The use of adjuvant
steroids following Kasai porteoenterostomy (KPE) for
biliary atresia is controversial. The aim of this study was twofold: a systematic review of published literature and an update of the clinical Kings College Hospital series to look for evidence of an effect of age on the outcome in a group of BA infants treated with high-dose
steroids. This clinical study included infants treated between January 2006 and June 2014 who underwent KPE by day 70 of life and who received high-dose
steroids (oral
prednisolone starting 5 mg/kg/day). They were subdivided into cohorts according to age at which KPE was performed. The outcome measured was clearance of
jaundice (<20 µmol/L) by 6 months and native liver survival. R × C χ(2) analysis and log-rank tests were used, respectively, and P ≤ 0.05 was regarded as significant. 104 infants were included with a median age at KPE of 45 (range 12-70) days. 71/104 (67 %) cleared their
jaundice by 6 months of age. Age-cohort analysis showed a trend (P = 0.03) favouring early KPE (e.g. 100 % of 11 infants operated on <30 days clearing their
jaundice compared to 66 % of those operated on between 61 and 70 days). There was a significant native liver survival benefit for those operated on <45 days (5 year NLS estimate 69 versus 46 %; P = 0.05). Clearance of
jaundice is related to the age at KPE in infants who receive high-dose
steroids. Native liver survival appears to be improved as a result of this. This is the first study to show tangible longer-term benefit from high-dose
steroids in
biliary atresia.