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Wilms' tumor
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Tumor is limited to the kidney and is completely excised. The surface of the renal capsule is intact. The tumor is not ruptured or biopsied (open or needle) prior to removal. No involvement of renal sinus vessels. No residual tumor apparent beyond the margins of excision.
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Tumor extends beyond the kidney but is completely excised. No residual tumor apparent at or beyond the margins of excision. Any of the following conditions may also exist:
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Tumor involvement of the blood vessels of the renal sinus and/or outside the renal parenchyma. The tumor has been biopsied prior to removal or there is local spillage of tumor during surgery, confined to the flank.
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Unresectable primary tumor. Lymph node metastasis. Positive surgical margins. Tumor spillage involving peritoneal surfaces either before or during surgery, or transected tumor thrombus.
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MRV
genital ulcers (including anal ulcers and spots in the genital region and swollen testicles or epididymitis in men) skin lesions (papulo-pustules, folliculitis, erythema nodosum, acne in post-adolescents not on corticosteroids) eye inflammation (iritis, uveitis, retinal vasculitis, cells in the vitreous) pathergy reaction (papule >2 mm dia. 24-48 hrs or more after needle-prick). The pathery test has a specificity of 95% to 100%, but the results are often negative in American and European patients[2]
y y
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mouth ulcers arthritis/arthralgia nervous system symptoms stomach and/or bowel inflammation deep vein thrombosis superficial thrombophlebitis cardio-vascular problems of an inflammatory origin inflammatory problems in chest and lungs problems with hearing and/or balance extreme exhaustion changes of personality, psychoses any other members of the family with a diagnosis of Behet disease.
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Cephalohematoma: between periosteum and bone surface, dont cross suture line, more distinct Caput succedaneum: fluid collection in presenting part, cross suture line, boggy and indistint
11 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. (Phenylketonuria PKU) (Congenital Hypothyroidism CHT) (Homocystinuria) (Galactosemia GAL) (Glucose-6-Phosphate Dehydrogenase Deficiency G6PD Deficiency) (Congenital Adrenal Hyperplasia CAH) (Maple Syrup Urine Disease MSUD) A (Medium Chain Acyl-CoA Dehydrogenase Deficiency MCAD) (Glutaric Acidemia Type I GAI) (Isovaleric Acidemia IVA) (Methylmalonic Acidemia MMA)
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Clinical Features
Dermatomyositis
Polymyositis
Age at onset
Children/adults
Sex
F=M
F>M
M>F
Ethnic group
All
Familial association
No
No
Other disorders
Clinical Features
Dermatomyositis
Polymyositis
Cutaneous* and muscle weakness: symmetrical prox. legs > arms neck
Muscle weakness: symmetrical prox. legs > arms asymmetric prox./distal leg and arm muscle, wrist/fingers flexors deltoids
EMG
Myopathic
Myopathic
Myopathic or neurogenic
Muscle enzymes
High or normal
High
Normal or high
Muscle biopsy
Endomysial inflammatory cell infiltrates surrounding and invading non-necrotic muscle fibers, vacuolated m. fibers
Response to immunosuppression
Yes
Yes
No
The clinical features of Horner's syndrome can be remembered using the mnemonic, "Horny PAMELa" for Ptosis, Anhidrosis, Miosis, Enophthalmos and Loss of ciliospinal reflex.
ysfunction
Effects
vestibular nuclei
palatal myoclonus
contralateral deficits in pain and temperature sensation from body (limbs and torso)
nucleus ambiguus - (which affects vagus nerve and glossopharyngeal nerve - localizing lesion (all other deficits are present in lateral pontine syndrome as well)
ipsilateral laryngeal, pharyngeal, and palatal hemiparalysis: dysphagia,hoarseness, diminished gag reflex (efferent limb - CN.X)
M1: The sphenoidal segment, so named due to its origin and loose lateral tracking of the sphenoid bone. Although known also as the horizontal segment, this may be misleading since the segment may descend, remain flat, or extend posteriorly the anterior (dorsad) in different individuals. The M1 segment perforates the brain with numerous anterolateral central (lateral lenticulostriate) arteries, which irrigate the basal ganglia . M2: Extending anteriorly on the insula, this segment in known as the insular segment. It is also known as the Sylvian segment when the opercu lar segments are included. The MCA branches may bifurcate or sometimes trifurcate into trunks in this segment which then extend into branches that terminate towards the cortex. M3: The opercular segments and extends laterally exteriorly from the insula to wards the cortex. This segment is sometimes grouped as part of M2. M4: These finer terminal or cortical segments irrigate the cortex. They begin at the external of the Sylvian fissure and extend distally away on the cortex of the brain.
Unilateral occlusion of Middle Cerebral Arteries at the stem (proximal M1 segment) results in:
y y y
Contralateral hemiplegia affecting face, arm, and leg (lesser). Homonymous hemianopia - Ipsilateral head/eye deviation. If on left: global aphasia.
Bilateral occlusion of Anterior Cerebral Arteries at their stems results in infarction of the anteromedial surface of the cerebral hemispheres:
o o o o
Paraplegia affecting lower extremities and sparing face/hands. Incontinence Abulic and motor aphasia Frontal lobe Symptoms: personality change, contralateral grasp reflex.
Unilateral occlusion (distal to Ant. Comm. origin) of Anterior Cerebral Artery produces contralateral sensorimotor deficits mainly involving the lower extremity with sparing of face and hands (think of the humunculus).
===============================
In human anatomy, the posterior communicating artery is one of a pair of right -sided and left -sided blood vessels in the circle of Willis . It connects the three cerebral arteries of the same side. Anter iorly, it is one portion of the terminal trifurcation of the internal carotid artery . The anterior cerebral artery and the middle cerebral artery are the other two branches of the trifurcation. Posteriorly, it communicates w ith the posterior cerebral artery .
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Aneurysms of the posterior communicating artery are the second most common circle of Willis aneurysm[1] (the most common are anterior communicating artery aneurysms) and can lead to oculomotor nerve palsy
MEN 2
Feature
MEN 1
MEN 2A
MEN 2B
FMTC (none)
Eponym
Wermer syndrome
Sipple syndrome(multiple)
- - - 100% - -
Pheochromocytoma
-
-
>95%
-
Gene(s)
MEN1 (131100)
RET (164761)
RET (164761)
ibuprofen
Gastric carcinoma:
y
Histologically, there are two major types of gastric adenocarcinoma (Lauren classification): intestinal type or diffuse type. Adenocarcinomas tend to aggressively invade the gastric wall, infiltrating the muscularis mucosae, the submucosa, and thence the muscularis propria. Intestinal type adenocarcinoma tumor cells describe irregular tubular structures, harboring pluristratification, multiple lumens, reduced stroma ("back to back" aspect). Often, it associates intestinal metaplasia in neighboring mucosa. Depending on glandular architecture, cellular pleomorphism and mucosecretion, adenocarcinoma may present 3 degrees of differentiation: well, moderate and poorly differentiated. Diffuse type adenocarcinoma (mucinous, colloid, linitis plastica, leather-bottle stomach) Tumor cells are discohesive and secrete mucus which is delivered in the interstitium producing large pools of mucus/colloid (optically "empty" spaces). It is poorly differentiated. If the mucus remains inside the tumor cell, it pushes the nucleus to the periphery"signet-ring cell".
The criteria for point assignm ent is that a certain breakpoint be met at anytime during that 48 hour period, so that in some situations it can be calculated shortly after admission. It is applicable to both gallstoneand alcoholic pancreatitis. Alternatively, pancreatitis severity can be assessed by any of the following:
[2]
APACHE II score 8 Organ failure Substantial pancreatic necrosis (at least 30% glandular necrosis accor ding to contrast-enhanced CT)
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Or
Score 0 to 2 : 2% mortality Score 3 to 4 : 15% mortality Score 5 to 6 : 40% mortality Score 7 to 8 : 100% mortality
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At admission: "GA LAW" (glucose, age, LDH, AST, WBC count) At 48 hours: "C HOBBS" (as in Calvin and Hobbes): (calcium, hematocrit, O2, BUN, Base deficit,
Adrenal zona glomerulosa: mineralocorticoid/ aldosterone zona fasciculata: glucocorticoids/cortisol, androgen(a few) zona reticularis: precursor androgens including dehydroepiandrosterone (DHEA) and androstenedione
ASA score
ASA 1 ASA 2
ASA 3 ASA 4
ASA 5 E
24
Cornea:
It comprises five major layers, namely, epithelium (outer portion, having the ability to regenerate), Bowman's membrane (hard, protective layer), stroma (thickest layer with collagen fibrils), Descemet's membrane (thin layer) and endothelium (one -celled thick innermost layer). Second branchial cleft cysts The second branchial cleft accounts for 95% of branchial anomalies, and they are most frequently identified along the anterior border of the upper third of the sternocleidomastoid muscle and adjacent to the muscle. However, these cysts may present anywhere along the course of a second branchial fistula, which proceeds from the skin of the lateral neck, between the internal and external carotid arteries, and into the palatine tonsil (see the following image). Therefore, a second branchial cleft cyst is part of the differential diagnosis of a parapharyngeal mass. First branchial cleft cysts First branchial cleft cysts are divided into type I and type II. Type I cysts are located near the external auditory canal. Most commonly, they are inferior and posterior to the tragus (base of the ear), but they may also be in the parotid gland or at the angle of the mandible. Type II cysts are associated with the submandibular gland or found in th e anterior triangle of the neck (see the image below). Third branchial cleft cysts Third branchial cleft cysts are rare. A third branchial fistula extends from the same skin location as a second branchial fistula (recall that the clefts merge during develo pment); however, a third branchial fistula courses posterior to the carotid arteries and pierces the thyrohyoid membrane to enter the larynx, terminating on the lateral aspect of the pyriform sinus. Third branchial cleft cysts occur anywhere along that course (eg, inside the larynx), but they are characteristically located deep to the sternocleidomastoid muscle (see the image below). Fourth branchial cleft cysts Fourth branchial cleft cysts are extremely rare. A fourth branchial fistula arises from the lat eral neck and parallels the course of the recurrent laryngeal nerve (around the aorta on the left and around the subclavian artery on the right), terminating in the apex of the pyriform sinus; therefore, fourth branchial cleft cysts arise in various locati ons, including the thyroid gland and mediastinum.
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y y
akathisia: restlessness pseudoparkinsonism: drug-induced parkinsonism (muscular lead-pipe rigidity, bradykinesia/akinesia, resting tremor, and postural instability; more frequent in adults and the elderly). Although Parkinson's Disease is primarily a disease of the nigrostriatal pathway and not the extrapyramidal system, loss of dopaminergic neurons in the substantia nigra leads to dysregulation of the extrapyramidal system. Since this system regulates posture and skeletal muscle tone, a result is the characteristic bradykinesia of Parkinson's.
tardive dyskinesia: Often miscategorized as an EPS, tardive dyskinesia (involuntary, irregular muscle movements, usually in the face) has been shown to have an association with EPS. [2]
y y
Periodic episodes of rotary vertigo or dizziness. Fluctuating, progressive, unilateral (in one ear) or bilateral (in both ears) hearing loss, usually in lower frequencies. [4] Unilateral or bilateral tinnitus. A sensation of fullness or pressure in one or both ears.
y y
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c feve Rhe schoff bodies are nodules found in the hearts of individuals with rheumatic fever. hey result from inflammation in the heart muscle and are characteristic of rheumatic heart disease.
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pericarditis with a rub, or a new heart murmur. Subcutaneous nodules: painless, firm collections of collagen fibers over bones or tendons. They commonly appear on the back of the wrist, the outside elbow, and the front of the knees. Erythema marginatum: a long lasting rash that begins on the trunk or arms as macules and spreads outward to form a snake like ring while clearing in the middle. This rash never starts on the face and it is made worse with heat. Sydenham's chorea (St. Vitus' dance): a characteristic series of rapid movements without purpose of the face and arms. This can occur very late in the disease. Minor criteria Fever Arthralgia: Joint pain without swelling (Cannot be included if Polyarthritis is present as a major symptom) Raised erythrocyte sedimentation rate or C reactive protein Leukocytosis ECG showing features of heart block, such as a prolonged PR interval[8] (Cannot be included if Cariditis is present as a major symptom) Supporting evidence of streptococcal infection: elevated or rising antistreptolysin O titre or DNAase.[1] Previous episode of rheumatic fever or inactive heart disease Breat cancer staging:
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no tumor can be found in the breast, but cancer cells are found in the axillary lymph nodes (the lymph nodes under the arm), OR the tumor measures 2 centimeters or less and has spread to the axillary lymph nodes, OR the tumor is larger than 2 centimeters but not larger than 5 centimeters and has not spread to the axillary lymph nodes
y y
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the tumor is larger than 2 but no larger than 5 centimeters and has spread to the axillary lymph nodes, OR the tumor is larger than 5 centimeters but has not spread to the axillary lymph nodes
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no tumor is found in the breast. Cancer is f ound in axillary lymph nodes that are clumped together or sticking to other structures, or cancer may have spread to lymph nodes near the breastbone, OR the tumor is 5 centimeters or smaller and has spread to axillary lymph nodes that are clumped together or sticking to other structures, OR the tumor is larger than 5 centimeters and has spread to axillary lymph nodes that are clumped together or sticking to other structures
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the tumor may be any size and has spread to the chest wall and/or skin of the breast AND may have spread to axillary lymph nodes that are clumped together or sticking to other structures, or cancer may have spread to lymph nodes near the breastbone Inflammatory breast cancer is considered at least stage IIIB.
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there may be no sign of cancer in the breast or, if there is a tumor, it may be any size and may have spread to the chest wall and/or the skin of the breast, AND the cancer has spread to lymph nodes above or below the collarbone, AND the cancer may have spread to axillary lymph nodes or to lymph nodes near the breastbone
y y
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the cancer has spread to other organs of the body -- usually the lungs, liver, bone, or brain
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Early stage
y y y y
y y y
size (T stands for tumor) lymph node involvement (N stands for no de) whether it has metastasized (M stands for metastasis)
y y y
TX means the tumor can't be measured or found. T0 means there isn't any evidence of the primary tumor. Tis means the cancer is "in situ" (the tumor has not started growing into the breast tissue).
The numbers T1-T4 describe the size and/or how much the cancer has grown into the breast tissue. The higher the T number, the larger the tumor and/or the more it may have grown into the breast tissue.
y y y
NX means the nearby lymph nodes can't be measured or found. N0 means nearby lymph nodes do not contain cancer. The numbers N1-N3 describe the size, location, and/or the number of lymph nodes involved. The higher the N number, the more the lymph nodes are involved.
y y y
MX means metastasis can't be measured or found. M0 means there are no distant metastases. M1 means that distant metastases were found.
+TWJ]FRUQJF932GWJFXYHFSHJW\TZQIRJFSYMFYYMJUWNRFW^GWJFXYYZRTW
y y y
is less than 2 centimeters across (T1) does not have lymph node involvement (N0) has not spread to distant parts of the body (M0)
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Cholestatic Diseases: primary biliary cirrhosis, sclerosing cholangitis, secondary biliary cirrhosis, biliary atresia, cystic fibrosis Chronic Hepatitis: hepatitis B, hepatitis C, hepatitis D, autoimmune chronic active hepatitis, cryptogenic cirrhosis, chronic drug toxicity or toxin exposure Alcoholic Cirrhosis: Patients with alcoholic cirrhosis are considered for transplant if they meet current criteria for abstinence and rehabilitation.
Abstinence of alcohol for six months. Ongoing participation in formal alcohol treatment program. Presence of adequate psychosocial supports as determined by social service and psychiatry consultants.
Patients who do not meet the above criteria at the time of referral will be given the opportunity to fulfill these criteria and undergo re -evaluation. Formal input from the psychiatry staff is required to assess the risk of return to alcohol use following liver transplantation.
y
Metabolic Diseases: hemochromatosis, Wilson's disease, Alpha-1-antitrypsin deficiency, glycogen storage disease, tyrosinemia, familial amyloidotic polyneuropathy, other metabolic disorders treatable by liver replacement. Fulminant Acute Hepatic Necrosis: viral hepatitis, drug toxicity, toxin, Wilson's disease. Primary Hepatic Tumors: selected patients with hepatocellular carcinoma
While each patient is evaluated on an individual basis, the presence of one or more of the following will frequently preclude acceptance as a candidate for liver transplantation
HIV infection Active alcohol or substance abuse Systemic infections Life limiting co-existing medical conditions: advanced heart, lung or neurologic conditions. Uncontrolled psychiatric disorder Inability to comply with pre- and post-transplant regimens
Alagille syndrome is a AD genetic disorder that affects the liver, heart, kidney, and oth er systems of the body, NOTCH2 is also associated with Alagille syndrome
S/S: jaundice, xanthoma, liver biopsy: few bile duct, Tetralogy of Fallot, spinal X ray: butterfly -shaped bone lesion, typical facial features (including a broad, prominent forehead, deep -set eyes, and a small pointed chin). The kidneys and central nervous system may also be affected.
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y y y y y y y
Papillary thyroid carcinoma Papillary renal cell carcinoma serous papillary ovarian adenocarcinoma (cystadenocarcinoma) endometrial adenocarcinomas (Papillary serous carcinoma ~3%-4%) meningioma mesothelioma somatostatinoma (pancreas)[2]
e 1 (NF 1)
ple iform neurofibroma on the nec of a patient; ple iform neurofibromas are a cause ofmorbidity in the affected individuals.
q r q
atient with multiple small cutaneous neurofibromas and a 'caf au lait spot' ( ottom of photo, to the b biopsy has been taken of one of the lesions
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right of centre .
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Two or more neurofibromas on or under the skin or one plexiform neurofibroma (a large cluster of tumors involving multiple nerves); Neurofibromas are the subcutaneous bumps that are characteristic of the disease and increase in number with age. Freckling of the groin or the axilla (arm pit). Caf au lait spots (pigmented, light brown macules located on nerves, with a smooth edges("coast of California") [4] birthmarks). Six or more measuring 5 mm in greatest diameter in prepubertal individuals and over 15 mm in greatest diameter in postpubertal individuals. Skeletal abnormalities, such as sphenoid dysplasia or thinning of the cortex of the long bones of the body (i.e. bones of the leg, potentially resulting in bowing of the legs)[1] Lisch nodules (hamartomas of iris), freckling in the iris. Tumors on the optic nerve, also known as an optic glioma. Macrocephaly in 30-50% of the pediatric population without any hydrocephalus.[5] Epilepsy (seizures) Juvenile posterior lenticular opacity [1]
y y
y y y y y
bilateral acoustic neuromas (tumors of the vestibulocochlear nerve or cranial nerve 8 (CN VIII) also known as schwannoma) often leading to hearing loss. In fact, the hallmark of NF 2 is hearing loss due to acoustic neuromas around the age of twenty. the tumors may cause:
o o
headaches balance problems, and peripheral vertigo often due to schwannoma and involvement of the inner ear. facial weakness/paralysis due to involvement or compression of the facial nerve (cranial nerve 7 or CN VII) patients with NF2 may also develop other brain tumors, as well as spinal tumors. Deafness and Tinnitus.
o o
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y y y y y
Muscle weakness in the arms and legs Loss of coordination Vision impairment Hearing impairment Slurred speech
y y y
Curvature of the spine (scoliosis) High plantar arches (pes cavus deformity of the foot) Diabetes (about 20% of people with Friedreich's ataxia develop carbohydrate intolerance and 10% develop diabetes mellitus) Heart disorders (e.g., atrial fibrillation, and resultant tachycardia (fast heart rate) and hypertrophic cardiomyopathy )
Friedreich's ataxia is an autosomal recessive congenital ataxia and is caused by a mutation in gene FXN, the mutant gene contains expanded GAA triplet repeats in the first intron
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Wada test is conducted with the patient awake. Essentially, a barbiturate (which is usually sodium amobarbital) is introduced into one of the internal carotid arteries via a cannula or intra-arterial catheter from the femoral artery. The drug is injected into one hemisphere at a time. The eff ect is to shut down any language and/or memory function in that hemisphere in order to evaluate the other hemisphere. The test is usually performed prior to ablative surgery for epilepsy and sometimes prior to tumor resection.
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y y
Latency of onset (usually 5 10 seconds) Torsional (rotational) nystagmus. If no torsional nystagmus occurs but there is upbeating or downbeating nystagmus, a central nervous system (CNS) dysfunction is indicated. Upbeating or downbeating nystagmus. Upbeating nystagmus indicates that the vertigo is present in the posterior semicircular canal of the teste d side. Downbeating nystagmus indicates that the vertigo is in the anterior semicircular canal of the tested side. Fatigable nystagmus. Multiple repetition of the test will result in less and less nystagmus. Reversal. Upon sitting after a positive maneuver the direction of nystagmus should reverse for a brief period of time.
y y
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o o o o o
Vertigo: Spinning dizziness, which must have a rotational component. Short duration (Paroxysmal): Lasts only seconds to minutes Positional in onset: Can only be induced by a change in position. Nausea is often associated Visual disturbance: It may be difficult to read or see during an attack due to the associated nystagmus. Pre-Syncope (feeling faint) or Syncope (fainting) is unusual. Emesis (Vomiting) is uncommon but possible.
o o
Signs
o
Rotatory (torsional) nystagmus, where the top of the eye rotates towards the affected ear in a beating or twitching fashion, which has a latency and can be fatigued (if you repeatedly continue placing yourself in the position to cause vertigo the symptoms should lessen each time).
Hirano bodies are intracellular aggregates of actin and actin-associated proteins observed in the neurons (nerve cells) of individuals afflicted with certain neurodegenerative disorders , such as Alzheimer's disease and Creutzfeldt-Jakob disease. They are often described as rod-shaped, crystal-like, and eosinophilic
Conditions affecting the dorsal columns of the spinal cord, such as tabes dorsalis (neurosyphilis), in which it was first described. [1] Conditions affecting the sensory nerve s (sensory peripheral neuropathies), such as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Friedreich's Ataxia
Romberg's test is not a test of cerebellar function, as it is commonly misconstrued. Patients with cerebellar ataxia will, generally, be unable to balance even with the eyes open;[5] therefore, the test cannot proceed beyond the first step and no patient with cerebellar ataxia can correctly be described as Romberg's positive. Rather, Romberg's test is a test of the proprioception receptors and pathways function. A positive Romberg's test has been shown to be 90% sensitive for lumbar spinal stenosis. Cushing triad is hypertension (systolic), bradycardia and respiratory depression.
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The Jendrassik maneuver is a medical maneuver wherein the patient flexes both sets of fingers into a hook-like form and interlocks those sets of fingers together. The tendon below the patient's knee is then hit with a reflex hammer to elicit the patellar reflex.
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Jolly's test is a medical test used to distinguish the muscle weakness found in myasthenia gravis and Eaton-Lambert syndrome. Jolly's test is a type of electromyography which uses repeated stimulation of motor nerves to demonstrate the fatiguability found with repeated muscle contraction which is seen in myasthenia but not Eaton-Lambert syndrome.
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Myotonic dystrophy is the most common form of muscular dystrophy affecting adults, which affects at least 1 in 8,000 people worldwide . Myotonic dystrophy (dystrophia myotonica, DM) is a chronic, slowly progressing, highly variable inherited multisystemic disease. It is characterized by wasting of the muscles (muscular dystrophy), cataracts, heart conduction defects, endocrine changes, and myotonia.
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Investigation:
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Diagnostic Criteria:
y
Symptomatic myeloma: 1. Clonal plasma cells >10% on bone marrow biopsy or (in any quantity) in a biopsy from other tissues (plasmacytoma) 2. A monoclonal protein (paraprotein) in either serum or urine(except in cases of true non-secretory myeloma) 3. Evidence of end-organ damage felt related to the plasma cell disorder (related organ or tissue impairment, ROTI, commonly referred to by the acronym "CRAB"):
HyperCalcemia (corrected calcium >2.75 mmol/L) Renal insufficiency attributable to myeloma Anemia (hemoglobin <10 g/dL) Bone lesions (lytic lesions or osteoporosis with compression fractures)
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2-microglobulin (
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