Proliferative diabetic retinopathy in a patient with maturity-onset diabetes of the young (MODY).

Maturity-onset diabetes of the young (MODY) has been described as being characteristically free from severe complications. This has led to speculation that the type of diabetes may be important in the pathogenesis of complications in diabetes. We report a case of classical MODY in which severe proliferative diabetic retinopathy developed. The retinopathy was detected shortly after the diagnosis of diabetes was made when the patient was 32 years old, and did not progress subsequently. No further complications developed during the subsequent 29 years in which normal postprandial plasma glucose levels were maintained with chlorpropamide therapy (mean 4.7, range 4.1-6.0 mmol I-1). This case demonstrates that severe retinopathy can occur in MODY and we suggest that in this patient there may have been a period of hyperglycaemia prior to diagnosis which was sufficient to lead to the microvascular complication.