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Children With Biliary Atresia Have Substantial Morbidity in Early Childhood and a High Risk of Liver Transplantation

Mads Damkjær Orcid Logo, Joachim Tan, Maria Loane Orcid Logo, Joanne Given, Elisa Ballardini, Clara Cavero‐Carbonell, Mika Gissler, Sue Jordan, Anna Pierini, Anke Rissmann Orcid Logo, David Tucker, Ester Garne Orcid Logo, Joan K. Morris Orcid Logo

Birth Defects Research, Volume: 118, Issue: 2

Swansea University Author: Sue Jordan

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DOI (Published version): 10.1002/bdr2.70024

Abstract

BackgroundBiliary atresia is a rare but severe congenital anomaly associated with substantial morbidity and mortality in early childhood. Population-based estimates of survival, surgical management, and liver transplantation across Europe remain limited. This study aimed to describe mortality and mo...

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Published in: Birth Defects Research
ISSN: 2472-1727 2472-1727
Published: Wiley 2026
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URI: https://cronfa.swan.ac.uk/Record/cronfa71487
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This study aimed to describe mortality and morbidity among children born with biliary atresia using multinational population-based data.MethodsWe investigated children diagnosed with biliary atresia across nine registries from five countries within the European surveillance of congenital anomalies network (EUROCAT), covering births from 1995 to 2014. The data were linked to hospital databases and adjusted for regional differences and follow-up length.ResultsOur cohort included 171 children, with an infant mortality rate of 12.3% (95% CI: 7.8&#x2013;17.6) and a mortality rate before age five of 18.5% (95% CI: 10.7&#x2013;27.7). Among these children, 151 had undergone surgery, including 133 who received the Kasai procedure by the age of 1&#x2009;year at a median age of 57&#x2009;days (95% CI: 51&#x2013;62&#x2009;days). By age five, 37% (adjusted percentage, 95% CI: 30&#x2013;44) had undergone liver transplantation, with the median age at transplantation being 318&#x2009;days (95% CI: 244&#x2013;391&#x2009;days). Median age at death in the first year was over 6&#x2009;months and was not immediately after surgery.ConclusionThe high mortality and the substantial need for liver transplantation within the first year of life underline the severity of biliary atresia. 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spelling 2026-02-23T15:38:23.4707497 v2 71487 2026-02-23 Children With Biliary Atresia Have Substantial Morbidity in Early Childhood and a High Risk of Liver Transplantation 24ce9db29b4bde1af4e83b388aae0ea1 Sue Jordan Sue Jordan true false 2026-02-23 BackgroundBiliary atresia is a rare but severe congenital anomaly associated with substantial morbidity and mortality in early childhood. Population-based estimates of survival, surgical management, and liver transplantation across Europe remain limited. This study aimed to describe mortality and morbidity among children born with biliary atresia using multinational population-based data.MethodsWe investigated children diagnosed with biliary atresia across nine registries from five countries within the European surveillance of congenital anomalies network (EUROCAT), covering births from 1995 to 2014. The data were linked to hospital databases and adjusted for regional differences and follow-up length.ResultsOur cohort included 171 children, with an infant mortality rate of 12.3% (95% CI: 7.8–17.6) and a mortality rate before age five of 18.5% (95% CI: 10.7–27.7). Among these children, 151 had undergone surgery, including 133 who received the Kasai procedure by the age of 1 year at a median age of 57 days (95% CI: 51–62 days). By age five, 37% (adjusted percentage, 95% CI: 30–44) had undergone liver transplantation, with the median age at transplantation being 318 days (95% CI: 244–391 days). Median age at death in the first year was over 6 months and was not immediately after surgery.ConclusionThe high mortality and the substantial need for liver transplantation within the first year of life underline the severity of biliary atresia. This highlights the urgent need for further research into pregnancy exposures that may contribute to this rare but severe congenital anomaly to develop primary prevention strategies. Journal Article Birth Defects Research 118 2 Wiley 2472-1727 2472-1727 biliary atresia; liver transplantation; morbidity 8 2 2026 2026-02-08 10.1002/bdr2.70024 COLLEGE NANME COLLEGE CODE Swansea University Another institution paid the OA fee European Commission 2026-02-23T15:38:23.4707497 2026-02-23T15:30:23.8014762 Faculty of Medicine, Health and Life Sciences School of Health and Social Care - Nursing Mads Damkjær 0000-0001-7410-8573 1 Joachim Tan 2 Maria Loane 0000-0002-1206-3637 3 Joanne Given 4 Elisa Ballardini 5 Clara Cavero‐Carbonell 6 Mika Gissler 7 Sue Jordan 8 Anna Pierini 9 Anke Rissmann 0000-0002-9437-2790 10 David Tucker 11 Ester Garne 0000-0003-0430-2594 12 Joan K. Morris 0000-0002-7164-612x 13 71487__36288__286a3c7e066645039162c1d6acc0ba73.pdf 71487.VoR.pdf 2026-02-23T15:34:00.4968718 Output 342174 application/pdf Version of Record true © 2026 The Author(s). This is an open access article under the terms of the Creative Commons Attribution License. true Eng http://creativecommons.org/licenses/by/4.0/
title Children With Biliary Atresia Have Substantial Morbidity in Early Childhood and a High Risk of Liver Transplantation
spellingShingle Children With Biliary Atresia Have Substantial Morbidity in Early Childhood and a High Risk of Liver Transplantation
Sue Jordan
title_short Children With Biliary Atresia Have Substantial Morbidity in Early Childhood and a High Risk of Liver Transplantation
title_full Children With Biliary Atresia Have Substantial Morbidity in Early Childhood and a High Risk of Liver Transplantation
title_fullStr Children With Biliary Atresia Have Substantial Morbidity in Early Childhood and a High Risk of Liver Transplantation
title_full_unstemmed Children With Biliary Atresia Have Substantial Morbidity in Early Childhood and a High Risk of Liver Transplantation
title_sort Children With Biliary Atresia Have Substantial Morbidity in Early Childhood and a High Risk of Liver Transplantation
author_id_str_mv 24ce9db29b4bde1af4e83b388aae0ea1
author_id_fullname_str_mv 24ce9db29b4bde1af4e83b388aae0ea1_***_Sue Jordan
author Sue Jordan
author2 Mads Damkjær
Joachim Tan
Maria Loane
Joanne Given
Elisa Ballardini
Clara Cavero‐Carbonell
Mika Gissler
Sue Jordan
Anna Pierini
Anke Rissmann
David Tucker
Ester Garne
Joan K. Morris
format Journal article
container_title Birth Defects Research
container_volume 118
container_issue 2
publishDate 2026
institution Swansea University
issn 2472-1727
2472-1727
doi_str_mv 10.1002/bdr2.70024
publisher Wiley
college_str Faculty of Medicine, Health and Life Sciences
hierarchytype
hierarchy_top_id facultyofmedicinehealthandlifesciences
hierarchy_top_title Faculty of Medicine, Health and Life Sciences
hierarchy_parent_id facultyofmedicinehealthandlifesciences
hierarchy_parent_title Faculty of Medicine, Health and Life Sciences
department_str School of Health and Social Care - Nursing{{{_:::_}}}Faculty of Medicine, Health and Life Sciences{{{_:::_}}}School of Health and Social Care - Nursing
document_store_str 1
active_str 0
description BackgroundBiliary atresia is a rare but severe congenital anomaly associated with substantial morbidity and mortality in early childhood. Population-based estimates of survival, surgical management, and liver transplantation across Europe remain limited. This study aimed to describe mortality and morbidity among children born with biliary atresia using multinational population-based data.MethodsWe investigated children diagnosed with biliary atresia across nine registries from five countries within the European surveillance of congenital anomalies network (EUROCAT), covering births from 1995 to 2014. The data were linked to hospital databases and adjusted for regional differences and follow-up length.ResultsOur cohort included 171 children, with an infant mortality rate of 12.3% (95% CI: 7.8–17.6) and a mortality rate before age five of 18.5% (95% CI: 10.7–27.7). Among these children, 151 had undergone surgery, including 133 who received the Kasai procedure by the age of 1 year at a median age of 57 days (95% CI: 51–62 days). By age five, 37% (adjusted percentage, 95% CI: 30–44) had undergone liver transplantation, with the median age at transplantation being 318 days (95% CI: 244–391 days). Median age at death in the first year was over 6 months and was not immediately after surgery.ConclusionThe high mortality and the substantial need for liver transplantation within the first year of life underline the severity of biliary atresia. This highlights the urgent need for further research into pregnancy exposures that may contribute to this rare but severe congenital anomaly to develop primary prevention strategies.
published_date 2026-02-08T05:33:24Z
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