Deck 16 Flashcards
What is transthyretin and what can it do to heart?
Carrier protein from liver- mutations»_space; amyloid protein that infiltrates heart»_space; restrictive cardiomyopathy
What is tetrahydrobiopterin a cofactor for? What condition if defective?
Serotonin, DOPA; can be a cause of PKU - Qid 1500
What condition leads to absence/hypoplasia of vermis and dilated 4th ventricle (non-commun hydroceph)
Dandy- Walker
What would lead to acute morbidity in RF?
HF from pancarditis
Tetanus vaccine illicits what immune response?
Humoral- specific for toxin
What is the principle site of uric acid precipitation?
Collecting duct and DCT (most acidic > uric acids precipitate)
What CNS tumors stain for synaptophysin? GFAP?
Synaptophysin: neuronal origin (neuroectoderm, neuroendocrine); GFAP: gliomas (astrocytomas, oligodendrogliomas, glioblastoma, ependymomas)
Mechanism of action of PCP?
NMDA antagonist: dissociation, violent behavior, hallucinations, vertical nystagmus, Qid 1165
What is normal/ abnormal babinski response?
Normal = plantar flexion; Abnormal = dorsiflexion & toe spreading
What occurs within pancreatic islets in type II diabetes?
Amyloid deposition
What is also seen with familial retinoblastoma?
Osteosarcoma
What is defective in hyper IgM? What are features?
CD 40 ligand (from CD4 Tcell) activates B cell > splice to desire heavy chain region; High IgM, low everything else, sinopulmonary, GI, opportunistic infections (crypto, pneumocystis)
What is first line for alcohol withdrawal agitation? What about with liver dz?
Benzos -> long lasting (chlordiazepoxide, diazepam); liver disease = (ones that don’t undergo oxidative metabolism in liver and have no active metabolites => LOT) Lorazepam, oxazepam, temazepam
What does PAS stain for?
Glycogen when used with diastase (“pass the sugar”): glycogen storage diseases, whipple disease (diastase resistant) - Qid 132
Whats a kussmaul sign?
Increase in JVP on inspiration: restrictive cardiomyopathy, constrictive pericarditis, right sided heart tumors