title: The subtle signs of Wolfram (DIDMOAD) syndrome: not all juvenile diabetes is type 1 diabetes.
abstract: 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.
authors.affiliation: Department of General Pediatrics and Neonatology, Center of Child and Adolescent Medicine, Justus Liebig University, Giessen, Germany. Claudia.Boettcher@paediat.med.uni-giessen.de
authors.forename: Claudia
authors.initials: C
authors.lastname: Boettcher
authors.forename: Burkhard
authors.initials: B
authors.lastname: Brosig
authors.forename: Klaus P
authors.initials: KP
authors.lastname: Zimmer
authors.forename: Stefan A
authors.initials: SA
authors.lastname: Wudy
mesh_terms: D000293:Adolescent; D017668:Age of Onset; D002648:Child; D002653:Child Behavior Disorders / Q000175:diagnosis; D003922:Diabetes Mellitus, Type 1 / Q000175:diagnosis*; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007859:Learning Disabilities / Q000175:diagnosis; D008297:Male; D008565:Membrane Proteins / Q000235:genetics; D035781:Siblings; D014929:Wolfram Syndrome / Q000175:diagnosis* / Q000453:epidemiology / Q000503:physiopathology / Q000523:psychology
chemical_list: D008565:Membrane Proteins; C114987:wolframin protein