Indication

Primary dysbetalipoproteinemia

Primary dysbetalipoproteinemia, also known as type III hyperlipoproteinemia or familial dysbetalipoproteinemia, is a rare inherited metabolic disorder of lipid clearance affecting the cardiovascular and integumentary systems. Read more about Primary dysbetalipoproteinemia

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Drugs that treat it

Common Primary dysbetalipoproteinemia therapies.

Showing 1–9 of 164 drugs

SIMVASTATIN

ANTIHYPERLIPIDEMICS HMGCO-A REDUCTASE INHIBITORS (STATINS)·HYPERCHOLESTEROLEMIA·+13
MCKESSON PACKAG

SIMVASTATIN

ANTIHYPERLIPIDEMICS HMGCO-A REDUCTASE INHIBITORS (STATINS)·HYPERCHOLESTEROLEMIA·+13
NORTHSTAR RX LL

SIMVASTATIN

ANTIHYPERLIPIDEMICS HMGCO-A REDUCTASE INHIBITORS (STATINS)·HYPERCHOLESTEROLEMIA·+13
AUROBINDO PHARM

PRAVASTATIN SODIUM

ANTIHYPERLIPIDEMICS HMGCO-A REDUCTASE INHIBITORS (STATINS)·HYPERCHOLESTEROLEMIA·+11
DR.REDDY'S LAB
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About Primary dysbetalipoproteinemia.

Primary dysbetalipoproteinemia, also known as type III hyperlipoproteinemia or familial dysbetalipoproteinemia, is a rare inherited metabolic disorder of lipid clearance affecting the cardiovascular and integumentary systems.

It is primarily caused by homozygosity for the apolipoprotein E2 allele or other mutations in the *APOE* gene, which disrupt the binding of apolipoprotein E to hepatic receptors and impair the clearance of chylomicron and very-low-density lipoprotein remnants.

This defect results in mixed hyperlipidemia with roughly equimolar elevations in plasma cholesterol and triglycerides due to the accumulation of atherogenic remnant lipoproteins (beta-VLDL).

Clinically, affected individuals are at substantial risk for premature coronary and peripheral vascular disease and may develop characteristic pathognomonic physical signs such as palmar xanthomas (xanthoma striata palmaris) and tuberoeruptive xanthomas.

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