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GARGOYLISM, HUNTER SYNDROME
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DeCS
Descriptor
English
:
Mucopolysaccharidosis II
Descriptor
Spanish
:
Mucopolisacaridosis II
Descriptor
Portuguese
:
Mucopolissacaridose II
Synonyms
English
:
Gargoylism, Hunter Syndrome
Hunter's Syndrome
Iduronate 2-Sulfatase Deficiency
Iduronate Sulfatase Deficiency
Sulfoiduronate Sulfatase Deficiency
Tree Number:
C10.597.606.643.455.750
C16.320.322.500.750
C16.320.400.525.750
C16.320.565.202.715.645
C16.320.565.595.600.645
C17.300.550.575.645
C18.452.648.202.715.645
C18.452.648.595.600.645
Definition
English
:
Systemic lysosomal storage
disease
marked by progressive physical deterioration and caused by a deficiency of L-sulfoiduronate sulfatase. This
disease
differs from
MUCOPOLYSACCHARIDOSIS I
by slower progression, lack of corneal clouding, and X-linked rather than autosomal recessive inheritance. The mild form produces near-normal
intelligence
and life span. The severe form usually causes death by age 15.
Indexing Annotation
English
:
DF: MPS II
See Related
English
:
Iduronate Sulfatase
Mucopolysaccharidosis I
History Note
English
:
1992; for MUCOPOLYSACCHARIDOSIS 2 and HUNTER'S SYNDROME use LIPOCHONDRODYSTROPHY 1976-1991
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:
29956
Unique Identifier:
D016532
Occurrence in VHL
:
Similar:
DeCS
CID-10
SciELO
LILACS
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