Abstract
Maturity-onset Diabetes of the Young (MODY) is autosomal dominant-inherited, yet clinically heterogeneous type of diabetes. In case of prognostically benign GCK-MODY no specific antidiabetic treatment is required (with pregnancy as an exception).
However, in MODY of transcription factors, especially HNF1A-MODY, but also in less common HNF4A-MODY, the therapy, traditionally based on sulphonylurea derivatives (alternatively on glinides), is necessary. In case of failure of this therapy (usually after the long duration of HNF1A-MODY and HNF4A-MODY) initiation of insulin therapy is required.
Insulin is also the first drug of choice in HNF1B-MODY. Use of other hypoglycaemic agents is yet experimental and the promising effects of particularly incretin therapy and gliflozins (probably rather in combination) will be needed to verify in larger studies.
This review summarizes current available information about therapeutical options in MODY patients.