Tuesday, September 9, 2014

1. Defense mechanisms: Disassociation vs Repression 
- Disassociation: disruptions in memory, identity, consciousness, or perception to retain illusion of control; ie amnesia following a cancer diagnosis, being unable to remember such appointment despite others' reminding her.
- Repression: blocking upsetting ideas from entering consciousness - blockage of inner states (vs denial, blocks external sensory data). I.e. repressing memory of personal cancer diagnosis, and then not understanding why one is anxious when their child reaches the age that the patient was diagnosed with cancer.
- Psychiatrists tend to think of disassociation as a clinical, measurable phenomenon while repression is more of a  Freudian psychoanalytic concept that may be dated; I guess for the purposes of USMLE it sounds like disassociation may be used for discrete events while repression is a broader suppressive concept..?
2. CSF studies: 
- Albumino-cytologic disassociation (elevated protein with normal cell count) - Guillian Barre
- Oligoclonal bands - 85-90% of MS cases. However can be elevated in other neurological conditions as well.
- IgG index, the other test used for MS, seems to have less value than Oligoclonal bands.
3. ADPCKD: 
- Increased incidence of abdominal wall/inguinal hernias and colonic diverticula.
- Cysts in kidney, liver, pancreas
- Increased incidence of berry aneurysms in circle of willis
- Mitral valve prolapse
4. Henoch Schonlein purpura:
- Immune-mediated vasculitis: deposition of IgA in various tissues
- Most common in kids < 15
- Palpable purpura - normal platelet and coagulation studies
- IgA nephropathy - UA shows red cell casts, hematuria, mild proteinuria, Cr can be normal or elevated
- Abdominal pain - increased risk for intussusception - occurs in up to 4% of cases, and is frequently small bowel or ileo-ileal (rather than the usual ileo-colic of non HSP kids which can be reduced with air enemas); usually requires surgical management
- GI bleeding
- Arthralgias
- Scrotal swelling
5. DVT management
- First time DVT with clear reversible inciting incident: 3 months of coumadin (bridge with heparin)
- If post surgical, OK to start heparin in someone who is hemodynamically stable 48-72 hours after surgery UNLESS IT'S NEUROSURGERY then consult neurosurgical team for when its OK to restart anticoagulation
- Clot-lysing agents like streptokinase - not shown to improve survival with DVTs; may decrease post-phlebitic syndrome. Not commonly used.
- First time DVT in someone with no clear inciting incident - indefinite anticoagulation if bleeding risk low; 3-6 months then re-evaluate if bleeding risk high.
6. Turner syndrome: 
- Increased risk of osteoporosis: low estrogen from gonadal dysgenesis. These patients get estrogen supplementation for development reasons which also helps with osteoporosis prevention.
- Coarctation of aortic valve, aortic valve abnormalities
7. Lysosomal storage diseases: 
- Niemann-Pick: sphingomelinase deficiency, sphingomyelin accumulates in reticuloendothelial system and brain - hypotonia, hepatosplenomegaly, cervical lymphadenopathy, protuding abdomen, cherry-red macula
- Tay Sachs: hexosaminidase A deficiency - hyperacusis, mental retardation, seizures, cherry red spot, NO accumulation in reticuloendothelial system
- Gaucher's: glucocerebrosidase deficiency - hepatosplenomegaly, pancytopenia
- Krabbe's: galactocerebrosidase deficiency - hyperacusis, irritability, seizures
- Mucopolysaccharidosis I (Hurler's) - a-L-iduronase coarse facial features, HSM, corneal clouding/retinal degeneration
- Mucopolysaccharidosis II (Hunter's) - iduronate sulfatase deficiency - dermatan and heparan sulfate accumulation, HSM, frequent infections
8. Side effects of clozapine:
- See post from January 10, 2014 for thorough description of side effects.
- Briefly: agranulocytosis (need weekly CBC for 1st 6 mos then titrate down, 3% incidence in caucasians), myocarditis, seizures (give c depakote), metabolic syndrome
9. Postpericardotomy syndrome: 
- After pericardotomy, can develop reactive pericarditis (post op inflammatory reaction), pericardial effusion, even tamponade
- In small children can present with vomiting, decreased appetite, abdominal pain
- In older children presents as adults - pleuritic chest pain exacerbated by lying supine or inspiration,
10. Diabetic nephropathy
- Glomerular hyperfiltration -> Basement membrane thickening -> Mesangial expasnion -> nodular sclerosis
- Glomerular hyperfiltration can be seen within days of DM diagnosis; the ability of ACE-I to decrease this is believed to be the mechanism of their effectiveness in the treatment of diabetic nephropathy.

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