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Descriptor English: Hyperlipoproteinemia Type III
Descriptor Spanish: Hiperlipoproteinemia Tipo III
Descriptor hiperlipoproteinemia tipo III
Entry term(s) disbetalipoproteinemia
disbetalipoproteinemia familiar
enfermedad de beta-ancha
hipercolesterolemia autosómica recesiva
Scope note: Trastorno autosómico recesivo caracterizado por acumulación de lipoproteínas de densidad intermedia (IDL o lipoproteínas con banda beta ancha).Las IDL presentan una relación TRIGLICÉRIDOS a COLESTEROL superior a la de las LIPOPROTEÍNAS DE MUY BAJA DENSIDAD. Este trastorno es debido a una mutación de las APOLIPOPROTEINAS E, un componente de unión al receptor de las VLDL y los QUILOMICRONES, que resulta en disminución del aclaramiento y elevadas concentraciones plasmáticas de colesterol y triglicéridos, respecto de los triglicéridos que la VLDL normal.
Descriptor Portuguese: Hiperlipoproteinemia Tipo III
Descriptor French: Hyperlipoprotéinémie de type III
Entry term(s): Autosomal Recessive Hypercholesterolemia
Autosomal Recessive Hypercholesterolemias
Broad Beta Disease
Broad-beta Hyperlipoproteinemia
Dysbetalipoproteinemia
Dysbetalipoproteinemia, Familial
Familial Dysbetalipoproteinemia
Familial Hypercholesterolemia with Hyperlipemia
Hyperlipoproteinemia, Broad beta
Hyperlipoproteinemia, Broad-beta
Hyperlipoproteinemia, Type III
Hyperlipoproteinemias, Type III
Recessive Hypercholesterolemia, Autosomal
Type III Hyperlipoproteinemia
Type III Hyperlipoproteinemias
Tree number(s): C16.320.565.398.483
C18.452.584.500.500.644.485
C18.452.584.563.483
C18.452.648.398.483
RDF Unique Identifier: https://id.nlm.nih.gov/mesh/D006952
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.
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: 80
History Note: 80
DeCS ID: 7133
Unique ID: D006952
Documents indexed in the Virtual Health Library (VHL): Click here to access the VHL documents
Date Established: 1980/01/01
Date of Entry: 1979/04/23
Revision Date: 2022/04/13
Hyperlipoproteinemia Type III - Preferred
Concept UI M0010834
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.
Preferred term Hyperlipoproteinemia Type III
Entry term(s) Broad Beta Disease
Broad-beta Hyperlipoproteinemia
Dysbetalipoproteinemia
Dysbetalipoproteinemia, Familial
Familial Dysbetalipoproteinemia
Familial Hypercholesterolemia with Hyperlipemia
Hyperlipoproteinemia, Broad beta
Hyperlipoproteinemia, Broad-beta
Hyperlipoproteinemia, Type III
Hyperlipoproteinemias, Type III
Type III Hyperlipoproteinemia
Type III Hyperlipoproteinemias
Autosomal Recessive Hypercholesterolemia - Related but not broader or narrower
Concept UI M000749425
Preferred term Autosomal Recessive Hypercholesterolemia
Entry term(s) Autosomal Recessive Hypercholesterolemias
Recessive Hypercholesterolemia, Autosomal



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