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

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

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish
Pages (from-to)521-528
Number of pages8
JournalDiabetes Care
Issue number5
Publication statusPublished - 1986

ASJC Scopus subject areas

  • Internal Medicine
  • Endocrinology, Diabetes and Metabolism


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