Structured Summary
Abstract
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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Classification
Broader headings
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Synonyms
16 entry terms
- Broad Beta Disease
- Dysbetalipoproteinemia
- Dysbetalipoproteinemia, Familial
- Familial Dysbetalipoproteinemia
- Familial Hypercholesterolemia with Hyperlipemia
- Hyperlipoproteinemia, Broad-beta
- Hyperlipoproteinemia, Type III
- Broad-beta Hyperlipoproteinemia
- Hyperlipoproteinemia, Broad beta
- Hyperlipoproteinemias, Type III
- Type III Hyperlipoproteinemia
- Type III Hyperlipoproteinemias
- Autosomal Recessive Hypercholesterolemia
- Hypercholesterolemia, Autosomal Recessive
- Autosomal Recessive Hypercholesterolemias
- Recessive Hypercholesterolemia, Autosomal
MeSH Record
Aspects Covered
34 allowable subheadings
Indexed with the subheadings blood, cerebrospinal fluid, chemically induced, classification, complications, diagnosis, diagnostic imaging, diet therapy, drug therapy, economics, embryology, enzymology, epidemiology, ethnology, etiology, genetics, history, immunology, metabolism, microbiology, mortality, nursing, parasitology, pathology, physiopathology, prevention & control, psychology, radiotherapy, rehabilitation, surgery, therapy, urine, veterinary, virology.
MeSH Record
History Note
1980; for AUTOSOMAL RECESSIVE HYPERCHOLESTEROLEMIA use HOMOZYGOUS FAMILIAL HYPERCHOLESTEROLEMIA 2022
MeSH Record
Previous Indexing
- Hyperlipidemia/familial & genetic (1966-1979)
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AMA Style
References
- National Library of Medicine. Hyperlipoproteinemia Type III. Medical Subject Headings (MeSH). 2026. Unique ID D006952. http://id.nlm.nih.gov/mesh/2026/D006952
- Hyperlipoproteinemia Type III. In: Wikipedia. https://en.wikipedia.org/wiki/Familial_dysbetalipoproteinemia
- Hyperlipoproteinemia Type III. In: Wikidata. https://www.wikidata.org/wiki/Q2069207