USMLE Step 1 Rapid Review Questions and Answers.

acanthocytosis, ataxia, night blindness, steatorrhea, failure to thrive - abetalipoproteinemia: AR inability to synthesize apoB-48 and apoB-100 --> inability to export lipid absorbed in intestine as chylomicron accelerated atherosclerosis, tendon xanthoma, corneal arcus - familial hypercholesterolemia: absent or decreased LDL receptors arginine derivatives - creatine urea (urea cycle intermediate) NO asymptomatic increase in fructose in blood and urine - fructokinase deficiency (essential fructosuria) autonomic receptor that causes vasoconstriction - alpha-1 autonomic receptor that causes vasodilation - beta-2, M3 back pain, hemoglobinuria following oxidative stress from infection, fava beans, sulfonamides, other drugs (enzyme deficiency, mechanism) - G6PDase deficiency (dec NADPH --> dec glutathione reductase --> free radi