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familial dysbetalipoproteinemia |
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dysbetalipoproteinemia /dys·be·ta·lipo·pro·tein·emia/ (-ba″tah-lip″o-pro″te-ne´me-ah)
1. the accumulation of abnormal β-lipoproteins in the blood. familial dysbetalipoproteinemia an inherited disorder of lipoprotein metabolism caused by interaction of a defect in apolipoprotein E with genetic and environmental factors causing hypertriglyceridemia; its phenotype is that of a type III hyperlipoproteinemia. dysbetalipoproteinemia [dis-ba″tah-lip″o-pro″tēn-e´me-ah] the accumulation of abnormal low-density lipoproteins (β-lipoproteins) in the blood. familial dysbetalipoproteinemia familial hyperlipoproteinemia, type III.
familial dysbetalipoproteinemia Broad beta lipoproteinemia, hyperlipoproteinemia type III Metabolic disease An uncommon 1:1,000 to 10,000 AD condition with a defective apoE–the apoE2/2 phenotype, poor catabolism of
beta-migrating remnants, and ↑ production of TG-rich lipoproteins–eg, VLDL Clinical Palmoplantar tuberoeruptive xanthomas, ASHD < age 50, peripheral vascular and CAD, which may be accompanied by hyperthyroidism Lab ↑ TGs, ↑
cholesterol, floating beta lipoproteins Management Diet–weight loss, exercise, drugs–eg bile acid-binding resins and nicotinic acid; in post-menopausal ♀, low-dose estrogens Want to thank TFD for its existence? Tell a friend about us, add a link to this page, add the site to iGoogle, or visit the webmaster's page for free fun content. |
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