title: Steroids after the Kasai procedure for biliary atresia: the effect of age at Kasai portoenterostomy.
abstract: 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.
authors.affiliation: Department of Paediatric Surgery, King's College Hospital, Denmark Hill, London, SE5 9RS, UK.
authors.forename: Athanasios
authors.initials: A
authors.lastname: Tyraskis
authors.affiliation: Department of Paediatric Surgery, King's College Hospital, Denmark Hill, London, SE5 9RS, UK. Markdav2@ntlworld.com.
authors.forename: Mark
authors.initials: M
authors.lastname: Davenport
mesh_terms: D000367:Age Factors; D001656:Biliary Atresia / Q000601:surgery*; D002761:Cholangitis / Q000517:prevention & control*; D005938:Glucocorticoids / Q000494:pharmacology*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008099:Liver / Q000601:surgery; D011171:Portoenterostomy, Hepatic; D011183:Postoperative Complications / Q000517:prevention & control*; D011239:Prednisolone / Q000494:pharmacology*
chemical_list: D005938:Glucocorticoids; D011239:Prednisolone
keywords: Adjuvant steroids; Biliary atresia; Kasai portoenterostomy; Prednisolone