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.

fa·mil·i·al hy·per·lip·o·pro·te·in·e·mi·a type III

[MIM*107741]
hyperlipoproteinemia characterized by increased plasma levels of LDL, β-lipoproteins, pre-β-lipoproteins, cholesterol, phospholipids, and triglycerides; hypertriglyceridemia induced by a high carbohydrate diet, and glucose tolerance is abnormal; frequent eruptive xanthomas and atheromatosis, particularly coronary artery disease; biochemical defect lies in apolipoproteins. There are many varieties, one of which is caused by mutation in the APOE gene on chromosome 19q.
References in periodicals archive ?
It is indicated as an adjunct to diet for the treatment of adult patients with hypertriglyceridemia, as an adjunct to diet for the treatment of adult patients with primary dysbetalipoproteinemia (type III hyperlipoproteinemia), and as an adjunct to other lipid-lowering treatments (eg, LDL apheresis) or as monotherapy if such treatments are unavailable, to decrease LDL-C, total cholesterol, and ApoB in adult patients with homozygous familial hypercholesterolemia.
Shortly after generation of ApoE knock-outs, another approach to generating dyslipidemic mice was taken by creating animals that bear a human gene variant associated with familial dysbetalipoproteinemia. APOE*3-Leiden dominant mutation was present in a Dutch family.
Non-denaturing polyacrylamide gradient gel electrophoresis for the diagnosis of dysbetalipoproteinemia. J Lipid Res 2003;44(1):212-217.
Visseren, "Autosomal dominant familial dysbetalipoproteinemia: A pathophysiological framework and practical approach to diagnosis and therapy," Journal of Clinical Lipidology, vol.
Pathogenesis of type III hyperlipoproteinemia (dysbetalipoproteinemia): questions, quandaries, and paradoxes.
Non denaturing polyacrolamide gradient gel electrophoresis for the diagnosis of dysbetalipoproteinemia. J Lipid Res 2003;44(1):212-217.
Food and Drug Administration today approved the first generic version of Crestor (rosuvastatin calcium) tablets for the following uses: in combination with diet for the treatment of high triglycerides (hypertriglyceridemia) in adults; in combination with diet for treatment of patients with primary dysbetalipoproteinemia (Type III Hyperlipoproteinemia), a disorder associated with improper breakdown of cholesterol and triglycerides; either alone or in combination with other cholesterol treatment(s) for adult patients with homozygous familial hypercholesterolemia, a disorder associated with high low-density lipoprotein (LDL) cholesterol.
[USPRwire, Fri Jun 13 2014] GlobalData's clinical trial report, "Dysbetalipoproteinemia (Type III Hyperlipoproteinemia) Global Clinical Trials Review, H1, 2014" provides data on the Dysbetalipoproteinemia (Type III Hyperlipoproteinemia) clinical trial scenario.
(12) known glycogen storage disease or dysbetalipoproteinemia; (13) known phenotypic hemochromatosis (HII greater than 1.9 or removal of more than 4 g of iron by phlebotomy); (14) prominent bile duct injury (florid duct lesions or periductal sclerosis) or bile duct paucity; (15) chronic cholestasis; (16) vascular lesions (vasculitis, cardiac sclerosis, acute or chronic Budd-Chiari, hepatoportal sclerosis, or peliosis); (17) concomitant severe underlying systemic illness that in the opinion of the investigator would interfere with completion of followup; (18) inability to undergo MRI.
The differential diagnosis of mixed hyperlipidemia also includes familial combined hyperlipidemia (FCHL), familial dysbetalipoproteinemia, and familial hypertriglyceridemia.
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).
Remnant-like particle cholesterol levels in patients with dysbetalipoproteinemia or coronary artery disease.