Long-Term Prognosis of Patients with Biliary Atresia : A 25 Year Summary
Resource
JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION v.42 n.2 pp.190- 195
Journal
JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION
Journal Volume
v.42
Journal Issue
195
Pages
-
Date Issued
2006
Date
2006
Author(s)
HUNG, PEI-YIN
CHEN, CHIU-CHIANG
CHEN, WEI-JAO
LAI, HONG-SHIEE
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 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 μmol/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 delineted 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.
Subjects
Biliary atresia
Prognosis
Kasai portoenterostomy
Type
journal article