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79 Cards in this Set
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- Back
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patient has acutely painful abdomen, c/o recent history of dark "wine-colored" urination, and feeling confused. what is the dx and what is the affected enzyme?
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acute intermittent porphyria
enzyme = porphobilinogen deaminase |
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homeless man comes in with blistering on neck and arms. he seems to be extra-hairy on his face and darkened skin located primarily on his face. you also can smell alcohol in his breath.
1) what is the likely dx? 2) which type of GI disease is he likely to have? 3) what is the affected enzyme? |
1) porphyria cutanea tarda
2) hepatitis C 3) uroporphyrinogen decarboxylase |
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what is the rate-limiting step in heme synthesis?
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aminolevulinic acid synthase
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what immunosuppressant:
1) causes phocomelia 2) nephrotoxic in 75% of pts |
1) thalidomide
2) cyclosporine |
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which immunosuppressant:
1) inhibits secretion of IL-2 and other cytokines 2) alkylating agent that requires bioactivation in liver |
1) tacrolimus, cyclosporine
2) cyclophosphamide |
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which protozoal organism:
1) ixodes tick is the vector 2) suramin or melarsoprol is the treatment 3) maltese cross seen in RBCs |
1) babesia
2) trypanosoma 3) babesia |
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what is the result of a glycolytic enzyme deficiency? what about a deficiency in pyruvate DH?
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RBC hemolysis will be seen in glycolytic enzyme deficiency
neurologic defects will be seen in pyruvate DH defiencieny |
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what segment of the renal tubule:
segment responsible for concentrating urine |
collecting duct
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what segment of the renal tubule:
site of secretion of organic anions and cations |
proximal tubule
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what segment of the renal tubule:
always impermeable to water |
thick ascending loop of henle
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what segment of the renal tubule:
site of Na/2Cl/K cotransporter |
thick ascending loop of henle
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what segment of the renal tubule:
site of isotonic fluid reabsorption |
PT
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what segment of the renal tubule:
site responsible for diluting urine |
ascending loop of Henle
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what segment of the renal tubule:
water reabsorption in the loop of henle |
descending loop of henle
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what segment of the renal tubule:
site where PTH acts on Ca reabsorption (to increase it) |
early distal tubule
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what segment of the renal tubule:
site where Ca/Mg are reabsorbed, and where loop diuretics act upon to "lose calcium" |
thick ascending loop of henle
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what shifts K out of cells, what shifts K in?
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out - low insulin, beta-blockers, acidosis, digoxin, cell lysis (eg. leukemia)
in - insulin, beta-agonists, alkalosis, cell proliferation (they will all be grabbing for K) |
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what is wrong in patients with:
pH 7.47, HCO3 14, CO2 22 |
respiratory alkalosis with metabolic compensation
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what is wrong in patients with:
pH 7.34, HCO3 31, CO2 62 |
respiratory acidosis with metabolic compensation
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pt with recent kidney transplant on cyclosporin for immunosuppression requires an antifungal anggent for candidiasis --> what drug would result in cyclosporin toxicity?
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ketoconazole (interferes with/inhibitsw cytochrome p-450)
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what is the maximal serum glucose concentration at which glucose can be absorbed in the tubules?
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350 mg/dL
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a pt w/ heart failure exacerbation needs medical diuresis but has a sulfa allergy --> what diuretic can be used?
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ethacrynic acid
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what type of diuretic is the following drug?
bumetanide |
loop diuretic
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what type of diuretic is the following drug?
metolazone |
thiazide
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what type of diuretic is the following drug?
chlorthalidone |
thiazidea
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what type of diuretic is the following drug?
amiloride |
K sparing
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what diuretic would be most useful i a pateint w/ edema a/w nephrotic syndrome?
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loop diuretic
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a 40 y/o pt of yours weighs 100 kg. what is her estimated plasma volume?
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use the 60-40-20 rule.
60 for total body water 40 for ICF 20 for ECF And of the ECF, 1/4 is plasma volume (the other 3/4 is interstitial volume) so in this case, plasma volume would be 5 L |
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which glomerular dz would you suspect the most in a pt with the following findings:
IF: granular pattern of immune complex deposition; LM: diffuse capillary thickening |
membranous glomerulonephritis
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which glomerular dz would you suspect the most in a pt with the following findings:
IF: granular pattern of immune complex deposition; LM: hypercellular glomerulli |
acute post-streptococcal GN
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which glomerular dz would you suspect the most in a pt with the following findings:
IF: linear pattern of immune complex deposition |
goodpasture's syndrome
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which glomerular dz would you suspect the most in a pt with the following findings:
deposition of IgG, IgM, IgA, and C3 in the mesangium |
IgA nephropathy
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which glomerular dz would you suspect the most in a pt with the following findings:
most common nephrotic syndrome in adults |
membranous GN
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which glomerular dz would you suspect the most in a pt with the following findings:
nephrotic syndrome a/w hep B |
membrano-proliferative GN
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which glomerular dz would you suspect the most in a pt with the following findings:
nephrotic syndrome a/w HIV |
focal segmental glomerulosclerosis
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which glomerular dz would you suspect the most in a pt with the following findings:
EM: subendothelial humps and tram-track appearance |
membrano-proliferative GN
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which glomerular dz would you suspect the most in a pt with the following findings:
nephritis, deafness, cataracts |
Alport's
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which glomerular dz would you suspect the most in a pt with the following findings:
LM: crescent formation in the glomeruli |
rapidly progressive (crescentic) GN
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which glomerular dz would you suspect the most in a pt with the following findings:
LM: segmental sclerosis and hyalinosis |
focal segmental glomerulosclerosis
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which glomerular dz would you suspect the most in a pt with the following findings:
LM: wire-loop appearance |
lupus nephritis
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which glomerular dz would you suspect the most in a pt with the following findings:
apple-green birefringence with congo red stain under polarized light |
amyloidosis
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which glomerular dz would you suspect the most in a pt with the following findings:
EM: spiking of the GBM due to electron dense subepithelial deposits |
membranous GN
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under what circumstances would you see:
1) bacterial cast 2) waxy cast 3) fatty cast |
1) acute pyelonephritis
2) chronic renal failure 3) nephrotic syndrome |
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under what circumstance would you see epithelial cell cast? what about granular casts?
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renal tubular damage; acute tubular necrosis
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glomerular histology reveals multiple mesangial nodules --> this lesion is indicative of what dz?
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kimmelstein-wilson lesion in diabetic GN
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UTI caused by proteus vulgaris --> what type of renal stone is this patient at risk for?
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ammonium magnesium phosphate stone (struvite)
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a patient reports a long-term history of acetaminophen use --> what is she at increased risk for?
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renal papillary necrosis (chronic phenacetin use is a common cause)
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what are the risk factors for transitional cell carcinoma?
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"Pee SAC": phenacetin, smoking, aniline dyes, cyclophosphamide
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renal pathology rapid review:
most common tumor of urinary tract system |
transitional cell carcinoma
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renal pathology rapid review:
hitologic appearance of renal cell carcinoma |
polygonal clear cell
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renal pathology rapid review:
fever + rash + hematuria + eosinophilia |
acute interstitial nephritis
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renal pathology rapid review:
cancer a/w Schistosoma haematobium |
squamous cell carcinoma
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what is the tx for heparin-induced thrombocytopenia?
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lepirudin, bivalirudin
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what pathologic form of RBC would you see in:
abetalipoproteinemia |
acanthocytes
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what is the cause of anemia in the following?
microcytic anemia + >3.5% HbA2 |
beta-thal minor
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what is the cause of anemia in the following?
megaloblastic anemia not correctable w/ B12 or folate what is the tx? |
orotic aciduria
tx = oral uridine administration |
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what is the cause of anemia in the following?
HIV positive patient w/ macrocytic anemia |
zidovudine
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what is the cause of anemia in the following?
normocytic anemia and elevated Cr |
chronic kidney dz --> dec EPO levels
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what does the ham's test do?
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check for PNH
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what does DEB test do? what about the (+) ristocetin test?
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check for Fanconi's anemia; von Willebrand's dz
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what do you think of when you hear +Tdt and + CALLA antigen?
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ALL
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after a normal spontaneous vaginal delivery, the new mom bleeds profusely from her vagina and later from her gums --> what abnromal lab values would you suspect?
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this is likely amniotic fluid embolism --> DIC
so increased PT/PTT, increased BT, dec platelet count, increased D-dimer |
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a 11 y/o child presents w/ chronic non-healing ulcer on his foot and imaging shows a small calcified spleen --> what drug can imrpove his sypmtoms?
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sickle cell anemia; hydroxyurea
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what is the mechanism of action:
streptokinase |
incrase plasminogen conversion to plasmin (breaking down fibrin clots)
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what is the mechanism of action:
clopidogrel |
blocks ADP receptor, which downregulates gp2b/3a receptors from platelet surface
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what is the mechanism of action:
abciximab |
blocks gp2b/3a recceptors on platelets, decrasing pt aggregation
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what is the mechanism of action:
tirofiban |
same abciximab
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what is the mechanism of action:
ticlopidine |
same as clopidogrel
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what is the mechanism of action:
eptifibatide |
same as abciximab
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what is the structure of HbH?What dz results in HbH production?
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3 alpha chains are defective; alpha-thalassemia
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what is the structure of Hb Barts? What dz results in Hb Barts production?
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no alpha chains are produced; all four are gamma chains -- this results in hydrops fetalis
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what is the difference b/w beta-thal major and minor?
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beta-thal minor (heterozygote) = beta chain is UNDERPRODUCED; pt usually asymptomatic
beta-thal major (homozygote) = beta chain is completely absent --> severe anemia requiring blood transfusion (resulting in 2ndary hemochromatosis) |
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what is the signficance of t(12;21)?
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it is ALL, that actually will have a better prognosis
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an unknown leukemia presents that is TRAP positive. what is the most likely dx and tx?
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hairy cell leukemia; tx w/ cladribine
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in von Willebrand's disease, on which cells does ddavp act upon as part of the treatment?
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endothelial cells (helps them to release more vWF)
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what is the mechanism of pathogenesis of ITP?
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anti-GpIIb/IIIa antibodies --> peripheral platelet destruction
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what dz is caused by a deficiency of ADAMTS 13; what is the pathogenesis and presentation?
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thrombotic thrombocytopenic purpura (TTP)
ADAMTS 13 is a vWF metalloprotease --> which if you are deficient in it, you will have decreased degradation of vWF multimers; increasingly large vWF multimers --> increased platelet aggregation and thrombosis p/w schistocytes, increased LDH on the lab; pt will have neurolgic symptoms, fever, and MAHA |
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what condition is caused by inactivation of ferrochelatase and ALA dehydratase?
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lead poisoning (will see basophilic stippling on peripheral smear)
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what are the 5 p's of acute intermittent porphyria?
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1) painful abdomen (neurogenic pain, will have nl CT scan)
2) pink urine 3) polyneuropathy 4) psychological disturbances 5) precipitated by drugs |
