Search on: PROGERIA 
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Descriptor English:   Progeria 
Descriptor Spanish:   Progeria 
Descriptor Portuguese:   Progeria 
Synonyms English:   Hutchinson-Gilford Syndrome  
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:  
blood cerebrospinal fluid
chemically induced classification
complications diet therapy
diagnosis drug therapy
economics ethnology
embryology enzymology
epidemiology etiology
genetics history
immunology metabolism
microbiology mortality
nursing pathology
prevention & control physiopathology
parasitology psychology
radiography rehabilitation
radionuclide imaging radiotherapy
surgery therapy
ultrastructure urine
ultrasonography veterinary
virology  
Record Number:   11804 
Unique Identifier:   D011371 

Occurrence in VHL:
 

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