Mixed Flashcards
Pt with X-linked disease that consists of aniokeratomas, cardiac and renal involement and painful neuropathy. Deficiency in?
Fabry. a-galactosidase
Hepatosplenomegaly, pancytopenia, bone and joint pain, and macrophages that look like crumpled paper. Deficiency in?
Gaucher’s. B-glucocerbrosidase/
Gargoylism, cateracts, hepatosplenomegaly. Accumulation of? Decifiency in?
Hurler. Dermatan and heparan sulfate. a-L-iduronidase
Gargoylism, normal eyes, hepatomegaly? Accumulation of? Deficiency in?
Hunter. Dermatan and heparan sulfate. Iduronate sulfatase.
Hepatosplenomegaly, cherry red-spot, progressive neurodegeneration.
Foam cells. Deficiency?
Sphinomyelinase
Cherry-red spot, neurodegeneration. No hepatosplenomegaly. Deficiency?
Tay-sachs. b-hexosaminidase A/
Neurodegeneration andoptic atrophy. Deficiency?
Krabbe. Galactocerebrosidase.
Muscle wasting, dementia, ataxia. Deficiency in?
Metachromatic leukodystrophy. Arylsulfatase A
Obese pts have increased insulin resistance because?
high FFA
Hyperacute rejection. Cause?
Preformed antibodies (like those for blood type) attacking
VLCFA broken down where?
Peroxisomes
Hyperacute rejection. Cause?
Preformed antibodies (like those for blood type) attacking
VLCFA broken down where?
Peroxisomes
Stratified columnar found where?
conjunctiva of eye and male urethra
Lung stop expanding because?
DECREASE in firing of slowly adapting cells
Drug that causes loss of green vision?
Ethanbutol. (for TB)
Chemo receptor that responds to O2?
Peripheral
Cause of congential diaphragmatic hernia (abdominal contents in thorax?)
failure of pleuroperitoneal folds to form
Tx of typical pneumonia?
Ceftriaxone
Findings in sarcoidosis?
GRAIN Gamma-globulinema RA ACE interstitial fibrosis Non-caseating granulomas
Eosinophils are activated by with IL? Released by?
IL-5. Released by Th2
Cytokines that increase ESR? Mech?
IL-1, IL-6, TNF-a. Increase acute phase reactants, esp fibrinogen, which increases aggregation and ESR
