Wolfram syndrome.
Article
in English
| IMSEAR
| ID: sea-94925
ABSTRACT
Wolfram syndrome is a rare neurodegenerative and genetic disorder, which should be suspected in patients with young onset non-immune insulin dependent diabetes mellitus and optic atrophy. Patients are most likely to develop diabetes insipidus, deafness, urinary tract, and neurological abnormalities. 60% of the people with Wolfram syndrome die at age 35, usually due to central respiratory center failure following brain stem atrophy. Though there is no treatment to reverse the underlying mechanism of neurodegeneration, early diagnosis and adequate hormonal replacement could improve quality of life and survival.
Full text:
Available
Index:
IMSEAR (South-East Asia)
Main subject:
Wolfram Syndrome
/
Female
/
Humans
/
Optic Atrophy
/
Risk Factors
/
Adolescent
/
Diabetes Mellitus, Type 1
/
Mutation
Type of study:
Etiology study
/
Risk factors
/
Screening study
Language:
English
Year:
2008
Type:
Article
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