Test 85 Flashcards
RVH
Cor pulmonale
caused by pulmonary HTN seen in F 20-40
Essential HTN and aortic stenosis cause what type of hypertrophy
LEFT VH
Pica
compulsive consumption of nonfood
common in pregnancy
usually ice
CGD inheritance
X linked mutation that affects NADPH oxidase
reccurent bacterial and fungal infections
dx CGD
ABSENCE of normal BLUE and fluroscent GREEN pigment produced by NITROBLUE TETRAZOLIUM test and DIHYRORHODAMINE flow cytometry
Nitroblue tetrazolium testing
Add NBT to sample of pt’s neutrophils
properly functioning neutrophils produce ROS that can REDUCE the yellow NBT to DARK BLUE that preceipitates in cells
Dihydrorhodamine
assesses production of superoxide radicals by measuring conversion of DHR to RHOdamine which shoiuld come out fluorescent green
ADA def
AR
SECOND MC cause of SCID
PROFOUND decrease in B and T cells
mutlisubunit complex that acts as terminal enzyme of mitochondrial respiratory chain
Cyt C oxidase
MPO
Enzyme found in neutrophil azurophilic granules
catalyzes production of BLEACH from hydrogen peroxide and chlorine
Tx HIGH LDL
Hepatitis
myopathy
Statins
HMG CoA reductase
Tx LOW HDL Cutaneous vasodilation (Red flushed face) hyperglycemia (acanthosis nigricins) Hyperuricemia/ GOUT Hepatitis
Niacin
INHIBITS lipolysis in adipose
REDUCES hepatic VLDL synthesis
Tx HIGH TG
Gallstones
Myopathy (WORSE w/ Statins)
Fibrates (gemfibrozil, and ‘brates)
Upregulate LPL> increase TG clearance
Tx high LDL
Gi upset
hypertriglyceridemia
malabsorption
cholestyramine, colestipol, coleselvam
prevent intestinal esorption of bile acids, so liver must use choleseterol to make more
MO of Nitrate drugs
Mimic actions of NO
transformed to NO at SM membrane> INCREASED cGMP> DECREASEd intracellular Ca and myosin dephosphorylation
Receptor for insulin and ILGF
TK
leads to TRANSIENT recurrent pulmonary infiltrates and eventual proximal BRONCHIECTASIS that can complicate asthma
Aspergillus fumigatus
*often occurs in STEROID DEPENDENT asthmatics
pulmonary pathogen in pts w/ CF or neutropenia
p. aeruginosa
Colchicine
inhibits LEUKOCYTE migration and phagocytosis by blocking TUBULIN polymeratization
TX for acute gouty arthritis in ELDERLY pts or those w/ renal failure by INHIBITING phospholipiase A2
Glucocorticoids
Zieluton
blocks 5 lipoxygenase and inhibits leukotriene formation
Why does urinary pH DECREASE in metabolic acidosis?
Bicar is combletely resorbed from teh tubular fluid
to excrete H you need to the two most important renal buffers (NH3 and H2PO4)
CEREBELLAR HEMANGIOBLASTOMAS
CYSTS/CANCER in kidney, liver, pancrease
incrased risk for RCC (can be bilateral)
VHL disease
Neurofibromas
optic nerve gliomas
Lisch nodules
cafe au lait spots
NF1
AKA Von Recklinghaunsen’s disease
bilateral cranial nerve II SCHWANNOMAS
multiple MENINGIOMAS
NF 2
cutaneous FACIAL ANGIOMAS and LEPTOMENINGEAL ANGIOMAS
usually inolved V1 and V2 distribution of trigemnial nerve
TRAM TRACK calcifications on skull
Sturge Weber syndrome
think about weber test being on the head….