Genetic testing plays a pivotal role in the management of maturity onset diabetes of the young (MODY), driving the choice of the treatment, the assessment of the risk for complications, and the need for genetic counseling.

Patients with glucokinase (GCK)-MODY should not be treated, except during pregnancy and when the fetus is healthy.

Sulphonylureas are effective in managing patients with HNF1A- and HNF4A–MODY, while additional treatment with other oral hypoglycemic agents may improve blood glucose control.

Avoid treating carriers of HNF1A and HNF4A mutations with sulphonylureas during pregnancy.