Maturity Onset Diabetes of the Young (MODY)

Features of Maturity Onset Diabetes of the Young (MODY) include the family history, which is consistent with autosomal dominant inheritance (with 50% chance of transmission to offspring), and the hypersensitivity to sulphonylureas.

The commonest type of MODY (MODY 3) is due to mutation in the HNF1alpha gene, which encodes a transcription factor involved in beta cell development. Other HNFs are implicated in further types of MODY, and in general the hyperglycaemia in these subtypes is progressive, with 30-40% requiring insulin, and risk of microvascular complications which is commensurate with that in type 1 diabetes, matched for glycaemia. Glucokinase mutations reset the set point for glucose homeostasis, and tend to result in mild, non-progressive hyperglycaemia, with low risk of complications.