Pathogenic HNF1A Variant in an Indonesian Family: Atypical Management of MODY3 Guided by Patient Comorbidity.

Background/Objective: Maturity-onset diabetes of the young (MODY) is an autosomal dominant form of monogenic diabetes frequently misdiagnosed as type 1 or type 2 diabetes. Identifying the specific subtype is crucial, as several subtypes, such as Case Report: A 19-year-old male with a history of diabetes presented with right-sided weakness, aphasia, and facial asymmetry. He was admitted for gamma knife radiosurgery to treat a left basal ganglia arteriovenous malformation. One month prior to this admission, he had undergone surgical evacuation of an intracranial hemorrhage-a complication of his arteriovenous malformation-at another hospital. He was discharged on a basal-bolus insulin regimen due to hyperglycemia during that hospitalization and was subsequently referred to our center. During the current admission, glycemic control was achieved, allowing gradual reduction of insulin dose and transition to oral sitagliptin/metformin XR upon discharge. Genetic testing later confirmed a pathogenic c.160C>T variant in the Discussion: Although sulfonylureas are typically the first-line treatment for Conclusion: This case underscores that while genetic confirmation of