Long-term prognosis of patients with biliary atresia: a 25 year summary.
OBJECTIVE
The purpose of this study was to delineate the long-term prognosis of biliary atresia (BA) in Taiwan.


METHODS
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.
Hung
Pei-Yin
PY
Department of Pediatrics, National Taiwan University Hospital, No. 7 Chung-Shan South Road, Taipei, Taiwan.
Chen
Chiu-Chiang
CC
Chen
Wei-Jao
WJ
Lai
Hong-Shiee
HS
Hsu
Wen-Ming
WM
Lee
Po-Hung
PH
Ho
Ming-Chih
MC
Chen
Tony H H
TH
Ni
Yen-Hsuan
YH
Chen
Huey-Ling
HL
Hsu
Hong-Yuan
HY
Chang
Mei-Hwei
MH
D000293:Adolescent; D001656:Biliary Atresia / Q000453:epidemiology / Q000401:mortality* / Q000473:pathology* / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D002761:Cholangitis / Q000453:epidemiology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007565:Jaundice / Q000453:epidemiology; D016031:Liver Transplantation; D008297:Male; D011171:Portoenterostomy, Hepatic / Q000401:mortality; D011379:Prognosis; D015996:Survival Rate; D013624:Taiwan / Q000453:epidemiology; D013997:Time Factors; D016896:Treatment Outcome