To date, mutations in 14 essential genes controlling insulin secretion have been reported including ABCC8 and KCNJ11. ABCC8 and KCNJ11 genes encode the KATP channel subunits SUR1 and Kir6.2, respectively, and mutations in these genes represent the most prevalent cause of CHI. This evidence concerns the gene KCNJ11 and congenital isolated hyperinsulinism.