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Nutritional and Metabolic Diseases > Metabolic Diseases > Lipid Metabolism Disorders > Lipid Metabolism, Inborn Errors > Hyperlipoproteinemia Type III
Nutritional and Metabolic Diseases > Metabolic Diseases > Metabolism, Inborn Errors > Lipid Metabolism, Inborn Errors > Hyperlipoproteinemia Type III
Congenital, Hereditary, and Neonatal Diseases and Abnormalities > Genetic Diseases, Inborn > Metabolism, Inborn Errors > Lipid Metabolism, Inborn Errors > Hyperlipoproteinemia Type III
... > Nutritional and Metabolic Diseases > Metabolic Diseases > Lipid Metabolism Disorders > Dyslipidemias > Hyperlipidemias > Hyperlipoproteinemias > Hyperlipoproteinemia Type III

Preferred term

Hyperlipoproteinemia Type III  

Type

  • Topical Descriptor

Broader concept

Entry terms

  • Broad Beta Disease
  • Broad-beta Hyperlipoproteinemia
  • Dysbetalipoproteinemia
  • Dysbetalipoproteinemia, Familial
  • Familial Dysbetalipoproteinemia
  • Familial Hypercholesterolemia with Hyperlipemia
  • Hyperlipoproteinemia, Broad beta
  • Hyperlipoproteinemia, Broad-beta
  • Hyperlipoproteinemias, Type III
  • Hyperlipoproteinemia, Type III
  • Type III Hyperlipoproteinemia
  • Type III Hyperlipoproteinemias

Scope note

  • 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.

History note

  • 1980; for AUTOSOMAL RECESSIVE HYPERCHOLESTEROLEMIA use HOMOZYGOUS FAMILIAL HYPERCHOLESTEROLEMIA 2022

In other languages

  • tyypin III hyperlipoproteinemia

    Finnish

  • dysbeetalipoproteinemia
  • Fredrickson-typ III-hyperlipoproteinemi
  • hyperlipoproteinaemia, typus III
  • hyperlipoproteinemia, tyyppi III
  • remnant hyperlipidemi
  • remnantti hyperlipidemia
  • Hyperlipoproteinemi typ III

    Swedish

  • Bred beta-sjukdom
  • Dysbetalipoproteinemi

URI

http://www.yso.fi/onto/mesh/D006952

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