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HISTIOCYTOSIS, SEA-BLUE
Descriptors Found:
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DeCS
Descriptor
English
:
Sea-Blue Histiocyte Syndrome
Descriptor
Spanish
:
Síndrome del Histiocito Azul-Marino
Descriptor
Portuguese
:
Síndrome do Histiócito Azul-Marinho
Synonyms
English
:
Histiocytosis, Sea-Blue
Tree Number:
C10.228.140.163.100.435.825.775
C15.604.250.410.800
C16.320.565.189.435.825.775
C16.320.565.398.641.803.850
C16.320.565.595.554.825.775
C18.452.132.100.435.825.775
C18.452.584.687.803.850
C18.452.648.189.435.825.775
C18.452.648.398.641.803.850
C18.452.648.595.554.825.775
Definition
English
:
A congenital
disease
caused by an inborn error involving
APOLIPOPROTEINS E
leading to abnormal
LIPID METABOLISM
and the accumulation of
GLYCOSPHINGOLIPIDS
, particularly
SPHINGOMYELINS
in the
HISTIOCYTES
. This disorder is characterized by
SPLENOMEGALY
and the sea-blue
histiocytes
in the
spleen
and
bone marrow
after May Grunwald staining.
See Related
English
:
Niemann-Pick Diseases
History Note
English
:
1984
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:
27828
Unique Identifier:
D012618
Occurrence in VHL
:
Similar:
DeCS
CID-10
SciELO
LILACS
LIS