Abstract
Objective. The dosage, duration, and the benefits of high-dose steroid treatment and outcome in biliary atresia (BA) remain controversial. In this study, we evaluated the impact of high-dose steroid therapy on the outcome of BA after the Kasai procedure. Methods. Intravenous prednisolone administration was started 1 week after surgery, followed by 8 to 12 weeks of oral prednisolone. Total bilirubin (TB) levels (3, 6, and 12 months after surgery), early onset of cholangitis, and two-year native liver survival were evaluated. Results. 53.4%, 56.9%, and 58.1% of the patients in the high-dose steroid group were jaundice-free 3, 6, and 12 months after surgery, respectively; these values were significantly higher than the 38.7%, 39.4%, and 43.3% of the low-dose steroid group. One year after surgery, the incidence of cholangitis in the high-dose group (32.0%) was lower than that in the low-dose group (48.0%). Infants with native liver in the high-dose group had a better two-year survival compared to those in the low-dose steroid group (53.7% versus 42.6%). Conclusions. The high-dose steroid protocol can reduce the incidence of cholangitis, increase the jaundice-free rate, and improve two-year survival with native liver after the Kasai operation.
Highlights
Biliary atresia (BA) is a progressive fibroobliterative disease of the biliary tract occurring in infants [1]
Twelve children in the low-dose steroid group and 7 in the high-dose group were lost to follow-up after 12 months and were included in the analysis of the postoperative bilirubin level and incidence of cholangitis but not in the analysis of the survival with native liver rates
Karrer and Lilly [15] proposed using a very highdose “blast-” type steroid treatment, which is supported by potential choleretic and anti-inflammatory properties that may benefit children with cholangitis
Summary
Biliary atresia (BA) is a progressive fibroobliterative disease of the biliary tract occurring in infants [1]. The survival rate in BA infant patients with native liver is less than 10% at 2 to 3 years following diagnosis without certain form of biliary drainage procedure [2]. Some studies have demonstrated improved outcome with routine postsurgery steroid therapy [3,4,5,6,7] These studies used different maximum doses of steroids (1–10 mg/kg/d), a distinct dosetapering schedule, and various treatment times (generally 1–3 months). Some of these studies showed that steroid therapy was not beneficial for BA patients [8, 9]. Due to the distinct regimens used and discrepancy of results obtained, the efficacy and safety of corticosteroids in improving biliary drainage remain controversial
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