In summary, our results strongly support the role of an ABCB4 gene defect in LPAC syndrome and replace it in the context of ABCB4 gene-associated liver diseases in adults including attenuated form of PFIC 3, "atypical intrahepatic cholestasis of pregnancy", LPAC syndrome which might lead to adulthood biliary cirrhosis or bile duct dilatations associated with gallstones. Here, ABCB4 is linked to liver disorder.