UWorld mock blocks week 2/5-2/10

Question 1

Cystinuria

Answer 1

defective intestinal absorption of cystine, ornithine, argentine, and lysine.

Defective renal tubular reabsorption of cystine, ornithine, arginine, and lysine.

Recurrent nephrolithasis with Cystine stones - hexagonal crystals

Question 2

MCA

Answer 2

Covers both Broca and Wernicke’s

Question 3

MCAD

Answer 3

Medium chain acyl CoA dehydrogenase - beta oxidation of fatty acids in the mitochondria

Deficiency results in hypoketotic hypoglycemia

Question 4

Carnitine deficiency

Answer 4

Transfer of fatty acids into mitochondria for oxidation

Muscle weakness, cardiomyopathy, hypoketotic hypoglycemia, elevated muscle triglycerides

Question 5

Locus ceruleus

Answer 5

pigmented neurons in the posterior rostral pons at the lateral floor of the fourth ventricle

Produces norepinephrine

Question 6

C-ANCA

Answer 6

We”c”ener’s granulomatosis

Kidney, nasopharynx, and lungs

Granulomas

Question 7

P-ANCA

Answer 7

Microscopic polyangitis OR Churg Strauss (eosinophilic)

Question 8

hartnup Dz

Answer 8

Pellagra like skin eruptions and cerebellar ataxia

Mutation in neutral amino acid transporter

Supplement w/ Niacin and high protein diet

Question 9

Glyburide vs. glipizide

Answer 9

sulfonylureas - block K+ channel –> insulin release from beta cells

Glyburide (and glimeperide) are longer acting and have a higher rate of hypoglycemia

Glipizide is short acting - less likely to cause hypoglycemia

Question 10

Acarbose

Answer 10

Alpha-glucosidase inhibitor - prevents intestinal absorption of glucose

Blunts postprandial hyperglycemia

Question 11

Metformin

Answer 11

Decreases hepatic glucose production, decreases intestinal glucose absorption, increases peripheral glucose uptake.

Question 12

Thiazidolinediones

Answer 12

Cardiac SE’s (heart failure, edema)

increase insulin sensitivity by increasing glucose utilization and decreasing glucose production in adipose, liver, and muscle.

Question 13

Sitagliptin

Answer 13

Increases insulin release from beta cells

Effect is glucose dependent - minimal resk of hypoglycemia

Question 14

Ezetimibe

Answer 14

Reduces intestinal cholesterol absorption

–> less cholesterol reaches liver –> liver increases LDL receptor concentration –> decreases cholesterol in circulation

Question 15

Colesevelam

Answer 15

Bile acid sequestrate

Binds bile salts and depletes them –> cholesterol 7-alpha hydroxyls is unregulated to convert cholesterol into bile acids

Question 16

Fibrates

Answer 16

Activate PPAR-alpha –> stimulate lipoprotein lipase –> decrease triglycerides (increase gallstone formation)

Question 17

Fenoldopam

Answer 17

Peripheral D1 agonist

Given IV in hypertensive emergency - especially renal insufficiency

–> arteriolar dilation, increased renal perfusion, increased diuresis/naturesis

Question 18

calcipotriene

Answer 18

topical vit. D analog used to treat psoriasis –> inhibition of keratinocyte proliferation and differentiation

Question 19

Gaucher

Answer 19

beta-glucocerebrosidase deficiency –> glucocerebroside accumulates

Lipid laden macrophages - gaucher cells - wrinkled tissue paper on bone marrow aspirate

hepatosplenomegaly, easy bleeding.bruising, fatigue d/t pancytopenia

massive splenomegaly

Question 20

Pleiotropy

Answer 20

a single genetic mutation that results in multiple phenotypic variations

Question 21

Aspirin toxicity

Answer 21

Primary respiratory alkalosis

Secondary metabolic acidosis/respiratory alkalosis