familial dysbetalipoproteinemia

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
References in periodicals archive ?
5 mmol/L (400 mg/dL), in familial dysbetalipoproteinemia, or when there is abnormal VLDL composition.
The hereditary types of hyperlipoproteinemia are familial lipoprotein lipase deficiency and/or apoprotein C-II deficiency (type I or V), familial hypercholesterolemia (type IIa or IIb), familial dysbetalipoproteinemia type III), familial hypertriglyceridemia (type IV), and combined hypercholesterolemia (type IIa, IIb, and/or IV) (table).

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