| Morbidity and mortality in the Wolfram syndrome. | |
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MedLine Citation:
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PMID: 8722052 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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OBJECTIVE: To determine the major causes of morbidity and mortality in the autosomal recessive Wolfram syndrome, which is defined by diabetes and bilateral progressive optic atrophy with onset in childhood or adolescence. RESEARCH DESIGN AND METHODS: We abstracted and reviewed the medical records of 68 confirmed cases of Wolfram syndrome identified through a nationwide survey of endocrinologists, ophthalmologists, institutes, and homes for the blind. We also reviewed all available autopsy records. RESULTS: The most common causes of morbidity and mortality were the neurological manifestations of this syndrome and the complications of urinary tract atony. There was a lower frequency of diabetic ketoacidosis, no histologically proven diabetic glomerulosclerosis, and less severe, more slowly progressive, diabetic retinopathy than in classic type I diabetic patients. Mortality in Wolfram syndrome is much higher than in type I diabetes; 60% of Wolfram syndrome patients die by age 35. Recognition of these clinical differences from classic type I diabetes is important for the proper management of Wolfram syndrome patients. CONCLUSIONS: Identification of Wolfram syndrome patients among all diabetic patients presenting in childhood or adolescence is important because the management of patients with this syndrome is different from that of patients with classic type I diabetes. |
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Authors:
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B T Kinsley; M Swift; R H Dumont; R G Swift |
Publication Detail:
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Type: Journal Article; Multicenter Study; Research Support, U.S. Gov't, P.H.S. |
Journal Detail:
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Title: Diabetes care Volume: 18 ISSN: 0149-5992 ISO Abbreviation: Diabetes Care Publication Date: 1995 Dec |
Date Detail:
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Created Date: 1996-10-11 Completed Date: 1996-10-11 Revised Date: 2007-11-15 |
Medline Journal Info:
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Nlm Unique ID: 7805975 Medline TA: Diabetes Care Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 1566-70 Citation Subset: IM |
Affiliation:
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Joslin Diabetes Center, New England Deaconess Hospital, and Harvard Medical School, Boston, Massachusetts, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Adult Age Factors Child Congenital Abnormalities / epidemiology Diabetes Mellitus, Type 1 / epidemiology*, mortality Diabetic Ketoacidosis / epidemiology, physiopathology Diagnosis, Differential Humans Life Tables Medical Records Morbidity Retrospective Studies United States / epidemiology Wolfram Syndrome / epidemiology*, mortality, physiopathology |
| Grant Support | |
ID/Acronym/Agency:
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MH-45128/MH/NIMH NIH HHS |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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