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Type III Hyperlipoproteinemia: Diagnosis, Molecular Defects, Pathology, and Treatment
- H. BRYAN BREWER, Jr., M.D.;
- LOREN A. ZECH, M.D.;
- RICHARD E. GREGG, M.D.;
- DAVID SCHWARTZ, M.D.; and
- ERNST J. SCHAEFER, M.D.
Abstract
Type III hyperlipoproteinemia is characterized by increased plasma levels of triglycerides and cholesterol, palmar-tuberoeruptive xanthoma, and premature cardiovascular disease. Three major classes of molecular defects will predispose patients to develop type III hyperlipoproteinemia: a deficiency in apolipoprotein E, a structural defect in the E apolipoprotein, and a functional defect in the liver receptor system. Most patients with type III hyperlipoproteinemia have a structural defect in apolipoprotein E associated with increased synthesis and decreased catabolism of apolipoprotein E, delayed catabolism of chylomicron remnants, and development of plasma lipoprotein abnormalities characteristic of type III hyperlipoproteinemia. Analysis of cardiovascular disease in patients with type III hyperlipoproteinemia showed extensive coronary and peripheral vascular atherosclerosis indistinguishable from the atherosclerosis of non-hyperlipidemic and other dyslipoproteinemic patients. The xanthoma and elevated plasma cholesterol and triglyceride levels in patients with type III hyperlipoproteinemia respond to dietary and drug therapy.
Article and Author Information
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▸An edited transcript of a Clinical Staff Conference at the Clinical Center, Bethesda, Maryland, 23 January 1981, sponsored by the National Heart, Lung, and Blood Institute, National Institutes of Health, U.S. Department of Health and Human Services.
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▸Authors who wish to cite a section of the conference and specifically indicate its author can use this example of the form of the reference:
GREGG RE. Molecular and metabolic defects, pp. 628-32. In: Brewer HB, moderator. Type III hyperlipoproteinemia: diagnosis, molecular defects, pathology, and treatment. Ann Intern Med. 1983;98(5 pt 1):623-40.
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▸Requests for reprints should be addressed to H. Bryan Brewer, Jr., M.D.; Building 10, Room 7N117, National Institutes of Health; Bethesda, MD 20205.
