Wednesday, July 2, 2014

1. Hemophilia - recurrent hemarthrosis and skeletal muscle bleeds are most common manifestations. GI bleeds are less common.
2. Restrictive cardiomyopathy:
- Diastolic dysfunction - people will experience significant clinical findings of heart failure (JVD, edema, wet lungs, etc), while systolic function on echo is preserved.
Causes:
- Hemochromatosis - can be reversed with treatment (ie phlebotomy)
- Amyloidosis - not reversible; symptomatic treatment only
- Sarcoidosis - treatable with steroids; will slow progression but will not reverse
- Scleroderma - treatable with steroids; will slow progression but will not reverse
3. Senile purpura: ecchymoses in elderly patients in places exposed to repeated minor trauma; due to age-related loss of elastic fibers in perivascular connective tissue.
4. DDx poor platelet adhesion
- Von willebrands: generally presents quite young
- Antiplatelet drugs (aspirin, plavix, abciximab...)
- CKD (2/2 uremia... some renal transplant surgeons wont operate when BUN>100 due to increased bleed risk)
5. Types of tremor:
- Essential: generally bilateral action tremor or hands, may have isolated head tremor, improves with alcohol
- Parkinsonian: may be unilateral or bilateral of hands and feet, rarely involves head, improves with voluntary movement, worse with mental distraction
- Cerebellar: intention tremor, often associated with ataxia, dysmetria, gait problems
- Physiologic: onset with sympathomimetics (drugs, hyperthyroidsm, anxiety, caffeine); worse with movement, can affect face and extremities
- Orthostatic: tremor of legs that occurs right after standing up
6. Bradycardia, hypotension, AV block, wheezing, hypoglycemia, neuro effects (seizures, delirium) = beta blocker toxicity. Treat with IV fluids and atropine first; if that doesn't work, then glucagon; after that consider calcium, glucose + insulin, epi; if medical therapy fails can consider temporary pacemaker.
7. Sepsis in sickle cell: strep pneumo most common cause; HIB is a distant second. Thus these kids should get the standard 13-valent pnuemo vaccine + HIB vaccine and also the 23-valent polysaccharide pneumo vaccine usually given to old people and the neisseria vaccine as well.
8. Duodenal hematoma in kids - manage conservatively with NG suction and IV nutrition, as these typically resolve in 1-2 weeks.
9. Neuroblastoma: 
- Most common extracranial solid tumor of childhood
- Neural crest cell origin - may originate from anywhere along sympathetic chain or adrenal gland.
- Firm, nodular mass
- Can have calcifications and hemorrhages
- Up to 70% will have mets at presentation - long bones, skull, bone marrow, liver, lymph nodes, skin.
- HVA and VMA elevated, but no pheo symptoms (fainting, sweating, palpitations, HTN)
10. Renal embryology 
- Wilms tumor - from metanephros (embryologic precursur of renal parenchyma)
- Mesonephros - gives rise to seminal vesicles, epididymis, ejaculatory ducts and ductus deferens
- Paramesoneprhon - fallopian tubes, uterus, vagina

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