In the future, it is expected that AIMS patients will be identified with additional mutations in ABCC9. Such mutations may prove to be autosomal dominant or may exhibit only partial LoF, as is observed in the analogous pancreatic KATP disorder, congenital hyperinsulissm (CHI [MIM: 256450]), which arises from LoF mutations in ABCC848. Here, ABCC9 is linked to congenital isolated hyperinsulinism.