Therefore, for surgical instruments or other items requiring
sterilization, autoclaving at 132 C for 4.5 hours is recommended.
Prions are not inactivated by formalin (choice B). This is very important for
pathological examinations or autopsies in which formalin-fixed tissues are
processed.
The normal prion protein (PrPc) isoform is sensitive to proteinase K (choice
D). In contrast, proteinase K removes only small number of amino acids from
the abnormal isoform, PRPsc.
Prions are resistant to inactivation by UV-irradiation at 254 nm (choice E).


Question 4 of 6
Which of the following agents is the most likely cause of this disease?
/ A. BoPrPsc
/ B. GSS prion
/ C. HuPrPsc
/ D. Kuru prion
/ E. OvPrPsc

Explanation - Q: 2.4 Close

The correct answer is C. Prions are proteinaceous infectious particles that
lack nucleic acid. They are responsible for the pathogenesis of TSE
(transmissible spongiform encephalopathy). The causative agent for CJD is
designated as HuPrPsc. Several features distinguish prions from viruses:
they can exist in multiple molecular forms, they are non-immunogenic, they
do not possess nucleic acid, and they consist of a single protein. Prions are
abnormally folded proteins, which infect their normally folded counterparts in
the brain, setting off a domino-like effect in which the normal proteins refold
into an abnormal form. This influence is continued even after the cell
undergoes division and new protein is made. Therefore, prions confer a
heritable phenotype that is based on a change in protein conformation alone
and has no underlying alteration in the DNA. Sometimes, "rogue" prions are
produced by genetic mutations. A normally folded, cellular PrPc form occurs
in the brains of all mammals so far studied. The gene for PrPc, which in
humans is called PRNP, is located on the short arm of chromosome 20, and
encodes a 35 kDa glycoprotein with two glycosylation sites. The amino acid
sequences of PrPc and PrPsc should be the same, because they are coded
by the same gene. PrPc is expressed on the cell membrane of neurons
attached by the glycoinositol phospholipid anchor. It is believed to be
involved in synaptic function, and it has been suggested that it can protect
the brain from dementia and other degenerative changes associated with
aging. The secondary structure of PrPc consists of three alpha helices and a
single beta-sheet. For unknown reasons, this secondary structure adopts an
alternative conformation (PrPsc) that is rich in beta-sheet structure. This
change in secondary structure leads to alterations in the physiochemical
properties, which results in greater resistance to protease action, more firm
membrane-anchoring, and formation of amyloid plaques. Once formed,
molecules in the misfolded conformation have a tendency to stimulate the
formation of additional PrPsc by post-translational recruitment of PrPc.
BoPrPsc (choice A) is involved in the pathogenesis of BSE (bovine
spongiform encephalopathy). The tridimensional folds of BoPrPsc and its
human counterpart are, in fact, practically identical. Differences occur in the
electrostatic surface charge distribution of the globular region.
Point mutations at a number of different codons of the prion protein gene are
associated with GSS disease (choice B). As a result of the mutations, a
substitution at the corresponding residues of the prion protein occurs.
Amyloidosis coexists with severe spongiform degeneration in patients with
the codon 102 mutations, and with neurofibrillary degeneration in patients
with mutations at codons 145, 198, and 217.
The Kuru prion (choice D) is transmissible in brain or eye tissue to primates,
including humans. Kuru prion is thought to be spread either by ingestion of
brain tissue or by self-inoculation into sores or conjunctiva. The agent is not
passed maternally or by mother's milk.
OvPrPsc (choice E) is a causative agent of scrapie, a degenerative brain
disease of sheep. The "sc" superscript is initially derived from the term
scrapie because scrapie is the prototypic prion disease.

Question 5 of 6
Which of the following would most likely transmit this disease?
/ A. BIood
/ B. Direct contact
/ C. Direct inoculation
/ D. Droplets
/ E. Semen

Explanation - Q: 2.5 Close

The correct answer is C. In contrast to viral diseases, the human prion
diseases are not communicable, but they are transmissible. Research has
determined that prions remain infectious in the environment for years. Prions
can be transmitted to other species, chimpanzees, and mice by inoculation of
infected brain tissue. When prions are introduced peripherally or through the
eye, the infection travels via the lymphoreticular system before it goes to the
central nervous system. Transmission requires direct inoculation past the
natural defensive barriers. In medical institutions, the transmission of CJD
has been reported when infectious material has been directly introduced into
patients via transplanted tissue (corneal transplants and dura mater grafts),
cadaver-derived growth hormone and gonadotropin, and contaminated
surgical instruments (iatrogenic CJD). During the last several years, new
cases of so-called new variant CJD (vCJD) were noted in young people in
Britain. Since substantial evidence has suggested that prions can only
accumulate at high levels in the brain, spinal cord, and retina, it is postulated
that these patients were infected by eating food contaminated with the
infective agent. As a result, specific types of bovine offal (particularly
homogenates of pooled bovine brains, used as binders for hamburgers and
sausages) has been banned for use in food for human consumption.
However, ingestion (other than cannibalism) is an inefficient route of
transmission of prions. Also, there is no direct evidence that the prion
responsible for bovine spongiform encephalopathy ("mad cow disease") is
infectious in humans. Therefore, infection of humans from eating the BSE
prion is unlikely, but remains a possibility.
The current understanding is that there is no conclusive evidence that
infectious prions circulate in blood or plasma (choice A). Therefore, the risk
of transmission of CJD among humans from the use of blood or biological
products of human origin remains theoretical.
The disease is not transmitted by direct contact (choice B). Even living with
someone who has CJD is not a risk factor for developing the disease.
There is no evidence of aerosol transmission (choice D) of prions from one
human to another.
No reports are available that semen (choice E) can transmit CJD.


Question 6 of 6
Two years later, the patient dies and is autopsied. Microscopic examination of
sections from the brain would most likely reveal which of the
following findings?
/ A. Bizarre, enlarged astrocytes
/ B. Loss of substantia nigra neurons
/ C. Macrophages with vacuoles containing metachromatic material
/ D. Spongiform degeneration
/ E. Whorls composed of meningeal cells

Explanation - Q: 2.6 Close

The correct answer is D. On light microscopy, the pathologic hallmarks of
CJD are spongiform degeneration, astrogliosis, and the lack of an
inflammatory response. Spongiform changes occur in putamen, caudate
nucleus, cerebral cortex, thalamus, and cerebellum. The amyloid plaques
that are seen in about 10% of cases are histologically different from those
seen in scrapie or Kuru.
Bizarre, enlarged astrocytes (choice A) suggest anaplastic astrocytoma or
glioblastoma multiforme.
Loss of substantia nigra neurons (choice B) suggests Parkinson disease.
Macrophages with metachromatic material (choice C) suggest
metachromatic leukodystrophy.
Whorls of meningeal cells (choice E) suggest meningioma.


A 68-year-old woman presents to her primary care physician complaining of
clumsiness and urinary incontinence. The daughter tells the
physician that her mother "walks oddly" and has been falling with increasing
frequency. She says that her mother's symptoms began a year or
two before, and gradually became worse, starting with a changing gait, followed
by urinary urgency and incontinence. More recently, she says
her mother has had difficulty remembering things and has "ruined her credit
rating" because she forgets to pay her bills. Her medical history
includes gall bladder surgery 10 years ago and hysterectomy 15 years ago for
abnormal bleeding. Family history is negative for strokes,
aneurysms, or intracranial bleeds. She quit smoking fifteen years ago, and does
not drink alcohoI. On physical examination, the physician
notes that the patient has an ataxic gait. She does not have a tremor, and on
neurologic examination, she is found to have normal strength and
muscle tone. MRI reveals enlargement of the ventricles without cortical atrophy.
Question 1 of 5
Which of the following is the most likely diagnosis?
/ A. AIzheimer disease
/ B. Migraine headaches
/ C. NormaI-pressure hydrocephalus
/ D. Parkinson disease
/ E. Seizures
/ F. Stroke

Explanation - Q: 3.1 Close

The correct answer is C. This patient has the classic triad of normal
pressure hydrocephalus, consisting of urinary incontinence, mental status
changes, and gait disturbance. NPH is a disease usually found in older
adults. Most cases are idiopathic, and are due to impaired cerebrospinal fluid
absorption. Subarachnoid hemorrhage or meningitis are risk factors for the
future development of NPH. The CT makes the diagnosis likely because of
the enlarged ventricles.
Alzheimer disease (choice A) is the most common cause of dementia.
However, a gait disturbance is not a typical early symptom, and cortical and
hippocampal atrophy are often visualized on CT.
Migraine headaches (choice B), seizures (choice E) and strokes (choice F)
are episodic disturbances. Seizures and migraine headaches do not usually
cause a decline, while the decline of multiple strokes is stepwise.
Parkinson disease (choice D) would most likely present with rigidity and a
pill-rolling tremor. Normal pressure hydrocephalus can present with a broad-
based, shuffling, bradykinetic gait similar to Parkinson disease, although in
this case, the patient's gait appears ataxic. MRI scans often show cortical
atrophy

Question 2 of 5
The most pertinent part of the neurological examination in this case would be
which of the following?
/ A. Deep tendon reflexes
/ B. Gait evaluation
/ C. Looking for nystagmus
/ D. Pinprick sensation
/ E. Testing of cranial nerve l

Explanation - Q: 3.2 Close

The correct answer is B. The classic triad of clinical symptoms of normal
pressure hydrocephalus includes abnormal gait, urinary incontinence, and
dementia. Therefore, evaluation of gait would be important with this disorder.
The gait disturbance and urinary dysfunction is thought to be the result of
distortion of the corona radiata (white matter that carries descending and
ascending cortical projections) resulting from the distended ventricles.
Deep tendon reflexes (choice A) may be useful to distinguish an upper
motor neuron from a lower motor neuron lesion.
Nystagmus (choice C) on end gaze is present in many normal individuals.
There are pathological types of nystagmus associated with vestibular or
brain stem lesions.
Pinprick sensation (choice D) should be evaluated particularly carefully if
you suspect a peripheral neuropathy, peripheral sensory nerve lesion, or
central lesion to the spinal-thalamic-cortical system.
Cranial nerve I (choice E) should be carefully tested if the patient reports
any smell abnormality, or if damage along the olfactory pathway is
suspected.


Question 3 of 5
The patient has a lumbar puncture and does better for about two days. The
mechanism underlying this improvement is which of the following?
/ A. BIood products have been removed from the cerebrospinal fluid
/ B. Brainstem pressure has been relieved
/ C. Cervical cord pressure has been relieved
/ D. PIacebo effect
/ E. Stretching of frontal lobe tracts is decreased

Explanation - Q: 3.3 Close

The correct answer is E. Normal-pressure hydrocephalus exerts its effects
by stretching frontal lobe tracts. When spinal fluid is removed, this tension
may be temporarily alleviated. If the patient responds with an improvement in
gait, mental status, and/or a reduction in bladder hyperactivity, he or she may
be a good candidate for shunt surgery.
Subarachnoid hemorrhage is a common antecedent to normal-pressure
hydrocephalus, presumably through inflammation of the arachnoid
granulations. However there is no such history in this case and even if there
were, there would be no utility to doing a lumbar puncture for such a reason
(choice A).
Pressure on the brainstem (choice B) would probably have caused cranial
nerve deficits or changes in consciousness, while cervical cord pressure
(choice C) would have probably caused problems in both upper and lower
extremities. Additionally, increased pressure over the cervical cord and/or
brainstem are both relative contraindications for lumbar puncture.
There may be a minimal placebo effect (choice D), but choice E is clearly a
better answer.

Question 4 of 5
Which of the following central nervous system areas are involved in the control of
urination?
/ A. Frontal lobes, medulla, and pons
/ B. Frontal lobes, pons, and spinal cord
/ C. Medulla, pons, and temporal lobes
/ D. Pons, medulla, and spinal cord
/ E. Spinal cord, occipital lobes, and pons

Explanation - Q: 3.4 Close

The correct answer is B. There are urinary control centers located in the
frontal lobes, pons, and sacral spinal cord. The exact way that these urinary
control centers work in humans is not well understood. Simply put, there is a
cortical area (probably in the medial aspect of the primary motor cortex) that
controls the pontine micturition center. Activation of the pontine micturition
center leads to a coordinated detrusor contraction and sphincter relaxation
via thoracolumbar sympathetic, sacral parasympathetic, and sacral somatic
centers that are involved regulation of the bladder. In normal pressure
hydrocephalus, it is thought that stretching and eventual death of frontal lobe
fibers results in dysfunction of the frontal lobe urinary center.
Urinary control fibers pass through the medulla (choices A, C, and D), but
there is not a control center located there.
The temporal and occipital lobes are not known to play a role in urination
control (choices C and E).

Question 5 of 5
The cerebrospinal fluid production rate in a normal adult is approximately which
of the following?
/ A. 5 mL/day
/ B. 50 mL/day
/ C. 500 mL/day
/ D. 5000 mL/day
/ E. 50000 mL/day

Explanation - Q: 3.5 Close

The correct answer is C. CSF formation is at the rate of about 500 mL per
day. The primary site for production is the choroid plexus. It is absorbed in
the arachnoid granulations. The pathology of normal-pressure hydrocephalus
is thought to be more related to inadequate absorption, rather than excessive
production.







A 56-year-old man is brought to the psychiatrist with a three-year history of
progressive speech difficulties associated with altered social
behavior. The family states that he became aggressive, talked inappropriately to
strangers, and showed insensitivity, contrary to his past
consideration to others. He has had problems with speech production, using
some phrases repeatedly, and showing a decreased vocabulary.
He started to eat a great deaI, and has developed obsessional cravings for
sweets. As a result, the patient has gained 40 pounds over the
past year. His neighbors caught him stealing things from their back yard. The
patient repeats the examiner's words and imitates the
examiner's gestures. Testing reveals difficulties in naming common objects or
pictures.
Question 1 of 4
Which of the following is the most likely diagnosis?
/ A. AIzheimer disease
/ B. Huntington disease
/ C. KIuver-Bucy syndrome
/ D. Pick disease
/ E. Progressive supranuclear palsy


Explanation - Q: 4.1 Close

The correct answer is D. Pick disease is a rare form of neurodegenerative
disorder characterized by a distinct progressive dementing process.
Presenile onset (under 65 years old), initial personality change, progressive
language dysfunction, hyperorality (overeating with obsessional craving for
certain types of food), and disinhibition are the key features of this disease.
Compared to Alzheimer disease, memory loss and impairment of intellect
occur at later stages of the disease. The symptoms of Pick disease occur
because the frontal and temporal lobes are affected. Circumscribed ('knife-
like") lobar atrophy is the hallmark of Pick disease, and sometimes can be so
severe that the postmortem brain weight can be as low as 800 g. The
atrophy affects the anterior temporal and frontal lobes, the orbital frontal
lobe, and the medial temporal lobe, but spares the posterior part of the
superior temporal gyrus and the pre- and postcentral gyri. These lobes are
important for language skills, impulse control, energy and enthusiasm,
problem solving, and maintaining socially appropriate behavior. In most
cases of Pick disease, the cause cannot yet be determined. However, there
is a strong genetic component in certain families. A mutation on chromosome
17 has been identified, which leads to production of an abnormal tau protein.
Key features of Alzheimer disease (choice A) are progressive dementia,
diffuse cortical atrophy, senile plaques, and neurofibrillary tangles.
Huntington disease (choice B) is characterized by chorea, athetoid
movements, dementia, and is associated with the atrophy of the caudate,
putamen and frontal cortex.
Originally described in primates with bilateral temporal lobe lesions
(amygdala), Kluver-Bucy syndrome (choice C) refers to hypersexuality,
gluttony, and an obsession to touch and seize any objects in the visual field.
Progressive supranuclear palsy (choice E) belongs to the tauopathy group
of diseases, together with Pick disease and corticobasal degeneration. It is a
neurodegenerative disorder characterized by supranuclear ophthalmoplegia,
pseudobulbar palsy, parkinsonism, axial dystonia, and dementia at the end-
stage of the disease.

Question 2 of 4
Imaging studies are ordered for this patient. Which of the following will most likely
be found?
/ A. Bitemporal lobar atrophy
/ B. Circumscribed cerebral atrophy (frontal and temporaI)
/ C. Diffuse cortical atrophy
/ D. Enlarged lateral ventricles
/ E. T2 hyperdensities

Explanation - Q: 4.2 Close

The correct answer is B. CT and MRI are useful to identify cortical
shrinkage and low density of white matter in the involved frontal and
temporal lobes. Brain SPECT imaging shows greatly decreased, or absent
perfusion to the affected lobes.
Bitemporal lobar atrophy (choice A), particularly involving the amygdala, is
seen on imaging studies in cases of the rare Kluver-Bucy syndrome.
Brain MRI images in patients with Alzheimer disease show diffuse
cerebral/cortical atrophy (choice C).
Enlarged, dilated lateral ventricles (choice D) are seen on head CT scan in
patients with Huntington disease.
T2 hyperdensities (choice E) on brain MRI are typical findings in patients
with multiple sclerosis.

Question 3 of 4
Which of the following would a brain biopsy most likely show?
/ A. "Ballooned" cells and argyrophilic bodies
/ B. Lewy bodies
/ C. Sclerosis in plaques
/ D. Senile plaques
/ E. Spongiform degeneration

Explanation - Q: 4.3 Close

The correct answer is A. Microscopically, in Pick disease, there is a marked
loss of cortical neurons with gliosis. Neuronal loss and degeneration are
usually maximal in the limbic system, including the hippocampus, entorhinal
cortex, and amygdala. The argyrophilic intraneuronal inclusions (Pick bodies)
are the hallmark of classic Pick disease. They are usually homogenous,
smooth-edged, and intensely argyrophilic. Ultrastructurally, Pick bodies are
composed of bundles of disorganized straight filaments that may be mixed
with coiled fibrils; the filaments and fibrils are made from aggregated tau
proteins. Pick bodies are negative for alpha-synuclein, which differentiates
them from cortical Lewy bodies. In contrast to Alzheimer disease, dendritic
branches of neurons containing Pick bodies are not labeled with anti-tau
antibodies. Pick bodies are most numerous in limbic and paralimbic cortices
and the ventral temporal lobe. In addition to Pick bodies, the cerebral cortex
may contain neurons with distended cytoplasm called "ballooned cells" or
Pick cells. Characteristically, Pick cells are seen in the nonpyramidal cells of
layers 2, 3 and 6 of the cerebral cortex.
Spherical inclusions (Lewy bodies, choice B) in melanin-depleted neurons of
substantia nigra are found in Parkinson disease.
Perivascular infiltration of monocytes and lymphocytes appearing as
indurated areas in pathologic specimens (sclerosis in plaques, choice C) is
seen in multiple sclerosis.
Typical pathologic features of Alzheimer disease are senile plaques (choice
D), composed of beta amyloid protein.
Spongiform degeneration (choice E) is a characteristic pathologic finding in
Creutzfeldt-Jakob disease.

Question 4 of 4
Abnormal amounts of which of the following proteins would most likely be found
in this patient?
/ A. Amyloid beta protein
/ B. Apolipoprotein E
/ C. Myelin basic protein
/ D. Ubiquitin
/ E. Tau
Explanation - Q: 4.4 Close

The correct answer is E. Tau proteins belong to the microtubule-associated
proteins (MAP) family. In humans, they are expressed in brain, mainly in
neurons, but also in glial cells, particularly in pathological conditions. It is
possible to detect tau mRNA also in muscles, heart, kidney, and other
tissues. The human tau gene is located on the long arm of chromosome 17.
Alternative mRNA splicing results in 6 mature isoforms, ranging from 352 to
441 amino acids. In normal brains, the ratio of shorter forms of tau to the
longer ones is 1:1. In Pick disease, the ration is abnormal, i.e., there are
more shorter forms of tau than the long ones. Tau proteins can undergo
nonenzymatic glycosylation and can be phosphorylated at multiple sites.
They are believed to promote normal microtubule assembly and stability, and
are involved in axonal transport. The biochemical analysis in all studied
cases of Pick disease reveals a major 55 and 64 kD tau doublet. This
characteristic electrophoretic pattern of pathological tau in this disease is well
correlated with the presence of Pick bodies. The remaining options are not
affected in Pick disease.
Amyloid beta protein (choice A) is a characteristic finding in Alzheimer
disease.
ApoE (choice B) protein transports cholesterol and other fatty molecules in
blood. ApoE is an integral part of the metabolic pathway of acetylcholine, and
appears to protect neurons by acting as an antioxidant.
Myelin basic protein (choice C) is one of the components of myelin and a
marker of its presence. Its level in CSF is increased in patients with active
demyelination disorders.
Ubiquitin (choice D) is a small, heat-stable stress protein that is found only in
eukaryotic cells. This protein can be found in Pick disease, but its presence
is not as consistent and specific as tau.




A 72-year-old man has impaired concentration and an inability to recollect names
and appointments. These memory problems have become
increasingly worse over a period of months and begin to interfere with his social
and financial activities. Subsequently, the patient becomes
depressed, and his wife persuades him to see a physician. A neurologic
examination confirms the presence of moderately severe short-term
memory loss associated with disturbances in language, such as difficulty in
naming familiar objects and verbal comprehension. MRI of the
head reveals diffuse cerebral atrophy without focal lesions or tumors.
Question 1 of 6
Which of the following is the most likely diagnosis?
/ A. AIzheimer disease
/ B. Creutzfeldt-Jakob disease
/ C. Huntington disease
/ D. Parkinson disease
/ E. Pick disease

Explanation - Q: 5.1 Close

The correct answer is A. This patient has Alzheimer disease (AD), the most
common cause of dementia in the Western world. His symptoms are typical
of early AD and the diffuse cerebral atrophy is also characteristic; MRI scans
may later show hippocampal atrophy. The common form of this disease
typically affects people over age 60. Approximately 4 million people in the
United States have AD, translating to a staggering annual health care cost of
over $80 billion. AD can be divided in to clinical stages, although the disease
progression varies with the individual. In the early stage of AD, patients
experience recent memory loss, personality changes, language difficulties
(especially word finding), emotional lability, and diminished judgement. Some
patients are aware of their difficulties, causing frustration and anxiety; others
are seemingly unaware of their symptoms. Mild to moderate depression is
common in early stages. Surprisingly, sociability may not be affected at this
early stage. The intermediate stage is characterized by a worsening memory,
both recent and remote. Various apraxias are common; patients may have
difficulties with sequential motor tasks, leading to difficulties with dressing,
eating, bathing, and toilet functions. Their sense of time and place are lost,
leading to a host of behavioral problems, including wandering and becoming
lost. Sometimes they can be quite aggressive, agitated, and uncooperative,
which can alternate with being socially withdrawn and passive. They can still
ambulate, but are at risk for accidents resulting from their confusion. In end-
stage AD, patients are unable to walk or perform tasks of daily living and
their recent and remote memory is gone. Death generally results from
malnutrition, secondary infections, and heart disease. The typical duration of
the disease is 8 to 10 years.
Creutzfeldt-Jacob disease (choice B) is a degenerative central nervous
system disease caused by infectious proteins known as prions. Although
these patients suffer from dementia, CJD is associated with myoclonus,
ataxia, and rigidity, and symptoms that progress so rapidly, that death
generally occurs within a year of onset. Patients often do not have brain
abnormalities on gross examination.
Huntington disease (choice C) is an autosomal dominant degenerative brain
disease characterized by chorea and behavioral disturbances. Onset of
symptoms generally occurs in the 40s or 50s. Memory is often not impaired
until late in the disease.
Parkinson disease (choice D) is a slowly progressing, degenerative brain
disease, which is characterized by resting tremor, rigidity, bradykinesia, and
disturbances of gait and posture. AD patients with advanced disease may
appear parkinsonian due to a shuffling gait and generalized muscle rigidity,
but they rarely have the resting tremor. Although Parkinson patients can
have dementia, AD's cognitive deficits far outweigh motor deficits.
Pick disease (choice E) can sometimes be difficult to distinguish from AD
clinically, but imaging studies show only temporal and frontal lobe atrophy. In
early Pick disease, behavioral changes are often more marked than memory
loss.

Question 2 of 6
If the patient undergoes a biopsy of the frontal cortex in the nondominant
hemisphere, which of the following findings would most likely support
the probable diagnosis?
/ A. Neuron loss without specific inclusions
/ B. Round argentophilic cytoplasmic inclusions
/ C. Round eosinophilic cytoplasmic inclusions
/ D. Senile plaques and neurofibrillary tangles
/ E. Spongiform change with mild neuron loss and gliosis

Explanation - Q: 5.2 Close

The correct answer is D. The three most important microscopic findings in
AD are neuritic or senile plaques, cytoplasmic neurofibrillary tangles (NFTs),
and amyloid angiopathy. The plaques are collections of twisted and dilated,
silver-staining, neuritic processes, which surround a central core. The
primary component of the core is A amyloid (derived from amyloid
precursor protein, APP). The core also contains other substances such as
Apo E, proteoglycans, and 1-antichymotrypsin. Degenerating neurons,
macrophages, and microglia surround the plaque core. NFTs, present in
neuronal cytoplasm, are bundles of filaments that displace or surround the
nucleus. They appear primarily as paired helical filaments ultrastructurally, a
major component of which is the abnormally phosphorylated tau protein.
Although NFTs are characteristic of AD, they do occur in other neurologic
disease.
Neuron loss without specific inclusions (choice A) occurs in various
disorders, such as very rare forms of fronto-temporal dementia.
Round argentophilic cytoplasmic inclusions (choice B) are found in Pick
disease. This diagnosis is ruled out by the "diffuse" nature of the of cerebral
atrophy in this case. In Pick disease, atrophy is circumscribed to the frontal
and anterior temporal lobes.
Round eosinophilic cytoplasmic inclusions (choice C) are called Lewy
bodies. Lewy bodies are characteristic of dementia with Lewy bodies (diffuse
Lewy body disease), and are also seen in patients with Parkinson disease.
Spongiform change with mild neuron loss and gliosis (choice E)
characterizes Creutzfeldt-Jakob disease.
Question 3 of 6
The cholinergic deficit thought to contribute to the learning and memory
dysfunction in this disease is associated with degeneration of which of
the following?
/ A. Large neurons in the basal nucleus of Meynert
/ B. Pigmented neurons in the locus coeruleus
/ C. Pigmented neurons in the substantia nigra
/ D. Pyramidal neurons of the hippocampus
/ E. Small neurons in the caudate nucleus

Explanation - Q: 5.3 Close

The correct answer is A. The most severe pathology seen on autopsy are
in the nucleus basalis of Meynert, the hippocampus, and temporal cortex.
The nucleus basalis of Meynert contains large cholinergic neurons that
project to the cerebral cortex, basolateral amygdala, basal ganglia, and
thalamus. The reduction in acetylcholine (ACh) that occurs in AD is thought
to be due to degeneration of this nucleus; this loss of ACh is thought to
underlie the memory and learning dysfunction of AD. Current treatment for
AD involves increasing levels of ACh.
Loss of pigmented neurons in the locus coeruleus (choice B) occurs in AD,
resulting in decreased central norepinephrine levels, however this is not
thought to be involved in the memory deficits.
Loss of pigmented dopaminergic neurons in the substantia nigra (choice C)
is characteristic of Parkinson disease.
Pyramidal neurons of the hippocampus (choice D) do degenerate in AD, but
are not cholinergic. The cholinergic deficit in the hippocampus is secondary
to the degeneration of neurons in the septum that project to the hippocampus
(analogous to the degeneration of the basal nucleus of Meynert, which
results in the neocortical cholinergic deficit).
Small neurons in the caudate nucleus (choice E) degenerate in Huntington
disease. These neurons are primarily GABAergic.
Question 4 of 6
Which of the following drugs can be used to treat this condition?
/ A. Diazepam
/ B. Haloperidol
/ C. L-Dopa
/ D. Scopolamine
/ E. Tacrine

Explanation - Q: 5.4 Close

The correct answer is E. Cholinesterase inhibitors are currently the only
agents approved by the FDA for the treatment of AD. These presumably
increase acetylcholine levels in the brain. Four cholinesterase inhibitors that
have been approved are: tacrine, donepezil hydrochloride, rivastigmine, and
galantamine.
Diazepam (choice A) is a benzodiazepine, used for sedation.
Haloperidol (choice B) is a dopamine antagonist, used as an antipsychotic.
L-Dopa (choice C), the precursor to dopamine, is used to replenish
dopamine in Parkinson disease.
Scopolamine (choice E) is an anticholinergic, and would be expected to
exacerbate this patient's symptoms.

Question 5 of 6
Which of the following syndromes would most likely produce clinical and
neuropathological features similar to those in this patient?
/ A. Cri-du-chat syndrome
/ B. DiGeorge syndrome
/ C. Down syndrome
/ D. KIinefelter syndrome
/ E. Turner syndrome

Explanation - Q: 5.5 Close

The correct answer is C. Almost all patients with Down syndrome, if they
survive into their forties, show clinical and neuropathological changes similar
to those seen in Alzheimer disease. Down syndrome is caused by trisomy
21. This chromosome is the locus of the gene for amyloid precursor protein
(APP). APP is therefore overproduced in these patients, which leads to
overproduction of beta-amyloid (Abeta), and to the typical features of
Alzheimer disease. Studies showed that the predominant type of Abeta in
Down syndrome is Abeta42, which is basically the same finding as in
Alzheimer disease.
Cri-du-chat syndrome (choice A) is caused by a deletion in chromosome 5p.
DiGeorge syndrome (choice B) is caused by a chromosome 22q deletion.
Klinefelter syndrome (choice D) is characterized by a 47,XXY (and variants
with additional X and Y chromosomes) karyotype.
Turner syndrome (choice E) involves a loss of X-chromosomal material,
resulting in a (45,XO) karyotype.

Question 6 of 6
Impairment of short-term memory in this patient suggests involvement of which of
the following brain regions?
/ A. Cerebral cortex, basal ganglia, and cerebellum
/ B. Hippocampus
/ C. Left inferior parietal cortex
/ D. Medial geniculate nucleus
/ E. Right parietal lobe

Explanation - Q: 5.6 Close

The correct answer is B. The hippocampus is a structure located in the
medial temporal lobe. It can be divided into five different areas: the dentate
gyrus at the tip of the hippocampus; CA3 (cornu ammonis) and CA1 with the
small CA2 between them; the subiculum at the base of the hippocampus,
and entorhinal area, which is a part of parahippocampal gyrus. The tract
called the perforant path is responsible for the input of the hippocampus. The
dentate neurons send axons that are called mossy fibers, to the CA3 area.
CA3 sends axons called Schaffer collaterals to CA1, which then sends fibers
to the subiculum, which represents the output of the hippocampus. From
here, the information can flow directly to the hypothalamus and mamillary
bodies, or back to entorhinal cortex and farther to sensory cortex. Although
AD affects the entire brain, the CA1 region of the hippocampus can be
severely affected early in the course of the disease. When the hippocampal
region is damaged, the major symptom is anterograde amnesia, the loss of
ability to store new declarative memories.
Nondeclarative forms of memory (skill learning, classical conditioning) are
based on the function of the cerebral cortex, basal ganglia and cerebellum
(choice A).
Dyscalculia suggests involvement of the left inferior parietal cortex (choice
C).
The medial geniculate nucleus (choice D) is a relay center for audition.
Involvement of the right parietal lobe could explain constructional apraxia
seen in this patient (choice E).


A 52-year-old woman consults a physician because she has been having
increasingly frequent episodes of abdominal cramps and diarrhea.
These episodes are accompanied by an uncomfortable flushing of her skin. She
thinks they are sometimes precipitated by eating, alcohoI, or
emotional distress, but she has also recently been having episodes that had no
obvious trigger.
Question 1 of 5
Her physician suspects that she may have a hormone-secreting tumor. Ectopic
secretion of which of the following substances would be most
Iikely to cause diarrhea?
/ A. Gastrin
/ B. GIucagon
/ C. Histamine
/ D. Insulin
/ E. Serotonin

Explanation - Q: 1.1 Close

The correct answer is E. Excess serotonin can act on smooth muscle to
produce diarrhea, colic, and malabsorption.
Excess gastrin secretion (choice A) can cause peptic ulceration secondary
to stimulation of gastric glands.
Excess glucagon secretion (choice B) can cause rash and impaired glucose
tolerance.
Excess histamine secretion (choice C), as well as excess bradykinin
secretion, can coexist with excess serotonin secretion, and can contribute to
the flushing seen in this patient.
Excess insulin secretion (choice D) can cause hypoglycemia.

Question 2 of 5
Which of the following laboratory tests would be most appropriate to test for
excess secretion of the hormone causing the diarrhea?
/ A. Dexamethasone test
/ B. Hemoglobin A1C
/ C. Urinary 5-hydroxyindoleacetic acid
/ D. Urinary aminolevulinic acid
/ E. Urinary porphobilinogen

Explanation - Q: 1.2 Close

The correct answer is C. 5-hydroxyindoleacetic acid is the major urinary
metabolite of serotonin.
The dexamethasone test (choice A) is used to screen for Cushing
syndrome.
Hemoglobin A1c (choice B) is a marker for long-term glycemic control.
Urinary porphobilinogen (choice E) and urinary aminolevulinic acid (choice
D) are used in the diagnosis of the porphyrias.


Question 3 of 5
CT scan demonstrates several probable metastatic tumors in the patient's liver.
These are most likely which of the following histologic types?
/ A. Burkitt lymphoma
/ B. Carcinoid tumor
/ C. Hepatocellular carcinoma
/ D. Kaposi sarcoma
/ E. Squamous cell carcinoma

Explanation - Q: 1.3 Close

The correct answer is B. The patient's clinical presentation is typical for
carcinoid syndrome, which is a clinical manifestation of hormone-secreting
carcinoid tumors. It is usually seen in the presence of metastatic disease
involving the liver. None of the other tumors listed would be expected to
secrete hormones.


Question 4 of 5
The metastases most likely originated from which of the following?
/ A. Central nervous system
/ B. Gastrointestinal tract
/ C. Reproductive tract
/ D. Respiratory tract
/ E. Urinary tract

Explanation - Q: 1.4 Close

The correct answer is B. Approximately 90% of carcinoid tumors occur in
the gastrointestinal tract, with the most common site in the gastrointestinal
tract being the small intestine (39%, data from www.carcinoid.org web site).
Involvement of the appendix (26%) and rectum (15%) is also fairly common.
Other sites include colon (5-7%), stomach (2-4%), pancreas (2-3%), liver
(less than 1%), bronchial system (10%, choice D), and rarely gonads
(choice C), gallbladder and bile ducts, urinary bladder and kidneys (choice
E), prostate, breast, and thymus. The central nervous system (choice A)
does not appear to be a significant site of origin.
Question 5 of 5
These tumors are thought to be derived from which of the following cell lines?
/ A. Endothelial cells
/ B. Enterochromaffin cells
/ C. Fibroblasts
/ D. Lymphocytes
/ E. Smooth muscle cells

Explanation - Q: 1.5 Close

The correct answer is B. Carcinoid tumors are thought to be derived from
the glandular endocrine hormone-producing cells, known as
enterochromaffin cells, which are widely distributed through the body. They
occupy a borderline category between benign and malignant, because only a
fairly small percentage of them eventually metastasize, and pathologists are
unable, histologically, to tell which ones will or will not. Most of the malignant
ones are derived from the small intestine (1/5 metastasize and 1/3 of those
cause carcinoid syndrome), and they tend to produce the clinical symptoms
of carcinoid syndrome illustrated in the case history only when they have
metastasized to the liver. The tumors probably secrete hormones prior to
metastasis; the absence of carcinoid syndrome in these patients is attributed
to the fact that the venous drainage of the gut passes through the liver via
the portal system, clearing the blood of excess vasoactive substances. Also,
some of the tumors do not secrete clinically significant amounts of hormones.
Serotonin, histamine, and bradykinin are the most common hormones
secreted that produce symptoms. Carcinoid tumors are found incidentally in
approximately 0.5% of appendectomy specimens; appendiceal carcinoids
only rarely metastasize. The tumors may also be a component of the multiple
endocrine neoplasia (MEN) syndromes. Tumors that are identified
incidentally before metastasis can be cured surgically. No effective
chemotherapy or radiotherapy is available for metastatic disease, but the
tumors grow so slowly that 10-15 year survival times are not unusual. The
somatostatin analog octreotide can help control symptoms.
Endothelial cells (choice A) give rise to vascular tumors such as Kaposi
sarcoma and angiosarcoma.
Fibroblasts (choice C) can give rise to a variety of fibromas and
fibrosarcomas.
Lymphocytes (choice D) can give rise to leukemias and lymphomas.
Smooth muscle cells (choice E) can give rise to leiomyomas and
leiomyosarcomas.

A 32-year-old woman goes to an emergency department because she has
developed severe, watery diarrhea. On questioning, she reports
that three days previously, she spent the weekend along the Gulf of Mexico, and
ate at a raw oyster bar. About 36 hours later, she developed
vomiting and an abrupt, painless, watery diarrhea. The volume of diarrhea has
been copious, and she has subsequently developed intense
thirst, oliguria, muscle cramps, and weakness. At the time of being seen in the
emergency department, she is noted to have marked loss of
tissue turgor, sunken eyes, and wrinkling of the skin of her fingers. Laboratory
studies demonstrate hemoconcentration and severe metabolic
acidosis with potassium depletion.
Question 1 of 5
Which of the following is the most likely cause of the woman's problems?
/ A. Amoeba infection
/ B. Bacterial infection
/ C. Food poisoning
/ D. Irritable bowel syndrome
/ E. Viral infection

Explanation - Q: 2.1 Close

The correct answer is B. This patient is severely ill and dehydrated. Severe
watery diarrhea that persists is most likely to be due to a bacterial infection.
Amoeba infection (choice A) usually produces a bloody diarrhea that does
not have the watery characteristic of cholera.
Food poisoning (choice C) usually begins within the first eight hours after
ingestion of contaminated food, and is often over within 24 hours.
Irritable bowel syndrome (choice D) is usually a chronic, but relatively mild,
problem.
Viral infection (choice E) can cause gastroenteritis, but it is not usually as
debilitating as this patient's disease.


Question 2 of 5
An organism cultured from the patient's stool is subsequently characterized as an
oxidase-positive, gram-negative curved rod with polar
flagella that shows "shooting star" mobility and grows best on TCBS medium.
Which of the following is the most likely pathogen?





Explanation - Q: 2.2 Close

The correct answer is E. The features noted are those of Vibrio cholerae,
the causative organism of the severe diarrheal disease, cholera. The
diarrhea is the result of the action of the cholera enterotoxin, which acts by
ADP-ribosylating adenylate cyclase, leading to increased cyclic AMP with
secondary increased efflux of Cl
-
and H
2
O. World-wide, cholera is usually
spread by fecally contaminated water, but in areas such as Europe and the
United States, where the general level of hygiene, sewage control, and water
supply is good, the occasional cases seen are often the result of
contaminated food. Some of the shellfish from the Gulf of Mexico naturally
contain the organism, and ingestion of these shellfish in raw or poorly cooked
form has caused cholera.
Escherichia(choice A) is oxidase-negative.
Pseudomonas(choice B) is an oxidase-positive, gram-negative rod that
characteristically produces the blue pigment, pyocyanin.
Salmonella(choice C) is a motile, gram-negative rod that is a non-lactose
fermenter that produces H
2
S.
Shigella(choice D) are non-motile gram-negative rods.



Explanation - Q: 2.3 Close

The correct answer is A. Vibrio cholera serogroup O1 is the major cause of
epidemic cholera, and up until 1992 was the only cause. Since then, the
O139 serogroup (choice E) has been an important pathogen in India and
Bangladesh, but has not yet become important outside Asia. No other
serotypes are known to cause epidemic cholera.

Question 4 of 5
In the United States, approximately what percentage of patients who acquire this
infection subsequently die?
/ A. 1% or less
/ B. 5-10%
/ C. 20-30%
/ D. 50-70%
/ E. 90% or more

Explanation - Q: 2.4 Close

The correct answer is A. The mortality of cholera varies markedly
depending upon the general health of the individuals who become infected
and the availability of adequate medical care. In extreme situations, such as
in refugee camps with little sanitation, little food, and no available medical
facilities, case fatality rates of 25-50% are encountered. In Europe, the
Americas, and more recently Asia, case fatality rates in most situations are
kept at 1% or less. A major reason for the decreased fatality rates in most
parts of the world is that the World Health Organization has been
encouraging endemic areas to pre-plan for epidemics and have available
large numbers of previously prepared packages of oral rehydration salts.
Question 5 of 5
The patient responds with IV fluids, and is very much better within 24 hours.
While this woman did not require antibiotic therapy, which of the
following antibiotics is used for this disease?
/ A. First generation cephalosporin
/ B. Metronidazole
/ C. Penicillin
/ D. Tetracycline
/ E. Third generation cephalosporin

Explanation - Q: 2.5 Close

The correct answer is D. Most patients with cholera in this country who
have disease severe enough to cause significant dehydration are rehydrated
initially with IV fluids, and are then switched to oral fluids several hours later.
In countries with more likelihood of having an epidemic and poorer medical
availability, packages of oral rehydration salts to which water is added are
commonly (and very effectively) used to treat cholera. Antibiotics are often
not employed in milder cases. In severe cases, antibiotic therapy can reduce
the volume and duration of the diarrhea experienced by the patient. The
usual antibiotic of choice is tetracycline, although some resistant strains are
emerging. Useful alternative drugs include ciprofloxacin, erythromycin,
doxycycline, and furazolidone. No vaccine against cholera is presently
available in the United States, although two vaccines (which are not
completely effective) are available elsewhere in the world. The other agents
listed in the choices are not used to treat cholera.



A 25-year-old woman is hospitalized for multiple injuries to her arms and legs
from an automobile accident. She is treated with several
intravenous broad-spectrum antibiotics because of significant concern that her
wounds are becoming infected with a mixed flora of organisms.
She responds over the next several days to the antibiotics with an initial
decrease in fever. However, on the fourth day after her accident, she
develops severe diarrhea with fever, vomiting, cramping abdominal pain,
tenesmus, abdominal distension, and fluid losses severe enough to
require IV fluids.
Question 1 of 5
Proctoscopic examination demonstrates discrete yellow plaques up to 2 cm
diameter which are scattered over the colonic mucosa. Which of
the following would most likely be demonstrated on colonic biopsy?
/ A. Acid fast bacteria
/ B. Neoplastic polyps
/ C. Parasitic eggs
/ D. Pseudomembrane formation
/ E. Small granulomas

Explanation - Q: 3.1 Close

The correct answer is D. This patient's history and proctoscopic
examination are typical of severe pseudomembranous colitis (also
sometimes called antibiotic associated colitis). Patients with milder disease
may have only diarrhea. On pathologic examination, pseudomembranes
composed of fibrin, neutrophils, necrotic material, and bacteria are seen
overlying partially disrupted glands with prominent submucosal edema.
Acid fast bacteria (choice A) would suggest tuberculosis or atypical
mycobacterial infection.
Neoplastic polyps (choice B) are seen as isolated findings or as part of
familial polyposis syndromes.
Pseudomembranous colitis is not related to parasitic infection (choice C).
Small granulomas (choice E) could suggest either Crohn disease or
tuberculosis.


Explanation - Q: 3.2 Close

The correct answer is B. The clostridia are gram-positive, anaerobic, spore-
forming rods that can produce a variety of nasty diseases. This patient's
disease, pseudomembranous colitis, is due to bacterial gut overgrowth with
Clostridium difficile, usually in the aftermath of broad-spectrum antibiotic
therapy. Both children and adults may become infected. Probably in most
cases, the patients already had small numbers of the organisms in their guts
before antibiotic therapy, although isolated examples of transmission within
wards have also been documented.
Clostridium botulinum(choice A) causes botulism.
Clostridium perfringens(choice C) and Clostridium septicum(choice D)
cause gas gangrene.
Clostridium tetani(choice E) causes tetanus.
Question 3 of 5
Which of the following tests would be most helpful in confirming the diagnosis?
/ A. CT scan
/ B. MRI scan
/ C. "Scotch tape" test
/ D. Specific toxin in stool
/ E. Stool for ova and parasites

Explanation - Q: 3.3 Close

The correct answer is D. Clostridium difficile produces toxins A and B,
which can now be rapidly identified in stool samples. This is the best
confirmatory test following proctoscopy, as the results will be back much
faster than the pathology report on a tissue sample. The toxins cause
necrosis of the superficial gut mucosa that, in turn, leads to
pseudomembrane formation. True infection of the gut wall by the bacteria
does not occur. (The bacteria like to live in and eat the necrotic material and
fibrin of the pseudomembrane.)
CT scan (choice A) and MRI scan (choice B) are expensive tests that will
not contribute to the diagnosis.
The "Scotch tape" test (choice C) involves using tape to collect pinworm
eggs from the perirectal skin.
Clostridium difficile is a bacterium rather than a larger parasite, so stool for
ova and parasites (choice E) would not be helpful.


Question 4 of 5
This patient should not undergo barium contrast studies because of the
increased risk in her disease of which of the following
/ A. AIIergic reaction
/ B. Gut perforation
/ C. Predisposition for cancerous transformation
/ D. Secondary appendicitis
/ E. Trapping of dye in diverticula



Explanation - Q: 3.4 Close

The correct answer is B. In addition to the immediate risks related to
hypotension, dehydration, and electrolyte imbalance in these sometimes
critically ill patients, complications that can incur include colonic perforation
(which may be induced by barium contrast studies) and toxic megacolon.
Pseudomembranous colitis does not alter allergic reactions (choice A),
predispose for appendicitis (choice D) or cancer (choice C), or induce
formation of diverticula (choice E).

Question 5 of 5
Which of the following drugs is most likely to be effective in this case?
/ A. 3rd generation cephalosporin
/ B. Amoxicillin
/ C. Ampicillin
/ D. CIindamycin
/ E. Vancomycin

Explanation - Q: 3.5 Close

The correct answer is E. Clostridium difficile is resistant to most antibiotics,
which is why it tends to cause a bowel bacterial overgrowth when broad-
spectrum antibiotics are used. The two antibiotics to which it is usually
sensitive, and that are consequently most often used to treat
pseudomembranous colitis, are vancomycin and metronidazole. The other
agents listed in the choices are frequently reported causes of
pseudomembranous colitis.

A 17-year-old boy is seen in an emergency department with severe acute
abdominal symptoms that suggest appendicitis. The patient had a
similar episode previously that led to an appendectomy, but the appendix was
free of inflammation at pathologic examination. On questioning,
the boy reports having had intermittent diarrhea with moderate chronic abdominal
pain for several years, which he had been afraid to tell
anyone about after having had a "normaI" appendix removed. On physical
examination, the boy is noted to be thin, with short stature, and have
a palpable fullness in the right lower quadrant of his abdomen. UItrasound
examination of the abdomen shows some thickening of bowel
mesentery, but no distinct masses. Colonoscopy demonstrates sharply
demarcated segments of diseased bowel with patchy mucosal ulcers
separated by adjacent normal bowel involving both the distal ileum and right side
of the colon.

Question 1 of 6
Which of the following is the most likely diagnosis?
/ A. Colon cancer
/ B. Crohn disease
/ C. Hirschsprung disease
/ D. Pseudomembranous colitis
/ E. UIcerative colitis

Explanation - Q: 4.1 Close

The correct answer is B. This patient most likely has Crohn disease, as
indicated by his chronic abdominal complaints and the distal small intestinal
and colonic ulceration with skip lesions (normal bowel separating involved
areas). In addition to the distal small intestine and colon, Crohn disease can
affect the perianal area, entire small intestine, stomach, and esophagus.
Crohn disease may present with chronic diarrhea with systemic complaints,
acute abdomen, or extraintestinal manifestations. The peak incidence for
onset of symptoms occurs between 14 to 24 years. Crohn disease is thought
to be due to a genetic predisposition that leads to an autoimmune reaction in
the intestine that may be triggered in response to an environmental, dietary,
or infectious agent. About 1 in 6 people with Crohn disease have a relative
with Crohn disease, or, less commonly, ulcerative colitis.
Colon cancer (choice A) would be highly unlikely in a 17-year-old, and
usually causes a single mass lesion or stricture.
Hirschsprung disease (choice C) causes megacolon in young children.
Pseudomembranous colitis (choice D) is due to overgrowth of Clostridium
difficile and is usually seen following treatment with broad-spectrum
antibiotics.
Ulcerative colitis (choice E) usually extends from the rectum proximally and
does not have skip lesions.

Question 2 of 6
Which of the following findings on right colon mucosal biopsy would be most
suggestive of the patient's likely diagnosis?
/ A. Absence of nerve cell bodies in submucosa
/ B. Bacteria-Iaden pseudomembrane
/ C. Crypt abscesses
/ D. Neoplastic epithelial cells
/ E. Small granulomas



Explanation - Q: 4.2 Close

The correct answer is E. Microscopic features of Crohn disease include
transmural inflammation, small granulomas (most characteristic, but only
present in about 50% of cases), and variable degrees of inflammation.
Absence of nerve cell bodies in submucosa (choice A) suggests
Hirschsprung disease.
A bacteria-laden pseudomembrane (choice B) suggests
pseudomembranous colitis;
Crypt abscesses (choice C) suggest ulcerative colitis.
Neoplastic epithelial cells (choice D) suggest colon cancer or neoplastic
polyps.

Question 3 of 6
The patient is treated initially with corticosteroids, and then these are tapered and
he is switched to a maintenance therapy with sulfasalazine.
Sulfasalazine is an unusual medication that combines which of the following?
/ A. A histamine derivative and a beta blocker
/ B. A Iipoxygenase inhibitor and a penicillin derivative
/ C. A mast cell degranulation inhibitor and an anticoagulant
/ D. A salicylate derivative and a sulfonamide derivative
/ E. A serotonin antagonist and a proton pump inhibitor

Explanation - Q: 4.3 Close

The correct answer is D. Acute exacerbations of Crohn disease can be
treated with corticosteroids, but then the patient is usually switched to
maintenance therapy with immunomodulating drugs, such as azathioprine
and 6-mercaptopurine, or sulfasalazine or related drugs. Sulfasalazine has a
sulfapyrine component, which is a sulfonamide derivative and a 5-
aminosalicyclic acid (5-ASA) component, which is a salicylate derivative.
Most of the drug activity appears to be related to the 5-ASA component, and
most of the toxicity is related to the sulfapyrine, so alternatives to
sulfasalazine, such as mesalamine, are being developed that preserve the 5-
ASA activity without retaining the sulfapyrine component. The other answers
are distracters.

Question 4 of 6
Several years later, the patient develops recurrent urinary tract infections with
mixed flora bacteria isolated from the urine. This pattern
suggests that which of the following may have developed?
/ A. BIadder stone
/ B. Fistula
/ C. Kidney stone
/ D. Systemic immunosuppression
/ E. Urethral strictures

Explanation - Q: 4.4 Close

The correct answer is B. The patient has probably developed a fistula
between the intestine and the bladder. Fistulas are a relatively common
complication of Crohn disease. Surgery is used to correct recurrent intestinal
obstruction and intractable fistulas, but may be unsatisfactory because of
disease recurrence.
The other choices do not commonly complicate Crohn disease and would not
specifically predispose for mixed flora infections.
Question 5 of 6
At a still later date, the patient develops chronic lower back pain and is
diagnosed with ankylosing spondylitis. Which HLA type has been
associated with this extracolonic manifestation?
/ A. HLA-B27
/ B. HLA-B35
/ C. HLA-Cw6
/ D. HLA-DR3
/ E. HLA-DR5

Explanation - Q: 4.5 Close

The correct answer is A. Ankylosing spondylitis is one of the seronegative
spondyloarthropathies. It should be suspected in any young person
complaining of chronic lower back pain and can be confirmed by radiographs
or CT scans of sacroiliac joints. The disease usually progresses to involve
the whole vertebral column, producing ankylosis and respiratory failure
secondary to restrictive lung disease. HLA-B27 positivity has been
associated with Crohn disease patients who develop extracolonic
manifestations including ankylosing spondylitis, sacroiliitis, uveitis, and
primary sclerosing cholangitis.
You should associate HLA-B35 (choice B) with vitiligo, duodenal ulcer, and
subacute thyroiditis.
You should associate HLA-Cw6 (choice C) with psoriasis.
You should associate HLA-DR3 (choice D) with celiac disease, Goodpasture
syndrome, type I diabetes mellitus, and systemic lupus erythematosus.
You should associate HLA-DR5 (choice E) with juvenile rheumatoid arthritis
and pernicious anemia.

Question 6 of 6
Examination of the patient's legs reveals necrotic ulcers with ragged bluish-red
overhanging edges together with areas containing plaques
with pustules. Which of the following is the most likely diagnosis?
/ A. Eruptive xanthomata
/ B. Lupus vulgaris
/ C. Raynaud's phenomenon
/ D. Psoriasis
/ E. Pyoderma gangrenosum

Explanation - Q: 4.6 Close

The correct answer is E. Pyoderma gangrenosum can precede the onset of
chronic inflammatory bowel disease. It is treated by systemic and topical
corticosteroids. It may also be associated with myeloproliferative disorders
and rheumatoid arthritis.
Xanthomata (choice A) may be located on the tendons on the back of the
hands, the Achilles tendon and patellar tendon, buttocks, and back. They are
usually yellow papules up to 5 mm in diameter. They suggest familial
hypercholesterolemia.
Lupus vulgaris (choice B) is a progressive form of cutaneous tuberculosis
occurring in a person with a moderate or high degree of immunity. The two
types are the plaque form (a tiny reddish-brown, flat plaque that extends
gradually) or the ulcerative form (scarring and ulceration over the areas of
necrosis).
Raynaud's phenomenon (choice C) presents as cold and cyanotic digits
along with atrophy of the finger pulp and, in severe cases, gangrene. It is an
exaggerated physiological response wherein the fingers turn white, and then
when rewarmed, will turn blue, and then red as part of rebound hyperemia. It
may be associated with underlying connective tissue diseases such as
systemic sclerosis.
Psoriasis (choice D) presents as a thick plaque-like scale that is usually
silver or salmon-pink in color. It is usually well defined on the extensor
surfaces of elbows or knees. It is often associated with asymmetrical
arthropathy mainly involving the terminal interphalangeal joints.


Several individuals develop bloody diarrhea after eating hamburgers at a fast
food chain. Testing of stool samples from these patients
demonstrates the presence of verotoxin.

Question 1 of 5
Follow-up cultures from the patients and from food samples taken from the
restaurant would most likely demonstrate which of the following?





Explanation - Q: 5.1 Close

The correct answer is B. Escherichia coli is usually a normal commensal in
the gut, but strains producing gastrointestinal disease do occur. In this case,
this patient has enterohemorrhagic E. coli (EHEC), as evidenced by the
bloody diarrhea. These strains are also sometimes known as verotoxin-
producing E. coli (VTEC), and the most common serotype is O157:H7. The
bacteria can be acquired in poorly cooked beef, unpasteurized milk, and
water or a variety of foods contaminated with cow manure (e.g., alfalfa
sprouts). It is thought that mild disease may be common, but is usually not
specifically diagnosed. In its more severe form, severe cramping
accompanies a diarrhea that is initially watery but becomes grossly bloody.
Vomiting also occurs, albeit less commonly. High fever does not usually
occur, although low fever may. The disease is usually self limited, typically
ending within 8 days.
EaggEC (choice A) causes persistent diarrhea with vomiting and low-grade
fever in the developing world.
EIEC (choice C) causes watery diarrhea with fever and abdominal pain that
sometimes progresses to dysentery.
EPEC (choice D) causes watery diarrhea in babies.
ETEC (choice E) causes traveler's diarrhea.



Explanation - Q: 5.2 Close

The correct answer is D. Verotoxins are plasmid-associated toxins that are
most similar to the Shiga-like toxins 1 and 2, produced by Shigella. All of
these toxins inhibit protein synthesis in the small intestine by damaging the
60S ribosome.
Clostridium botulinum(choice A) produces a toxin that causes paralysis.
Clostridium tetani(choice B) produces a toxin that causes spastic paralysis.
Pseudomonas(choice C) produces exotoxin A, which ADP ribosylates EF-2,
thereby inhibiting protein synthesis (similar to diphtheria toxin).
Vibrio cholerae(choice E) produces a toxin that causes diarrhea by ADP-
ribosylating adenylate cyclase, causing rising cAMP levels.



Question 3 of 5
One of the younger children who had the diarrhea develops a severe
complication. Which of the following complications most likely occurred?
/ A. Congestive heart failure
/ B. Disseminated intravascular coagulation
/ C. Hemolytic uremic syndrome
/ D. Hepatic abscess
/ E. Polycystic kidney disease



Explanation - Q: 5.3 Close

The correct answer is C. Up to 15% (depending on the particular outbreak)
of young children who have E. coli O157:H7 develop hemolytic uremic
syndrome (HUS), which is characterized by hemolytic anemia,
thrombocytopenia, and renal failure. The other answers are distracters


Question 4 of 5
One of the elderly individuals who had the diarrhea develops a severe
complication. Which of the following is the most likely complication?
/ A. Aplastic anemia
/ B. Myelodysplastic syndrome
/ C. Non-Hodgkin lymphoma
/ D. Thrombotic thrombocytopenic purpura
/ E. UIcerative colitis

Explanation - Q: 5.4 Close

The correct answer is D. Thrombotic thrombocytopenic purpura (TTP),
despite its very different name, appears to be very closely related to
hemolytic uremic syndrome, and both can complicate E. coli O157:H7
infection. Some authors think the two conditions are actually the same
(sometimes called TTP-HUS), with varying degrees of renal failure and the
addition of fever and neurologic symptoms commonly in TTP. Both young
children and the elderly are particularly vulnerable to developing severe
complications of E. coli O157:H7 infection, but young children tend to be
diagnosed with HUS while the elderly tend to be diagnosed with TTP. These
are the only two potentially fatal complications of this infection that are seen
with any significant frequency. The other choices are distracters.

Question 5 of 5
Introduction of which of the following treatments has markedly altered the course
of the most serious complications of this condition in adults
and children?
/ A. Bone marrow transplant
/ B. PIasma exchange
/ C. Renal transplant
/ D. Thyroxine supplementation
/ E. Total parenteral nutrition

Explanation - Q: 5.5 Close

The correct answer is B. The pathophysiology of TTP-HUS and its
relationship to the enterohemorrhagic E. coli strains are still unclear. (Cases
may also be related to pregnancy, drugs, bone marrow transplantation,
metastatic cancer, or may occur idiopathically.) Formerly, TTP-HUS was
considered to be almost uniformly fatal, except in epidemic disease in young
children, but the recovery rate is now 85% when plasma exchange is
continued daily until evidence of disease activity has subsided. Patients
should be monitored for relapses, which may recur over a period of many
years. The other therapies listed are not usually used in these diseases.


An 11-month-old girl who attends a day care center develops vomiting and
severe, watery diarrhea. The child is taken to an emergency
department on the third day of the illness, because the mother is concerned that
she has not been able to hold any food or liquids down without
vomiting. Physical examination demonstrates an obviously ill child with listless
behavior, Ioss of skin turgor, dry mucous membranes, and
weight 3 pounds less than 1 week previously. Her blood pressure is 64/40 mm
Hg.


Question 1 of 5
Which of the following is the most common cause of viral gastroenteritis in
children under the age of three?
/ A. Astrovirus
/ B. Calicivirus
/ C. Enteric adenovirus
/ D. Norwalk virus
/ E. Rotavirus

Explanation - Q: 6.1 Close

The correct answer is E. The most common cause of severe dehydrating
diarrhea in infants and young children is Group A rotavirus. The peak
incidence of infection occurs from 3 to 15 months. After age three, severe
diarrhea is rare, but mild disease can occur. This virus accounts for about
half of the cases of diarrhea requiring hospitalization in children in the United
States. Rotaviruses are in the Reoviridae family and have a genome
consisting of 11 double-stranded RNA segments. The other agents listed can
also cause viral gastroenteritis in children and/or adults.

Question 2 of 5
In the United States, this virus tends to cause illness during which of the following
periods?
/ A. Predominately fall months
/ B. Predominately spring months
/ C. Predominately summer months
/ D. Predominately winter months
/ E. Year-Iong with no seasonal preference


Explanation - Q: 6.2 Close

The correct answer is D. In temperate climates, rotavirus has a winter
seasonal pattern. In the United States, epidemics occur from November to
April. In tropical climates, disease caused by rotavirus occurs year round.

Question 3 of 5
The virus is predominately spread by which of the following routes?
/ A. Contaminated blood
/ B. Contaminated food
/ C. Contaminated water sources
/ D. Direct fecaI-oral route
/ E. Inhaled aerosols
Explanation - Q: 6.3 Close

The correct answer is D. Rotavirus is a notoriously contagious virus, and
the infective dose is thought to be as small as 10-100 infectious viral
particles. While the initial yearly source of the virus may be from
contaminated estuary waters (choice C), the vast majority of cases are
spread from person to person via direct contact with stool on diapers, objects
such as toys, or fingers. While not always necessary, the presence of
rotavirus can be confirmed by using enzyme immunoassay on stool
specimens. Day care centers are notorious sources of minor epidemics of
the virus, and day care workers should be encouraged to be very careful.
Reasonable precautions include removal of kids with diarrhea from the day
care environment, prompt diaper changing before the children have a chance
to touch the stool, and routine use of fresh disposable gloves during
diapering.
Contaminated blood (choice A) and inhaled aerosols (choice E) are not
usual routes of infection.
Contaminated food (choice B), typically contaminated by food handlers who
have recently changed an infected child's diapers, is an uncommon source of
infection.


Question 4 of 5
This child's poor skin turgor, dry mucus membranes, significant weight loss, and
low blood pressure all suggest which of the following?
/ A. Dehydration
/ B. Disseminated intravascular coagulation
/ C. IIeus
/ D. Sepsis
/ E. Starvation

Explanation - Q: 6.4 Close

The correct answer is A. These findings strongly suggest that the child is
severely dehydrated and in need of intravenous fluid replacement.
Approximately 500,000 children are seen in emergency room and outpatient
clinic visits for rotavirus infection yearly, and of these, about 50,000
hospitalizations (usually to correct dehydration) and 20 deaths (usually
caused by failure to correct the dehydration in a timely fashion) occur yearly
in the United States. World-wide, rotavirus infection is thought to cause
around 600,000 deaths, primarily in young children. This child with severe
dehydration should at least be given IV fluids, and might require
hospitalization.
Disseminated intravascular coagulation (choice B) would cause multiple
petechiae and purpura, and does not usually complicate rotavirus infection.
Ileus (choice C) is a paralyzed bowel, which is the opposite of the problem
seen in gastroenteritis.
Sepsis (choice D) is not usually diagnosed in viral infections, but is seen in
severe bacterial infections, often with bacteremia.
Three or four days of not eating is not long enough to induce starvation
(choice E).

Question 5 of 5
In 1989, the FDA Iicensed a live attenuated vaccine against the causative agent
of this disease for use in infants. It was later withdrawn for
which of the following reasons?
/ A. Data suggested a link between the vaccine and intussusception in some
infants during the first 1-2 weeks following vaccination.
/ B. It was questioned if there was association between the vaccine and the
development of autism.
/ C. Seizures occurred in a significant number of recipients.
/ D. The vaccine was not widely used because it was too expensive.
/ E. The vaccine was thought to be associated with the development of arthritis
in recipients.

Explanation - Q: 6.5 Close

The correct answer is A. The live attenuated rotavirus vaccine was a virus
mixture of reassorted strains that were primarily animal viruses except for
one human-virus gene segment. The vaccine had a 49-68% efficacy against
any diarrhea due to rotavirus and a 61-100% efficacy against severe
disease. However, during the first 11 months of use, post-licensure studies
identified intussusception, a form of intestinal obstruction in which a segment
of the bowel prolapses into a more distal segment, as an uncommon but
potentially life-threatening side effect. Follow up studies estimated that 1
additional case of intussusception occurred for every 5,000- 10,000
vaccinees. The vaccine has currently been withdrawn from use in the US.

A 45-year-old man presents to a physician because of severe chronic diarrhea
accompanied by a 18 kg (40 Ib) weight loss. The diarrhea
began several years ago, and has become steadily worse. It is often
accompanied by excessive flatulence, and the man notes that his stools
usually float. The man additionally has felt generally poorly and sometimes has
experienced joint symptoms. Intestinal biopsy demonstrates
Iarge numbers of foamy macrophages distending the lamina propria. A periodic
acid-Schiff (PAS) stain shows granules within the
macrophages.
Question 1 of 5
Which of the following is the most likely diagnosis?
/ A. Celiac sprue
/ B. Intestinal lymphangiectasia
/ C. Lactase deficiency
/ D. Tropical sprue
/ E. Whipple disease

Explanation - Q: 7.1 Close

The correct answer is E. This patient has Whipple disease, which is a rare
illness characterized by diarrhea, often severe malabsorption, and weight
loss. The condition most commonly affects men aged 30 to 60. The disease
may present either abruptly or insidiously. If it develops insidiously, non-
gastrointestinal manifestations may bring the patient to medical attention.
These may include pleuritic pain, pleural effusion, anemia, joint problems,
cardiac problems, neuropsychiatric problems, eye problems, or hepatic
dysfunction.
All of the other diseases listed can also cause chronic diarrhea.
Celiac sprue (choice A) shows villous atrophy on biopsy, and is related to
gluten sensitivity.
Intestinal lymphangiectasia (choice B) shows dilated lymphatic channels on
biopsy.
Lactase deficiency (choice C) is due to an enzymatic abnormality and is
characterized by a histologically normal intestinal epithelium.
Tropical sprue (choice D) usually shows moderate broadening and
shortening of villi, and an often intense inflammatory reaction.



Explanation - Q: 7.2 Close

The correct answer is D. Electron microscopy of the intestinal mucosa
shows that the PAS-positive granules in macrophages in Whipple disease
are actually bacterial forms. The causative organism is Tropheryma whippelii,
and, if necessary, its DNA can be identified with PCR technology. This is
necessary because attempts to culture the organism have not, to date, been
successful. At the moment, this test is available only through reference
laboratories. In patients who have extraintestinal manifestations,
macrophages containing the bacteria may be found in extraintestinal sites.
Bartonella(choice A) species can cause cat scratch disease, trench fever,
and disseminated infections.
Borrelia(choice B) causes Lyme disease.
Francisella(choice C) causes tularemia.
Vibrio(choice E) causes cholera and other diarrheal illnesses.



Explanation - Q: 7.3 Close

The correct answer is D. When a patient complains of excessive flatulence,
the first step is to ask them to describe the amount and frequency of the
flatulence, since some people with normal intestinal gas production have
unrealistic expectations about the possibility of completely eliminating
flatulence. In cases in which excessive flatulence really is present, you
should think of gastrointestinal disorders that can cause malabsorption, since
many cases of excessive flatulence are related to bacterial digestion of
unabsorbed nutrients (notably carbohydrates) with resultant gas production
as a by-product. These disorders may include a wide variety of underlying
conditions including pancreatic disease, liver disease, genetic enzymatic
abnormalities of the intestinal mucosa, and acquired intestinal tract disease.
Colonic obstruction (choice A), small intestinal obstruction (choice E), and
GI hypomotility (choice C) can also cause gas to be retained in the bowel
and be visible on abdominal x-ray films, but do not, by themselves, increase
gas production.
GI hypermotility (choice B) often accompanies acute diarrheal illness, but
the excessive flatulence that may also be present in that setting is secondary
to malabsorption rather than the hypermotility.

Question 4 of 5
BIood studies on this patient demonstrate a microcytic hypochromic anemia. The
nutrient whose deficiency would most likely account for the
patient's anemia is primarily absorbed in which of the following sites?
/ A. Colon
/ B. Duodenum
/ C. Esophagus
/ D. IIeum
/ E. Stomach

Explanation - Q: 7.4 Close

The correct answer is B. Microcytic, hypochromic anemia suggests iron
deficiency. Patients with Whipple disease frequently develop anemia related
to poor duodenal absorption of either folate (producing a macrocytic anemia)
or iron. Other substances absorbed in the duodenum include water, calcium,
fats, sugars, proteins, many vitamins, magnesium, and sodium.
The colon (choice A) absorbs water and electrolytes.
The esophagus (choice C) does not absorb nutrients.
The ileum (choice D) can absorb bile salts, vitamin B12, and chloride.
The stomach (choice E) can absorb water and alcohol.

Question 5 of 5

This patient's illness is most effectively treated with which of the following?
/ A. Antibiotics
/ B. Chemotherapy
/ C. Radiation therapy
/ D. Steroid therapy
/ E. Surgery

Explanation - Q: 7.5 Close

The correct answer is A. The symptoms of Whipple disease often
dramatically improve after antibiotic therapy, although the therapy must often
be continued for prolonged intervals to prevent relapse and the microscopic
intestinal changes may not resolve for 2 years. Many antibiotics are effective,
including chloramphenicol, tetracycline, ampicillin, penicillin, and
trimethoprim-sulfamethoxazole. The other therapies listed in the choices are
not usually employed with Whipple disease.










Within a two-day period, a pediatrician in a rural community sees sixteen children
between the ages of 2 and 6 with gastroenteritis. The illness
began with a fever and abdominal pain. The diarrhea was initially watery, but in
some patients, subsequently became mucoid and bloody.
Three days before the onset of illness, all of the children had attended a picnic at
a city park where they played in a wading pool later found to
have an inadequate level of chlorination. Stool cultures performed on these
children show a nonmotile gram-negative rod that does not ferment
Iactose.


Question 1 of 4

Which of the following organisms is the most likely cause of the illnesses?



Explanation - Q: 8.1 Close

The correct answer is D. Shigella is a common cause of bacterial diarrhea
worldwide. It is an obligate human pathogen. In children, Shigella
gastroenteritis typically presents with diarrhea, abdominal cramps, and fever.
The stools are characteristically small in volume, bloody, and mucoid.
Shigella species are members of the family Enterobacteriaceae. They are
nonmotile and nonlactose fermenters. There are 4 serogroups of Shigella: S.
dysenteriae, S flexneri, S. boydii and S. sonnei. The severity of the disease
varies with the infecting serogroup. S. sonnei causes mild disease. S.
dysenteriae and S. flexneri commonly cause dysentery. In the United States,
S. sonnei and S. flexneri infections are most common, and most cases of
shigellosis occur in daycare centers or residential institutions. Infections
caused by S. dysenteriae are usually imported from Mexico or Central
America.
Campylobacter jejuni(choice A) is a leading cause of acute diarrhea
worldwide. Campylobacter gastroenteritis is essentially a food-borne
disease, although infection also can be acquired through direct contact with
animals or their products. Campylobacter jejuni inhabits the intestinal tracts
of a wide range of animals, especially poultry. The most common clinical
features in children are diarrhea, fever, abdominal pain, and vomiting. Blood
in the stools is present in more than one-half of the children with
Campylobacter gastroenteritis. Campylobacter needs to be cultured on
specific selective media, and incubated under microaerophilic and
capnophilic conditions. They have a distinctive appearance on Gram's stain.
They are gram-negative, seagull-shaped organisms. Microscopic
appearance as well as testing for oxidase and catalase production are all
that is needed to confirm a diagnosis.
Enterohemorrhagic E. coli (EHEC)(choice B) was first noted as a pathogen
in the early 1980s. It was found to be the cause of an outbreak of bloody
diarrhea acquired after eating hamburgers at a fast food chain. EHEC strains
colonize cattle, and the transmission of EHEC is most commonly associated
with the consumption of undercooked hamburger meat. This is probably
because grinding distributes the bacteria throughout the meat rather than
leaving it on the surface where it could easily be killed by cooking. However,
EHEC outbreaks have also been associated with contaminated lettuce,
sprouts, and apple cider. E. coli is a member of the Enterobacteriaceae. It is
motile and ferments lactose. The enterohemorrhagic E. coli causing the early
outbreaks belonged to serogroup O157:H7, which could be distinguished
from other E. coli by its inability to ferment sorbitol. However, in the last few
years, major outbreaks of non-O157 EHEC disease have occurred in several
countries.
Salmonella enteritidis(choice C) is the number one cause of food-borne
illness in the United States, and is responsible for more deaths than any
other food-borne pathogen. Salmonella enteritidis has many animal
reservoirs. Typical sources of human infection are poultry, and especially
eggs, since the bacteria can be passed transovarially from chickens to intact
eggs. Salmonella is a member of the Enterobacteriaceae. It is motile and a
nonlactose fermenter.
Yersinia enterocolitica(choice E) can cause both outbreaks and sporadic
cases of gastroenteritis. Bloody diarrhea, fever, and vomiting are more
common in children than adults. It is also a member of the family
Enterobacteriaceae. It is motile and a nonlactose fermenter.
Question 2 of 4
Which of the following is the infecting dose of the organism needed to cause this
disease?
/ A. 10 to 100 organisms
/ B. 104-106 organisms
/ C. 105-108organisms
/ D. 108organisms
/ E. 109 organisms

Explanation - Q: 8.2 Close

The correct answer is A. Data on the number of organisms required for
clinical illness was obtained from studies on human volunteers and
outbreaks in which the source of infection could be quantitatively cultured.
Shigella is not as susceptible to acid as many other bacterial pathogens.
Because of this, Shigella can survive passage through the stomach, so only
a few organisms (10 to 100 organisms) are needed to cause disease. Since
the amplification of bacterial numbers in contaminated food or water is not
essential to achieve an infectious dose, Shigella can be transmitted directly
from person-to-person, as well as from contaminated food and water.
10
4
to 10
6
organisms (choice B) is the infectious dose for Campylobacter
jejuni.
10
5
-10
8
organisms (choice C) is the infectious dose for Salmonella
enteritidis.
10
8
organisms (choice D) is the infectious dose for enteropathogenic E. coli.
10
9
organisms (choice E) is the infectious dose for Yersinia enterocolitica.


Explanation - Q: 8.3 Close

The correct answer is B. Shiga toxin and Shiga-like toxin are two
component cytotoxins that inhibit cellular protein synthesis by enzymatically
cleaving 28S rRNA, which prevents EF-dependent binding of tRNA to
ribosomes. Shiga toxin is found in Shigella dysenteriae. Shiga-like toxin
differs from Shiga toxin by one amino acid, and is found in other species of
Shigella and in enterohemorrhagic E. coli (EHEC), where it is carried on a
lysogenic phage. All Shigella also require a large virulence plasmid, which
contains genes that are required for invasion, and intra- and intercellular
spread.
Cytolethal distending toxin (choice A) is a single-stranded DNAase made by
Campylobacter jejuni.
A heat stable enterotoxin that causes vomiting (choice C) describes the
enterotoxin of Staphylococcus aureus. It acts on neural receptors in the
upper GI tract leading to stimulation of vomiting center.
A plasmid-encoded enterotoxin that stimulates adenylate cyclase (choice D)
describes LT toxin of enteropathogenic E. coli. LT toxin is 80% homologous
with cholera toxin and has the same mechanism of action. The B subunits
bind to a ganglioside on the cell membrane and the A subunit is then
internalized, where it catalyzes the ADP-ribosylation of Gs protein, destroying
the mechanism to turn off the stimulation of adenyl cyclase. This results in
increased levels of cAMP, leading to an increase in prostaglandin E and the
secretion of salt and water.
A tyrosine phosphatase induced by low calcium concentrations (choice E)
describes the YopH gene product of Yersinia. This tyrosine phosphatase is
broad-spectrum and dephosphorylates all tyrosine-phosphorylated host
proteins, subverting phagocytosis.


Question 4 of 4

AIthough infection with this pathogen is generally self-Iimited, several of the
children require treatment with trimethoprim-sulfamethoxazole. This
medication works via which of the following mechanisms?

/ A. Inhibits DNA gyrase
/ B. Inhibits folate synthesis
/ C. Inhibits protein synthesis by binding to the 30s ribosomal subunit
/ D. Inhibits protein synthesis by binding to the 50s subunit
/ E. Inhibits RNA polymerase

Explanation - Q: 8.4 Close

The correct answer is B. Both trimethoprim and sulfamethoxazole inhibit
microbial folate synthesis. When combined, they have a synergistic effect.
Inhibiting DNA gyrase (choice A) is the mechanism of action of the
fluoroquinolones, such as ciprofloxacin and levofloxacin.
Inhibiting protein synthesis by binding to the 30s ribosomal subunit (choice
C) is the mechanism of action of the tetracyclines such as doxycycline and
minocycline.
Inhibiting protein synthesis by binding to the 50s subunit (choice D) is the
mechanism of action of the macrolides, which include erythromycin and the
long acting macrolides, azithromycin and clarithromycin.
Inhibiting RNA polymerase (choice E) is the mechanism of action of the
rifamycins, rifampin, and rifabutin.




A 32 year-old successful female lawyer in a ball gown is dropped off at the
emergency room. She complains of an unusual sensation in her
chest and recently had a nose bleed. She is irritable and talks about "Iiberals,"
trying to shut her office down because she "stands for what is
right." She explains being dropped off at the emergency room as a conspiracy
against her. She reports that tonight's party was to celebrate
her promotion to full partner. She throws her supper tray across the room and
says "Oh please, you don't think l would be so stupid as to eat
that, I know what you are up to." When interviewed, she refuses to speak to the
doctor who enters and then lunges at the "jealous hag trying to
frame her." The patient refuses vital signs but is noted to have mydriasis.
Question 1 of 6
Which of the following is the most appropriate pharmacotherapy?
/ A. Lorazepam
/ B. Methylphenidate
/ C. Midazolam
/ D. Sertraline
/ E. Zolpidem

Explanation - Q: 1.1 Close

The correct answer is A. Lorazepam is often used for treating agitation in
the emergency room due to its quick absorption. Onset of action is within
about thirty minutes.
Methylphenidate (choice B) is a stimulant used to treat attention deficit
disorder. Stimulants would worsen agitation and psychosis.
Midazolam (choice C) is a rapid acting parenteral benzodiazepine used in
anesthesiology. It is not indicated for psychiatry disorders.
Sertraline (choice D) is a SSRI, used to treat depression and anxiety
disorders. Treatment of agitation is the primary goal at this point.
Zolpidem (choice E) is an imidazopyridine that binds the GABA-
benzodiazepine receptor complex. It is used to induce sleep.
Question 2 of 6
This patient is at risk for which of the following complications?
/ A. Anxiety and amotivational syndrome
/ B. Ataxia and respiratory depression
/ C. Bone marrow depression and immunosuppression
/ D. Brain damage and peripheral neuropathies
/ E. Intracranial hemorrhage and myocardial infarction



Explanation - Q: 1.2 Close

The correct answer is E. The patient's symptoms are consistent with
cocaine intoxication. Pupillary dilation, hypertension, tachycardia,
hallucinations, aggressive behavior and paranoia can all be seen with
amphetamine or cocaine use. Acutely, cocaine ingestion can produce
intracranial hemorrhage or myocardial infarction in certain individuals. Long-
term snorting can lead to nosebleeds.
Anxiety and amotivational syndrome (choice A) is commonly associated with
long term marijuana use.
Ataxia and respiratory depression(choice B) is associated with alcohol,
barbiturate or benzodiazepine use.
Brain damage and peripheral neuropathies (choice D) and bone marrow
depression and immunosuppression (choice C) are complications of inhalant
(glues, solvents and cleaners) abuse.


Question 3 of 6
If, in addition to the above history, the patient is also pregnant, which of the
following complications might occur?
/ A. CIeft palate abnormalities
/ B. Congenital hypothyroidism
/ C. Ebstein's anomaly
/ D. Neural tube defects
/ E. PIacental abruption

Explanation - Q: 1.3 Close

The correct answer is E. Maternal cocaine use is associated with placental
abruption. Drug-induced hypertension may play a role.
Cleft palate abnormalities (choice A) are associated with carbamazepine.
Ebstein's anomaly (choice C) and congenital hypothyroidism (choice B) are
side effects of lithium.
Neural tube defects (choice D) are associated with valproic acid.

Question 4 of 6
Which of the following most accurately describes the mechanism responsible for
this patient's presentation?
/ A. BIockade of dopamine receptors
/ B. BIockade of NMDA receptors
/ C. Increased affinity of binding to GABA receptors
/ D. Increased release of monoamines in the synaptic cleft
/ E. Inhibition of monoamine reuptake in the synaptic cleft


Explanation - Q: 1.4 Close

The correct answer is E. Cocaine inhibits the reuptake of norepinephrine,
dopamine, and serotonin (monamines) from the synaptic cleft.
Blockade of dopamine receptors (choice A) is the mechanism of action
responsible for the efficacy and some side effects of the antipsychotic
medications.
Blockade of NMDA receptors (choice B) is the mechanism of action of PCP.
Increased affinity of binding to GABA receptors (choice C) is a mechanism
of both benzodiazepines and barbiturates, in addition to potentiation of the
chloride channels.
Increased release of monamines (choice D) is the mechanism of action of
amphetamines. Amphetamine intoxication would be less likely than cocaine
intoxication in this successful professional woman. The recent history of a
nose bleed also tends to suggest cocaine abuse.
Question 5 of 6
The patient responds very little to the first medication, but begins pacing and
ranting at the nurses. When a police officer appears, she attacks
her when she hallucinates and hears a voice saying the officer is "really working
for the other side." Which of the following is the most
appropriate pharmacotherapy?
/ A. Amitriptyline
/ B. CIonazepam
/ C. Cyproheptadine
/ D. Haloperidol
/ E. Propanolol

Explanation - Q: 1.4 Close

The correct answer is E. Cocaine inhibits the reuptake of norepinephrine,
dopamine, and serotonin (monamines) from the synaptic cleft.
Blockade of dopamine receptors (choice A) is the mechanism of action
responsible for the efficacy and some side effects of the antipsychotic
medications.
Blockade of NMDA receptors (choice B) is the mechanism of action of PCP.
Increased affinity of binding to GABA receptors (choice C) is a mechanism
of both benzodiazepines and barbiturates, in addition to potentiation of the
chloride channels.
Increased release of monamines (choice D) is the mechanism of action of
amphetamines. Amphetamine intoxication would be less likely than cocaine
intoxication in this successful professional woman. The recent history of a
nose bleed also tends to suggest cocaine abuse.


Question 5 of 6
The patient responds very little to the first medication, but begins pacing and
ranting at the nurses. When a police officer appears, she attacks
her when she hallucinates and hears a voice saying the officer is "really working
for the other side." Which of the following is the most
appropriate pharmacotherapy?
/ A. Amitriptyline
/ B. CIonazepam
/ C. Cyproheptadine
/ D. Haloperidol
/ E. Propanolol

Explanation - Q: 1.5 Close

The correct answer is D. Haloperidol is often used in the emergency setting
and the ICU to treat psychotic aggression due to its rapid onset of action and
multiple formulations (oral, IV, IM).
Amitriptyline (choice A) is a tricyclic antidepressant with marked
anticholinergic properties. It would not be appropriate for this patient.
Clonazepam (choice B) does not have a rapid onset of action and is not
used in this setting. More rapidly acting benzodiazepines can be used in the
emergency setting. This patient had assaulted someone due to her psychotic
state and the psychosis needs treatment.
Cyproheptadine (choice C) has been used to target the sexual side effects
induced by treatment with SSRIs.
Propranolol (choice E) is a nonspecific beta-blocker. It is useful for "stage
fright", but would not be effective to treat this patient's psychotic aggression.
Question 6 of 6
The patient refuses the medication and staff overhears her say she plans to "get
rid of the imposter, once and for alI," to "make the world a
safe place again." The patient escapes from the emergency department. The
husband usually returns from working the night shift at this time of
day. In compliance with the Tarasoff-II Supreme Court Decision, the doctor is
expected to do which of the following?
/ A. Chase the patient
/ B. Dispatch the police to warn the husband
/ C. Document the findings
/ D. Leave a message for the husband
/ E. Take out papers to have the patient involuntarily committed

Explanation - Q: 1.6 Close

The correct answer is B. The Tarasoff-II Supreme Court decision indicates
a duty to protect potential victims (not just warn them, as found in the original
Tarasoff ruling). The treating physician "bears a duty to exercise reasonable
care to protect the foreseeable victim of that danger."
Chasing a patient (choice A) is dangerous, inappropriate, and outside the
bounds of the safe practice of medicine.
Documenting the findings (choice C) is crucial, but it does not meet the
requirements of the Tarasoff rulings.
Leaving a message for the husband (choice D) does not meet the
requirements of the Tarasoff rulings.
Taking out papers to have the patient involuntarily committed (choice E) is a
crucial step, and the patient is a danger to others, but it may take several
hours for the papers to be signed, before the police can look for the patient.
Tarasoff indicates that the foreseeable victim should receive a warning if
he/she is in danger.


A 29-year-old man is brought via EMS to the emergency department. Bystanders
report the patient was staggering and mumbling, and then
collapsed. The paramedic recognizes the patient, and believes the patient to be a
homeless drug abuser. Past medical history and allergies
are unknown. BIood pressure is 130/75 mm Hg, pulse is 60/min, and respirations
are 8/min. The patient is comatose, with eyes closed, and
does not withdraw to pain. He does not smell of alcohoI. He has multiple tattoos
and needle tracks on both arms. Pupils are pinpoint and
sclerae are not jaundiced. Respirations are shallow. Abdominal examination is
unremarkable.
Question 1 of 4
Which of the following is the most appropriate pharmacotherapy?
/ A. Deferoxamine
/ B. FIumazenil
/ C. N-Acetylcysteine
/ D. Naloxone
/ E. Naltrexone


Explanation - Q: 2.1 Close

The correct answer is D. Hypoventilation, pupillary miosis, and a comatose
state is highly suggestive of an opioid overdose. The opioid in question is
probably heroin, given the needle tracks on his arms, Naloxone, an opioid
antagonist, is used to treat the acute opioid overdose.
Deferoxamine (choice A) is used to treat iron overload.
Flumazenil (choice B) is used to treat benzodiazepine overdose.
N-Acetylcysteine (choice C) is used to treat acetaminophen overdose. This
might also be appropriate had the patient ingested an excess of an
acetaminophen-opioid combination (e.g., hydrocodone and acetaminophen).
Naltrexone (choice E) is used to prevent relapse in previously opiate-
dependent individuals.



Question 2 of 4

Which of the following is frequently present in patients with this condition?
/ A. Aseptic necrosis of the hip
/ B. Diabetes insipidus
/ C. Hepatitis C
/ D. Intermittent porphyria
/ E. Subacute bacterial peritonitis

Explanation - Q: 2.2 Close

The correct answer is C. Hepatitis B, C, and HIV are all associated with IV
drug use. This patient has needle marks on his arms and his presentation is
consistent with opioid overdose.
Aseptic necrosis of the hip (choice A) is associated with long term steroid
treatment.
Diabetes insipidus (choice B) is associated with lithium.
Intermittent porphyria (choice D) can be precipitated by barbiturate intake in
vulnerable persons.
Subacute bacterial peritonitis (choice E) is associated with ascites and other
signs of cirrhotic liver disease. This patient does not smell of alcohol, and the
examination did not reveal ascites, caput medusa, edema, or bulging flanks.

Question 3 of 4
The patient is admitted to the hospitaI. An arterial blood gas is most likely to
show which of the following?
/ A. Metabolic acidosis
/ B. Metabolic alkalosis
/ C. Marked anion gap
/ D. Respiratory acidosis
/ E. Respiratory alkalosis

Explanation - Q: 2.2 Close

The correct answer is C. Hepatitis B, C, and HIV are all associated with IV
drug use. This patient has needle marks on his arms and his presentation is
consistent with opioid overdose.
Aseptic necrosis of the hip (choice A) is associated with long term steroid
treatment.
Diabetes insipidus (choice B) is associated with lithium.
Intermittent porphyria (choice D) can be precipitated by barbiturate intake in
vulnerable persons.
Subacute bacterial peritonitis (choice E) is associated with ascites and other
signs of cirrhotic liver disease. This patient does not smell of alcohol, and the
examination did not reveal ascites, caput medusa, edema, or bulging flanks.

Question 3 of 4
The patient is admitted to the hospitaI. An arterial blood gas is most likely to
show which of the following?
/ A. Metabolic acidosis
/ B. Metabolic alkalosis
/ C. Marked anion gap
/ D. Respiratory acidosis
/ E. Respiratory alkalosis


Explanation - Q: 2.3 Close

The correct answer is D. Opioids are central CNS depressants, and are
associated with hypoventilation and CO
2
retention, which results in
respiratory acidosis.
Metabolic acidosis (choice A) is associated with substrates such as ketones,
uric acid, salicylates, methyl alcohol, ethylene glycol, and lactic acid.
Metabolic alkalosis (choice B) is associated with loss of acid (vomiting) or
excess bicarbonate or alkali.
A marked anion gap (choice C) is seen in diabetic ketoacidosis, among
other causes.
Respiratory alkalosis (choice E) is associated with hyperventilation and loss
of CO
2
.


Question 4 of 4
Which of the following treatments can be used for long-term maintenance after
this patient is discharged from the hospitaI?
/ A. Disulfiram
/ B. FIumazenil
/ C. Naloxone
/ D. Naltrexone
/ E. Polyethylene glycol

Explanation - Q: 2.4 Close

The correct answer is D. Naltrexone, an opioid antagonist with a longer
half-life than naloxone, is used to prevent relapse in previously opiate-
dependent individuals. It is most successful in the highly motivated patient.
Naltrexone can also be used in combination with clonidine for rapid
detoxification. Clonidine, an alpha 2 agonist, decreases some of the
autonomic symptoms during withdrawal. Later, the patient can alternatively
be maintained on opioid maintenance agonists, such as methadone, levo-
alpha-acetylmethadol (LAAM), or buprenorphine.
Disulfiram (choice A) is used to prevent relapse with alcohol. Persons who
drink alcohol become very ill due to inhibition of aldehyde dehydrogenase.
Flumazenil (choice B) is used to treat benzodiazepine overdose.
Naloxone (choice C) is used to treat acute opioid overdose.
Polyethylene glycol (choice E) is a laxative, which produces whole bowel
irrigation. This may be indicated for patients with massive oral overdoses of
opioids, or in "body packers" (people who smuggle heroin, often in balloons,
in their GI tract) if one of the packages ruptures.


A 46-year-old homeless man is brought to the emergency department after the
EMS receive a call to evaluate a "man down" on the street. No
one had witnessed the event, but the paramedic recognizes the patient as a
chronic substance abuser with a history of alcohol and drug use.
Past medical history is significant for cirrhosis. On examination, the patient is
somnolent, but arousable and is not confused. He smells of
alcohoI, is mildly jaundiced and says "Ieave me alone, Iet me go, I just fell
asleep." His blood pressure is 140/86 mm/Hg, pulse is 78/min, and
respirations are 15/min. The patient refuses laboratory tests and states "I'd rather
die than go back in the hospitaI." The staff knows the patient
has changed his mind in the past and allows the patient to sleep it off for a few
hours. The patient awakens, has a fight with the nurse and
twenty minutes later is found extremely difficult to arouse with an empty bottle of
clonazepam on the floor.


Question 1 of 5

Which of the following is the most appropriate pharmacotherapy?
/ A. Acetylcysteine
/ B. Deferoxamine
/ C. FIumazenil
/ D. Naloxone
/ E. Naltrexone


Explanation - Q: 3.1 Close

The correct answer is C. Flumazenil is used to reverse benzodiazepine
(e.g., clonazepam) overdose. This agent competitively inhibits the action of
benzodiazepines at the benzodiazepine recognition site on the
GABA/benzodiazepine receptor complex. Importantly, flumazenil does not
antagonize the effects of other CNS drugs affecting GABAergic neurons
including ethanol, barbiturates, opioids, or general anesthetics.
Acetylcysteine (choice A) is used in acetaminophen overdose.
Deferoxamine (choice B) is used in iron overdose.
Naloxone (choice D) is used in opioid overdose.
Naltrexone (choice E) is used to promote abstinence from opioid abuse.


Question 2 of 5

The patient recovers from the event in the emergency department and is
admitted to the ICU to evaluate possible gastric bleeding. When
asked about a history of drug and alcohol use, the patient gives a long history of
"taking everything l could get my hands on." Which of the
following substances is associated with a fatal withdrawal syndrome?
/ A. CIozapine
/ B. CIonazepam
/ C. Cocaine
/ D. Heroin
/ E. Pentobarbital

Explanation - Q: 3.2 Close

The correct answer is E. Barbiturate withdrawal (similar to alcohol
withdrawal) can lead to delirium, convulsions, and death. Pentobarbital is
given in a "challenge" or test dose to estimate the amount of barbiturate
needed to adequately treat withdrawal.
Clozapine (choice A) is an antipsychotic medicine reserved for refractory
schizophrenia; seizures may develop if doses are missed.
Clonazepam (choice B) is a benzodiazepine with a long half-life. Withdrawal
is associated with anxiety and insomnia.
Cocaine (choice C) can be fatal in overdose, but not withdrawal. Withdrawal
symptoms usually include tiredness, craving for cocaine, lack of energy, and
depressed mood (sometimes with suicidality).
Heroin (choice D) can be fatal in overdose, but not withdrawal. Opioid
detoxification can be treated symptomatically (clonidine, phenergan,
hyoscyamine, ibuprofen, or cyclobenzaprine). Patients in opioid withdrawal
have extreme discomfort from nausea, diarrhea, and myalgia often
accompanied by depressed mood. Withdrawal is extremely unpleasant, but
generally not fatal.

Question 3 of 5

The patient gives a history of a code team revival after taking an intentional
overdose on "vodka, sleeping pills, and pain-killers." Which of the
following physiologic processes is usually responsible for a fatal overdose on
these substances?
/ A. Agranulocytosis
/ B. Hypertensive crisis
/ C. QTc prolongation
/ D. Respiratory depression
/ E. Stevens-Johnson syndrome

Explanation - Q: 3.3 Close

The correct answer is D. Respiratory depression is a property shared by
alcohol, benzodiazepines, barbiturates, opiates, and drugs like chloral
hydrate (an older sedative-hypnotic). The effects of these different drugs are
additive, making them even more dangerous.
Agranulocytosis (choice A) is a rare (about 1%) side effect associated with
clozapine treatment. Undetected infections can be fatal. Patients taking
clozapine receive frequent complete blood counts.
Hypertensive crisis (choice B) is associated with tyramine ingestion
concurrent with monoamine oxidase inhibitor treatment. It can be fatal.
QTc prolongation (choice C) is associated with several older antipsychotic
medications (thioridazine, droperidol) and especially with tricyclic
antidepressants. QTc prolongation is associated with the development of
Torsades de Pointes and cardiac arrest.
Stevens-Johnson syndrome (choice E) is associated with carbamazepine,
an anticonvulsant used to treat bipolar disorder. Stevens-Johnson syndrome
has also been reported to be associated with some of the newer
anticonvulsant medications as well.
Question 4 of 5

After four days in the hospitaI, the patient develops elevated blood pressure,
tachycardia, severe tremor, and states that "the last time l
stopped drinking l started seeing things and had seizures." Which of the following
is appropriate pharmacotherapy in this patient?
/ A. Diazepam
/ B. CIorazepate
/ C. FIurazepam
/ D. Halazepam
/ E. Lorazepam

Explanation - Q: 3.4 Close

The correct answer is E. The patient's report is classic for alcohol
withdrawal with impending delirium tremens, and benzodiazepines are
indicated. Lorazepam has a shorter half-life than the other drugs listed and
no active metabolites. It is metabolized only by phase II (conjugation) in the
liver. Benzodiazepines metabolized by phase II only are better tolerated in
patients with impaired liver function (elderly patients, patients with cirrhosis).
In summary, lorazepam is the drug of choice when accumulation is a
concern, because lorazepam has a short half-life, no active metabolites, and
is biotransformed only by phase II metabolism. The remaining drugs
(choices A, B, C, and D) all undergo phase I metabolism also. Temazepam
and oxazepam are examples of other benzodiazepines that are metabolized
by phase II only


Question 5 of 5
The patient recovers and returns to the hospital after sustaining an unwitnessed
head injury. Initial workup reveals no changes in mental status
or findings on CT of the head, but the patient develops a seizure disorder and is
started on carbamazepine, and phenobarbital is added six
weeks later. The patient is at risk for which of the following?
/ A. Decreased carbamazepine levels
/ B. Gingival hyperplasia
/ C. Hypernatremia
/ D. Increased phenobarbital levels
/ E. Vitamin D toxicity

Explanation - Q: 3.5 Close

The correct answer is A. Carbamazepine levels decrease over time by
p450 autoinduction, and also the addition of phenobarbital (one of the most
potent of enzyme-inducers) will decrease the levels of other anticonvulsants
the patient is concurrently taking.
Gingival hyperplasia (choice B) is associated with phenytoin administration.
Hypernatremia (choice C) is not associated with these medicines, but
carbamazepine and the newer anticonvulsants are associated with hypo-
osmolar hyponatremia.
Carbamazepine does not inhibit P450 (actually, it induces P450) and
therefore would not increase levels of phenobarbital (choice D).
Vitamin D (choice E) can be depleted by carbamazepine, but vitamin D is
not increased (or toxic). Carbamazepine can also decrease folate levels.





A 25-year-old woman consults a physician because she has developed severe
dysphagia and constipation that has led to a recent weight loss
of 15 pounds. She is referred to a gastroenterologist, to whom she reports that
she has a great deal of trouble swallowing solid foods, but not
Iiquids, and has also been having nearly constant gastroesophageal reflux
symptoms. She has awakened during the night several times
coughing and feeling as if she had aspirated acid stomach contents.
Esophagogastroduodenoscopy and colonoscopy are performed, and
demonstrate massive dilatation of the esophagus and colon.
Question 1 of 5
Which of the following parasitic diseases would most likely produce
megaesophagus and megacolon?
/ A. Chagas disease
/ B. Cysticercosis
/ C. Hydatid disease
/ D. Malaria
/ E. Threadworm infection

Explanation - Q: 1.1 Close

The correct answer is A. Megaesophagus and megacolon are unusual
pathologic changes, and should bring Chagas disease to mind, which is also
known as American trypanosomiasis. Megacolon, but not megaesophagus,
can also be due to Hirschsprung disease, but this is usually diagnosed in
infants and young children and is due to abnormal neural development in the
gut.
Cysticercosis (choice B) can cause cyst formation in the brain and other
organs.
Hydatid disease (choice C) can cause cysts in the liver and other organs.
Malaria (choice D) can be complicated by fever, chills, anemia,
splenomegaly, and in severe cases, renal failure, coma, thrombocytopenia,
and respiratory distress syndrome.
Threadworm infection (choice E) can cause skin rashes, eosinophilia,
abdominal pain, and, in severe cases, massive GI bleeding, severe
malnutrition, and severe pulmonary symptoms
Question 2 of 5
The infecting species is which of the following?
/ A. FIuke
/ B. Nematode
/ C. PIasmodia
/ D. Tapeworm
/ E. Trypanosome

Explanation - Q: 1.2 Close

The correct answer is E. Chagas disease is caused by infection with
Trypanosoma cruzi, which is an intracellular protozoan parasite that takes a
leishmanial form within muscle cells.
Examples of fluke (choice A) or trematode diseases include schistosomiasis
(blood flukes), paragoniasis (lung fluke), and various liver flukes (e.g.,
clonorchiasis, fascioliasis, fasciolopsiasis).
Examples of nematode (choice B), or round worm diseases, include the
various intestinal nematodes (e.g., ascariasis, trichuriasis, ancylostomiasis,
strongyloidiasis), tissue nematodes (e.g., toxocariasis, trichinosis,
dracunculosis), and the filarial nematodes (e.g., elephantiasis,
onchocerciasis).
Plasmodia (choice C) cause malaria.
Tapeworm infections (choice D) include intestinal infections
(diphyllobothriasis, Taenia infections), cysticercosis (due to Taenia solium),
and Echinococcus infections.


Question 3 of 5
On further questioning, the woman reports having lived for a year in Bolivia as a
teenager. At one point, she had had an automobile accident
and had been transfused with blood at a local hospital there. The person whose
blood she received had probably acquired the infection via a
bite by which of the following?
/ A. FIea
/ B. Mosquito
/ C. Reduviid bug
/ D. Tick
/ E. Tsetse fly

Explanation - Q: 1.3 Close

The correct answer is C. Reduviid bugs are insects that live in poorly
constructed adobe housing and feed on human or animal blood.
Trypanosomes they ingest during their feedings multiply in the insect gut and
are deposited in fecal material near the wound when the insect bites another
individual. Contamination through the wound leads to invasion into, and
reproduction within, host cells. Release of the trypanosomes back into the
blood stream makes them available for transmission either via another bug
bite or via blood transfusion. Transmission of Chagas disease via blood
transfusion has also become an important mode of transmission in South
America. Santa Cruz, Bolivia is thought to have a prevalence of infected
blood in blood banks of 53%, which is much higher than the rate of hepatitis
or HIV infection. In other parts of South America, the infection rate is much
lower, due primarily to a massive attempt by the World Health Organization
to eliminate both the reduviid bug (through better housing and insecticide
use) and screen for Chagas disease in blood products in endemic regions.
These attempts have been most successful in eradicating or nearly
eradicating the infection in Chile, Uruguay, and Brazil. Transmission of
Chagas disease via blood transfusions has not been a significant problem in
the United States, primarily because one of the questions asked before a
blood donation is taken is whether or not a person has traveled outside the
United States during the previous year. While Chagas disease can persist for
decades, the period in which significant numbers of parasites are in the
blood is usually only during the comparatively short (and often
asymptomatic) acute phase of the illness.
Flea-borne illnesses (choice A) to remember include bubonic plague and
typhus.
Associate mosquito bites (choice B) with malaria.
Associate tick bites (choice D) with Lyme disease, babesiosis, and Rocky
Mountain spotted fever.
Associate Tsetse fly bites (choice E) with African trypanosomiasis (sleeping
sickness).


Question 4 of 5
While this woman's gastrointestinal tract disease brought her to medical
attention, involvement of which of the following is the principal source
of morbidity and mortality in this condition?
/ A. Heart
/ B. Kidneys
/ C. Liver
/ D. Lungs
/ E. Pancreas

Explanation - Q: 1.4 Close

The correct answer is A. Chagas disease is conventionally divided into
three stages. The short acute period may be asymptomatic or may be
characterized by fever, swelling of lymph glands, hepatosplenomegaly, and
local inflammation at the site of the bite. This is followed by an asymptomatic
period, which may last years to the rest of the patient's life. In one-third of
patients, clinically evident disease becomes apparent 10-20 years after
infection. 27% of infected patients develop cardiac symptoms (which may
cause flaccid cardiomyopathy, cardiac aneurysm formation, or sudden
death); 6% develop digestive system damage with megaviscera (thought to
be related to neural involvement rather than muscle involvement); and 3%
develop neural involvement. You should also be aware that coexisting AIDS
infection may predispose for an unusually rapid and severe course of
Chagas disease.
The kidneys (choice B), liver (choice C), lungs (choice D), and pancreas
(choice E) are not particular targets in this disease.


Question 5 of 5
While no drug therapy is useful in the therapy of chronic cases, treatment can be
used in newly diagnosed acute cases. Which of the following
medications would be most useful in treating the acute infection?
/ A. Chloroquine
/ B. Doxycycline
/ C. Nifurtimox
/ D. Primaquine
/ E. Pyrimethamine-sulfadoxine

Explanation - Q: 1.5 Close

The correct answer is C. The diagnosis in suspected acute cases can be
established with review of thin or thick blood smears. (The diagnosis in latent
and chronic cases usually requires special blood culture techniques or PCR-
amplified detection of parasite DNA in blood.) The only effective drugs are
nifurtimox and benznidazole, which are given in courses up to 4 months
because the organisms are difficult to eradicate. The other choices listed are
drugs used in malaria prophylaxis or treatment.


A 65-year-old man presents to his physician because he has been having
increasing difficulty swallowing over the past 2 months. He is still
able to swallow liquids, but swallowing solid food now causes severe pain and a
sense of fullness behind his sternum. He has lost 18 pounds
since his swallowing difficulties began. The patient is referred to a
gastroenterologist, who demonstrates a mass lesion of the distal
esophagus, which on biopsy is shown to contain cancer.



Question 1 of 6

Which of the following is most important in separating the esophagus from the
larynx, and must consequently be carefully passed behind
during endoscopy?
/ A. Arytenoids
/ B. Cricoid cartilage
/ C. Epiglottis
/ D. Pharynx
/ E. Vocal cords

Explanation - Q: 2.1 Close

The correct answer is C. Endoscopists are very careful when guiding the
endoscope past the epiglottis, which is a pear-shaped portion of elastic
cartilage that can be moved during swallowing to close the larynx, preventing
swallowed material from eventually entering the lungs.
The arytenoids (choice A) are the site of the attachment of the vocal cords
(choice E) within the larynx.
The cricoid cartilage (choice B) is in the more distal portion of the larynx.
The pharynx (choice D) is shared by the respiratory and gastrointestinal
tracts.



Question 2 of 6

Which of the following nerves provides the efferent impulses necessary for the
esophageal actions that occur during swallowing?
/ A. GIossopharyngeal
/ B. Hypoglossal
/ C. Spinal accessory
/ D. Trigeminal
/ E. Vagus

Explanation - Q: 2.2 Close

The correct answer is E. The vagus nerve supplies the efferent input into
the esophagus that is necessary for swallowing.
The glossopharyngeal nerve (choice A) provides taste and sensation on the
palate, but the only muscle it supplies is the stylopharyngeus.
The hypoglossal nerve (choice B) moves the tongue during the initiation of
swallowing, but does not innervate the esophagus.
The spinal accessory nerve (choice C) plays no role in swallowing. This
nerve mediates head and shoulder movement and innervates laryngeal
muscles.
The trigeminal nerve (choice D) provides general sensation to the mouth
and motor innervation to the muscles of mastication.


Question 3 of 6
Which of the following approximately represents the proportion of different
esophageal cancer types now being observed in the United States?
/ A. 1/10 adenocarcinoma and 9/10 squamous cell carcinoma
/ B. 1/3 adenocarcinoma and 2/3 squamous cell carcinoma
/ C. 1/2 adenocarcinoma and 1/2 squamous cell carcinoma
/ D. 2/3 adenocarcinoma and 1/3 squamous cell carcinoma
/ E. 9/10 adenocarcinoma and 1/10 squamous cell carcinoma

Explanation - Q: 2.3 Close

The correct answer is C. More recent statistics indicate that the incidence
of adenocarcinoma and squamous cell carcinoma of the esophagus are now
roughly equal. Formerly, approximately 2/3 of the esophageal cancers were
squamous in origin (choice B). Adenocarcinoma of the esophagus is often
found in the distal esophagus.

Question 4 of 6
Precancerous metaplasia of the esophageal epithelium gives rise to a mucosa
resembling which of the following?
/ A. Mesothelium
/ B. Respiratory epithelium
/ C. Small intestine
/ D. Squamous epithelium
/ E. Stomach

Explanation - Q: 2.4 Close

The correct answer is C. This is an indirect question about Barrett's
esophagus, which is an important precursor of adenocarcinoma of the
esophagus. While Barrett's esophagus was initially defined to be either
gastric-type or intestinal-type metaplasia of the esophagus, more recent
studies have shown that the actual problem lesion is more likely to be
intestinal metaplasia (diagnosed when isolated goblet cells are seen in the
epithelium) rather than gastric metaplasia (choice E).
Metaplasia to mesothelium (choice A) or ciliated respiratory epithelium
(choice B) does not usually occur in the esophagus.
The normal epithelium of most of the esophagus is squamous (choice D).


Question 5 of 6
Frequent use of which of the following has recently been found to probably have
a protective effect against development of esophageal
cancer?
/ A. Acetaminophen
/ B. AIcohol
/ C. Aspirin
/ D. Cigarettes
/ E. Codeine

Explanation - Q: 2.5 Close

The correct answer is C. An interesting new research observation that may
be exploited in the future is that the incidence of esophageal cancer appears
to be much lower in people who use aspirin frequently.
Cigarettes (choice D) and alcohol use (choice B) have been implicated as
risk factors for esophageal cancer.
Acetaminophen (choice A) and codeine (choice E) have no known effects
on the incidence of esophageal cancer.


Question 6 of 6
Currently, esophageal cancer has which of the following long-term survival rates?
/ A. Less than 5%
/ B. 30%
/ C. 50%
/ D. 70%
/ E. More than 95%

Explanation - Q: 2.6 Close

The correct answer is A. Esophageal cancer is one of the very bad
cancers, presently with poor long-term survival. The underlying problem is
that the esophagus is only about 3 mm thick, and both metastatic disease
and direct spread (often unresectable) to mediastinal structures is common.
Active research is presently being undertaken to modify this prognosis by
using chemotherapy and radiation therapy prior to surgery, but these
modalities have not yet come into widespread use.


A 30-year-old man consults a physician because he has been having increasing
difficulty swallowing both solids and liquids. Physical
examination of the patient is noncontributory. Barium swallow studies show a
mostly dilated esophagus with slow passage of barium into the
stomach. The very distal part of the esophagus appears narrowed into a "bird's
beak." Esophageal manometry shows incomplete relaxation of
the lower esophageal sphincter in response to swallowing, high resting lower
esophageal pressure, and absent esophageal peristalsis.

Question 1 of 5

The manometry and barium swallow studies most strongly support which of the
following diagnoses?
/ A. Achalasia
/ B. Adenocarcinoma
/ C. Barrett esophagus
/ D. Squamous cell carcinoma
/ E. Systemic sclerosis

Explanation - Q: 3.1 Close

The correct answer is A. The most likely diagnosis is achalasia. This
condition is a neurogenic esophageal disorder that can occur at any age, but
frequently is diagnosed when individuals are between the ages of 20 and 40.
Characteristically, the swallowing difficulties involve both solid food and
liquids. The manometry findings illustrated are typical; the barium swallow
findings may be as illustrated or may instead show diffuse esophageal
dilation without the "bird's beak" near the lower esophageal sphincter.
Larger cancers of the esophagus (choices B and D) would be more likely to
cause either a mass or an ulceration, which would be visible on barium
swallow.
Very small cancers and Barrett's esophagus (choice C) would require
esophagogastroduodenoscopy with biopsy for diagnosis, and would be
unlikely to cause dysphagia.
Systemic sclerosis (choice E) can involve the esophagus as well, however,
physical examination would usually show obvious skin involvement.

Question 2 of 5
This patient's condition is most likely due to which of the following?
/ A. Acid reflux
/ B. Cancerous destruction
/ C. Candida infection
/ D. Fibrosis of the esophageal wall
/ E. Lack of ganglion cells


Explanation - Q: 3.2 Close

The correct answer is E. Individuals who have achalasia have been found
to have a deficiency of inhibitory ganglion cells within the esophageal wall.
This lack causes an imbalance in excitatory and inhibitory neurotransmission,
with the result that the lower esophageal sphincter tends to have a higher-
than-normal muscle tone and relaxes only with difficulty.
Acid reflux (choice A) can cause esophageal irritation, ulceration, and also
predisposes for Barrett's metaplasia with subsequent risk of adenocarcinoma
of the esophagus.
Cancerous destruction (choice B) would produce a mass, or area of stricture
or ulceration.
Candida infection (choice C) of the esophagus resembles thrush of the
mouth, and causes a usually superficial infection.
Fibrosis of the esophageal wall (choice D) can be the consequence of
ulceration (due to reflux or ingestion of harsh chemicals such as lye) or
systemic sclerosis.


Question 3 of 5
Which of the following regulators would most likely inhibit the lower esophageal
sphincter in normal individuals?
/ A. Acetylcholine and substance P
/ B. Substance P and nitric oxide
/ C. Substance P only
/ D. Vasoactive intestinal polypeptide and acetylcholine
/ E. Vasoactive intestinal polypeptide and nitric oxide

Explanation - Q: 3.3 Close

The correct answer is E. Physiologically important inhibitors of the lower
esophageal sphincter include nitric oxide and vasoactive intestinal
polypeptide. Physiologically important substances that stimulate the lower
esophageal sphincter include acetylcholine and substance P.


Question 4 of 5
Which of the following medications is used to directly relax the lower esophageal
sphincter?
/ A. Diphenoxylate
/ B. Famotidine
/ C. Granisetron
/ D. Isosorbide dinitrate
/ E. Metoclopramide

Explanation - Q: 3.4 Close

The correct answer is D. Commonly used medications to relax the lower
esophageal sphincter in patients with achalasia include nitrates such as
isosorbide dinitrate (remember that nitric oxide physiologically inhibits the
lower esophageal sphincter) and calcium channel blockers such as
nifedipine (which inhibit calcium flow into the smooth muscle of the lower
esophageal sphincter, thereby inhibiting contraction.) For patients in whom
medical therapy fails, other options include paralysis of the lower esophageal
sphincter with intrasphincteric injection of botulinum toxin, pneumatic
dilatation, and a Heller myotomy (which interrupts the muscles of the lower
esophageal sphincter).
Diphenoxylate (choice A) is a opiate antidiarrheal, and would not be useful
for achalasia.
Famotidine (choice B) is an H2 antagonist that would be useful in reducing
stomach acidity, but would not directly affect lower esophageal pressure.
Other drugs in this class include cimetidine, ranitidine, and nizatidine.
Granisetron (choice C) is a 5HT3 antagonist and is used to prevent nausea
and vomiting in patients who receive chemotherapy, and after general
anesthesia. Other members of this drug class include ondansetron and
dolasetron.
Metoclopramide (choice E) stimulates gastric motility in patients with
gastroparesis and is also a antiemetic agent.


Question 5 of 5
Worldwide, which of the following parasitic diseases is most likely to produce a
disorder that clinically resembles this patient's condition?
/ A. Ascariasis
/ B. African sleeping sickness
/ C. Chagas disease
/ D. Cysticercosis
/ E. Malaria

Explanation - Q: 3.5 Close

The correct answer is C. Chagas disease, which is found in South and
Central America and is due to infection with Trypanosoma cruzi, can involve
the heart, colon, and esophagus. The esophageal involvement clinically
closely resembles achalasia.
The adult worms of ascariasis (choice A), or roundworm infection, live
principally in the intestine, and can obstruct the intestine or a bile duct; the
larvae can migrate to the liver, heart, and lungs.
African sleeping sickness (choice B), caused by Trypanosoma brucei and
Trypanosoma gambiense, causes lymphadenopathy, rash, and CNS
involvement.
Cysticercosis (choice D), due to the larval form of the pork tapeworm Taenia
solium, can involve subcutaneous tissue, muscle, viscera (but not specifically
the esophagus), and, most seriously, the CNS.
Malaria (choice E), due to various Plasmodium species, involves the blood,
liver, kidney, spleen, and brain, but does not have a specific predilection for
the esophagus.


A 35-year-old woman consults a physician because she has been having trouble
swallowing. She also often experiences chronic heartburn.
The physician performs a screening physical examination, and notices that the
skin of her hands appears tight and shiny. On specific
questioning, she reports having often experienced color changes in her hands
from white to blue to red.
Question 1 of 5
Which of the following is the most likely cause of the patient's difficulties with
swallowing?
/ A. Achalasia
/ B. Adenocarcinoma
/ C. Chagas disease
/ D. Scleroderma
/ E. Squamous carcinoma

Explanation - Q: 4.1 Close

The correct answer is D. The tip-off is the reference to the patient's skin
changes that are typical for scleroderma, also known as systemic sclerosis.
Scleroderma is a disease that may be either predominately limited to the skin
or involve many body systems, including the musculoskeletal system,
gastrointestinal tract (with esophageal involvement most often symptomatic),
cardiorespiratory system, and renal system. Esophageal dysfunction is a
common complication of scleroderma. In most patients, the skin changes are
obvious, even if the patient has not been previously diagnosed. Rarely, the
skin changes may be noticed at an earlier stage, in which the skin of the
hands appears puffy and edematous, but not scarred.
Achalasia (choice A) and Chagas disease (choice C) are also important
causes of dysphagia, but in achalasia, the physical examination is usually
normal, and in Chagas disease, you should be able to elicit a history of
possible exposure in Central or South America.
Reflux esophagitis with risk of progression to Barrett esophagus and
adenocarcinoma (choice B) can occur in scleroderma, but it is unlikely, at
this early stage in the patient's disease, that she already has
adenocarcinoma.
The risk of squamous cell carcinoma (choice E) is not increased in this
patient.


Question 2 of 5
The color changes described on the patient's hand are most likely due to which
of the following?
/ A. Arteriolar spasm
/ B. BIood clots at sites of vascular injury
/ C. Large artery spasm
/ D. PIatelet clots
/ E. Stasis blood clots

Explanation - Q: 4.2 Close

The correct answer is A. The color changes described are typical for
Raynaud's phenomenon, which occurs because of changes in perfusion due
to arteriolar spasm. Raynaud's phenomenon is common in scleroderma,
largely because the subintimal hyperplasia of small vessels characteristic of
scleroderma can reduce the luminal diameter by more than 75%. Some
authors argue that the vascular changes seen in scleroderma are actually
the insult that triggers the subsequent development of fibrosis.
Blood clots at sites of vascular injury (choice B) are typical in clots that form
in arteries on surfaces such as a fractured atherosclerotic clot.
Larger artery spasm (choice C) of vessels damaged by atherosclerosis is
thought to contribute to some myocardial infarctions, but is not thought to be
an important pathologic mechanism in scleroderma.
Platelet clots (choice D) can be seen in some diseases in which platelet
function is abnormal, such as essential thrombocytopenia.
Stasis blood clots (choice E) commonly are found in venous thromboses.




Question 3 of 5
Additional findings on physical examination include noting that the skin changes
are limited to areas distal to the elbow and knee, the
presence of calcified nodules on the extensor surfaces of the forearms, and the
presence of telangiectasias on the forearms. This suggests
that this patient has which of the following?
/ A. Bauer syndrome
/ B. Charcot syndrome
/ C. CREST syndrome
/ D. Crigler-Najjar syndrome
/ E. Dandy-Walker syndrome

Explanation - Q: 4.3 Close

The correct answer is C. These findings, together with esophageal
dysfunction and Raynaud's phenomenon (both of which this patient has), are
called the CREST syndrome, also known as limited cutaneous scleroderma.
This form of scleroderma has a better long-term prognosis than when the
skin changes also involve the trunk (diffuse scleroderma) and more internal
organs are additionally involved.
Bauer syndrome (choice A) is aortitis and aortic endocarditis as a
complication of rheumatoid arthritis.
Charcot syndrome (choice B) is intermittent claudication.
Crigler-Najjar syndrome (choice D) is a severe familial liver disease.
Dandy-Walker syndrome (choice E) is a malformation of the central nervous
syndrome.




Question 4 of 5
If this woman's involved skin were biopsied, which of the following would most
likely be seen?
/ A. CIeft separating the dermis and subcutaneous tissues
/ B. Epithelial cell hyperplasia
/ C. Marked dermal fibrosis
/ D. Narrowing of the basal lamina of small capillaries
/ E. Thickening of rete pegs


Explanation - Q: 4.4 Close

The correct answer is C. In scleroderma, early changes (at the point at
which the hands appear swollen, rather than with tight, thick skin) show
edema with perivascular infiltrates of CD4+ T cells. At this stage, the
collagen fibers are swollen and beginning to degenerate. The smaller
vessels may show basal lamina thickening (not narrowing as in choice D)
and endothelial (not epithelial as in choice B) cell damage and proliferation.
With time, the characteristic marked dermal fibrosis develops, which tends to
both narrow (not thicken as in choice E) the rete pegs and attach the dermis
tightly (compare with cleft formation as in choice A) to subcutaneous tissues.


Question 5 of 5
More than 90% of the patients with the limited cutaneous form of this disorder
make which of the following autoantibodies?
/ A. Anti-centromere
/ B. Anti-DNA topoisomerase l
/ C. Anti-double-stranded DNA
/ D. Anti-Golgi
/ E. Anti-ScI-70

Explanation - Q: 4.5 Close

The correct answer is A. All forms of scleroderma are thought to have a
strong autoimmune component, and glucocorticoids and azathioprine are
used to suppress the inflammatory complications of scleroderma. (Other
drugs that can be used in therapy include penicillamine, which inhibits
collagen cross-linking, NSAIDS for pain, and ACE inhibitors to protect the
kidney if hypertension or renal damage occurs.) The anti-centromere
antibody is quite specific for CREST syndrome (96% of cases), and is only
seen in a minority of patients with diffuse scleroderma (mainly those with
Raynaud's phenomenon) and rarely in systemic lupus erythematosus and
mixed connective tissue disease.
Anti-DNA topoisomerase I (choice B), also called anti-Scl-70 (choice E)
occurs commonly (64-75%) in diffuse scleroderma, but only rarely in CREST
syndrome.
Anti-double-stranded DNA (choice C) is fairly specific for systemic lupus
erythematosus, although it only occurs in 50-60% of lupus cases.
Anti-Golgi antibodies (choice D) are seen most often in systemic lupus
erythematosus and Sjgren syndrome.

A 40-year-old woman with welI-controlled diabetes comes to the urgent care
clinic complaining of ear pain and low-grade fever for the past
five weeks. She reports that there has also been copious greenish drainage on
her pillow each morning. Her pain has been worsening, which
brought her to the clinic. On examination, she is exquisitely tender when her
external ear is manipulated. A Iimited examination of the ear canal
shows red, inflamed skin with copious greenish exudate. There is no tenderness
over the mastoid process.
Question 1 of 3
Which of the following is the most likely diagnosis?
/ A. Acute otitis media
/ B. Chronic otitis media
/ C. Dental infection
/ D. Mastoiditis
/ E. Otitis externa

Explanation - Q: 1.1 Close

The correct answer is E. Patients with diabetes, or patients who sustain
damage to the external ear canal (usually with swabs) are susceptible to
otitis externa. This patient has many of the findings: ear pain, pruritus, and
discharge. On examination, the ear canal is erythematous and edematous.
Tenderness to manipulation of the external ear is also indicative of otitis
externa, and is not seen in otitis media.
Acute otitis media (choice A) presents with fever and ear pain. The external
ear canal is unaffected in otitis media, and there is no pain with manipulation
of the external ear.
Chronic otitis media (choice B) is inflammation of the middle ear and/or the
mastoid. It may present with otorrhea, but inflammation of the ear canal and
tenderness to manipulation of the external ear canal are not seen. Thus,
otitis externa is a better diagnosis.
Dental infections (choice C) may occasionally present as ear pain, but this is
unlikely.
Mastoiditis (choice D) is characterized by swelling, erythema and tenderness
over the mastoid. Diagnosis is confirmed by cloudiness and loss of margins
of the mastoid on radiograph. None of these findings are present here.






Question 2 of 3
This patient is started on oral antibiotics, but her infection progresses. She
continues to have pain and discharge, but now she has a facial
droop on the ipsilateral face. Which of the following cranial nerves is most likely
affected to create this symptom?
/ A. Cranial nerve l
/ B. Cranial nerve VII
/ C. Cranial nerve VIII
/ D. Cranial nerve XI
/ E. Cranial nerve XII

Explanation - Q: 1.2 Close

The correct answer is B. Cranial nerve VII, the facial nerve, innervates the
muscles of facial expression. Lesions of this nerve produce a facial droop. In
diabetics (even if the blood sugar is under tight control), there is a propensity
to develop a severe form of otitis externa known as malignant otitis externa.
In this condition, the infection in the external ear canal erodes into adjacent
soft tissues, mastoid, and temporal bone. Eventually the infection spreads
across the base of the skull. Cranial nerves are occasionally affected as are
the meninges. Treatment is surgical debridement with appropriate
intravenous antibiotics. Pseudomonas aeruginosa is often implicated in such
cases.

Question 3 of 3
Computed tomography scans of the head show extensive infection involving the
mastoid, temporal bone, and skull base. PIans for surgical
debridement are made. Until cultures are taken at the time of operation, empiric
antibiotic therapy should be directed toward which of the
following organisms?


Explanation - Q: 1.3 Close

The correct answer is C. When a diabetic patient shows symptoms of
malignant otitis externa, the causative organism is Pseudomonas 95% of the
time. Empiric therapy directed at eradicating this organism should be started
immediately. Alterations to therapy can be made when cultures and
sensitivity are obtained at time of operation.
Enterococcus(choice A), E. coli(choice B), and Strep. pneumoniae(choice
E), would be atypical for malignant otitis externa.
Staph. epidermidis(choice D) occasionally causes this condition, but
uncommonly (<5%). Thus, empiric treatment for this organism is
inappropriate.


A three-year-old boy is brought to urgent care complaining of three days of fever,
fussiness, and ear pain. The patient's mother also reveals
that the child has had a two week history of rhinorrhea and cough that resolved
several days ago. On physical examination, the tympanic
membrane is erythematous, opaque, and the ossicles are not seen. No pain is
elicited by manipulating the tragus.
Question 1 of 3
Which of the following is the most likely diagnosis?
/ A. Acute otitis media
/ B. Acute rhinoviral infection
/ C. Otitis externa
/ D. Otitis media with effusion
/ E. Mastoiditis

Explanation - Q: 2.1 Close

The correct answer is A. Acute otitis media is characterized, in children, by
fever, fussiness, and ear pain. Often a viral syndrome is found prior to onset
of ear symptoms. On examination, the tympanic membrane is erythematous,
opaque, and lacks mobility with insufflation. This child has the classic
findings.
Acute rhinoviral infection (choice B) is incorrect because it is too specific.
While many ear infections are viral, the type of virus cannot be definitively
elucidated on clinical history alone.
Otitis externa (choice C) is an infection of the external ear. It presents in a
fashion similar to otitis media, but the ear is exquisitely tender when the
external ear is manipulated. The fact that the tragus can be manipulated
without discomfort eliminates this diagnosis.
Otitis media with effusion (OME; choice D) is fluid in the middle ear without
infection and thus does not present as fever and pain. Usually this condition
occurs as a consequence of acute otitis media and presents as "plugged
ears," hearing loss, vertigo, and clumsiness. On examination, fluid is seen
behind the tympanic membrane, and decreased tympanic membrane
mobility is demonstrated on pneumatic otoscopy and tympanogram. If this
condition persists, hearing loss may develop.
Mastoiditis (choice E) is a complication of acute otitis media. It is
characterized by swelling, erythema and tenderness over the mastoid
process. Diagnosis is confirmed by cloudiness and loss of margins of the
mastoid on radiograph


Explanation - Q: 2.2 Close

The correct answer is E. Streptococcus pneumoniae is an alpha hemolytic,
gram-positive coccus that is the most commonly isolated pathogen in the
setting of acute otitis media in the pediatric setting.
Haemophilus influenzae(choice A) is a gram-negative coccobacillus
commonly found in otitis media. Its grows on chocolate agar and is not alpha
hemolytic.
Moraxella catarrhalis(choice B) can cause acute otitis media, but is not the
most common cause, nor is it alpha hemolytic.
Pseudomonas aeruginosa(choice C) is a common pathogen in otitis externa.
It is a gram-negative rod that grows on MacConkey's agar.
Staphylococcus aureus(choice D) can rarely cause otitis media and tends to
be found in chronic suppurative otitis media. It is beta hemolytic.










Question 3 of 3
Which of the following would be the most appropriate pharmacotherapy?
/ A. Ceftriaxone
/ B. Gentamicin
/ C. Methicillin
/ D. Penicillin G
/ E. Trimethoprim-sulfamethoxazole

Explanation - Q: 2.3 Close

The correct answer is D. Penicillin G is still effective in treating most
streptococcal isolates, although resistance is an emerging problem.
Ceftriaxone (choice A) is a third-generation cephalosporin and would not be
the best agent for Streptococcus. As the cephalosporins progress from first
to second to third generation, they have increasing gram-negative activity
and decreasing gram-positive activity. Thus, penicillin is a better choice.
Gentamicin (choice B) and trimethoprim-sulfamethoxazole (choice E) are
effective against gram-negative organisms and thus would not be used in
this case.
Methicillin (choice C) is used to treat organisms that are beta-lactamase
positive such as Staphylococcus. Thus, penicillin is more appropriate to treat
this infection.


A 52-year-old man presents to the general medicine clinic complaining of
progressive hearing loss in his right ear. He says that over the past
year, he has had difficulty hearing conversations in noisy places such as
restaurants. In the past few months, he has experienced occasional
ringing in his right ear. His hearing in his left ear has not been affected. He
denies headache, vertigo, otalgia, facial pain, or weakness. He has
no medical problems and does not take any medications. Otoscopic examination
reveals pearly gray tympanic membranes bilaterally without
any bulge or retraction. A Weber's test is performed, and the patient reports
hearing the vibration loudest in his left ear.
Question 1 of 6
Which of the following is the most likely diagnosis?
/ A. Acoustic neuroma
/ B. Cholesteatoma
/ C. Meniere disease
/ D. Meningioma
/ E. Otosclerosis
Explanation - Q: 3.1 Close

The correct answer is A. Acoustic neuroma is the most common cause of
unilateral sensorineural hearing loss in adults. The result of the Weber's test
indicates a sensorineural deafness in his right ear (see explanation for
question 2 for more details). Actually, the term acoustic neuroma is
something of a misnomer, since it is not "acoustic" (originating from the
auditory nerve), nor is it a neuroma (it is a schwannoma). Some have
recommended that it be called a "vestibular schwannoma." They account for
about 80% of tumors in the cerebellopontine angle; the remaining 20% are
primarily meningiomas. Acoustic neuromas most often occur as isolated
lesions in patients 40 to 60 years of age, but may also be a manifestation of
neurofibromatosis type 2 (in which case they are typically bilateral). It is a
slowly growing, benign tumor that arises from the vestibular portion of cranial
nerve VIII. Because destruction of vestibular fibers is slow and the patients
have time to equilibrate, most patients present with hearing loss, rather than
vertigo. As small tumors, they can present with hearing loss and tinnitus, and
as they grow and continue to impinge on structures in the cerebellopontine
angle, they may cause additional cranial nerve dysfunction and cerebellar
dysfunction.
Cholesteatomas (choice B) are epidermoid inclusion cysts that occur in the
middle ear and petrous apex. They most typically start as an infection in the
middle ear that accumulates dead cells and keratin. They usually present
with intermittent drainage from the ear and conductive hearing loss.
Meniere disease (choice C) is characterized by repeated episodes of vertigo
lasting from minutes to days. This disease is also accompanied by tinnitus
and progressive sensorineural hearing loss. The etiology is thought to be an
increase in the volume of labyrinthine endolymph, but the exact
pathophysiology is not known.
Meningioma (choice D) is the second most common tumor of the
cerebellopontine angle.
Otosclerosis (choice E) is caused by bony changes in the tympanic cavity
that result in immobility of the stapes. Conductive hearing loss is its most
distinctive clinical feature. Tinnitus and vertigo can also occur. Auditory
symptoms usually begin between ages 15-35 and familial occurrence is
common. This disease is bilateral in the majority of cases. Additionally, a
patient with conductive hearing loss will hear the vibration produced in the
Weber's test louder in the diseased ear.




Question 2 of 6
Which of the following findings would most likely be observed in this patient?
/ A. Air conduction equals bone conduction in his left ear
/ B. Air conduction equals bone conduction in his right ear
/ C. Air conduction is greater than bone conduction in his right ear
/ D. Bone conduction is greater than air conduction in his left ear
/ E. Both air and bone conduction are enhanced in his right ear

Explanation - Q: 3.2 Close

The correct answer is C. This patient has sensorineural deafness in his
right ear due to his acoustic neuroma. In the normal ear, air conduction is
greater than bone conduction. An ear with sensorineural deafness will still
have air conduction greater than bone conduction, however both will be
qualitatively decreased.
This can be determined with the Rinne test, in which a vibrating tuning fork is
placed on the mastoid bone (bone conduction) behind an ear until it is no
longer heard by the patient. Then, the tuning fork is held at the external
auditory canal (air conduction). In sensorineural deafness, the Rinne test is
still normal, though both components may be qualitatively decreased. In
conduction deafness, bone conduction will be better than air conduction
(abnormal Rinne test).
Another type of auditory testing is the Weber test. In the Weber test, a
vibrating tuning fork is placed on the middle of the forehead and the patient
compares the loudness of the sound in both ears. This delivers vibrations via
bone conduction, thereby bypassing the ossicles. The Weber test compares
bone conduction in the two ears. If the sound is not heard in the middle, the
sound is said to be lateralized, and a hearing loss is present. In patients with
unilateral sensorineural hearing loss, such as in acoustic neuromas, the
sound is softer on the affected side, and is louder or "localized" to the
unaffected ear. This patient has sensorineural hearing loss on the right side
and during a Weber test, the sound will lateralize to the unaffected or left ear.
Conversely, a conduction-deaf ear will hear the sound as louder than in the
normal ear (you can do this experiment yourself by putting an ear plug in one
ear). Therefore, a patient with unilateral conduction deafness hears the
vibration louder in the diseased ear, whereas a patient with unilateral
sensorineural deafness hears the vibration louder in the normal ear.
His left ear is normal, therefore, air conduction should be greater than bone
conduction, ruling out choices A and D.
Air conduction equals bone conduction in his right ear (choice B) is incorrect
because air conduction is still greater than bone conduction with
sensorineural deafness.
Both air and bone conduction are enhanced in his right ear (choice E) is
incorrect because both should be diminished in his right ear.



Question 3 of 6
The patient's disease may be associated with which of the following disease
processes?
/ A. Hypothyroidism
/ B. Neurofibromatosis type 2
/ C. Paget disease
/ D. Von HippeI Lindau syndrome
/ E. Wallenberg syndrome

Explanation - Q: 3.3 Close

The correct answer is B. Neurofibromatosis type 2 is an autosomal
dominant disorder characterized by a propensity to develop bilateral eighth
nerve schwannomas or multiple meningiomas. The other diseases listed are
not particularly associated with acoustic neuromas.


Question 4 of 6
The patient is lost to follow-up, but returns a couple of years later complaining of
worsening hearing loss and tinnitus in his right ear and
increasing dysequilibrium. On examination, stimulation of his right cornea with a
wisp of cotton does not elicit blinking in either eye, whereas
stimulation of his left cornea produces blinking in both eyes. What is the most
likely location of this new lesion?
/ A. Left abducens nerve
/ B. Left facial nerve
/ C. Left trigeminal nerve
/ D. Right abducens nerve
/ E. Right facial nerve
/ F. Right trigeminal nerve

Explanation - Q: 3.4 Close

The correct answer is E. In the corneal reflex, a wisp of cotton is touched to
the cornea. Afferent information travels up the ipsilateral trigeminal nerve (V),
and efferent information travels down the facial nerve (VII) bilaterally to elicit
the blink. The fact that this patient had no blink reflex in either eye when his
right eye was stimulated, indicates a problem in the afferent arc of the reflex
(right V).
A left abducens nerve (VI) (choice A) lesion would result in an inability to
abduct the left eye. (The abducens nerve innervates the lateral rectus
muscle.)
If there was a left facial nerve (VII) (choice B) lesion, stimulation of his right
cornea would have produced a blink reflex in the right eye; stimulation of his
left cornea would have also produced a blink reflex only in his right eye.
If there was a lesion in the left trigeminal nerve (choice C), stimulation of the
left cornea would not elicit a reflex in either eye; stimulation of the right
cornea would elicit a blink reflex in both eyes.
A right abducens nerve (VI) (choice D) lesion would result in an inability to
abduct the left eye. (The abducens nerve innervates the lateral rectus
muscle.)
If there was a right facial nerve (VII) (choice E) lesion, stimulation of his right
cornea would have produced a blink reflex in the left eye; stimulation of his
left cornea would have also produced a blink reflex only in his left eye.


Question 5 of 6
Additional testing reveals that he does not taste sugar or salt well on the anterior
two-thirds of the right side of his tongue. Which of the
following nerves is most likely lesioned?
/ A. Right facial nerve
/ B. Right glossopharyngeal nerve
/ C. Right hypoglossal nerve
/ D. Right trigeminal nerve
/ E. Right vagus nerve

Explanation - Q: 3.5 Close

The correct answer is A. The right facial nerve (VII) innervates taste buds in
the right anterior two-thirds of the tongue.
The right glossopharyngeal nerve (IX) (choice B) innervates taste buds in
the right posterior one-third of the tongue.
The right hypoglossal nerve (XII) (choice C) innervates intrinsic and extrinsic
muscles of the right tongue, thereby mediating tongue movement.
The right trigeminal nerve (V) (choice D) conveys somatosensory
information from the anterior two-thirds of the right tongue.
The right vagus nerve (X) (choice E) innervates taste buds in the right
epiglottic region.

Question 6 of 6
Which of the following is the most appropriate therapy for this patient?
/ A. Endolymphatic shunting
/ B. Hydrochlorothiazide
/ C. Positional exercises
/ D. Scopolamine
/ E. Surgical resection

Explanation - Q: 3.6 Close

The correct answer is E. Surgical resection by a variety of approaches is
the only treatment for acoustic neuromas. 75% of acoustic neuromas will
grow at a gradual pace if left unresected. As these tumors enlarge, they may
cause symptoms by compressing other cranial nerves in the region and lead
to further disability. Because of this, surgical resection is usually
recommended. If hearing is absent or poor at the time of surgical resection,
even successful removal of the acoustic neuroma will not restore any hearing
already lost. Hearing preservation is best when the tumor is less than 2 cm in
size and does not involve the internal auditory canal fundus or cochlear
aperture.
Endolymphatic shunting (choice A) is a surgical technique used to treat
Meniere disease. It reduces the volume of labyrinthine endolymph, which is
thought to be the cause of vertigo in Meniere disease. It is not a treatment for
acoustic neuromas.
Hydrochlorothiazide (choice B) is a diuretic, which may help prevent attacks
in patients with Meniere disease, however, it does not have a role in acoustic
neuroma therapy.
Positional exercises (choice C) are used to treat symptoms associated with
benign positional vertigo and have no role in the treatment of acoustic
neuromas.
Scopolamine (choice D) is an anticholinergic medication used to treat
symptoms in vertigo in patients with Meniere disease. It has no role in the
treatment of acoustic neuromas.

A 59-year-old woman with a 10-year history of type 2 diabetes mellitus is noted
by her physician to have bilateral pitting edema of the ankles
and feet. No erythema is noted. On questioning, the patient also reports
shortness of breath on exertion and states that she has been using 3
pillows at night in order to sleep comfortably.
Question 1 of 6
This patient's symptoms are most suggestive of which of the following?
/ A. Cellulitis
/ B. Congestive heart failure
/ C. Gynecologic cancer
/ D. Lipedema
/ E. Thrombophlebitis

Explanation - Q: 1.1 Close

The correct answer is B. The combination of leg edema and pulmonary edema (as
indicated by shortness of breath and sleeping on multiple pillows) strongly suggests
that the patient has congestive heart failure involving both ventricles. Heart failure is
a pathologic state in which an abnormality of cardiac function leads to failure of the
heart to pump blood throughout the body at a rate sufficient to meet the body's
requirements. Some of the adaptive mechanisms that compensate for the failing
heart include increasing preload (through the Frank-Starling mechanism),
myocardial hypertrophy (to restore elevated ventricular wall stress to within normal
limits), redistribution of cardiac output from non-vital organs to vital organs, and
neurohumoral adjustments. Congestion develops behind the failing ventricle, with
left ventricular failure causing signs and symptoms of pulmonary congestion, and
right ventricular failure causing signs and symptoms of systemic congestion.
Cellulitis (choice A) is usually markedly erythematous.
Gynecologic cancer (choice C) and thrombophlebitis (choice E) are causes of
unilateral leg edema.
Lipedema (choice D) is not a true edema (and would not show pitting), but is
instead the deposition of fatty tissues around the ankles of obese individuals.


Question 2 of 6
A chest x-ray film demonstrates pulmonary venous congestion and interstitial
edema indicative of pulmonary edema. Which of the following
physiologic mechanisms is most likely the immediate cause of the pulmonary
edema?
/ A. Damage to endothelial cells
/ B. Damage to the epithelial lining of the alveoli
/ C. EIevated pulmonary capillary pressure
/ D. Low serum albumin
/ E. Poor lymphatic drainage of fluid

Explanation - Q: 1.2 Close

The correct answer is C. The cause of pulmonary edema in congestive
heart failure is an increase in the hydrostatic pressure at the level of the
capillaries of the lung. This increased pressure serves to drive fluid out of the
capillaries and into the alveoli. The other reasons cited in the choices can
also cause pulmonary edema, but occur in other clinical settings.
Damage to endothelial cells (choice A) can occur in vasculitis.
Damage to the epithelial lining of the alveoli (choice B) can occur in
pneumonia and respiratory distress syndrome.
Low serum albumin (choice D) can be seen in liver and kidney disease.
Poor lymphatic drainage (choice E) can be seen in lungs with chronic
damage due to severe emphysema or fibrosis.




Question 3 of 6
A chest x-ray film additionally demonstrates a markedly enlarged cardiac shadow
with dilation of both the right and left ventricles.
Echocardiography shows dilated, hypokinetic ventricles. The physician
prescribes several medications, including captopriI. Which of the
following best characterizes captopriI?
/ A. ACE inhibitor
/ B. AIpha blocker
/ C. Beta blocker
/ D. Diuretic
/ E. Positive inotrope

Explanation - Q: 1.3 Close

The correct answer is A. ACE inhibitors, including captopril, enalapril, and
lisinopril, are becoming mainstays in the treatment of congestive heart
failure, more formally known as dilated congestive cardiomyopathy. These
agents inhibit the conversion of angiotensin I to angiotensin II, thereby
interrupting the renin-angiotensin-aldosterone loop. The functional result is
vasodilation, with a reduction in both cardiac afterload and cardiac preload.
Furthermore, the ACE inhibitors, such as captopril and enalapril, are proven
to delay the progression of this condition by protecting the ventricles from
deleterious remodeling.
Alpha-blockers (choice B) are not commonly used in CHF, although
carvedilol has some alpha-blocking activity.
Carvedilol is a non-cardioselective alpha- and beta-blocker (choice C)
approved by the FDA for the treatment of both ischemic and non-ischemic
heart failure; its alpha blocking effects confer vasodilatory activity.
Catecholamine production is enhanced in the early development of CHF to
compensate for decreasing cardiac output. Beta-blockers are beneficial in
heart failure, since they diminish the chronic sympathetic nervous system
stimulation; they may also be beneficial through the following mechanisms:
improving cardiac output, increasing diastolic filling time, and decreasing
arrhythmias.
Loop diuretics (choice D), such as bumetanide and furosemide, are also
considered to be first-line agents in the treatment of heart failure. These
agents are indicated for the treatment of edema associated with CHF,
hepatic cirrhosis, and renal disease, as well as treatment of hypertension
(furosemide and torsemide).
Digoxin (choice E) causes a positive inotropic effect by inhibiting the Na
+
/K
+

ATPase in cardiac cell membranes. This inhibition leads to increased
intracellular sodium, which alters the driving force for the Na
+
/Ca
2+
exchange
mechanism, leading to a decrease of calcium removal from the cell. This
results in increased calcium storage in the sarcoplasmic reticulum, which
when released, increases contractile force. Digitalis also modifies autonomic
outflow.

Question 4 of 6
An increase in which of the following is the most likely explanation for the edema
in her legs?
/ A. Interstitial colloid osmotic pressure
/ B. Lymph flow
/ C. PIasma colloid osmotic pressure
/ D. Right atrial pressure
/ E. Stroke volume
Explanation - Q: 1.4 Close

The correct answer is D. Right atrial pressure rises in congestive heart
failure, which elevates venous pressure throughout the body. This increase
in venous pressure can cause excessive fluid loss from the microcirculation
and the development of peripheral edema.
Fluid loss from the capillaries washes protein molecules from the interstitial
compartment and thereby decreases interstitial colloid osmotic pressure
(choice A).
Increased lymph flow (choice B) is a consequence rather than a cause of
the edema.
Increased plasma colloid osmotic pressure (choice C) would tend to
decrease the development of edema.
Stroke volume (choice E) has no effect on the formation of peripheral
edema.


Question 5 of 6

As this patient's condition progresses, she is started on digoxin. Her most recent
digoxin blood level was 2.0 ng/mL. She will be at the highest
risk for developing digoxin toxicity if she has which of the following conditions?
/ A. Hypokalemia
/ B. Hyponatremia
/ C. Hypophosphatemia
/ D. Vitamin B12 deficiency
/ E. Vitamin K deficiency

Explanation - Q: 1.5 Close

The correct answer is A. Digoxin is a cardiac glycoside indicated for the
treatment of congestive heart failure, atrial fibrillation, and atrial flutter. The
therapeutic drug serum levels for digoxin are 0.5 to 2.2 ng/mL. Toxicity
typically occurs when digoxin levels are > 2.5 ng/mL, unless the patient is
hypokalemic, then toxicity can occur at any therapeutic concentration.
Hypokalemia sensitizes the myocardium to digoxin and may reduce the
positive inotropic effects of the medication. Other signs and symptoms of
digoxin toxicity include nausea, vomiting, anorexia, and appearance of
yellow-green halos in the visual field, as well as the development of cardiac
arrhythmias.
Although hyponatremia (choice B) and hypophosphatemia (choice C) can
result in the development of other pathological disturbances, they do not
potentiate digoxin toxicity.
Vitamin B
12
deficiency (choice D) is associated with the development of
pernicious anemia.
Vitamin K deficiency (choice E) is associated with the development of
clotting disorders. Furthermore, vitamin K deficiency is known to potentiate
warfarin toxicity.

Question 6 of 6
This patient's condition is associated with which of the following 5-year mortality
rates?
/ A. 5%
/ B. 30%
/ C. 50%
/ D. 70%
/ E. 95%

Explanation - Q: 1.6 Close

The correct answer is D. In temperate zones, dilated congestive
cardiomyopathy is most commonly the result of diffuse coronary artery
disease with diffuse ischemic myopathy, which produces chronic myocardial
fibrosis with diffuse loss of myocytes. Other causes are very diverse and can
include a wide variety of infections, granulomatous disease, metabolic
disorders, drugs and toxins, cancers, connective tissue disorders, familial
neuromuscular disorders, and pregnancy. The dilated chambers usually
function poorly, lea