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Licensed Unlicensed Requires Authentication Published by De Gruyter March 16, 2011

The subtle signs of Wolfram (DIDMOAD) syndrome: not all juvenile diabetes is type 1 diabetes

  • Claudia Boettcher EMAIL logo , Burkhard Brosig , Klaus P. Zimmer and Stefan A. Wudy
From the journal


Wolfram syndrome (also known as DIDMOAD=diabetes insipidus, diabetes mellitus, optic atrophy, deafness) is an autosomal recessive disorder characterized by the association of childhood non-immune insulin-dependent diabetes mellitus (DM) with progressive bilateral optic atrophy. Additional symptoms including signs of severe neurodegeneration and psychiatric illness are likely to evolve over time resulting in premature death. We report on two siblings of Turkish origin from our diabetes clinic who were diagnosed with Wolfram syndrome after 6 years and 2 years duration of DM, respectively. Subtle symptoms such as attitude changes, growing reading difficulties in the history of children or adolescents with antibody negative and ketone negative DM should alert the treating physician and lead to re-evaluation of the diagnosis, keeping in mind that not all juvenile DM is type 1 DM.

Corresponding author: Dr. C. Boettcher, Department of General Pediatrics and Neonatology, Center of Child and Adolescent Medicine, Justus Liebig University, Feulgenstr. 12, 35392 Giessen, Germany Phone: +49 641 99 43 400, Fax: +49 641 99 43 459

Published Online: 2011-03-16
Published in Print: 2011-03-01

©2011 by Walter de Gruyter Berlin New York

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