Wolfram syndrome

Common Name(s)

Wolfram syndrome

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). 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. Treatment is symptomatic and supportive.
 

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Wolfram syndrome" for support, advocacy or research.

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Wolfram syndrome" returned 65 free, full-text research articles on human participants. First 3 results:

A donor splice site mutation in CISD2 generates multiple truncated, non-functional isoforms in Wolfram syndrome type 2 patients.
 

Author(s): Monica Cattaneo, Lucia La Sala, Maurizio Rondinelli, Edoardo Errichiello, Orsetta Zuffardi, Annibale Alessandro Puca, Stefano Genovese, Antonio Ceriello

Journal:

 

Mutations in the gene that encodes CDGSH iron sulfur domain 2 (CISD2) are causative of Wolfram syndrome type 2 (WFS2), a rare autosomal recessive neurodegenerative disorder mainly characterized by diabetes mellitus, optic atrophy, peptic ulcer bleeding and defective platelet aggregation. ...

Last Updated: 31 Dec 1969

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Diabetes mellitus and optic atrophy: study of the Wolfram syndrome
 

Author(s): Bernardette Rivas-Gómez, Alfredo Adolfo Reza-Albarrán

Journal: Gac Med Mex. 2017 ;153(4):466-472.

 

Wolfram syndrome (WS), also known by the acronym DIDMOAD, is a rare and progresive hereditary disease of autosomal recessive inheritance which minimum ascertainment diagnostic criteria are the occurrence together of diabetes mellitus and optic atrophy before 15 years of age.

Last Updated: 31 Dec 1969

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Retinal thickness as a marker of disease progression in longitudinal observation of patients with Wolfram syndrome.
 

Author(s): Agnieszka Zmyslowska, Wojciech Fendler, Arleta Waszczykowska, Anna Niwald, Maciej Borowiec, Piotr Jurowski, Wojciech Mlynarski

Journal: Acta Diabetol. 2017 Nov;54(11):1019-1024.

 

Wolfram syndrome (WFS) is a recessively inherited monogenic form of diabetes coexisting with optic atrophy and neurodegenerative disorders with no currently recognized markers of disease progression. The aim of the study was to evaluate retinal parameters by using optical coherence ...

Last Updated: 31 Dec 1969

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Wolfram syndrome" returned 4 free, full-text review articles on human participants. First 3 results:

Wolfram Syndrome: Diagnosis, Management, and Treatment.
 

Author(s): Fumihiko Urano

Journal: Curr. Diab. Rep.. 2016 Jan;16(1):6.

 

Wolfram syndrome is a rare genetic disorder characterized by juvenile-onset diabetes mellitus, diabetes insipidus, optic nerve atrophy, hearing loss, and neurodegeneration. Although there are currently no effective treatments that can delay or reverse the progression of Wolfram syndrome, ...

Last Updated: 31 Dec 1969

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[Wolfram syndrome: from definition to molecular bases].
 

Author(s): Maria Regina F Ribeiro, Felipe Crispim, Márcio F Vendramini, Regina S Moisés

Journal: Arq Bras Endocrinol Metabol. 2006 Oct;50(5):839-44.

 

Wolfram syndrome (WS) is an autosomal recessive progressive neurodegenerative disorder characterized by diabetes mellitus and optic atrophy. Diabetes insipidus and sensorineural deafness are also noted frequently, explaining the acronym DIDMOAD (diabetes insipidus, diabetes mellitus, ...

Last Updated: 31 Dec 1969

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Wolfram (DIDMOAD) syndrome.
 

Author(s): T G Barrett, S E Bundey

Journal: J. Med. Genet.. 1997 Oct;34(10):838-41.

 

Wolfram syndrome (MIM 222300) is the association of juvenile onset diabetes mellitus and optic atrophy, also known as DIDMOAD (Diabetes Insipidus, Diabetes Mellitus, Optic Atrophy, and Deafness). Patients present with diabetes mellitus followed by optic atrophy in the first decade, ...

Last Updated: 31 Dec 1969

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Clinical Trial Information This information is provided by ClinicalTrials.gov

A Clinical Trial of Dantrolene Sodium in Pediatric and Adult Patients With Wolfram Syndrome
 

Status: Recruiting

Condition Summary: Wolfram Syndrome; Diabetes Mellitus; Optic Nerve Atrophy; Ataxia

 

Last Updated: 24 Mar 2017

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Tracking Neurodegeneration in Early Wolfram Syndrome
 

Status: Recruiting

Condition Summary: Type 1 Diabetes; Diabetes Insipidus; Diabetes Mellitus; Wolfram Syndrome

 

Last Updated: 31 Oct 2016

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Wolfram Syndrome International Registry and Clinical Study
 

Status: Recruiting

Condition Summary: Wolfram Syndrome; Diabetes Mellitus; Optic Nerve Atrophy; Deafness; Diabetes Insipidus; Ataxia

 

Last Updated: 1 Nov 2017

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