If confirmed, 4PB therapy could become the preferred choice, instead of topical steroids, antihistamine agents, and surgical procedures, for attenuating cholestatic pruritus in patients with PFIC1 and benign intrahepatic cholestasis type 1 (BRIC1), a hereditary disorder characterized by mutations in ATP8B1 and by recurrent and intermittent episodes of cholestasis with refractory cholestatic pruritus[30]. Here, ATP8B1 is linked to benign recurrent intrahepatic cholestasis type 1.