Sequential treatment of biliary atresia with kasai portoenterostomy and liver transplantation: A review

摘要: Biliary atresia is the most frequent cause of chronic cholestasis in infants. When left untreated, this condition leads to death from liver insufficiency within first 2 yr life. The modern therapeutic approach consists a sequential strategy with Kasai portoenterostomy as step and, case failure, transplantation. After portoenterostomy, no more than 20% 30% patients will live jaundice-free into adulthood. Illness another third be palliated, and these have extended survival, delaying transplantation later childhood (2 15 yr). remaining 40% not benefit operation die failure infancy. annual need for biliary one per million people. This indication represents 35% 67% reported series pediatric between 5% 10% indications transplantation, all ages included. Approximately four five children transplanted become long-term survivors good physical mental development; recurrence disease after has been observed. Because candidates are young (< 3 yr) small size 10 kg), there shortage size-matched donors (which alleviated by use innovative techniques such reduced split livers). resulting redistribution adult donor pool ethically justified equal quality results full-size or partial graft.