Outcomes of liver transplantation for Alagille syndrome after Kasai portoenterostomy: Alagille Syndrome with agenesis of extrahepatic bile ducts at porta hepatisJournal of Pediatric Surgery - Tập 54 - Trang 2387 - 2019
Kaneshiro Masakatshu, Ogawa Eri, Okajima Hideaki, Sonoda Mari, Uemoto Shiji, Okamoto Tatsuya, Gunadi
Background Alagille syndrome (ALGS) is an autosomal dominant disorder, characterized by a paucity of intrahepatic bile ducts, resulting in significant cholestasis, and peculiar extrahepatic features. Some ALGS patients show a considerable overlap with biliary atresia (BA), and they can undergo Kasai procedure. The purpose of this study is to show the manifestations of BA overlapped ALGS cases in o...... hiện toàn bộ
#Alagille syndrome #Biliary atresia #Kasai procedure #Liver transplantation #Survival