[Clinical variability of autosomal-dominant juvenile diabetes (MODY)]

Abstract

Occurrence prior to the age of 25 years, dominant inheritance, metabolic control without insulin for more than two years and mild course without late complications are considered characteristic of maturity-onset diabetes of young people (MODY). Diabetes or glucose tolerance disturbance was observed in 30 members of a family in five successive generations. The course of the disease within the family was variable: even after disease for several decades no diabetes-specific late complications were seen in 20 diabetics, six, in contrast, had serious complications such as proliferative retinopathy, nephropathy and coma. The different clinical course is expression of the considerable variability of this genetically uniform metabolic disorder. Our observations demonstrate that vascular complications in MODY are less frequent than in type II diabetes, however are not excluded. Thus, also MODY diabetics require life-long careful metabolic surveillance.