CHI is a diverse disease involving different genetic causes affecting proteins such as glutamate dehydrogenase, hepatic nuclear factors 4α and 1α, the inwardly rectifying potassium channel Kir6.2, monocarboxylic acid transporter 1, short-chain 3-OH acyl-CoA dehydrogenase, the sulfonylurea receptor 1, and uncoupling protein 2. This evidence concerns the gene KCNJ11 and congenital isolated hyperinsulinism.