En ganska ovanlig form av ärftlig hyperlipemi, kännetecknad av förekomst av lipoproteiner med onormal sammansättning. De viktigaste onormala lipoproteinerna kallas beta-VLDL och har ett annat apoproteininnehåll och större andel kolesterol i förhållande till triglycerid än normalt VLDL.
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.
Dysbetalipoproteinemi — Bred beta-sjukdom
Broad Beta Disease — Dysbetalipoproteinemia — Hyperlipoproteinemia, Broad-beta — Broad-beta Hyperlipoproteinemia — Hyperlipoproteinemia, Broad beta — Dysbetalipoproteinemia, Familial — Familial Hypercholesterolemia with Hyperlipemia — Familial Dysbetalipoproteinemia — Hyperlipoproteinemia, Type III — Hyperlipoproteinemias, Type III — Type III Hyperlipoproteinemia — Type III Hyperlipoproteinemias — Autosomal Recessive Hypercholesterolemia — Autosomal Recessive Hypercholesterolemias — Recessive Hypercholesterolemia, Autosomal — Hypercholesterolemia, Autosomal Recessive