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Diseases

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
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Overview

Wolfram syndrome, which is also known by the acronym DIDMOAD, is an inherited condition characterized by diabetes insipidus (DI), childhood-onset diabetes mellitus (DM), a gradual loss of vision caused by optic atrophy (OA), and deafness (D).[1] There are two types of Wolfram syndrome (type 1 and type 2) which are primarily differentiated by their genetic cause. Type 1 is caused by changes (mutations) in the WFS1 gene, while type 2 is caused by mutations in the CISD2 gene. Both forms are inherited in an autosomal recessive manner.[2][3] Treatment is symptomatic and supportive.[2]
Last updated: 12/8/2014

References

  1. Wolfram syndrome. Genetics Home Reference. 2012; http://ghr.nlm.nih.gov/condition/wolfram-syndrome. Accessed 1/21/2014.
  2. Tranebjærg L, Barrett T & Rendtorff ND. WFS1-Related Disorders. Gene Reviews. 2013; http://www.ncbi.nlm.nih.gov/books/NBK4144/. Accessed 1/21/2014.
  3. Wolfram Syndrome 2. OMIM. September 2014; http://omim.org/entry/604928.
Your Questions Answered
by the Genetic and Rare Diseases Information Center

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

  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
    Wolfram Syndrome 1
    Wolfram Syndrome 2
    Wolfram Syndrome, Mitochondrial Form
  • 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.