Answers

1. A. Type IIa or familial hypercholesterolemia. Because triglycerides are normal, the contribution of VLDL cholesterol to the total cholesterol is slight (about 105/5, or 21 mg) and taking into account HDL cholesterol, it is clear LDL accounts for the increase in cholesterol. This is confirmed by an intense p-lipoprotein band on electrophoresis. High LDL cholesterol without elevation of other lipids defines type IIa. The condition is probably genetic because of the premature death of his brothers due to heart attacks.

2. D. Reduced hepatic LDL-receptor activity. Familial hypercholesterolemia is most often due to deficient LDL-receptor activity. A less likely possibility, although not considered in this question, is reduced LDL clearance from the circulation due to defective apolipoprotein B100.

3. E. Simvastatin. Itraconazole inhibits cytochrome P450 3A4. This cytochrome is responsible for metabolism of simvastatin. Therefore, itraconazole can increase serum level of simvastatin and increase its toxicity.

4. E. Rhabdomyolysis. Cerivastatin increases the risk of death from rhabdomyolysis.

5. D. Type IV. The patient's very high triglycerides are due to elevated VLDL, because electrophoresis showed only an intense pre-p-band. The high cholesterol is due to VLDL being composed of about 20% cholesterol and not to elevated LDL. VLDL cholesterol is about 208 mg/dL (1042/5) and 72 mg/dL for HDL. LDL cholesterol (about 88 mg/dL) must account for the remainder.

6. D. Pancreatitis. Extremely high plasma triglycerides, as in this patient, present a serious risk of acute pancreatitis.

7. E. Niacin + gemfibrozil. Niacin and gemfibrozil each can reduce plasma triglycerides. In combination a greater reduction should be observed. Although atorvastatin is reported to lower triglycerides, it is mainly a hypocholesterolemic drug. A statin should not be combined with a fibrate. Cholestyramine is a hypocholesterolemic drug that may aggravate hypertriglyceridemia, and therefore, it should not be used in this patient.

8. D. Lipoprotein lipase. Fenofibrate is a hypotriglyc-eridemic drug that lowers plasma triglycerides by increasing the activity of lipoprotein lipase, the enzyme responsible for disassembly of triglycerides in serum lipoproteins (VLDL, IDL and chylomicrons).

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