A 19-year-old man presents with severe chest pain and is found to have an acute myocardial infarction. His fasting lipid panel shows total cholesterol of 680 mg/dL, triglycerides of 920 mg/dL, and a dense band on lipoprotein electrophoresis. His father died of an MI at age 32. Which genetic defect is most consistent with this presentation?

  1. A)Homozygous familial hypercholesterolemia (LDL receptor mutations)GABARITO
  2. B)Familial combined hyperlipidemia with genetic predisposition to early MI
  3. C)Type III hyperlipoproteinemia (apoE2/E2 genotype)
  4. D)Heterozygous familial hypercholesterolemia with secondary hypertriglyceridemia
  5. E)Familial chylomicronemia syndrome with secondary atherosclerosis

Explicação

Homozygous familial hypercholesterolemia results from two mutant alleles of the LDL receptor gene. This causes severe LDL cholesterol elevation (often >600 mg/dL), widespread xanthomas, acute coronary disease in childhood/adolescence, and aortic stenosis. The ... Ver explicação completa e trilha adaptativa →

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