Catalog

MODY 5 syndrome

No MMS code

Link to WHO Definition

MODY 5 syndrome – http://id.who.int/icd/entity/566324219

This refers to the hereditary form of diabetes caused by mutations in an autosomal dominant gene (sex independent, i.e. inherited from any of the parents) disrupting insulin production. One of the less common forms of MODY, with some distinctive clinical features, including atrophy of the pancreas and several forms of renal disease. Defect in HNF-1 beta gene. 5%–10% cases.