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Descriptor English: Hyperlipoproteinemia Type I
Descriptor Spanish: Hiperlipoproteinemia Tipo I
Descriptor hiperlipoproteinemia tipo I
Entry term(s) deficiencia de apolipoproteína C-II
deficiencia familiar de lipoproteína lipasa
hiperquilomicronemia familiar
Scope note: Rara condición familiar que se caracteriza por quilomicronemia masiva y disminución de los niveles de otras lipoproteínas. Se debe a un déficit de lipoproteín lipasa, que es una hidrolasa alcalina de los triglicéridos que cataliza un importante paso en la eliminación extrahepática de las lipoproteínas ricas en triglicéridos de la sangre.
Descriptor Portuguese: Hiperlipoproteinemia Tipo I
Descriptor French: Hyperlipoprotéinémie de type I
Entry term(s): Anapolipoproteinemia, C-II
Anapolipoproteinemias, C-II
Apolipoprotein C II Deficiency
Apolipoprotein C-II Deficiencies
Apolipoprotein C-II Deficiency
Burger Grutz Syndrome
Burger-Grutz Syndrome
Burger-Grutz Syndromes
C-II Anapolipoproteinemia
C-II Anapolipoproteinemias
Chylomicronemia, Familial
Chylomicronemias, Familial
Deficiencies, Apolipoprotein C-II
Deficiencies, Familial LPL
Deficiencies, LIPD
Deficiencies, Lipase D
Deficiencies, Lipoprotein Lipase
Deficiency, Apolipoprotein C-II
Deficiency, Familial LPL
Deficiency, LIPD
Deficiency, Lipase D
Deficiency, Lipoprotein Lipase
Essential Familial Hyperlipemia
Essential Familial Hyperlipemias
Familial Chylomicronemia
Familial Chylomicronemias
Familial Fat Induced Hypertriglyceridemia
Familial Fat-Induced Hypertriglyceridemia
Familial Fat-Induced Hypertriglyceridemias
Familial Hyperchylomicronemia
Familial Hyperchylomicronemias
Familial Hyperlipemia, Essential
Familial Hyperlipemias, Essential
Familial Hyperlipoproteinemia Type 1
Familial LPL Deficiencies
Familial LPL Deficiency
Familial Lipoprotein Lipase Deficiency
Fat-Induced Hypertriglyceridemia, Familial
Fat-Induced Hypertriglyceridemias, Familial
Hyperchylomicronemia, Familial
Hyperchylomicronemias, Familial
Hyperlipemia, Essential Familial
Hyperlipemia, Idiopathic, Burger-Grutz Type
Hyperlipemias, Essential Familial
Hyperlipoproteinemia Type Ia
Hyperlipoproteinemia Type Ias
Hyperlipoproteinemia Type Ib
Hyperlipoproteinemia Type Ibs
Hyperlipoproteinemia Type Is
Hyperlipoproteinemia, Type I
Hyperlipoproteinemia, Type Ia
Hyperlipoproteinemia, Type Ib
Hyperlipoproteinemias, Type I
Hyperlipoproteinemias, Type Ia
Hyperlipoproteinemias, Type Ib
Hypertriglyceridemia, Familial Fat-Induced
Hypertriglyceridemias, Familial Fat-Induced
LIPD Deficiencies
LIPD Deficiency
LPL Deficiencies, Familial
LPL Deficiency, Familial
Lipase D Deficiencies
Lipase D Deficiency
Lipase Deficiencies, Lipoprotein
Lipoprotein Lipase Deficiencies
Lipoprotein Lipase Deficiency
Lipoprotein Lipase Deficiency, Familial
Syndrome, Burger-Grutz
Syndromes, Burger-Grutz
Type I Hyperlipoproteinemia
Type I Hyperlipoproteinemias
Type Ia Hyperlipoproteinemia
Type Ia Hyperlipoproteinemias
Type Ib Hyperlipoproteinemia
Type Ib Hyperlipoproteinemias
Tree number(s): C16.320.565.398.465
C18.452.584.500.500.644.237
C18.452.584.563.465
C18.452.648.398.465
RDF Unique Identifier: https://id.nlm.nih.gov/mesh/D008072
Scope note: 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.
Allowable Qualifiers: BL blood
CF cerebrospinal fluid
CI chemically induced
CL classification
CO complications
DG diagnostic imaging
DH diet therapy
DI diagnosis
DT drug therapy
EC economics
EH ethnology
EM embryology
EN enzymology
EP epidemiology
ET etiology
GE genetics
HI history
IM immunology
ME metabolism
MI microbiology
MO mortality
NU nursing
PA pathology
PC prevention & control
PP physiopathology
PS parasitology
PX psychology
RH rehabilitation
RT radiotherapy
SU surgery
TH therapy
UR urine
VE veterinary
VI virology
Previous Indexing: Hyperlipidemia/familial & genetic (1966-1979)
Public MeSH Note: 2007; see LIPOPROTEIN LIPASE DEFICIENCY, FAMILIAL 1991-2006, see HYPERLIPOPROTEINEMIA 1980-1990
History Note: 2007 (1980)
Related: Apolipoprotein C-II MeSH
Lipoprotein Lipase MeSH
DeCS ID: 8246
Unique ID: D008072
Documents indexed in the Virtual Health Library (VHL): Click here to access the VHL documents
Date Established: 1991/01/01
Date of Entry: 1979/04/23
Revision Date: 2013/07/08
Hyperlipoproteinemia Type I - Preferred
Concept UI M0012579
Scope note 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.
Preferred term Hyperlipoproteinemia Type I
Entry term(s) Burger Grutz Syndrome
Burger-Grutz Syndrome
Burger-Grutz Syndromes
Chylomicronemia, Familial
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 Hyperchylomicronemia
Familial Hyperchylomicronemias
Familial Hyperlipemia, Essential
Familial Hyperlipemias, Essential
Familial Hyperlipoproteinemia Type 1
Familial LPL Deficiencies
Familial LPL Deficiency
Familial Lipoprotein Lipase Deficiency
Hyperchylomicronemia, Familial
Hyperchylomicronemias, Familial
Hyperlipemia, Essential Familial
Hyperlipemia, Idiopathic, Burger-Grutz Type
Hyperlipemias, Essential Familial
Hyperlipoproteinemia Type Ia
Hyperlipoproteinemia Type Ias
Hyperlipoproteinemia Type Is
Hyperlipoproteinemia, Type I
Hyperlipoproteinemia, Type Ia
Hyperlipoproteinemias, Type I
Hyperlipoproteinemias, Type Ia
LIPD Deficiencies
LIPD Deficiency
LPL Deficiencies, Familial
LPL Deficiency, Familial
Lipase D Deficiencies
Lipase D Deficiency
Lipase Deficiencies, Lipoprotein
Lipoprotein Lipase Deficiencies
Lipoprotein Lipase Deficiency
Lipoprotein Lipase Deficiency, Familial
Syndrome, Burger-Grutz
Syndromes, Burger-Grutz
Type I Hyperlipoproteinemia
Type I Hyperlipoproteinemias
Type Ia Hyperlipoproteinemia
Type Ia Hyperlipoproteinemias
Apolipoprotein C-II Deficiency - Narrower
Concept UI M0486996
Preferred term Apolipoprotein C-II Deficiency
Entry term(s) Anapolipoproteinemia, C-II
Anapolipoproteinemias, C-II
Apolipoprotein C II Deficiency
Apolipoprotein C-II Deficiencies
C-II Anapolipoproteinemia
C-II Anapolipoproteinemias
Deficiencies, Apolipoprotein C-II
Deficiency, Apolipoprotein C-II
Familial Fat Induced Hypertriglyceridemia
Familial Fat-Induced Hypertriglyceridemia
Familial Fat-Induced Hypertriglyceridemias
Fat-Induced Hypertriglyceridemia, Familial
Fat-Induced Hypertriglyceridemias, Familial
Hyperlipoproteinemia Type Ib
Hyperlipoproteinemia Type Ibs
Hyperlipoproteinemia, Type Ib
Hyperlipoproteinemias, Type Ib
Hypertriglyceridemia, Familial Fat-Induced
Hypertriglyceridemias, Familial Fat-Induced
Type Ib Hyperlipoproteinemia
Type Ib Hyperlipoproteinemias



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