{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,2]],"date-time":"2026-06-02T08:08:50Z","timestamp":1780387730242,"version":"3.54.1"},"reference-count":20,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2026,2,8]],"date-time":"2026-02-08T00:00:00Z","timestamp":1770508800000},"content-version":"vor","delay-in-days":7,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,2,1]],"date-time":"2026-02-01T00:00:00Z","timestamp":1769904000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/501100000780","name":"European Commission","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000780","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Birth Defects Research"],"published-print":{"date-parts":[[2026,2]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Biliary atresia is a rare but severe congenital anomaly associated with substantial morbidity and mortality in early childhood. Population\u2010based 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\u2010based data.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>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\u2010up length.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Our cohort included 171 children, with an infant mortality rate of 12.3% (95% CI: 7.8\u201317.6) and a mortality rate before age five of 18.5% (95% CI: 10.7\u201327.7). Among these children, 151 had undergone surgery, including 133 who received the Kasai procedure by the age of 1\u2009year at a median age of 57\u2009days (95% CI: 51\u201362\u2009days). By age five, 37% (adjusted percentage, 95% CI: 30\u201344) had undergone liver transplantation, with the median age at transplantation being 318\u2009days (95% CI: 244\u2013391\u2009days). Median age at death in the first year was over 6\u2009months and was not immediately after surgery.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>The 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.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/bdr2.70024","type":"journal-article","created":{"date-parts":[[2026,2,9]],"date-time":"2026-02-09T03:54:41Z","timestamp":1770609281000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Children With Biliary Atresia Have Substantial Morbidity in Early Childhood and a High Risk of Liver Transplantation"],"prefix":"10.1002","volume":"118","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7410-8573","authenticated-orcid":false,"given":"Mads","family":"Damkj\u00e6r","sequence":"first","affiliation":[{"name":"Department of Paediatrics and Adolescent Medicine, Lillebaelt Hospital University Hospital of Southern Denmark  Kolding Denmark"},{"name":"Department of Regional Health Research University of Southern Denmark  Kolding Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joachim","family":"Tan","sequence":"additional","affiliation":[{"name":"Population Health Research Institute, St George's University of London  London UK"},{"name":"NIHR GOSH Biomedical Research Centre, UCL Great Ormond Street Institute of Child Health  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1206-3637","authenticated-orcid":false,"given":"Maria","family":"Loane","sequence":"additional","affiliation":[{"name":"Faculty of Life &amp; Health Sciences Ulster University  Northern Ireland UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joanne","family":"Given","sequence":"additional","affiliation":[{"name":"Faculty of Life &amp; Health Sciences Ulster University  Northern Ireland UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisa","family":"Ballardini","sequence":"additional","affiliation":[{"name":"Neonatal Intensive Care Unit, University Hospital of Ferrara, IMER Registry (Emilia Romagna Registry of Birth Defects), Department of Medical Sciences, University of Ferrara  Ferrara Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Clara","family":"Cavero\u2010Carbonell","sequence":"additional","affiliation":[{"name":"Rare Diseases Research Unit, Foundation for the Promotion of Health and Biomedical Research in the Valencian Region  Valencia Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mika","family":"Gissler","sequence":"additional","affiliation":[{"name":"THL Finnish Institute for Health and Welfare, Department of Knowledge Brokers, Helsinki, Finland, Region Stockholm, Academic Primary Health Care Centre, Stockholm, Sweden; Karolinska Institutet, Department of Molecular Medicine and Surgery  Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sue","family":"Jordan","sequence":"additional","affiliation":[{"name":"Faculty of Health and Life Science Swansea University  Swansea UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Pierini","sequence":"additional","affiliation":[{"name":"Unit of Epidemiology of Rare Diseases and Congenital Anomalies, Institute of Clinical Physiology, National Research Council  Pisa Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9437-2790","authenticated-orcid":false,"given":"Anke","family":"Rissmann","sequence":"additional","affiliation":[{"name":"Malformation Monitoring Centre Saxony\u2010Anhalt, Medical Faculty Otto\u2010von\u2010Guericke\u2010University Magdeburg  Magdeburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Tucker","sequence":"additional","affiliation":[{"name":"Congenital Anomaly Registers &amp; Information Service for Wales (CARIS), Public Health Knowledge &amp; Research, Public Health  Swansea UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0430-2594","authenticated-orcid":false,"given":"Ester","family":"Garne","sequence":"additional","affiliation":[{"name":"Department of Paediatrics and Adolescent Medicine, Lillebaelt Hospital University Hospital of Southern Denmark  Kolding Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7164-612X","authenticated-orcid":false,"given":"Joan K.","family":"Morris","sequence":"additional","affiliation":[{"name":"Population Health Research Institute, St George's University of London  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,2,8]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpedsurg.2021.02.027"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPG.0000000000000986"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2013.01.040"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPG.0000000000001334"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/apa.16726"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.3390\/ijerph20054387"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamapediatrics.2016.3470"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1001\/jama.2020.0837"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.4292\/wjgpt.v13.i4.33"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPG.0b013e318282a913"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2022.12.041"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00383-021-05047-1"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmjopen-2020-047859"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPG.0000000000002450"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2019.10.054"},{"key":"e_1_2_10_17_1","first-page":"231","article-title":"Anesthesia Exposure in the Young Child and Long\u2010Term Cognition: An Integrated Review","volume":"87","author":"Rosenblatt A.","year":"2019","journal-title":"AANA Journal"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1440-1746.2007.05114.x"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1440-1746.2007.05235.x"},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0269874"},{"key":"e_1_2_10_21_1","doi-asserted-by":"publisher","DOI":"10.12688\/f1000research.16732.1"}],"container-title":["Birth Defects Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/bdr2.70024","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/bdr2.70024","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/bdr2.70024","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,6,2]],"date-time":"2026-06-02T07:40:38Z","timestamp":1780386038000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/bdr2.70024"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,2]]},"references-count":20,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2026,2]]}},"alternative-id":["10.1002\/bdr2.70024"],"URL":"https:\/\/doi.org\/10.1002\/bdr2.70024","archive":["Portico"],"relation":{},"ISSN":["2472-1727","2472-1727"],"issn-type":[{"value":"2472-1727","type":"print"},{"value":"2472-1727","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,2]]},"assertion":[{"value":"2024-12-13","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-01-01","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-02-08","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e70024"}}