Item: 1 of 2 ~ 1 • M khhttttp -<:J 1>- Jil hhttttp

~· !:';-~ hhttttp
QIO: 2617 ..L ar Pre v ious Next Lab~lues Notes Calcula t o r

•1
e rs
r s e s
rrs e s
rrs •
lA A] •
e
A young couple brings their 8-month-old boy to the pediatrician because of an upper respiratory infection. This is the tenth such infection the boy has
.2
o k
ook e o o k ke o o k
o k e o o kk
had In the past 4 months. Medical history is also significant for a cleft palate repair. The pediatrician is concerned, and subsequent genetic testing
o o
/ e
/ b
e b o / e
/ b
e o / e
/ b
e o
reveals a 22qll chromosomal deletion. Intravenous immunoglobulin and antibiotic prophylaxis are begun to compensate for the abnormal
b b / e
/ b
e b o
t .m mee development of a specific organ.

t m
.t.mee t m
.t.mee t m
.t.mee
/
/://t . s: /
: /
/ / s : /
: /
/ / s : /
: /
/ /
t tpp s t t
From which of the following embryonic structures is this organ derived?
hhtt hhtt pp s t t
hhtt pp s
:
A. First branchial (pharyngeal) arch

k eerrss B. Second branchial (pharyngeal) arch

k eerrss keerrss k e
b ooook b o o
o o k b oooo k b o o
o o k
/
ee e
/ eb c.
/
Third branchial (pharyngeal) pouch
ee e
/ e b ee/ e
/e b ee/ e
/ e b
t .
/:///t.mm o.
: / / t
/ .
t m
.m : // t/.
tm. m : / /t/.
t m
. m
tp ss:
p /
Third branchial (pharyngeal) cleft

t ppss: / tp pss: /
t
hht t
E. Fourth branchial (pharyngeal) cleft t
hht t t
hht t
F. Fourth branchial (pharyngeal) pouch

k eers
r s k e r
e s
r s k e r
e s
r s k e
bboooo k b o o
o o k b o o
o o k b o o
o o k
e/
e e
/ e ee/ e
/ e b ee/ e
/ e b ee/ e
/ e b
t
/:///t. m
. m : // t/.tm.m : / / t
/ .
t m
. m : / / t
/ .
t m
. m
t p ss
p : / t p ss
p : / t p ss
p : /
t
hht t t
hht t t
hht t

keerrss keerrss k eerrss k e

b oook
o b oook
o b o ook
o b oooo k
ee/ e
/ eb ee/ e
/ eb a ee/ e
/ e b s
ee/ ee
/ 8b
/ t
/t.m.m / .
/tt.mm Lock t
// t.m. m .
//tt.mmSuspend End Bl ock
 Item: 1 of 2 ~ 1 • M khhttttp -<:J 1>- Jil hhttttp
~· !:';-~ hhttttp
QIO: 2617 ..L ar Previous Next Lab~lues Notes Calculat or

•
1

keers
r s k ee s
rrs k e e s
rrs k e
.2
b o ook
o b o o
o o k b o o
o o k b o o
o o k
ee/ e
/ e b ee/ e
/ e b ee/ e
/ e b e/
e e
/ e b
/t
/://t.m. m : / / / m
.t.m
The correct a n swer is c. 6 0 0/o chose this.
t : / / t
/ m
.t.m : / / t
/ m
.t.m
tpps : / t ppss : / t ps
p s : /
This child has DiGeorge syndrome, which can be characterized by a variety of features including Cleft palate, Abnormal facies, T hymic aplasia, cardiac
s
t
hhtt t
hhtt t
hhtt
defects, and Hypocalcemia (remember the mnemonic " CATCH-22" ). Because of hypoplasra of the thymus, children wit h DiGeorge syndrome may have a
T-lymphocyte defidency. To prevent infection, t hese children are often given Intravenous immunoglobulins and antibiotic prophylaxis. The thymus normally
derives from the third branchial (pharyngeal) pouch.
biotics DiGeorge syndrome T cell Antibody Intravenous therapy Immunoglobulon therapy Pharynx Palate Hypoplasia Preventive healthcare

k errss
A is not correct. 2 0/o chose this.
e k eerrss keerrss k e
b ooook b o o
o o k b oooo k b o o o k
The first branchial arch plays no role in DiGeorge syndrome. For first-arch derivatives, think " M ": Mandible, Malleus, sphenoMandibular ligament; M uscles
o
ee/ e
/ eb ee/ e
/ e b ee/ e
/e b
Branchial arch Lateral pterygoid muscle Cranial nerves Pharyngeal arch DiGeorge synd orne Pterygoid bone Ugament ee/ e
/ e b
of Mastication (teM poralis, Masseter, Medial and lateral pterygoids). Derivatives of the first branchial arch are inne rvated by cranial nerve v.

/
/://t .
tm.m : / / t
/ .
t m
.m : // t/.
tm. m : / /t/.
t m
. m
t ss:
p /
B is not correct. 40/o ch ose t his.
p t ppss: / tp pss: /
t
hht t t
hht t t
hht t
The second branchial arch is not involved in DiGeorge syndrome. For second-arch derivatives, think " S" : Stapes, Styloid process, Stylohyoid ligament;
muscles of facial expression, Stapedius, Stylohyoid. Cranial nerve VII Innervates derivatives of the second arch.
Branchial arch DiGeorge syndrome Cranial nerves Facial muscles Pharyngeal arch Facial nerve

k ee s
0 is not co rrect . 100/o chose this.
rr s k e r
e s
r s k e r
e s
r s k e
The second, third, and fourth branchial clefts form temporary sinuses, but are obliterated before fetal m aturation . Thus they have no derivatives In the

bboooo k
adult.
b o o
o o k b o o
o o k b o o
o o k
e/
e e
/ e Sinus (anatomy) Paranasal sinuses
ee/ e
/ e b ee/ e
/ e b ee/ e
/ e b
t
/:///t. m
. m // t/.t
E i s no t co rrect . 70fo ch ose this.
: m.m : / / t
/ .
t m
. m : / / t
/ .
t m
. m
t p ss
p : / t p ss
p : / t p ss
p : /
The second, third, and fourth branchial clefts form temporary sinuses but are obliterated before fetal m aturation. Thus they have no derivatives In the
adult. t
hht t
Sinus (anatomy) Paranasal sinuses
t
hht t t
hht t
F i s n ot correct. 170/o ch ose this.

keerrss keerrss k e rrss

The fourth pharyngeal pouch gives rise to superior parathyroid glands and ultlmobranchial body, which forms the parafollicular
e
c cells (adjacent to the
k e
b oook
o
thyroid follicles).
b oook
o b o ook
o b oooo k
ee/ e
/ eb ee/ e
/ eb a ee/ e
/ e b s
ee/ ee
/ 8b
t
// t.m.m .
//tt.mm Lock t
// t.m. m .
//tt.mm
Suspend End Block
 Item: 1 of 2 ~. I • M hhttttp <:]
k t> al hhttttp
~· ~ hhttttp
QIO: 2617 .l. ar Previous Next lab 'Vfl1 ues Notes Calculator
- + - · - - + -

k e e rs s k eers
rs k e r
e s
r s
The second, third, and fourth branchial clefts form t emporary sinuses but are obliterat ed before fet al maturation . Thus they have no derivatives in the
r k e
.2
b o o
o o kadult.
Sinus (anatomy) Paranasal sinuses
b o o
o o k b o o
o o k b o o
o ok
ee/ e
/ e b chose this. e /
e e
/ e b ee/ e
/ e b e/
e e
/ e b
m
/://t/.t.m
is not correct.
F

: / / t
/ m17%
.t.m : / / t
/ m
.t.m : / / t
/ m
.t.m
tpp s : / t ppss : / t ps
p s : /
The fourth pharyngeal pouch gives rise to superior parathyroid glands and ultimobranchial body, which forms the parafollicular C cells (adjacent to the
s
t
hhtt
thyroid follicles).
t
hhtt t
hhtt
Parafollicular cell Ultimopharyngeal body Pharyngeal pouch (embryology) Thyroid Parathyroid gland Pharynx

k eerrss Bottom Line:

k eerrss keerrss k e
b ooook b o o
o o k b oooo k b o o o k
In DiGeorge syndrome the third and fourth branchial pouches do not develop. Absence of the third branchial pouch lea ds to T-lymphocyte deficiency,
o
ee/ e
/ eb ee/ e
/ e b ee/ e
/e b ee/
wherea s absence of the fourth branchial pouch causes hypocalcemia secondary to lack of parathyroid development.
e
/ e b
/
/://t .
tm.m : / / t
/ t m
.m : // t/tm
Pharyngeal pouch (embryology) Hypocalcaemia DiGeorge syndrome Parathyroid gland T cell
. . . m : / /t/.
t m
. m
tp ss:
p / t p pss: / tp pss: /
hht t t hht t t hht t t
lijl;fiiJI•l toryear:[ 2017 • ]
FI RST AI D FAC TS

k eerrss k e ers
r s k eerrss k e
b o o
o k
o Branchial pouch derivatives ebbooo o k b oooo k p
b o o
FA17
o o k 588.1

e/
e e
/ e b e e/ / e ee/ e
/ e b ee/ e
/ e b
/://t/.tm
.m / t . m.m / t . m
. m / t . m
. m
POUCH DERIVATIVES NOTES MNEMONIC

ss: /
: / / t ss: /
: / / t ss: /
: / / t
1st pouch
t
hht t p
t p Iiddie ear cavity, eustachian
tube, mastoid air cells. t t
1st
p
t
pouch
p contributes to Ear,
hhtendoderm-lined structures of I (ear), hht
tonsils,
t p
bottom-to-top:
t t p
ear. 2 (tonsils),
3 dorsal (bottom for inferior
keerrss 2nd pouch
keerrs
Epithelial lining of palatine
s k eerrss
parathyroids},
k e
b oook
o
tonsil.
b oook
o b o ook
o ... 1 / o
.' \

b oooo k
ee/ e
/ eb ee/ e
/ eb 6 ee/ e
/ e b s
ee/ ee
/ 0b
t
// t.m.m .
//tt.mm lock t
// t.m. m .
//tt.mm Suspend End Block
 Item: 1 of 2 ~. I • M khhttttp <:] t> al hhttttp
~· ~ hhttttp
QIO: 2617 .l. ar Previous Next lab 'Vfl1 ues Notes Calculator
--- -· - . .
1

keers
r s parathyroids.
k eers
r s k e r
e s
r s k e
.2
b o ook
o b o o o
Ventra l wings
o k b o o
o o k b o o
o ok
ee/ e
/ e b e / e
/ e b
- ultimobranchial body
e ee/ e
/ e b e/
e e
/ e b
/t
/://t.m. m : / / t
/ m
.t.m - parafollicular (C) cells of
: / / t
/ m
.t.m : / / t
/ m
.t.m
tp s
p s : / thyroid.
t ppss : / t ps
p s : /
t tt
hhsyndrome
DiGeorge
t t
hhtt development of 3rd and 4th pouches - hT-cell
Chromosome 22qll deletion. Aberrant htt deficiency
(thymic aplasia) and hypocalcemia (failure of parathyroid development). Associated with cardiac

k eerrss k errss
defects (conotrunca 1anomalies).
e keerrss k e
b ooook b o o
o o k b oooo k b o o
o o k
ee/ e
/ eb ee/ e
/ e b ee/ e
/e b ee/ e
/ e b FA17p112.1

/://t/.tm
.m Immunodeficiencies /t.m
p :
ss: / / /t .m
p :
ss:///t/.
tm. m
p :
ss:/ /
/t/.
t m
. m
t t
DISEASE
hht
B-cell disorders
t p DEFECT
tt
hht tp PRESENTATION
t
hhtt tp FINDINGS

X-linked (Bruton) Defect in BTK, a tyrosine Recurrent bacterial and Absent B cells in peripheral

k e r
e s
r s k e ers
r
agammaglobulinemia kinase gene - no B-cell s enteroviral infect ions after 6
k e e rrss blood, l lg of all classes.
k e
b o o
o o k b o o o k
maturation. X-linked recessi,·e
o months U maternal lgG).
b o o
o o k b o
Absent/scanty lymph nodes o
o o k
e/
e e
/ e b e / e
/ e b
(t in Boys).
e ee/ e
/ e b ee/ e
/ e b
and tonsils. Live vacci nes

/://t/.tm
.m
p : /
://t/.tm.m
ss Unknown. Most common 1° tMajority p : /
: /
/ t
/ .
t m
. m
ss Asymptomatic. p :
slgG,/
:
s lgM
/
/ t
/ .
t m
. m
contraindicated.

hh
deficiency
t
Selective lgAt
t t p immunodeficiency. h t
t t p
hCan see Airway and Gl hht
l lgA with normalt
t t p
levels. t susceptibility to
infections, Autoimmune giardiasis.

keerrss keerrss disease, Atopy, Anaphylaxis to

k eerrss k e
b oook
o b oook
o
lgA-containing products.
b o ook
o b oooo k
ee/ e
/ eb ee/ e
/ eb 6 ee/ e
/ e b s
ee/ ee
/ 0b
/ t
/t.m.m / .
/tt.mm lock t
// t.m. m .
//tt.mm Suspend End Block
 Item: 1 of 2 ~ 1 • M khhttttp -<:J 1>- Jil hhttttp
~· !:';-~ hhttttp
QIO: 2617 ..L ar Pre v ious Next Lab~lues Notes Calcula t o r

•
1

keers
r s k ee s
phagosome-lysosome fusion;
rrs albin ism, peripheral
k eerrss
Ivlild coagulation defects.
k e
.2
b o ook
o b ooo k
autosomal recessive.
o neuropalhy, progressi\·e
b o o oo k b o o
o o k
ee/ e
/ e b e e/ e
/ e b ee e
/ e b
neurodegeneralion, infiltrative
/ e/
e e
/ e b
/t
/://t.m. m : / / t
/ m
.t.m : / / t
/ m
Iymphoh istiocytosis.
.t.m : / / t
/ m
.t.m
tp s
D
p s : / t p pss : / t ps
p s : /
t
Chronic hht
t Defect of 'ADPII oxidase
t t
hhtt tsusceptibility to catalase <±> Abnormal dihydrorhodamine
hhtt
granulomatous - ' reactive oxygen organ1sms. (Aow cytometry) test (' green

k eerrss disease
k errss
species (eg, superoxide)
e keerrss fluorescence).
k e
b ooook b o o
o k
and ' respiratory burst
o b oooo k b o o
o o k
' itroblue tetrazolium dye

ee/ e
/ eb ee e
/ e b
in neutrophils; X-I inked
/ ee/ e
/e b ee/ e
/ e b
reduction test (obsolete) fails

/
/://t .
tm.m : / / t
/ .
t m
.
recessi\·e most common.
m : // t/.
tm. m : / /t/.
t m
to turn blue.
. m
tp ss:
p / t ppss: / tp pss: /
t
hht t t
hht t t t
hht FA17p61 .1
22q11 deletion licrodeletion at chromosome 22qll - v<Hiable CATC II-22.

k e ers s
syndromes
r r s
r s
presentations including C left palate, Abnormal
k e e k e r
e s
r s Due to aberrant development of 3rd and 4th
k e
b o o
o o k b o o
o o k o
facies, T hymic aplasia - T-cell deficiency,
b o o o k branchial pouches.
b o o
o o k
e/
e e
/ e b ee/ e
/ e b zo /
Cardiac defects, and Hypocalcemia to
ee e
/ e b ee/ e
/ e b
/://t/.tm
.m
p ss: /
://t/.tm.m parathyroid aplasia.
p ss: /
: /
/ t
/ .
t m
. m
DiGeorge syndrome-thymic, parathyroid, and
p ss: /
: /
/ t
/ .
t m
. m
hhtt t t p t
hhtt
cardiac defects.t p t
hhtt t p
Velocardiofacial syndrome-palate, facial, and
cardiac defects.
keerrss keerrss k eerrss k e
b oook
o b oook
o b o ook
o b oooo k
ee/ e
/ eb ee/ e
/ eb a ee/ e
/ e b s
ee/ ee
/ 8b
/ t
/t.m.m / .
/tt.mm Lock t
// t.m. m .
//tt.mm
Suspend End Bl ock
 Item: 2 of 2 ~ 1 • Ma r k hhttttp -<:J I> fJ hhttttp
£!1}>'
• !!":-~ hhttttp
QIO: 4598 ..L Prev ious Next Lab lues Not es Cal culat o r

•
1

ke rs
r s k e s
rrs
Several organs are responsible for hematopoiesis during development.
e e k e e s
rrs k e
.2
b o ook
o b o o
o o k b o o
o o k b o o
o o k
ee/ e
/ e b e / e
/ e b
Which of the following is the last to begin hematopoiesis?
e ee/ e
/ e b e/
e e
/ e b
/t
/://t.m. m : / / t
/ m
.t.m : / / t
/ m
.t.m : / / t
/ m
.t.m
tppss : / t ppss : / t ps
p s : / :
A. Bone marrow
t
hhtt t
hhtt t
hhtt
B. Liver

k eerrss c. Lungs

k eerrss keerrss k e
b ooook o. Spleen
b o o
o o k b oooo k b o o
o o k
ee/ e
/ eb ee/ e
/ e b ee/ e
/e b ee/ e
/ e b
/
/://t .
tm.m E. Yolk sac
: / / t
/ .
t m
.m : // t/.
tm. m : / /t/.
t m
. m
tp ss:
p / t ppss: / tp pss: /
t
hht t t
hht t t
hht t

k eers
r s k e r
e s
r s k e r
e s
r s k e
bboooo k b o o
o o k b o o
o o k b o o
o o k
e/
e e
/ e ee/ e
/ e b ee/ e
/ e b ee/ e
/ e b
t
/:///t. m
. m : // t/.tm.m : / / t
/ .
t m
. m : / / t
/ .
t m
. m
t p ss
p : / t p ss
p : / t p ss
p : /
t
hht t t
hht t t
hht t

keerrss keerrss k eerrss k e

b oook
o b oook
o b o ook
o b oooo k
ee/ e
/ eb ee/ e
/ eb a ee/ e
/ e b s
ee/ ee
/ 8b
/ t
/t.m.m / .
/tt.mm Lock t
// t.m. m .
//tt.mm
Suspend End Bl ock
 Item: 2 of 2 ~. I • M k hhttttp <:] t> al hhttttp
~· ~ hhttttp
QIO: 4598 .l. ar Previous Next lab 'Vfl1ues Notes Calculator

keers
r s k eers
rs k e r
e s
r s k e
2
b o ook
o b o o
o o k b o o
o o k b o o
o ok
ee/ e
/ e b ee/ e
/ e b ee/ e
/ e b e/
e e
/ e b
/t
/://t.m. m : / t m
.t.m
The co rrect a nswer is A. 71% cho se this.
/ / : / / t
/ m
.t.m : / / t
/ m
.t.m
tppss : / t ppss : / t ps
p s : /
Bone marrow is the last to undergo hematopoiesis and maintains hematopoiesis in adult life. It begins to synthesize blood at 18 weeks' gestation .
t
hhtt
Haematopoiesis Bone marrow Gestation Bone
t
hhtt t
hhtt
B is no t co rrect. 10% cho se this.
The liver is the second to begin hematopoiesis. It begins in gestational week 6 and ends at birth.

k e r
e ss
Haematopoiesis liver
r k eerrss keerrss k e
oock o o k oo k o o k
/ e bboo is no t co rrect. 6 % cho se this.

/ e b o
b o / e bboo / e b o
b o
m ee / e m ee /
The lungs do not participat e in hematopoiesis.
e m ee /e m ee / e
/
/://t .
t .m Haematopoiesis lung

: / /
/ t
/ .
t .m : ///t/.
t . m : / /
/t/.
t . m
tp ss:
D is no t co rrect. 10% cho se this.
t t p tt p
tpss: t tptpss:
hht hht
The spleen undergoes hematopoiesis in gestational weeks 10 through 28 .
Haematopoiesis Spleen
hht
E is no t co rrect. 3 % cho se this.

k e rs
r s k e r
e s
r s k e r s
r s
The yolk sac is the first to begin hematopoiesis. It begins in gestational week 3 and continues through week 8 .
e e k e
bboooo k Yolk sac Haematopoiesis Yolk

b o o
o o k b o o
o o k b o o
o o k
e/
e e
/ e ee/ e
/ e b ee/ e
/ e b ee/ e
/ e b
t
/:///t. m
. m : // t/.tm.m : / / t
/ .
t m
. m : / / t
/ .
t m
. m
Botto m Li ne:

t p ss
p : / t p ss
p : / t p ss
p : /
t
hht t t
hht t t
hht t
Fet al erythropoiesis occurs in the yolk sac (3 - 8 weeks), liver (6 - birth), spleen ( 10 -28 weeks), and bone marrow ( 18 weeks onward) .
Erythropoiesis Yolk sac Spleen liver Bone marrow Yolk Bone Fetus

k e errss keerrss k eerrss k e

b oo
ooI k
iii I •ti 1!1 It) fo r VP.il r :l /017 ..
b o
J
ook
o b o ook
o b oooo k
ee/ e
/ eb ee/ e
/ eb 6 ee/ e
/ e b s
ee/ ee
/ 0b
/ .m
/tt.m / .
/tt.mm lock t
// t.m. m .
//tt.mm
Suspend End Block
 Item: 2 of 2 ~. I • M k hhttttp <:] t> al hhttttp
~· ~ hhttttp
QIO: 4598 .l. ar Previous Next lab 'Vfl1ues Notes Calculator

keers s k eers
rs k e r
e s
r s
The yolk sac is the first to begin hem atopoiesis. It begins in gestational week 3 and continues through week 8.
r k e
2
b o ook
o
Yolk sac Haematopoiesis Yolk

b o o
o o k b o o
o o k b o o
o ok
ee/ e
/ e b ee/ e
/ e b ee/ e
/ e b e/
e e
/ e b
/t
/://t.m. m Botto m Line:
: / / t
/ m
.t.m : / / t
/ m
.t.m : / / t
/ m
.t.m
tppss : / t ppss : / t ps
p s : /
t
hhtt t
hhtt t
hhtt
Fetal erythropoiesis occurs in the yolk sac (3 - 8 weeks), liver (6 - birth), spleen ( 10 -28 weeks), and bone m arrow ( 18 weeks onward ).
Erythropoiesis Yolk sac Spleen liver Bone marrow Yolk Bone Fetus

kI e
iii e
I rrss1!1 I•J
;fi k eerrss k eerrss k e
b oooo k fo r year:l 2 0 17 ..
b o ooo k b ooo o k b o o
o o k
ee/ e
/ eb FI RST AI D FA CTS

ee/ e
/ e b ee/ e
/e b ee/ e
/ e b
/://t/.tm
.m
p : / /
/ t
/ .
t m
.m
ss: Fetal erythropoiesis occurs in: ttp :
ss:///t/.
tm. m
p :
ss:/ /
/t/.
t m
. m FA17 p 389.2

hhtt t t
Fetal erythropoiesisp hh t tp Young Liver Synthesizes h ht
Blood. t
t tp
• Yolk sac (3-8 weeks)
• Liver (6 weeks- birth)

k e r
e s
r s k e rs
r s
• Spleen (10-28 weeks)
e k e e rrss k e
b o o
o o k b o o o k
• Bone marrow (18 weeks to adult)
o b o o oo k b o o
o o k
e/
e e
/ e b Hemoglobin e e/ e
/
Embryonice bglobins: sand E. ee/ e
/ e b ee/ e
/ e b
/://t/.tm
.m development
p : /
://t/.tm.mFetal hemoglobin (HbF) = CJ.z'Yz·
ss Adult hemoglobin (HbA =nzP ttp ss: /
: /
/ t
/ .
t m
. mFrom feta l to adult hemoglobin:
p : /
: /
/
ss Beta.t
/ .
t m
. m
t
hht t t p hht t p
1) 2• Alpha Always; Gamma G oes,ttBecomes
hht t p
HbF has higher affinity for 0 2 due to less avid
binding of 2,3-BPG, allowing HbF' to extract

keerrss keer ss
0 2 from maternal hemoglobin (HbA 1 and
r k eerrss k e
b oook
o b ook
HbA 2) across the placenta. HbA2 (nzo2) is a
oo b o ook
o b oooo k
ee/ e
/ eb ee/ e
/ eb 6 ee/ e
/ e b s
ee/ ee
/ 0b
/ t
/t.m.m / .
/tt.mm lock t
// t.m. m .
//tt.mm
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