The molecular basis of CHI involves defects in key genes (ABCC8, KCNJ11, GLUD1, GCK, HADH, SLC16A1, HNF1A, HNF4A, UCP2, HK1, PGM1 and PMM2) which regulate insulin secretion. This evidence concerns the gene KCNJ11 and congenital isolated hyperinsulinism.