Children with biliary atresia have substantial morbidity in early childhood and a high risk of liver transplantation

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

doi:10.1002/bdr2.70024

Children with biliary atresia have substantial morbidity in early childhood and a high risk of liver transplantation

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

Public Health Wales NHS Trust

Research output: Contribution to journal › Article › peer-review

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Abstract

Background:
Biliary 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.
Methods:
We 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.
Results:
Our 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.
Conclusion:
The high mortality and the substantial need for liver transplantation within the first year of life underline the se-verity 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.

Original language	English
Article number	e70024
Pages (from-to)	1-6
Number of pages	6
Journal	Birth Defects Research
Volume	118
Issue number	2
Early online date	8 Feb 2026
DOIs	https://doi.org/10.1002/bdr2.70024
Publication status	Published (in print/issue) - 8 Feb 2026

Bibliographical note

Data Access Statement

The data that support the findings of this study are available from the participating registries of congenital anomalies, but restrictions apply to the availability of these data, which were used under license for the current study. These data are available for scientifically valid requests and with permission of the participating registries of congenital anomalies. To apply for the data please contact the corresponding author.

Funding

This project has received funding from the European Union’s Horizon 2020 research and innovation programme under grant agreement No. 733001. The funders had no role in the study.

Funder number
733001

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

SDG 3 Good Health and Well-being

Keywords

Morbidity
Liver transplantation
Biliary Atresia
Congenital Anomalies
Data linkage
biliary atresia
morbidity
liver transplantation
Humans
Risk Factors
Child, Preschool
Biliary Atresia/surgery
Infant
Male
Europe/epidemiology
Female
Registries
Infant, Newborn
Liver Transplantation/methods

Access to Document

10.1002/bdr2.70024

Birth Defects Research - 2026 - Damkjær - Children With Biliary Atresia Have Substantial Morbidity in Early Childhood and aFinal published version, 334 KBLicence: CC BY

Cite this

Damkjær, M., Tan, J., Loane, M., Given, J., Ballardini, E., Cavero‑Carbonell, C., Mika, G., Jordan, S., Pierini, A., Rissmann, A., Tucker, D., Garne, E., & Morris, J. K. (2026). Children with biliary atresia have substantial morbidity in early childhood and a high risk of liver transplantation. Birth Defects Research, 118(2), 1-6. Article e70024. https://doi.org/10.1002/bdr2.70024

@article{5587ff11194a4e418cee184dcd2413c4,

title = "Children with biliary atresia have substantial morbidity in early childhood and a high risk of liver transplantation",

abstract = "Background: Biliary 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. Methods: We 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. Results: Our 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. Conclusion: The high mortality and the substantial need for liver transplantation within the first year of life underline the se-verity 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.",

keywords = "Morbidity, Liver transplantation, Biliary Atresia, Congenital Anomalies, Data linkage, biliary atresia, morbidity, liver transplantation, Humans, Risk Factors, Child, Preschool, Biliary Atresia/surgery, Infant, Male, Europe/epidemiology, Female, Registries, Infant, Newborn, Liver Transplantation/methods",

author = "Mads Damkj{\ae}r and Joachim Tan and Maria Loane and Joanne Given and Elisa Ballardini and Clara Cavero‑Carbonell and Gissler Mika and Sue Jordan and Anna Pierini and Anke Rissmann and David Tucker and Ester Garne and Morris, \{Joan K.\}",

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Damkjær, M, Tan, J, Loane, M , Given, J, Ballardini, E, Cavero‑Carbonell, C, Mika, G, Jordan, S, Pierini, A, Rissmann, A, Tucker, D, Garne, E & Morris, JK 2026, 'Children with biliary atresia have substantial morbidity in early childhood and a high risk of liver transplantation', Birth Defects Research, vol. 118, no. 2, e70024, pp. 1-6. https://doi.org/10.1002/bdr2.70024

TY - JOUR

T1 - Children with biliary atresia have substantial morbidity in early childhood and a high risk of liver transplantation

AU - Damkjær, Mads

AU - Tan, Joachim

AU - Loane, Maria

AU - Given, Joanne

AU - Ballardini, Elisa

AU - Cavero‑Carbonell, Clara

AU - Mika, Gissler

AU - Jordan, Sue

AU - Pierini, Anna

AU - Rissmann, Anke

AU - Tucker, David

AU - Garne, Ester

AU - Morris, Joan K.

PY - 2026/2/8

Y1 - 2026/2/8

N2 - Background: Biliary 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. Methods: We 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. Results: Our 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. Conclusion: The high mortality and the substantial need for liver transplantation within the first year of life underline the se-verity 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.

AB - Background: Biliary 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. Methods: We 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. Results: Our 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. Conclusion: The high mortality and the substantial need for liver transplantation within the first year of life underline the se-verity 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.

KW - Morbidity

KW - Liver transplantation

KW - Biliary Atresia

KW - Congenital Anomalies

KW - Data linkage

KW - biliary atresia

KW - morbidity

KW - liver transplantation

KW - Humans

KW - Risk Factors

KW - Child, Preschool

KW - Biliary Atresia/surgery

KW - Infant

KW - Male

KW - Europe/epidemiology

KW - Female

KW - Registries

KW - Infant, Newborn

KW - Liver Transplantation/methods

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UR - https://www.scopus.com/pages/publications/105029570087

U2 - 10.1002/bdr2.70024

DO - 10.1002/bdr2.70024

M3 - Article

C2 - 41656190

SN - 2472-1727

VL - 118

SP - 1

EP - 6

JO - Birth Defects Research

JF - Birth Defects Research

IS - 2

M1 - e70024

ER -

Children with biliary atresia have substantial morbidity in early childhood and a high risk of liver transplantation

Abstract

Bibliographical note

Data Access Statement

Funding

UN SDGs

Keywords

Access to Document

Other files and links

Fingerprint

EUROlinkCAT

Cite this

Children with biliary atresia have substantial morbidity in early childhood and a high risk of liver transplantation

Abstract

Bibliographical note

Data Access Statement

Funding

UN SDGs

Keywords

Access to Document

Other files and links

Fingerprint

Projects

EUROlinkCAT

Cite this