Identification of a novel hepatocyte nuclear factor-1 alpha (HNF1A) variant in maturity onset diabetes of the young type 3 (HNF1A-MODY).

HNF1A mutations are a common cause of monogenic diabetes in patients presenting with early-onset diabetes and significant family history. Genetic testing in suspected patients allows for the identification of mutations causing monogenic diabetes. First-degree relatives of the affected individual should be considered for genetic testing. The use of sulfonylurea agents in patients with HNF1A-MODY can reduce dependence on insulin therapy and provide successful glycemic control.