There are studies that have proven that diazoxide is usually ineffective in treating patients with CHI caused by recessive biallelic mutations in the genes encoding the KATP channel (ABCC8/KCNJ11) associated with the diffuse form of the disease [44,45] as well as in the case of the presence of the monoallelic focal form of CHI [45,46]. Here, KCNJ11 is linked to congenital isolated hyperinsulinism.