Familial dysbetalipoproteinemia

Alternative names:

type III hyperlipoproteinemia

Definition:

An inherited disorder in which both cholesterol and triglycerides are elevated in the plasma.

Causes, incidence, and risk factors:

Familial dysbetalipoproteinemia is caused by a gene defect that results in an accumulation of large lipoprotein particles that contain both cholesterol and triglyceride. The disease is usually not evident by elevated blood levels or symptoms until the age of 20 or later. Atherosclerosis develops in the coronary arteries, internal carotid arteries that supply blood to the brain, and the abdominal aorta and its branches. The condition predisposes people to coronary artery disease and peripheral vascular disease. The condition is worsened by hypothyroidism, obesity, or diabetes mellitus. Risk factors are a family history of familial dysbetalipoproteinemia or coronary artery disease. The incidence is 1 out of 10,000 people.