Long-Term Prognosis of Patients with Biliary Atresia: A 25 Year Summary *Pei-Yin Hung, †Chiu-Chiang Chen, †Wei-Jao Chen, †Hong-Shiee Lai, †Wen-Ming Hsu, †Po-Hung Lee, †Ming-Chih Ho, ‡Tony H. H. Chen, *Yen-Hsuan Ni, *Huey-Ling Chen, *Hong-Yuan Hsu, and *Mei-Hwei Chang *Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan, †Department of Surgery, National Taiwan University Hospital, Taipei, Taiwan, ‡School of Preventive Medicine, College of Public Health, National Taiwan University, Taipei, Taiwan ABSTRACT Objective: The purpose of this study was to delineate the long- term prognosis of biliary atresia (BA) in Taiwan. Study Design: From 1976 to 2000, 185 children were diag- nosed with BA, 22 underwent exploratory laparotomy without Kasai operation, and 163 underwent Kasai operation, of which 141 cases had long-term follow-up and formed the basis of this study. The outcome was analyzed. Results: Among the 141 BA children studied who underwent Kasai operation, 115 (81.6%) had recoloration of stools, and 86 (61.0%) became jaundice-free (bilirubin ,20 mmol/L). The resolution of jaundice and the absence of repeated cholangitis contributed to better outcome. Five and 10 year survival rates with native liver were 35% and 31%, respectively. Liver transplantation was performed in 19 patients (all but 2 with a living-related donor), and 15 (79%) survived. Five and 10 year overall survival rates for BA patients were 41.9% and 40.2%, respectively. Conclusions: The study delineated the long-term outcome of BA in an Asian country other than Japan. Survival with native liver after a Kasai operation in Taiwan was similar to that in the American and European series. Limited donors for liver transplantation in the years of the study accounted for the poor overall prognosis of BA patients in this series. JPGN 42:190– 195, 2006. Key Words: Biliary atresia—Prognosis—Kasai portoenterostomy. Ó 2006 Lippincott Williams & Wilkins INTRODUCTION Biliary atresia (BA), a progressive obliterative chol- angiopathy, has a fatal outcome if left untreated (1). The incidence of BA in Taiwan is estimated to be 23.3 to 34.5 per 100,000 live births (unpublished personal data, MH Chang et al, infant stool card center, Taipei, Taiwan). This is higher than the 10.4 in Japan (2), 10.6 in Hawaii (3), and close to 29.4 in French Polynesia per 100,000 live births (4). In 1959, Kasai and Suzuki (5) introduced hepatic portoenterostomy to reestablish bile flow in BA. It is now well accepted that early diagnosis and timely surgery have a significant impact on long-term prognosis (2,6,7). Liver transplantation (LT) may be needed in cases of failure of Kasai operation and complications of cirrhosis (6) and is sometimes performed as primary therapy (8). Sequential treatment, by Kasai operation and LT if needed, is currently recommended for BA (9). In places such as Taiwan, where the availability of LT is limited, Kasai operation may be the only chance for survival of BA patients. The prognosis of Kasai operation and LT for BA has been widely reported (1,2,6,10). However, data from Asian countries other than Japan is lacking. The purpose of this study is to delineate the long-term prognosis and outcome of BA in Taiwan. MATERIALS AND METHODS During the period from January 1976 to December 2000, there were 185 cholestatic infants with a definite diagnosis of BA at the National Taiwan University Hospital. The data analyzed were collected up to December 2002. The diagnosis of BA was confirmed using the protocol, including stool color, serum bilirubin level and complete liver function profile, sonog- raphy, hepatobiliary scan, liver histology, as well as intra- operative findings and cholangiography (11). Neonatal hepatitis was excluded by definition in patients with of intrahepatic cholestasis and a patent extrahepatic biliary tree. Parenteral nutrition-related cholangitis was excluded by the absence of parenteral nutrition. a1-Antitrypsin level, and urine and serum amino acid profiles were checked to exclude a metabolic etiology. Alagille syndrome was excluded by failure to find paucity of interlobular bile ducts, characteristic facies, pulmo- nary artery stenosis, vertebral arch defect, and posterior embryotoxon. Received March 12, 2005; accepted October 16, 2005. Address correspondence and reprint requests to Dr. Mei-Hwei Chang, Department of Pediatrics, National Taiwan University Hospital, No.7, Chung-Shan South Road, Taipei, Taiwan (e-mail: mhchang@ ha.mc.ntu.edu.tw). Journal of Pediatric Gastroenterology and Nutrition 42:190–195 Ó February 2006 Lippincott Williams & Wilkins, Philadelphia 190 Copyright ' Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.