Adjuvant steroids following Kasai portoenterostomy for biliary atresia: a single-center 18-year experience comparing different steroid dose regimens Journal Article


Authors: Liang, N. E.; Danzer, E.; Hyun, J. S.; Chao, S. D.; Bruzoni, M.; Dünn, J. C. Y.
Article Title: Adjuvant steroids following Kasai portoenterostomy for biliary atresia: a single-center 18-year experience comparing different steroid dose regimens
Abstract: Purpose: In 2017, our hospital transitioned to a standardized post-KPE high-dose steroid protocol. We sought to compare outcomes for biliary atresia (BA) for this protocol against historical treatment with no or low-dose steroids. Methods: Between 2006 and 2024, 50 children underwent KPE for BA. Patients were stratified into three groups: no steroids, low-dose steroids (defined by a starting dose of 2–4 mg/kg/day), and high-dose steroids (starting dose of 10 mg/kg/day). After the initial taper, patients in both steroid groups continued with 2 mg/kg/day for 4–6 weeks. Results: 8 patients received no steroids, 21 received low-dose steroids, and 21 received high-dose steroids. Patients treated with high-dose steroids had significantly greater readmission rates compared to the no or low-dose steroid cohorts. There was an overall trend towards improved native liver survival for the high-dose steroid cohort at 1 and 5 years after KPE. There was no significant difference in rates of cholangitis, though the high-dose cohort tended to present with cholangitis within 30 days of discharge. Conclusions: We describe the 18-year experience of adjuvant steroid use in BA patients at a single institution. We show improved early postoperative biliary drainage with high-dose steroid use and identify a trend towards improved native liver survival with high-dose steroids. © 2025 Elsevier B.V., All rights reserved.
Keywords: survival; clinical article; controlled study; treatment outcome; retrospective studies; mortality; dose response; drug dose comparison; drug dose reduction; treatment duration; adjuvant therapy; comparative study; postoperative care; chemotherapy, adjuvant; drug megadose; follow up; low drug dose; cohort analysis; steroid; clinical protocol; dose-response relationship, drug; retrospective study; liver failure; hospitalization; prednisolone; infant; infant, newborn; heart failure; adjuvant chemotherapy; newborn; bile leakage; reoperation; glucocorticoid; surgery; surgical mortality; hospital discharge; hospital readmission; liver enzyme; drug therapy; trend study; liver abscess; drug dose regimen; drug pulse therapy; bilirubin blood level; wound dehiscence; small intestine obstruction; glucocorticoids; gram negative sepsis; hospital mortality; enzyme blood level; steroids; clinical outcome; biloma; corticosteroid therapy; procedures; pediatric surgery; postoperative inflammation; cholangitis; humans; human; male; female; article; biliary atresia; bilirubin glucuronide; body weight change; fascial dehiscence; kasai portoenterostomy; native liver survival; bile duct atresia; heart single ventricle; portoenterostomy; portoenterostomy, hepatic
Journal Title: Pediatric Surgery International
Volume: 41
Issue: 1
ISSN: 14379813
Publisher: Elsevier B.V.  
Date Published: 2025-01-01
Start Page: 258
Language: English
DOI: 10.1007/s00383-025-06162-z
PUBMED: 40830593
PROVIDER: scopus
DOI/URL:
Notes: Article -- Source: Scopus
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