Hyperlipoproteinemia Type III

**Definition:** 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.

**Tree numbers:** - C16.320.565.398.483 - C18.452.584.500.500.644.485 - C18.452.584.563.483 - C18.452.648.398.483

**Synonyms:** - Broad Beta Disease - Dysbetalipoproteinemia - Hyperlipoproteinemia, Broad-beta - Dysbetalipoproteinemia, Familial - Familial Hypercholesterolemia with Hyperlipemia - Familial Dysbetalipoproteinemia - Hyperlipoproteinemia, Type III