Preferred Label : Wolfram syndrome;
ICD-11 definition : Wolfram syndrome, also called DIDMOAD (Diabetes Insipidus, Diabetes Mellitus, Optic
Atrophy, and Neural Deafness), is a rare genetic disorder, causing diabetes mellitus,
optic atrophy, and neural deafness as well as diabetes insipidus. An autosomal recessive
form due to an inactivating mutation in the AVP portion of the gene, an X-liked recessive
form due to an unidentified gene on Xq28, and an autosomal recessive form due to mutations
of the WFS 1 gene are responsible for Wolfram syndrome.;
ICD-11 synonym : Diabetes insipidus, diabetes mellitus, optic atrophy, and deafness; DIDMOAD syndrome; DIDMOAD - [Diabetes insipidus, diabetes mellitus, optic atrophy, and deafness] syndrome;
Origin ID : 151381747;
Automatic exact mappings (from CISMeF team)
Wolfram syndrome, also called DIDMOAD (Diabetes Insipidus, Diabetes Mellitus, Optic
Atrophy, and Neural Deafness), is a rare genetic disorder, causing diabetes mellitus,
optic atrophy, and neural deafness as well as diabetes insipidus. An autosomal recessive
form due to an inactivating mutation in the AVP portion of the gene, an X-liked recessive
form due to an unidentified gene on Xq28, and an autosomal recessive form due to mutations
of the WFS 1 gene are responsible for Wolfram syndrome.
