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.

There are currently no organizations listed in Disease InfoSearch that support this condition. Create a listing.

 

 

General Support Organizations

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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 57 free, full-text research articles on human participants. First 3 results:

Selective cognitive and psychiatric manifestations in Wolfram Syndrome.
 

Author(s): Allison N Bischoff, Angela M Reiersen, Anna Buttlaire, Amal Al-Lozi, Tasha Doty, Bess A Marshall, Tamara Hershey,

Journal:

 

Wolfram Syndrome (WFS) is known to involve diabetes mellitus, diabetes insipidus, optic nerve atrophy, vision loss, hearing impairment, motor abnormalities, and neurodegeneration, but has been less clearly linked to cognitive, sleep, and psychiatric abnormalities. We sought to determine ...

Last Updated: 2 Jun 2015

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Association of optic atrophy and type 1 diabetes: clinical hallmarks for the diagnosis of Wolfram syndrome.
 

Author(s): José Luiz Pedroso, Leandro Tavares Lucato, Fernando Kok, Juliana Sallum, Orlando G P Barsottini, Acary Souza Bulle Oliveira

Journal: Arq Neuropsiquiatr. 2015 May;73(5):466-8.

 

Last Updated: 28 May 2015

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Early-onset central diabetes insipidus is associated with de novo arginine vasopressin-neurophysin II or Wolfram syndrome 1 gene mutations.
 

Author(s): Silverio Perrotta, Natascia Di Iorgi, Fulvio Della Ragione, Saverio Scianguetta, Adriana Borriello, Anna Elsa Maria Allegri, Marcella Ferraro, Claudia Santoro, Flavia Napoli, Annalisa Calcagno, Marta Giaccardi, Marco Cappa, Maria Carolina Salerno, Domenico Cozzolino, Mohamad Maghnie

Journal: Eur. J. Endocrinol.. 2015 Apr;172(4):461-72.

 

Idiopathic early-onset central diabetes insipidus (CDI) might be due to mutations of arginine vasopressin-neurophysin II (AVP-NPII (AVP)) or wolframin (WFS1) genes.

Last Updated: 5 Mar 2015

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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: 8 Jan 2016

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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: 12 Dec 2006

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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: 9 Dec 1997

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Symptoms, Diagnosis, and Treatment

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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: Not yet recruiting

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

 

Last Updated: 25 Aug 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: 21 Jul 2016

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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: 13 Jan 2016

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