[Maturity-onset diabetes of the young (MODY)--a heterogeneous disease picture]

A family with maturity-onset diabetes of the young (MODY), a rare subtype of non-insulin-dependent diabetes mellitus (NIDDM), is described. This familial form of diabetes is characterized by an early age of onset and an autosomal dominant inheritance. All three patients developed diabetes at an early age and were not obese. The diabetes was always well controlled and none of the patients developed late complications. The clinical and molecular-genetic characteristics of the different forms of MODY are described, as well as the diagnostic work-up and therapeutic management.