Mutations in ABCC8 and KCNJ11, which encode the SUR1 and Kir6.2 subunits of pancreatic ATP-sensitive potassium channel (KATP), are by far the most common cause of CHI and are estimated to account for 36%-69% of all cases[4–6]. This evidence concerns the gene KCNJ11 and congenital isolated hyperinsulinism.