MON-121 Successful Transition from Insulin to Sulfonylurea for a 26 Year Old Female with Neonatal Diabetes Secondary to KCNJ11 Gene Mutation

Abstract

Neonatal diabetes (NDM) is defined as diabetes that occurs in the first 6 months of life. Sporadic mutation is the main etiology of this type. ATP-sensitive potassium channels located in the beta cells of pancreas play a major role in insulin secretion and blood glucose hemostasis. Mutations that alter the function of these channels may lead to neonatal diabetes. We herein report a case of a 26 year old Irish female who was diagnosed with neonatal diabetes at the age of one month old and was labeled as type 1 diabetes mellitus, in which she was started on multiple daily injection of insulin with suboptimal control and frequent episodes of hypoglycemia. She has a positive family history of diabetes. Anti-GAD antibody and Islets cell antibody were both negative. However, at the age of 16 years, she underwent genetic testing and was diagnosed with KCNJ11 gene mutation. The gene is encoded for Kir6.2, which is a major subunit of ATP-sensitive potassium channels that lead to channels malfunction and development of diabetes. She was transferred to glibenclamide at the age of 16 but the trail was failed and restarted on insulin. Prior to the second trail of transition her HbA1C was 63 mmol/mol and her total daily dose of insulin was 50 units. At 23 years of age she was successfully shifted from insulin to high dose sulfonylurea (glibenclamide 15mg twice daily) with optimal control of blood glucose (HbA1C 44 mmol/mol), lower rates of hypoglycemic episodes and better quality of life.