Remission of Severe Neonatal Diabetes With Very Early Sulfonylurea Treatment

Mutations in KCNJ11 and ABCC8 can cause neonatal diabetes mellitus (NDM) (1) that may respond to sulfonylureas (2). We report three NDM infants treated with glyburide very early in life who were able to maintain good glycemic control with minimal dosing. This 6-year-old male (Case-1-male) was previously reported at birth (3). Mother and sister (Case-1-female) both had NDM and were given insulin for 24 and 6 years, respectively, then treated with high-dose glyburide; mother still requires some insulin. Case-1-male had NDM at 3 days and was given glyburide 0.2 mg/kg/day, weaned to 0.05 mg/kg/day, and maintained normal hemoglobin A1c for 5 years (Fig. 1 A ) continuing glyburide 0.075 mg thrice daily (mixing a crushed 5 mg tablet with 10 cc water, giving 0.15 cc for each dose, 0.012 mg/kg/day). All had a heterozygous mutation in KCNJ11 (R201H). Figure 1 A : Hemoglobin A …