Preferred Label : MODY 4 syndrome;
ICD-11 definition : 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 agensis in homozygotes and occasionally in heterozygotes.;
ICD-11 synonym : Maturity Onset Diabetes of the Young type 4;
Origin ID : 679107840;
Currated CISMeF NLP mapping
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 agensis in homozygotes and occasionally in heterozygotes.
