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Hyperlipoproteinemia Type III (Broad Beta Disease)

136  relevant articles (3 outcomes, 14 trials/studies) found for this Disease

Description: 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 APOLIPOPROTEIN E, a receptor-binding component of VLDL and CHYLOMICRONS, resulting in their reduced clearance and high plasma levels of both cholesterol and triglycerides.

Also Known As:
Broad Beta Disease; Familial Dysbetalipoproteinemia; Familial Hypercholesterolemia with Hyperlipemia; Hyperlipoproteinemia, Broad-beta; Broad-beta Hyperlipoproteinemia; Hyperlipoproteinemia, Broad beta; Dysbetalipoproteinemia; Dysbetalipoproteinemia, Familial

Relationship Network

Disease Context: Research Results

Related Diseases

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

Experts

1. Marais, A David: 2 articles (05/2005 - 01/2003)
2. Blom, Dirk J: 2 articles (05/2005 - 01/2003)
3. Smelt, A H M: 2 articles (08/2004 - 01/2003)
4. Bonet, Rosa: 1 article (10/2006)
5. Blanco-Vaca, Francisco: 1 article (10/2006)
6. Ordóñez-Llanos, Jordi: 1 article (10/2006)
7. Martín-Campos, Jesús M: 1 article (10/2006)
8. Rico, Naira: 1 article (10/2006)
9. Mayoral, Carme: 1 article (10/2006)
10. Lamarche, Benoît: 1 article (09/2006)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Hyperlipoproteinemia Type III:
1. Lipoproteins (Lipoprotein)IBA
2. CholesterolIBA
3. Triglycerides (Triacylglycerol)IBA
4. Vitamin A (Retinol)FDA Link
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"
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5. Hydroxymethylglutaryl-CoA Reductase Inhibitors (HMG-CoA Reductase Inhibitors)IBA
6. Gemfibrozil (Lopid)FDA LinkGeneric
7. Chylomicron RemnantsIBA
8. Corn Oil (Oil, Corn)IBA
9. cocoa butterIBA
10. ApolipoproteinsIBA

Therapies and Procedures

1. Liver Transplantation
2. Diet Therapy (Therapy, Diet)

Best Treatments:
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