Central Liver Nodules in Alagille Syndrome and Biliary Atresia After Kasai Portoenterostomy

摘要

Alhammad et al propose that large solitary nodules located close to the right portal vein in children with Alagille syndrome (ALGS) are likely benign if alpha-fetoprotein levels are normal and magnetic resonance imaging shows a central vessel. These nodules display less fibrosis and relative interlobular bile duct preservation, compared to the rest of the liver, which shows either extensive fibrosis or cirrhosis. Similar findings are described in explanted livers after Kasai portoenterostomy (3–6) (Fig. 1), but not in livers from children with biliary atresia (BA) directly referred to transplantation (3). The mechanisms leading to nodular transformation in cholestatic livers are unknown. At the molecular level, JAG1 mosaicism anddifferentialhaploinsufficiencydonotexplainthepresenceofbile ducts in centrally located regenerative nodules in ALGS