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MUCOPOLYSACCHARIDOSIS IV
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DeCS
Descriptor
English
:
Mucopolysaccharidosis IV
Descriptor
Spanish
:
Mucopolisacaridosis IV
Descriptor
Portuguese
:
Mucopolissacaridose IV
Synonyms
English
:
Eccentro-Osteochondrodysplasia
Morquio's Disease
Tree Number:
C16.320.565.202.715.655
C16.320.565.595.600.655
C17.300.550.575.655
C18.452.648.202.715.655
C18.452.648.595.600.655
Definition
English
:
Genetic disorder of mucopolysaccharide
metabolism
characterized by skeletal abnormalities,
joint instability
, development of cervical
myelopathy
, and excessive urinary
keratan sulfate
. There are two biochemically distinct forms, each due to a deficiency of a different enzyme.
History Note
English
:
1992; use MUCOPOLYSACCHARIDOSIS 4 1980-1991, use ECCENTROOSTEOCHONDRODYSPLASIA 1963-1979; for MORQUIO'S DISEASE use MUCOPOLYSACCHARIDOSIS 4 1980-1991
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:
30390
Unique Identifier:
D009085
Occurrence in VHL
:
Similar:
DeCS
CID-10
SciELO
LILACS
LIS