Catalog

MODY 4 syndrome

No MMS code

Link to WHO Definition

MODY 4 syndrome – http://id.who.int/icd/entity/679107840

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. Mutations of the IPF1 homeobox (Pdx1) gene. < 1% cases. Associated with pancreatic agenesis in homozygotes and occasionally in heterozygotes.