Immunosuppression as adjuvant therapy for biliary atresia

Background/Purpose: Despite improvements in the surgical management of bili ary atresia, the long-term incidence of progressive liver failure remains h igh. Because chronic inflammation involving both bile ducts and liver paren chyma contributes to the pathology, the authors have hypothesized that the liver damage may be altered using immunosuppressive therapy. The aim of thi s study was to examine the safety and efficacy of long-term steroid therapy in patients with biliary atresia. Methods: A retrospective analysis of all patients with biliary atresia trea ted with an hepatoportoenterostomy and postoperative steroid therapy at our 3 institutions was undertaken. Patients were treated uniformly with immuno suppressive doses of oral steroids for a minimum of 6 weeks after surgery. Results: Twenty-five infants with biliary atresia were treated with steroid therapy. Overall survival rate was 22 patients (88%) with a mean follow-up period of 50 months. Nineteen patients (76%) became jaundice free with nat ive liver function. Four patients (16%) did not respond to treatment and re quired transplantation. Age less than 12 weeks was a crucial predictor of s uccess of adjuvant steroid therapy. Cholangitis developed in 8 patients (32 %). There were no complications caused by steroid therapy. Conclusions: Steroid administration at immunosuppressive doses markedly imp roves the clinical outcome within the first 5 years after surgery as measur ed by jaundice-free status and survival without liver transplantation when compared with concurrent reports. These results suggest that immunosuppress ive therapy is safe and has a positive impact on the clinical course of thi s disease. However, a randomized study is needed to ultimately prove such a n hypothesis. J Pediatr Surg 36:80-85. Copyright (C) 2001 by W.B. Saunders Company.