Clinical trials in children with congenital cholestatic syndromes have shown that pruritus as a result of cholestasis is reduced by using ASBT inhibitors (ASBTi) and recently, two different intestine-restricted ASBTi, maralixibat13 and odevixibat,14 have been approved by the FDA and EMA for treatment of Alagille syndrome and PFIC, respectively.15 This evidence concerns the gene SLC10A2 and cholestasis.