Hyperlipoproteinemia Type III (Broad Beta Disease)

An autosomal recessively inherited disorder characterized by the accumulation of intermediate-density lipoprotein (IDL or broad-beta-lipoprotein). IDL has a CHOLESTEROL to TRIGLYCERIDES ratio greater than that of VERY-LOW-DENSITY LIPOPROTEINS. This disorder is due to mutation of APOLIPOPROTEINS E, a receptor-binding component of VLDL and CHYLOMICRONS, resulting in their reduced clearance and high plasma levels of both cholesterol and triglycerides.

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Networked: 147 relevant articles (4 outcomes, 16 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Hyperlipidemias (Hyperlipidemia)
2.	Dyslipidemias (Dyslipidemia)
3.	Hyperlipoproteinemias
4.	Hypertriglyceridemia
5.	Hyperlipoproteinemia Type II (Familial Hypercholesterolemia)

Experts

1.	Brisson, Diane: 4 articles (01/2015 - 12/2010)
2.	Gaudet, Daniel: 4 articles (01/2015 - 12/2010)
3.	Blom, Dirk J: 3 articles (12/2008 - 01/2003)
4.	Marais, A David: 3 articles (12/2008 - 01/2003)
5.	Bertolini, S: 2 articles (05/2015 - 04/2003)
6.	Villeneuve, Sylvia: 2 articles (01/2015 - 01/2014)
7.	Méthot, Julie: 2 articles (01/2011 - 12/2010)
8.	Tremblay, Karine: 2 articles (01/2011 - 12/2010)
9.	Okubo, Minoru: 2 articles (03/2010 - 03/2002)
10.	Murase, Toshio: 2 articles (03/2010 - 03/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Hyperlipoproteinemia Type III:

1.	Lipoproteins (Lipoprotein)IBA 11/01/2002 - "Dysbetalipoproteinaemia (type III hyperlipidaemia, broad-beta disease) is a highly atherogenic genetic disorder of lipoprotein metabolism. " 03/01/1994 - "Each also had dysbetalipoproteinemia (indicated by the presence of beta-very low density lipoprotein), but neither was hyperlipidemic, attesting to the importance of other factors for the full expression of HLP III. The mutant allele appears to contribute to the inheritance of HLP III in a recessive fashion. " 02/01/1993 - "Lipoproteins in familial dysbetalipoproteinemia. " 06/01/1992 - "An accumulation of lipoprotein-remnant particles may occur and familial dysbetalipoproteinemia (FD) develops. " 07/01/1991 - "Fish oil supplementation reduces beta-very low density lipoprotein in type III dysbetalipoproteinemia."
2.	ApolipoproteinsIBA 03/10/1993 - "[Apolipoprotein E phenotyping--useful in the study of hyperlipoproteinemia type III]." 05/01/2015 - "Genetically determined HTG includes Familial Dysbetalipoproteinemia due to deficiency of apolipoprotein EIII of VLDL and Familial HTG. " 08/01/2004 - "Apolipoprotein E and familial dysbetalipoproteinemia: clinical, biochemical, and genetic aspects." 05/15/1993 - "Apolipoprotein (apo) E3-Leiden, described in a large Dutch family, is associated with a dominantly inherited form of familial dysbetalipoproteinemia. " 06/01/1992 - "Familial dysbetalipoproteinemia: a genetically heterogenous disease caused by mutations of the ligand apolipoprotein E."
3.	CholesterolIBA 07/09/1989 - "The importance of cholesterol-rich VLDL in ASO and its relationship to familial dysbetalipoproteinemia are discussed." 12/31/1993 - "RLP-Cholesterol(C) levels in plasma have been determined in 363 male and female normolipidemic subjects (mean +/- S.D.: 72 +/- 16 mg/l) and have been found to be higher in patients with coronary heart disease and familial dysbetalipoproteinemia. " 03/15/2002 - "Apo var epsilon has been studied in disorders associated with elevated cholesterol levels or lipid derangements (i.e., hyperlipoproteinemia type III, coronary heart disease, strokes, peripheral artery disease, and diabetes mellitus). " 01/01/1984 - "This indicates that E2 homozygotes with hyperlipoproteinemia type III who occur rarely in the population but comprise about 1% of myocardial infarction patients have a markedly increased risk for coronary atherosclerosis, whereas the risk for E2 homozygotes with normal or subnormal plasma cholesterol (= primary dysbetalipoproteinemia) may be considerably lower than for the general population. " 05/01/2005 - "Screening for dysbetalipoproteinemia by plasma cholesterol and apolipoprotein B concentrations."
4.	Triglycerides (Triacylglycerol)IBA 10/08/1996 - "Gemfibrozil is effective in familial dysbetalipoproteinemia and is the drug of choice for patients with severely elevated serum triglycerides." 10/01/1996 - "We studied 23 patients with elevated plasma triglycerides to evaluate the influence of fish oil and fenofibrate therapy on hemorrheological parameters (15 patients with familial hypertriglyceridemia [FHTG] and eight with familial dysbetalipoproteinemia [FDL]). " 08/01/2008 - "Familial dysbetalipoproteinemia is an inherited disorder in which both cholesterol and triglycerides are elevated in the plasma of the blood, which pre-disposes people to coronary artery disease and peripheral vascular disease. " 04/01/1994 - "Type III hyperlipoproteinemia (dysbetalipoproteinemia) is characterized by elevated concentrations of plasma cholesterol and triglycerides due to an increase in very low density lipoprotein (VLDL) remnant lipoproteins. " 08/01/1989 - "Fenofibrate (300 mg daily) was given to 9 subjects (7 men, 2 women) with dysbetalipoproteinemia type III. The treatment brought about important plasma level reductions in cholesterol (-35%), triglycerides (-56%), VLDL-cholesterol (-63%) and VLDL-triglycerides (-59%). "
5.	Vitamin A (Retinol)FDA LinkGeneric 03/01/2000 - "Chylomicron processing in familial dysbetalipoproteinemia and familial combined hyperlipidemia studied with vitamin A and E as markers: a new physiological concept." 07/01/1976 - "Although additional experiments disclosed a similar delay in both vitamin A and triglyceride assimilation when basal triglyceride levels were high in these subjects, marked reduction of triglyceride levels did not correct the rise in the Sf greater than 400 vitamin A/triglyceride ratio between 6 and 24 hr. Experiments employing preparative electrophoresis confirmed the identity of VLDL containing a high vitamin A/triglyceride ratio with the beta-VLDL which accumulate in broad-beta disease..." 07/01/1976 - "Delayed clearance of chylomicron remnants following vitamin-A-containing oral fat loads in broad-beta disease (type III hyperlipoproteinemia)." 12/01/1998 - "Applied in a study in which 20 patients participated (eight patients with familial dysbetalipoproteinemia (FD), six patients with familial combined hyperlipidemia (FCH) and six controls) we found that vitamin A, no longer paralleled the apo B-48 concentrations from 9 h after a fat load, especially in the remnant fraction with Sf 15-100. " 07/01/1976 - "However, among those with broad-beta disease, an increasing vitamin A/triglyceride ratio between 6 and 24 hr was frequent within all VLDL subfractions and invariable among lipoproteins of Sf greater than 400 regardless of the degree of antecedent hypertriglyceridemia. "
6.	Chylomicron RemnantsIBA 07/01/1976 - "Delayed clearance of chylomicron remnants following vitamin-A-containing oral fat loads in broad-beta disease (type III hyperlipoproteinemia)." 05/01/2015 - "Familial dysbetalipoproteinemia (FD), also known as type III hyperlipoproteinemia, is a genetic dyslipidemia characterized by elevated very low density lipoprotein (VLDL) and chylomicron remnant particles that confers increased risk of cardiovascular disease (CVD). "
7.	Hydroxymethylglutaryl-CoA Reductase Inhibitors (HMG-CoA Reductase Inhibitors)IBA 02/01/1991 - "HMG-CoA reductase inhibitors have been proven effective in decreasing the plasma cholesterol levels in patients affected with various forms of hypercholesterolemia, familial dysbetalipoproteinemia, familial combined hyperlipidemia and in nephrotic and diabetic dyslipidemia. " 02/26/1998 - "Hepatic 3-hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) reductase inhibitors, or "statins," have been extremely efficacious in decreasing low-density lipoprotein (LDL) cholesterol levels in patients with hypercholesterolemia and in treating patients with dysbetalipoproteinemia, a relatively rare form of hyperlipidemia. "
8.	Gemfibrozil (Lopid)FDA LinkGeneric 10/08/1996 - "Gemfibrozil is effective in familial dysbetalipoproteinemia and is the drug of choice for patients with severely elevated serum triglycerides."
9.	Corn Oil (Oil, Corn)IBA 07/01/1976 - "To investigate a possible defect in the clearance of these remnants in the pathophysiology of broad-beta disease (type III hyperlipoproteinemia), subjects with this disorder and comparison subjects with endogenous hypertriglyceridemia (and type IV lipoprotein patterns) ingested an oral fat load (corn oil: cocoa butter, 1:1, 50 g/sq M) containing retinyl ester, 100 mg, with or without 15 muCi 15-(14) C-retinol (43.7 mCi/mg). "
10.	cocoa butterIBA 07/01/1976 - "To investigate a possible defect in the clearance of these remnants in the pathophysiology of broad-beta disease (type III hyperlipoproteinemia), subjects with this disorder and comparison subjects with endogenous hypertriglyceridemia (and type IV lipoprotein patterns) ingested an oral fat load (corn oil: cocoa butter, 1:1, 50 g/sq M) containing retinyl ester, 100 mg, with or without 15 muCi 15-(14) C-retinol (43.7 mCi/mg). "

Therapies and Procedures

1.	Liver Transplantation 02/01/1993 - "A case of type III hyperlipoproteinemia (HLP) (dysbetalipoproteinemia) acquired by liver transplantation is reported. "
2.	Diet Therapy (Therapy, Diet) 10/17/1989 - "Five of twelve members of the affected kindred are heterozygous for the mutant form of apoE, and four of the five have type III HLP, while the fifth member has dysbetalipoproteinemia on diet therapy. "