Familial dysbetalipoproteinemia

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Coronary artery disease

Type III hyperlipoproteinemia; Deficient or defective apolipoprotein E

The goal of treatment is to control underlying conditions such as obesity, hypothyroidism, and diabetes that can make dysbetalipoproteinemia appear in people who would otherwise not have it.

The restriction of excess calories and the reduction of saturated fats and cholesterol may significantly reduce cholesterol levels.

If high cholesterol and triglyceride levels persist with maximum dietary treatment, cholesterol lowering agents should be started. Nicotinic acid (niacin), clofibrate, statins or gemfibrozil are drugs that have effectively reduced cholesterol and triglycerides in people affected with dysbetalipoproteinemia.

Individuals with this form of hyperlipidemia have a significantly increased risk for coronary artery disease. With treatment, most people show a significant reduction in lipid levels.

• Premature myocardial infarction (heart attack or heart tissue death)
• Strokes
• Blocked arteries to the body (peripheral vascular disease)
• Intermittent claudication
• Gangrene of the lower extremities
• Xanthomas of the skin

Call your health care provider if symptoms worsen, do not improve with treatment, or new symptoms develop. Call a genetic counselor if there is a family history of dysbetalipoproteinemia.

Review Date: 04/20/2005
Reviewed By: Neal Sondheimer, M.D., PhD., Division of Genetics and Metabolism, Children's Hospital of Philadelphia, Philadelphia, PA. Review provided by VeriMed Healthcare Network.