Diseases

Hyperlipoproteinemia Type I

An inherited condition due to a deficiency of either LIPOPROTEIN LIPASE or APOLIPOPROTEIN C-II (a lipase-activating protein). The lack of lipase activities results in inability to remove CHYLOMICRONS and TRIGLYCERIDES from the blood which has a creamy top layer after standing.

National Library of MedicineMedical Subject Headings2026

Structured Summary

Abstract

An inherited condition due to a deficiency of either LIPOPROTEIN LIPASE or APOLIPOPROTEIN C-II (a lipase-activating protein). The lack of lipase activities results in inability to remove CHYLOMICRONS and TRIGLYCERIDES from the blood which has a creamy top layer after standing.

MeSH Record

Classification

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MeSH Record

See Also

MeSH Record

Synonyms

78 entry terms
  • Burger-Grutz Syndrome
  • Chylomicronemia, Familial
  • Familial Chylomicronemia Syndrom
  • Familial Hyperchylomicronemia
  • Familial Hyperchylomicronemia Syndrome
  • Familial Hyperlipoproteinemia Type 1
  • Familial LPL Deficiency
  • Familial Lipoprotein Lipase Deficiency
  • Hyperchylomicronemia, Familial
  • Hyperlipemia, Essential Familial
  • Hyperlipemia, Idiopathic, Burger-Grutz Type
  • Hyperlipoproteinemia Type Ia
  • Hyperlipoproteinemia, Type 1
  • Hyperlipoproteinemia, Type I
  • Hyperlipoproteinemia, Type Ia
  • LIPD Deficiency
  • Lipase D Deficiency
  • Lipoprotein Lipase Deficiency
  • Lipoprotein Lipase Deficiency, Familial
  • Burger Grutz Syndrome
  • Burger-Grutz Syndromes
  • Chylomicronemias, Familial
  • Deficiencies, Familial LPL
  • Deficiencies, LIPD
  • Deficiencies, Lipase D
  • Deficiencies, Lipoprotein Lipase
  • Deficiency, Familial LPL
  • Deficiency, LIPD
  • Deficiency, Lipase D
  • Deficiency, Lipoprotein Lipase
  • Essential Familial Hyperlipemia
  • Essential Familial Hyperlipemias
  • Familial Chylomicronemia
  • Familial Chylomicronemias
  • Familial Hyperchylomicronemias
  • Familial Hyperlipemia, Essential
  • Familial Hyperlipemias, Essential
  • Familial LPL Deficiencies
  • Hyperchylomicronemias, Familial
  • Hyperlipemias, Essential Familial
  • Hyperlipoproteinemia Type Ias
  • Hyperlipoproteinemia Type Is
  • Hyperlipoproteinemias, Type I
  • Hyperlipoproteinemias, Type Ia
  • LIPD Deficiencies
  • LPL Deficiencies, Familial
  • LPL Deficiency, Familial
  • Lipase D Deficiencies
  • Lipase Deficiencies, Lipoprotein
  • Lipoprotein Lipase Deficiencies
  • Syndrome, Burger-Grutz
  • Syndromes, Burger-Grutz
  • Type I Hyperlipoproteinemia
  • Type I Hyperlipoproteinemias
  • Type Ia Hyperlipoproteinemia
  • Type Ia Hyperlipoproteinemias
  • Apolipoprotein C-II Deficiency
  • C-II Anapolipoproteinemia
  • Familial Fat-Induced Hypertriglyceridemia
  • Hyperlipoproteinemia Type Ib
  • Hyperlipoproteinemia, Type Ib
  • Anapolipoproteinemia, C-II
  • Anapolipoproteinemias, C-II
  • Apolipoprotein C II Deficiency
  • Apolipoprotein C-II Deficiencies
  • C-II Anapolipoproteinemias
  • Deficiencies, Apolipoprotein C-II
  • Deficiency, Apolipoprotein C-II
  • Familial Fat Induced Hypertriglyceridemia
  • Familial Fat-Induced Hypertriglyceridemias
  • Fat-Induced Hypertriglyceridemia, Familial
  • Fat-Induced Hypertriglyceridemias, Familial
  • Hyperlipoproteinemia Type Ibs
  • Hyperlipoproteinemias, Type Ib
  • Hypertriglyceridemia, Familial Fat-Induced
  • Hypertriglyceridemias, Familial Fat-Induced
  • Type Ib Hyperlipoproteinemia
  • Type Ib Hyperlipoproteinemias

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

2007 (1980)

MeSH Record

Previous Indexing

  • Hyperlipidemia/familial & genetic (1966-1979)

MeSH Hierarchy

Tree Numbers

AMA Style

References

  1. National Library of Medicine. Hyperlipoproteinemia Type I. Medical Subject Headings (MeSH). 2026. Unique ID D008072. http://id.nlm.nih.gov/mesh/2026/D008072
  2. Hyperlipoproteinemia Type I. In: Wikidata. https://www.wikidata.org/wiki/Q68640538