Wolfram Syndrome (Syndrome, Wolfram)

A hereditary condition characterized by multiple symptoms including those of DIABETES INSIPIDUS; DIABETES MELLITUS; OPTIC ATROPHY; and DEAFNESS. This syndrome is also known as DIDMOAD (first letter of each word) and is usually associated with VASOPRESSIN deficiency. It is caused by mutations in gene WFS1 encoding wolframin, a 100-kDa transmembrane protein.

Also Known As:

Networked: 119 relevant articles (1 outcomes, 13 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Wolfram Syndrome (Syndrome, Wolfram)
2.	Diabetes Insipidus
3.	Mood Disorders (Mood Disorder)
4.	Anemia
5.	Low Tension Glaucoma

Experts

1.	Zatyka, Malgorzata: 4 articles (02/2015 - 07/2006)
2.	Oka, Yoshitomo: 4 articles (06/2009 - 07/2005)
3.	Tanizawa, Yukio: 4 articles (06/2009 - 06/2003)
4.	Barrett, Timothy G: 3 articles (02/2015 - 01/2008)
5.	Urano, Fumihiko: 3 articles (12/2014 - 10/2012)
6.	Agranat, Lily: 2 articles (06/2015 - 01/2013)
7.	Lipper, Colin H: 2 articles (06/2015 - 01/2013)
8.	Nechushtai, Rachel: 2 articles (06/2015 - 01/2013)
9.	Tamir, Sagi: 2 articles (06/2015 - 01/2013)
10.	Mittler, Ron: 2 articles (06/2015 - 01/2013)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Wolfram Syndrome:

1.	idebenoneIBA 12/01/2012 - "Visual improvement with the use of idebenone in the treatment of Wolfram syndrome."
2.	TungstenIBA 01/01/2003 - "Wolfram (DIDMOAD) syndrome: a multidisciplinary clinical study in nine Turkish patients and review of the literature." 12/02/1995 - "Neurodegeneration and diabetes: UK nationwide study of Wolfram (DIDMOAD) syndrome." 12/09/2014 - "Teaching NeuroImages: MRI of brain findings of Wolfram (DIDMOAD) syndrome." 11/01/2011 - "Presentation and clinical course of Wolfram (DIDMOAD) syndrome from North India." 01/01/2011 - "The subtle signs of Wolfram (DIDMOAD) syndrome: not all juvenile diabetes is type 1 diabetes."
3.	Mitochondrial DNA (mtDNA)IBA 06/01/2004 - "Study of the WFS1 gene and mitochondrial DNA in Spanish Wolfram syndrome families." 07/29/2011 - "The wide spectrum of clinical expression, affecting several organs and tissues, and the similarity in phenotype between patients with Wolfram syndrome and those with certain types of respiratory chain diseases suggests mitochondrial DNA (mtDNA) involvement in Wolfram syndrome patients. " 10/01/1997 - "LHON and DIDMOAD were significantly under-represented in the most frequent German haplogroup DC, but were concentrated in a mtDNA lineage defined by polymorphisms at nt 4216 + 11251 + 16126. " 05/01/1996 - "Autosomal recessive Wolfram syndrome associated with an 8.5-kb mtDNA single deletion." 04/01/1996 - "We report two Wolfram syndrome families harboring multiple deletions of mitochondrial DNA. "
4.	AutoantibodiesIBA 01/01/2010 - "The aim of the study was an evaluation of HLA subtypes and presence of β-cell autoantibodies in patients with molecularly confirmed Wolfram syndrome. " 01/01/2010 - "The aim of the study was an evaluation of HLA subtypes and presence of β-cell autoantibodies in patients with molecularly confirmed Wolfram syndrome. " 01/01/2010 - "Polish patients with Wolfram syndrome have a different profile of the HLA antigens with the presence of DR2, DQw1 and DRB3/4 allele and are negative for diabetes-related autoantibodies, which may confirm non-autoimmune β-cell destruction in this syndrome." 01/01/2010 - "Polish patients with Wolfram syndrome have a different profile of the HLA antigens with the presence of DR2, DQw1 and DRB3/4 allele and are negative for diabetes-related autoantibodies, which may confirm non-autoimmune β-cell destruction in this syndrome."
5.	Insulin (Novolin)FDA Link 03/01/2014 - "To investigate the cause of β-cell failure, we used induced pluripotent stem cells to create insulin-producing cells from individuals with Wolfram syndrome. " 09/01/2013 - "Wolfram syndrome should be considered as a differential diagnosis in insulin dependent diabetic children who present with neuropsychiatric problems. " 07/01/2011 - "An Asian Indian woman with Wolfram syndrome on insulin pump: successful pregnancy and beyond." 01/01/2010 - "Diabetes present in Wolfram syndrome is a result of the selective β cell loss and failed insulin secretion which is probably associated with non-autoimmune pathogenesis. " 01/01/2010 - "Diabetes present in Wolfram syndrome is a result of the selective β cell loss and failed insulin secretion which is probably associated with non-autoimmune pathogenesis. "
6.	Thiamine (Aneurin)FDA Link 11/01/1989 - "Treatment of DIDMOAD syndrome with thiamine." 03/01/1989 - "We propose that an inherited abnormality of thiamine metabolism is responsible for the multisystem degenerative disorder known as DIDMOAD syndrome." 01/01/2012 - "Furthermore, the finding of unexplained thiamine deficiency in a patient with Wolfram syndrome suggests a potential link between WFS1 biology and thiamine metabolism that has implications for the clinical management of Wolfram syndrome patients." 03/01/1989 - "Thiamine-responsive anemia in DIDMOAD syndrome." 05/01/1992 - "Thiamine dependent anemia in DIDMOAD (Wolfram) syndrome: Further studies and report of two additional cases."
7.	Vasopressins (Vasopressin)IBA 09/01/1986 - "Documented vasopressin deficiency in a child with Wolfram syndrome." 11/01/1998 - "The vasopressin precursor is not processed in the hypothalamus of Wolfram syndrome patients with diabetes insipidus: evidence for the involvement of PC2 and 7B2." 04/01/1989 - "The absence of vasopressin responses to non-osmotic stimuli in patients with complete diabetes insipidus suggests global lack of vasopressin secreting neurones, rather than an isolated osmoreceptor defect or selective vasopressin secreting neuronal loss, as the lesion producing diabetes insipidus in the DIDMOAD syndrome." 04/01/1989 - "Vasopressin secretion in the DIDMOAD (Wolfram) syndrome." 04/01/1989 - "The diabetes insipidus which accompanies the DIDMOAD (Wolfram) syndrome is thought to be hypothalamic in origin, though no formal study of vasopressin secretion in the syndrome has been published, and some data in the literature suggest a renal tubular defect. "
8.	Tyrosine 3-Monooxygenase (Tyrosine Hydroxylase)IBA 06/01/2003 - "The study could not support the involvement of tyrosine hydroxylase, catechol-O-methyl transferase and Wolfram syndrome 1 polymorphisms in mood disorders." 06/01/2003 - "The aim of the present study was to investigate tyrosine hydroxylase, catechol-O-methyl transferase and Wolfram syndrome 1 genes in mood disorders using a family-based association approach. " 06/01/2003 - "Multicentre Italian family-based association study on tyrosine hydroxylase, catechol-O-methyl transferase and Wolfram syndrome 1 polymorphisms in mood disorders."
9.	TransferasesIBA 06/01/2003 - "The study could not support the involvement of tyrosine hydroxylase, catechol-O-methyl transferase and Wolfram syndrome 1 polymorphisms in mood disorders." 06/01/2003 - "The aim of the present study was to investigate tyrosine hydroxylase, catechol-O-methyl transferase and Wolfram syndrome 1 genes in mood disorders using a family-based association approach. " 06/01/2003 - "Multicentre Italian family-based association study on tyrosine hydroxylase, catechol-O-methyl transferase and Wolfram syndrome 1 polymorphisms in mood disorders."
10.	Growth Hormone (Somatotropin)IBA 09/01/1995 - "Growth hormone deficiency and empty sella in DIDMOAD syndrome: an endocrine study."

Therapies and Procedures

1.	Pancreas Transplantation 11/27/2003 - "Solitary pancreas transplantation in Wolfram syndrome."
2.	Kidney Transplantation 09/15/2001 - "We discuss the etiology of our patient's postoperative events, and conclude that kidney transplantation may expose dormant manifestations-or aggravate existing manifestations-of Wolfram syndrome." 09/15/2001 - "There are no prior reports of kidney transplantation in patients with Wolfram syndrome. " 09/15/2001 - "Kidney transplantation unraveling Wolfram syndrome: a case report." 09/15/2001 - "Kidney transplantation was undertaken in a child with dysplastic kidneys, sensorineural hearing impairment and bilateral optic atrophy-a combination of features insufficient to define Wolfram syndrome. "
3.	Vitrectomy 03/01/1991 - "During the nine-year follow-up period, diabetic retinopathy deteriorated despite treatment by photocoagulation and vitrectomy, suggesting the importance of ophthalmological examinations in patients with DIDMOAD syndrome."
4.	Light Coagulation 03/01/1991 - "During the nine-year follow-up period, diabetic retinopathy deteriorated despite treatment by photocoagulation and vitrectomy, suggesting the importance of ophthalmological examinations in patients with DIDMOAD syndrome."