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 diagnosed 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 <34 micromol/L) [corrected]. 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.
Subject(s)
Biliary Atresia/mortality , Biliary Atresia/pathology , Portoenterostomy, Hepatic , Adolescent , Biliary Atresia/epidemiology , Biliary Atresia/surgery , Child , Child, Preschool , Cholangitis/epidemiology , Female , Follow-Up Studies , Humans , Infant , Infant, Newborn , Jaundice/epidemiology , Liver Transplantation , Male , Portoenterostomy, Hepatic/mortality , Prognosis , Survival Rate , Taiwan/epidemiology , Time Factors , Treatment OutcomeABSTRACT
AIM: To examine the differences of clinical behaviors between hepatocellular carcinomas (HCC) and hepatoblastomas (HB) in children. METHODS: From 1979 to 1997, we collected 73 HCC and 54 HB from two major medical centers in Taiwan. Demog-raphic, laboratory and radiological data, and survival curves were statistically compared. RESULTS: HCC clinically differed from HB in mean age (10.6 vs 2.5 years; P<0.001), status of hepatitis B infection (56/56 vs 4/35, P<0.001) and accompanying liver cirrhosis (26/40 vs 0/30, P<0.001), portal vein thrombi (22/56 vs 5/38, P = 0.006) and para-aortic lymphadenopathy (10/56 vs 1/38, P = 0.026). Due to a higher recurrence rate (7/12 vs 2/13, P = 0.041), stage I HCC compared poorly in survivals with stage I HB (P = 0.0183). Chemotherapy could only benefit HB as evidenced by 66.7% of resectability conversion and improve survivals for advanced HB, even with unsuccessful conversion. The survival difference between stage I HB and advanced HB with delayed complete resection was of borderline insignificance (P = 0.0507). CONCLUSION: HCC and HB were preliminarily distinguishable by some clinical clues. Delayed resection after chemotherapy was only possible for HB. However, further studies are needed to strengthen our observation that appropriate reliance upon chemotherapy to subsequently resect advanced HB could achieve the comparable survival to that of stage I HB.
