Maturity onset diabetes of young (MODY)

- monogenic diabetes mellitus
- early onset of diabetes with strong family history, often easy to control
- autosomal dominant
- MODY 3 (mutation of HNF-1a) is the commonest (70%), sensitive to treatment with sulphonylureas, raised HDL, preserved insulin sensitivity and glycosuria
- MODY 2 (mutation in glucokinase), usually asymptomatic and does not need treatment, but important to identify as it can lead to reduced birthweight in pregnancy
- MODY 1 (mutation of HNF-4a), presents later in life
- MODY 5 (mutation of HNF-1b), leads to renal cyst and proteinuria
- MODY 4 (IPF-1 mutation) and MODY 6 (NeuroD1 mutation) are rare

*HNF = hepatic nuclear factor
  IPF = insulin promoter factor