| Wolfram syndrome. | |
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MedLine Citation:
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PMID: 18697639 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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V Viswanathan; S Medempudi; M Kadiri |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: The Journal of the Association of Physicians of India Volume: 56 ISSN: 0004-5772 ISO Abbreviation: J Assoc Physicians India Publication Date: 2008 Mar |
Date Detail:
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Created Date: 2008-08-13 Completed Date: 2008-10-10 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 7505585 Medline TA: J Assoc Physicians India Country: India |
Other Details:
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Languages: eng Pagination: 197-9 Citation Subset: IM |
Affiliation:
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M.V. Hospital for Diabetes and Diabetes Research Centre, Chennai. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Diabetes Mellitus, Type 1 / physiopathology Female Humans Mutation Optic Atrophy / physiopathology Risk Factors Wolfram Syndrome / diagnosis*, genetics, physiopathology |
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