Abstract
MODY 3 belongs to the monogennic forms of diabetes. It is caused by the mutation of gene for transcription factor HNF-1 alpha, localized on the chromosome 12.
It is characterized by the discrepancy between impaired insulin response to glucose and well-preserved insulin response to sulphonylurea derivates. The theoretical presumption of sulphonylurea hypersensitivity is well-preserved insulin sensitivity in MODY 3 patients and probably prolonged clearence of sulphonylureas.
MODY 3 is typically manifested in young nonobese people by osmotic symptoms resulting from hyperglycaemia, without ketoacidosis and by the rapid development of chronic microvascular complications. Insulin treatment had been the only one therapeutical modality in the young MODY 3 patients till nowadays, but recently the safety and efficacy of the longterm sulphonylurea treatment has been reported.