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Diabetes Care, Vol 9, Issue 5 521-528, Copyright © 1986 by American Diabetes Association


ARTICLES

Wolfram's syndrome: a clinical, diagnostic, and interpretative contribution

C Blasi, F Pierelli, E Rispoli, M Saponara, E Vingolo and D Andreani

Four cases (belonging to two different families) of Wolfram's syndrome (WS), a rare congenital disease characterized in its complete form by insulin-dependent diabetes mellitus, optic atrophy, diabetes insipidus, deafness, and dilation of the urinary tracts are presented, and a review of the literature is included. Three of four patients are characterized by HLA-DR2 haplotype, which is rare in IDDM. The neurodegenerative nature of some symptoms and the possible pathogenesis of diabetes mellitus connected with it are discussed.
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Copyright © 1986 by the American Diabetes Association.