Multicenter Study on Early Predictors of Biliary Atresia Outcomes

Maria Hukkinen; Björn Fischler; Ulrika Liliemark; Vladimir Gatzinsky; Nils Ekvall; Runar Almaas; Hanna Elmi; Omid Madadi-Sanjani; Mikal Obed; Vibeke Brix Christensen; Thora Wesenberg Helt; Lars Soendergaard Johansen; Mark Davenport; Mikko P Pakarinen

doi:10.1016/j.jpedsurg.2025.162404

Multicenter Study on Early Predictors of Biliary Atresia Outcomes

J Pediatr Surg. 2025 Jun 7;60(9):162404. doi: 10.1016/j.jpedsurg.2025.162404. Online ahead of print.

Authors

Affiliations

¹ Section of Pediatric Surgery and Pediatric Liver and Gut Research Group, New Children's Hospital, University of Helsinki and Helsinki University Hospital, Finland; ERN Rare-Liver, France. Electronic address: maria.hukkinen@hus.fi.
² Department of Pediatrics, Karolinska University Hospital and CLINTEC, Karolinska Institutet, Stockholm, Sweden; ERN Rare-Liver, France.
³ Department of Pediatric Surgery, Queen Silvia Children's Hospital, Sahlgrenska University Hospital, Gothenburg, Sweden.
⁴ Department of Pediatrics, Queen Silvia Children's Hospital, Sahlgrenska University Hospital, Gothenburg, Sweden.
⁵ Department of Pediatric Research, Division of Paediatric and Adolescent Medicine, Oslo University Hospital, Pb 4950, Nydalen, Oslo, Norway; Institute of Clinical Medicine, University of Oslo, Oslo, Norway; ERN Rare-Liver, France.
⁶ Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany; ERN Rare-Liver, France.
⁷ Department of Clinical Physiology and Nuclear Medicine, Copenhagen University Hospital Rigshospitalet, Denmark; Department of Transplantation and Digestive Diseases, Copenhagen University Hospital Rigshospitalet, Denmark.
⁸ Department of Transplantation and Digestive Diseases, Copenhagen University Hospital Rigshospitalet, Denmark.
⁹ Department of Pediatric Surgery, King's College Hospital, London, UK.
¹⁰ Section of Pediatric Surgery and Pediatric Liver and Gut Research Group, New Children's Hospital, University of Helsinki and Helsinki University Hospital, Finland; Department of Women's and Children's Health, Karolinska Institute, Stockholm, Sweden; ERN Rare-Liver, France.

PMID: 40490075
DOI: 10.1016/j.jpedsurg.2025.162404

Abstract

Objectives: To compare liver biochemistry, aspartate aminotransferase-to-platelet ratio index (APRI) and patient characteristics for prediction of biliary atresia (BA) outcomes at time of portoenterostomy.

Methods: This was a retrospective observational study. Seven tertiary centers participated. Predictors of clearance of jaundice (COJ, bilirubin <20 μmol/L 6 months after portoenterostomy) and native liver survival (NLS, recorded at transplantation, death, or latest follow-up with NL) were analyzed with logistic regression and Cox regression. Cytomegalovirus (CMV) DNA detection in serum/urine or IgM antibodies were defined as CMV-associated BA.

Results: Out of 630 patients, 58 % achieved COJ. CMV-positive patients were older at portoenterostomy (74 vs 52 days, p < 0.001) whereas BA splenic malformation (BASM) patients were younger (median 47 vs 56 days, p = 0.001) and had equal COJ rates (56 %) compared to others. Absence of cirrhosis (p = 0.001) and CMV infection (p = 0.001), age <60 days at portoenterostomy (p < 0.001), and lower AST (p = 0.007-0.037) and APRI (p = 0.016) predicted COJ. While these variables also predicted NLS, in multivariable regression only COJ (hazard ratio, HR 15, p < 0.001), BASM (HR 1.9, p < 0.001), and portoenterostomy age >60 days (HR 1.3, p = 0.044) remained significant predictors of transplantation/death. Among COJ patients, only BASM predicted transplantation/death (HR 2.4, p = 0.002).

Conclusions: APRI is not superior to AST in the prediction of BA outcomes. However, in COJ patients, both are unrelated to NLS. Despite younger age at portoenterostomy and similar COJ rates compared to isolated BA, BASM strongly predicts shorter NLS. COJ is possible in CMV positive patients who, after achieving COJ, have similar NLS compared to other patients.

Keywords: Aspartate aminotransferase to platelet ratio-index; Biliary atresia; Clearance of jaundice; Cytomegalovirus; Liver transplantation; Native liver survival; Splenic malformation.