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DYSBETALIPOPROTEINEMIA, FAMILIAL
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DeCS
Descriptor
English
:
Hyperlipoproteinemia Type III
Descriptor
Spanish
:
Hiperlipoproteinemia Tipo III
Descriptor
Portuguese
:
Hiperlipoproteinemia Tipo III
Synonyms
English
:
Broad Beta Disease
Dysbetalipoproteinemia
Dysbetalipoproteinemia, Familial
Tree Number:
C16.320.565.398.483
C18.452.584.500.500.644.485
C18.452.648.398.483
Definition
English
:
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
English
:
80
Allowable Qualifiers
English
:
BL
blood
CF
cerebrospinal fluid
CI
chemically induced
CL
classification
CO
complications
DI
diagnosis
DH
diet therapy
DT
drug therapy
EC
economics
EM
embryology
EN
enzymology
EP
epidemiology
EH
ethnology
ET
etiology
GE
genetics
HI
history
IM
immunology
ME
metabolism
MI
microbiology
MO
mortality
NU
nursing
PS
parasitology
PA
pathology
PP
physiopathology
PC
prevention & control
PX
psychology
RA
radiography
RI
radionuclide imaging
RT
radiotherapy
RH
rehabilitation
SU
surgery
TH
therapy
US
ultrasonography
UR
urine
VE
veterinary
VI
virology
Record Number:
7133
Unique Identifier:
D006952
Occurrence in VHL
:
Similar:
DeCS
CID-10
SciELO
LILACS
LIS