{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T20:33:06Z","timestamp":1790109186963,"version":"4.0.1"},"reference-count":32,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2021,5,27]],"date-time":"2021-05-27T00:00:00Z","timestamp":1622073600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2021,5,27]],"date-time":"2021-05-27T00:00:00Z","timestamp":1622073600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Sci Rep"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>We present a 37\u00a0years\u2019 experience in the management of biliary atresia (BA) and discuss long-term complications after Kasai portoenterostomy (KPE). A retrospective territory-wide study from 1980 to 2017 on 231 patients with open KPE from three tertiary paediatric surgical centres was performed. Outcome parameters were clearance of jaundice (COJ), native liver survival (NLS) and long-term complications. Factors affecting the operative outcomes were analyzed. The median duration of follow up was 17.5 (IQR: 13.5\u201322) years. Over 66% of patients became jaundice-freed at 1\u00a0year after KPE. Seventy patients (30.3%) received liver transplant (LT) at a median age of 6.2 (IQR: 4.3\u20138.4) years. The NLS rates at 10 and 20\u00a0years were 70.7% and 61.5% respectively with no significant change over the study period. The median age at KPE was 59 (IQR: 49\u201367) days. KPE performed before 70\u00a0days was associated with higher odd ratios for successful drainage but the age of KPE did not have an impact on the long-term NLS. Among all native liver survivors (n\u2009=\u2009153), the median bilirubin level was 24 (IQR: 16\u201336) \u00b5mol\/L. Portal hypertension (PHT) and recurrent cholangitis were found in 51.6% and 27.5% of them respectively. With a vigilant follow up program, more than 60% of BA patients could remain stable with the disease and achieve long-term survival without LT. Although cholestasis, portal hypertension and recurrent cholangitis are common in long-term NLS, with a comprehensive follow management strategy, they do not always necessitate LT. Our study serves as an example for countries where deceased donor organs are scarce due to very low donation rate.<\/jats:p>","DOI":"10.1038\/s41598-021-90860-w","type":"journal-article","created":{"date-parts":[[2021,5,27]],"date-time":"2021-05-27T06:16:36Z","timestamp":1622096196000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":34,"title":["Life long follow up and management strategies of patients living with native livers after Kasai portoenterostomy"],"prefix":"10.1038","volume":"11","author":[{"given":"Patrick Ho Yu","family":"Chung","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edwin Kin Wai","family":"Chan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fanny","family":"Yeung","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Albert Chi Yan","family":"Chan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer Wai Cheung","family":"Mou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kim Hung","family":"Lee","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Judy Wing Suet","family":"Hung","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael Wai Yip","family":"Leung","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul Kwong Hang","family":"Tam","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kenneth Kak Yuen","family":"Wong","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,5,27]]},"reference":[{"issue":"4","key":"90860_CR1","doi-asserted-by":"publisher","first-page":"150950","DOI":"10.1016\/j.sempedsurg.2020.150950","volume":"29","author":"PHY Chung","year":"2020","unstructured":"Chung, P. H. Y., Zheng, S. & Tam, P. K. H. Biliary atresia: east versus west. Semin. Pediatr. Surg. 29(4), 150950 (2020).","journal-title":"Semin. Pediatr. Surg."},{"issue":"10099","key":"90860_CR2","doi-asserted-by":"publisher","first-page":"1072","DOI":"10.1016\/S0140-6736(17)32284-5","volume":"390","author":"PKH Tam","year":"2017","unstructured":"Tam, P. K. H. et al. Advances in paediatric gastroenterology. Lancet 390(10099), 1072\u20131082 (2017).","journal-title":"Lancet"},{"issue":"12","key":"90860_CR3","doi-asserted-by":"publisher","first-page":"1319","DOI":"10.1007\/s00383-017-4160-x","volume":"33","author":"M Nio","year":"2017","unstructured":"Nio, M. Japanese biliary atresia registry. Pediatr. Surg. Int. 33(12), 1319\u20131325 (2017).","journal-title":"Pediatr. Surg. Int."},{"issue":"9","key":"90860_CR4","doi-asserted-by":"publisher","first-page":"1689","DOI":"10.1016\/j.jpedsurg.2011.04.013","volume":"46","author":"M Davenport","year":"2011","unstructured":"Davenport, M. et al. Biliary atresia in England and Wales: results of centralization and new benchmark. J. Pediatr. Surg. 46(9), 1689\u20131694 (2011).","journal-title":"J. Pediatr. Surg."},{"issue":"4","key":"90860_CR5","doi-asserted-by":"publisher","first-page":"344","DOI":"10.1097\/MPG.0b013e318282a913","volume":"56","author":"C Jimenez-Rivera","year":"2013","unstructured":"Jimenez-Rivera, C., Jolin-Dahel, K. S., Fortinsky, K. J., Gozdyra, P. & Benchimol, E. I. International incidence and outcomes of biliary atresia. J. Pediatr. Gastroenterol. Nutr. 56(4), 344\u2013354 (2013).","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"issue":"1","key":"90860_CR6","doi-asserted-by":"publisher","first-page":"96","DOI":"10.1002\/lt.24640","volume":"23","author":"SS Sundaram","year":"2017","unstructured":"Sundaram, S. S., Mack, C. L., Feldman, A. G. & Sokol, R. J. Biliary atresia: Indications and timing of liver transplantation and optimization of pretransplant care. Liver Transpl. 23(1), 96\u2013109 (2017).","journal-title":"Liver Transpl."},{"issue":"2","key":"90860_CR7","doi-asserted-by":"publisher","first-page":"244","DOI":"10.1002\/lt.24677","volume":"23","author":"T Miloh","year":"2017","unstructured":"Miloh, T. et al. Immunosuppression in pediatric liver transplant recipients: unique aspects. Liver Transpl. 23(2), 244\u2013256 (2017).","journal-title":"Liver Transpl."},{"issue":"6","key":"90860_CR8","doi-asserted-by":"publisher","first-page":"F451","DOI":"10.1136\/adc.2009.159780","volume":"94","author":"E Livesey","year":"2009","unstructured":"Livesey, E. et al. Epidemiology of biliary atresia in England and Wales (1999\u20132006). Arch. Dis. Child Fetal Neonatal Ed. 94(6), F451\u2013F455 (2009).","journal-title":"Arch. Dis. Child Fetal Neonatal Ed."},{"issue":"6","key":"90860_CR9","doi-asserted-by":"publisher","first-page":"659","DOI":"10.1016\/j.jpeds.2007.05.051","volume":"151","author":"RA Schreiber","year":"2007","unstructured":"Schreiber, R. A. et al. Biliary atresia: the Canadian experience. J. Pediatr. 151(6), 659\u2013665 (2007).","journal-title":"J. Pediatr."},{"issue":"7","key":"90860_CR10","doi-asserted-by":"publisher","first-page":"997","DOI":"10.1016\/S0022-3468(03)00178-7","volume":"38","author":"M Nio","year":"2003","unstructured":"Nio, M. et al. Five- and 10-year survival rates after surgery for biliary atresia: a report from the Japanese Biliary Atresia Registry. J. Pediatr. Surg. 38(7), 997\u20131000 (2003).","journal-title":"J. Pediatr. Surg."},{"issue":"4","key":"90860_CR11","doi-asserted-by":"publisher","first-page":"1233","DOI":"10.1002\/hep.22182","volume":"47","author":"CH Hsiao","year":"2008","unstructured":"Hsiao, C. H. et al. Universal screening for biliary atresia using an infant stool color card in Taiwan. Hepatology 47(4), 1233\u20131240 (2008).","journal-title":"Hepatology"},{"issue":"6","key":"90860_CR12","doi-asserted-by":"publisher","first-page":"605","DOI":"10.1007\/s00383-014-3499-5","volume":"30","author":"KW Chan","year":"2014","unstructured":"Chan, K. W. et al. From laparoscopic to open Kasai portoenterostomy: the outcome after reintroduction of open Kasai portoenterostomy in infant with biliary atresia. Pediatr. Surg. Int. 30(6), 605\u2013608 (2014).","journal-title":"Pediatr. Surg. Int."},{"issue":"8","key":"90860_CR13","doi-asserted-by":"publisher","first-page":"931","DOI":"10.1007\/s00383-008-2190-0","volume":"24","author":"KK Wong","year":"2008","unstructured":"Wong, K. K., Chung, P. H., Chan, K. L., Fan, S. T. & Tam, P. K. Should open Kasai portoenterostomy be performed for biliary atresia in the era of laparoscopy?. Pediatr. Surg. Int. 24(8), 931\u2013933 (2008).","journal-title":"Pediatr. Surg. Int."},{"issue":"2","key":"90860_CR14","doi-asserted-by":"publisher","first-page":"264","DOI":"10.1016\/j.jpedsurg.2016.11.022","volume":"52","author":"MH Hussain","year":"2017","unstructured":"Hussain, M. H., Alizai, N. & Patel, B. Outcomes of laparoscopic Kasai portoenterostomy for biliary atresia: a systematic review. J. Pediatr. Surg. 52(2), 264\u2013267 (2017).","journal-title":"J. Pediatr. Surg."},{"issue":"2","key":"90860_CR15","doi-asserted-by":"publisher","first-page":"229","DOI":"10.1089\/lap.2017.0535","volume":"28","author":"SY Huang","year":"2018","unstructured":"Huang, S. Y., Yeh, C. M., Chen, H. C. & Chou, C. M. Reconsideration of laparoscopic Kasai operation for biliary atresia. J. Laparoendosc. Adv. Surg. Tech. A. 28(2), 229\u2013234 (2018).","journal-title":"J. Laparoendosc. Adv. Surg. Tech. A."},{"issue":"8","key":"90860_CR16","doi-asserted-by":"publisher","first-page":"3375","DOI":"10.1007\/s00464-019-07108-y","volume":"34","author":"C Shirota","year":"2020","unstructured":"Shirota, C. et al. Laparoscopic Kasai portoenterostomy is advantageous over open Kasai portoenterostomy in subsequent liver transplantation. Surg. Endosc. 34(8), 3375\u20133381 (2020).","journal-title":"Surg. Endosc."},{"issue":"4","key":"90860_CR17","doi-asserted-by":"publisher","first-page":"150948","DOI":"10.1016\/j.sempedsurg.2020.150948","volume":"29","author":"M Samyn","year":"2020","unstructured":"Samyn, M. Transitional care of biliary atresia. Semin. Pediatr. Surg. 29(4), 150948 (2020).","journal-title":"Semin. Pediatr. Surg."},{"issue":"2","key":"90860_CR18","doi-asserted-by":"publisher","first-page":"186","DOI":"10.1136\/archdischild-2015-310022","volume":"103","author":"T Grammatikopoulos","year":"2018","unstructured":"Grammatikopoulos, T., McKiernan, P. J. & Dhawan, A. Portal hypertension and its management in children. Arch. Dis. Child. 103(2), 186\u2013191 (2018).","journal-title":"Arch. Dis. Child."},{"issue":"9","key":"90860_CR19","doi-asserted-by":"publisher","first-page":"1346","DOI":"10.1002\/hep4.1560","volume":"4","author":"AF Kassam","year":"2020","unstructured":"Kassam, A. F. et al. Natural course of pediatric portal hypertension. Hepatol. Commun. 4(9), 1346\u20131352 (2020).","journal-title":"Hepatol. Commun."},{"issue":"5","key":"90860_CR20","doi-asserted-by":"publisher","first-page":"664","DOI":"10.1097\/MPG.0000000000000710","volume":"60","author":"M Duche","year":"2015","unstructured":"Duche, M., Ducot, B., Ackermann, O., Jacquemin, E. & Bernard, O. Progression to high-risk gastroesophageal varices in children with biliary atresia with low-risk signs at first endoscopy. J. Pediatr. Gastroenterol. Nutr. 60(5), 664\u2013668 (2015).","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"issue":"5","key":"90860_CR21","doi-asserted-by":"publisher","first-page":"537","DOI":"10.1097\/MPG.0b013e318282a22c","volume":"56","author":"C Wanty","year":"2013","unstructured":"Wanty, C., Helleputte, T., Smets, F., Sokal, E. M. & Stephenne, X. Assessment of risk of bleeding from esophageal varices during management of biliary atresia in children. J. Pediatr. Gastroenterol. Nutr. 56(5), 537\u2013543 (2013).","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"issue":"1","key":"90860_CR22","doi-asserted-by":"publisher","first-page":"71","DOI":"10.1016\/j.jhep.2019.03.005","volume":"71","author":"V Jain","year":"2019","unstructured":"Jain, V. et al. Prognostic markers at adolescence in patients requiring liver transplantation for biliary atresia in adulthood. J. Hepatol. 71(1), 71\u201377 (2019).","journal-title":"J. Hepatol."},{"issue":"3","key":"90860_CR23","doi-asserted-by":"publisher","first-page":"382","DOI":"10.1002\/hep4.1306","volume":"3","author":"M Hukkinen","year":"2019","unstructured":"Hukkinen, M. et al. Noninvasive evaluation of liver fibrosis and portal hypertension after successful portoenterostomy for biliary atresia. Hepatol. Commun. 3(3), 382\u2013391 (2019).","journal-title":"Hepatol. Commun."},{"issue":"3","key":"90860_CR24","doi-asserted-by":"publisher","first-page":"254","DOI":"10.1097\/MPG.0b013e318205993a","volume":"52","author":"SC Ling","year":"2011","unstructured":"Ling, S. C. et al. Primary prophylaxis of variceal hemorrhage in children with portal hypertension: a framework for future research. J. Pediatr. Gastroenterol. Nutr. 52(3), 254\u2013261 (2011).","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"issue":"2","key":"90860_CR25","doi-asserted-by":"publisher","first-page":"171","DOI":"10.1097\/MPG.0000000000002555","volume":"70","author":"SH Baek","year":"2020","unstructured":"Baek, S. H. et al. The epidemiology and etiology of cholangitis after Kasai portoenterostomy in patients with biliary atresia. J. Pediatr. Gastroenterol. Nutr. 70(2), 171\u2013177 (2020).","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"issue":"12","key":"90860_CR26","doi-asserted-by":"publisher","first-page":"2409","DOI":"10.1016\/j.jpedsurg.2018.08.028","volume":"53","author":"PHY Chung","year":"2018","unstructured":"Chung, P. H. Y., Tam, P. K. H. & Wong, K. K. Y. Does the identity of the bacteria matter in post-Kasai cholangitis? A comparison between simple and intractable cholangitis. J. Pediatr. Surg. 53(12), 2409\u20132411 (2018).","journal-title":"J. Pediatr. Surg."},{"issue":"5","key":"90860_CR27","doi-asserted-by":"publisher","first-page":"355","DOI":"10.4174\/jkss.2011.80.5.355","volume":"80","author":"JH Shin","year":"2011","unstructured":"Shin, J. H., Chang, E. Y., Chang, H. K., Kim, S. M. & Han, S. J. Home intravenous antibiotic treatment for intractable cholangitis in patients with biliary atresia following Kasai portoenterostomies. J. Korean Surg. Soc. 80(5), 355\u2013361 (2011).","journal-title":"J. Korean Surg. Soc."},{"issue":"5","key":"90860_CR28","doi-asserted-by":"publisher","first-page":"295","DOI":"10.1053\/j.sempedsurg.2017.09.003","volume":"26","author":"A Kelay","year":"2017","unstructured":"Kelay, A. & Davenport, M. Long-term outlook in biliary atresia. Semin. Pediatr. Surg. 26(5), 295\u2013300 (2017).","journal-title":"Semin. Pediatr. Surg."},{"issue":"1","key":"90860_CR29","doi-asserted-by":"publisher","first-page":"112","DOI":"10.1097\/MPG.0000000000000431","volume":"59","author":"RH Squires","year":"2014","unstructured":"Squires, R. H. et al. Evaluation of the pediatric patient for liver transplantation: 2014 practice guideline by the American Association for the Study of Liver Diseases, American Society of Transplantation and the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition. J. Pediatr. Gastroenterol. Nutr. 59(1), 112\u2013131 (2014).","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"issue":"4","key":"90860_CR30","doi-asserted-by":"publisher","first-page":"570","DOI":"10.1097\/MPG.0000000000001854","volume":"66","author":"CWY Wong","year":"2018","unstructured":"Wong, C. W. Y., Chung, P. H. Y., Tam, P. K. H. & Wong, K. K. Y. Long-term results and quality of life assessment in biliary atresia patients: a 35-year experience in a tertiary hospital. J. Pediatr. Gastroenterol. Nutr. 66(4), 570\u2013574 (2018).","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"issue":"9","key":"90860_CR31","doi-asserted-by":"publisher","first-page":"1892","DOI":"10.1016\/j.jpedsurg.2019.10.016","volume":"55","author":"PHY Chung","year":"2020","unstructured":"Chung, P. H. Y., Chok, K. S. H., Wong, K. K. Y., Tam, P. K. H. & Lo, C. M. Determining the optimal timing of liver transplant for pediatric patients after Kasai portoenterostomy based on disease severity scores. J. Pediatr. Surg. 55(9), 1892\u20131896 (2020).","journal-title":"J. Pediatr. Surg."},{"issue":"4","key":"90860_CR32","doi-asserted-by":"publisher","first-page":"467","DOI":"10.1016\/j.jpeds.2005.12.054","volume":"148","author":"BL Shneider","year":"2006","unstructured":"Shneider, B. L. et al. A multicenter study of the outcome of biliary atresia in the United States, 1997 to 2000. J. Pediatr. 148(4), 467\u2013474 (2006).","journal-title":"J. Pediatr."}],"container-title":["Scientific Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.nature.com\/articles\/s41598-021-90860-w.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.nature.com\/articles\/s41598-021-90860-w","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.nature.com\/articles\/s41598-021-90860-w.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,12,3]],"date-time":"2022-12-03T03:59:08Z","timestamp":1670039948000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.nature.com\/articles\/s41598-021-90860-w"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,5,27]]},"references-count":32,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2021,12]]}},"alternative-id":["90860"],"URL":"https:\/\/doi.org\/10.1038\/s41598-021-90860-w","relation":{},"ISSN":["2045-2322"],"issn-type":[{"value":"2045-2322","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,5,27]]},"assertion":[{"value":"27 January 2021","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"18 May 2021","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"27 May 2021","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"The authors declare no competing interests.","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"11207"}}