MODY (maturity onset diabetes of the young) is a rare, genetic form of diabetes characterised by three main features: a young age of onset (<25yrs in at least one family member), non-insulin-dependent diabetes and autosomal dominant inheritance. MODY accounts for 1% of patients with diabetes in the UK – approximately 20,000 cases. It is often misdiagnosed, which can have major implications for patient management and investigating family members. In this article we review what causes MODY, how it is diagnosed and how to manage patients and their families.
