The most common causes of PNDM are mutations in the genes (KCNJ11 and ABCC8) encoding the two protein subunits [Kir6.2 and sulfonylurea receptor 1 (SUR1), respectively] of the ATP-sensitive potassium (KATP) channel and in the gene encoding insulin itself (3,4,5,6,7,8). This evidence concerns the gene INS and permanent neonatal diabetes mellitus.