A Case of Wolfram Syndrome
Journal Title: Turkish Journal of Endocrinology and Metabolism - Year 2002, Vol 6, Issue 3
Abstract
Wolfram syndrome (WS) is the inherited association of juvenile-onset insulin-dependent diabetes mellitus and progressive bilateral optic atrophy. We have identified a complete DIDMOAD syndrome. Bilateral papillary atrophy was found in optic fundoscopic examination. The diagnosis of diabetes insipidus was determined after a water deprivation test. Fasting serum insulin levels was 0.4uU/ml and serum C-peptide level was 0.2 ng/ml. Antibodies to islet cell (ICA) and anti-insulin antibodies were negative. In MRI of the patient the posterior pituitary bright spot was absent. Deafness was found in odiometric examination. The disease is believed to account for 1/150 patients with young-onset insulin-requiring diabetes mellitus. Interestingly we follow- up almost 450 patients with type 1 diabetes mellitus per year, but we determined a Wolfram syndrome at first time. In the literature WS were found 27 patients in 589 type 1 diabetic patients. Whereas in UK population syndrome is rare and its prevalence is estimated to be 1/ 770 000 people. As a result we can say that frequency of syndrome may show ethnic differences and may be rare in our region.
Authors and Affiliations
Mithat Bahçeci, Alpaslan Tuzcu, Kadim Bayan, Metin Çelik
Keywords
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