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Diabetes caused by Kir6.2 mutation: Successful treatment with oral glibenclamide switched from continuous subcutaneous insulin infusion in the early phase of the disease

Neonatal diabetes mellitus (NDM) is very rare, with an estimated incidence of one in 500 000 neonates. Its etiology and long-term clinical course are not well known. In most cases, blood glucose levels during the neonatal and infantile periods are controlled by insulin treatment. Diabetes mellitus (DM) diagnosed before 6 months of age, classified as NDM, frequently results from mutations of the KCNJ11 gene that encodes the Kir6.2 subunit of the adenosine triphosphate (ATP)-sensitive potassium channel (KATP channel). These are activating mutations, resulting in hyperpolarization of the cell membrane, and, in the case of the pancreatic b-cells, inhibition of insulin secretion. Notably, it has been reported that in these patients, treatment with a sulfonylurea (SU), which binds to the sulfonylurea receptor 1 (SUR1) subunit and closes the KATP channel, can stimulate insulin secretion and improve blood glucose control. We report a case of NDM caused by the Kir6.2 mutation (R201C) in a young infant, in whom treatment initiated with continuous subcutaneous insulin infusion (CSII) could subsequently be switched successfully to oral sulfonylurea treatment with glibenclamide.