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. Here, ABCC8 is linked to congenital isolated hyperinsulinism.