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 ?
The differential diagnosis of mixed hyperlipidemia also includes familial combined hyperlipidemia (FCHL), familial dysbetalipoproteinemia, and familial hypertriglyceridemia.
However, this formula is not valid for nonfasting patients, when plasma triglyceride concentrations are [greater than or equal to] 4.5 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).
Familial dysbetalipoproteinemia. Abnormal binding of mutant apoprotein E to low density lipoprotein receptors of human fibroblasts and membranes from liver and adrenals of rats, rabbits and cows.

Medical browser ?
Full browser ?