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PROGERIA
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DeCS
Descriptor
English
:
Progeria
Descriptor
Spanish
:
Progeria
Descriptor
Portuguese
:
Progéria
Synonyms
English
:
Hutchinson Gilford Progeria Syndrome
Hutchinson Gilford Syndrome
Hutchinson-Gilford Progeria Syndrome
Hutchinson-Gilford Progeria Syndromes
Hutchinson-Gilford Syndrome
Progeria Syndrome, Hutchinson-Gilford
Progeria Syndromes, Hutchinson-Gilford
Tree Number:
C16.320.565.753
C18.452.648.753
Definition
English
:
An abnormal congenital condition, associated with defects in the
LAMIN TYPE A
gene, which is characterized by
premature aging
in
children
, where all the changes of cell senescence occur. It is manifested by premature greying; hair loss;
hearing loss
(
DEAFNESS
); cataracts (
CATARACT
);
ARTHRITIS
;
OSTEOPOROSIS
;
DIABETES MELLITUS
;
atrophy
of
subcutaneous fat
; skeletal hypoplasia; elevated urinary
HYALURONIC ACID
; and accelerated
ATHEROSCLEROSIS
. Many affected individuals develop malignant tumors, especially
SARCOMA.
See Related
English
:
Cockayne Syndrome
Werner Syndrome
Allowable Qualifiers
English
:
BL
blood
CF
cerebrospinal fluid
CI
chemically induced
CL
classification
CO
complications
DI
diagnosis
DG
diagnostic imaging
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
RT
radiotherapy
RH
rehabilitation
SU
surgery
TH
therapy
UR
urine
VE
veterinary
VI
virology
Record Number:
11804
Unique Identifier:
D011371
Occurrence in VHL
:
Similar:
DeCS