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Genetic and Rare Diseases Information Center (GARD)

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Wolfram syndrome


Other Names for this Disease

  • Diabetes insipidus and mellitus with optic atrophy and deafness
  • DIDMOAD
  • DIDMOAD syndrome
  • WFS
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Overview

Wolfram syndrome, which is also known by the acronym DIDMOAD, is characterized by diabetes insipidus (DI); childhood-onset diabetes mellitus (DM); a gradual loss of vision caused by optic atrophy (OA); and deafness (D). Other complications that affect the bladder and nervous system may also occur.[1] Mutations in two genes have been associated with Wolfram syndrome.[2] The majority of cases are associated with mutations in the WFS1 gene.[1][2] Mutations in the CISD2 gene have been identified in three consanguineous families of Jordanian descent.[2][3] The condition is inherited in an autosomal recessive manner. Treatment is symptomatic and supportive.[2]
Last updated: 9/8/2011

References

  1. WFS1. Genetics Home Reference (GHR). 2006; http://ghr.nlm.nih.gov/gene/WFS1. Accessed 9/7/2011.
  2. Martinez VN. Wolfram syndrome. Orphanet. 2007; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=3463. Accessed 9/7/2011.
  3. Wolfram Syndrome 2. Online Mendelian Inheritance in Man (OMIM). 2009; http://omim.org/entry/604928. Accessed 9/7/2011.
Your Questions Answered
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Please contact us with your questions about Wolfram syndrome. We will answer your question and update these pages with new resources and information.

Basic Information

  • Genetics Home Reference (GHR) contains information on Wolfram syndrome. This website is maintained by the National Library of Medicine.
  • The National Organization for Rare Disorders (NORD) is a federation of more than 130 nonprofit voluntary health organizations serving people with rare disorders. Click on the link to view information on this topic.

In Depth Information

  • The Online Mendelian Inheritance in Man (OMIM) database contains genetics resources that discuss the different types of Wolfram syndrome. Click on the links below to go to OMIM and review these resources.
    Wolfram Syndrome 1
    Wolfram Syndrome 2
  • Orphanet is a European reference portal for¬†information on rare diseases and orphan drugs.¬† Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Wolfram syndrome. Click on the link to view a sample search on this topic.
Other Names for this Disease
  • Diabetes insipidus and mellitus with optic atrophy and deafness
  • DIDMOAD
  • DIDMOAD syndrome
  • WFS
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.