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Children With Biliary Atresia Have Substantial Morbidity in Early Childhood and a High Risk of Liver Transplantation
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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
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...
| Published in: | Birth Defects Research |
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| ISSN: | 2472-1727 2472-1727 |
| Published: |
Wiley
2026
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| Online Access: |
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| URI: | https://cronfa.swan.ac.uk/Record/cronfa71487 |
| 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 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. |
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| Keywords: |
biliary atresia; liver transplantation; morbidity |
| College: |
Faculty of Medicine, Health and Life Sciences |
| Funders: |
European Commission |
| Issue: |
2 |