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MUCOPOLYSACCHARIDOSIS III
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DeCS
Descriptor
English
:
Mucopolysaccharidosis III
Descriptor
Spanish
:
Mucopolisacaridosis III
Descriptor
Portuguese
:
Mucopolissacaridose III
Synonyms
English
:
Polydystrophic Oligophrenia
Sanfilippo's Syndrome
Tree Number:
C16.320.565.202.715.650
C16.320.565.595.600.650
C17.300.550.575.650
C18.452.648.202.715.650
C18.452.648.595.600.650
Definition
English
:
Mucopolysaccharidosis characterized by
heparitin sulfate
in the urine, progressive mental retardation, mild
dwarfism
, and other skeletal disorders. There are four clinically indistinguishable but biochemically distinct forms, each due to a deficiency of a different enzyme.
History Note
English
:
92; was MUCOPOLYSACCHARIDOSIS 3 1991; was see under MUCOPOLYSACCHARIDOSIS 1975-90
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:
30389
Unique Identifier:
D009084
Occurrence in VHL
:
Similar:
DeCS
CID-10
SciELO
LILACS
LIS