{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,25]],"date-time":"2026-08-25T15:56:58Z","timestamp":1787673418774,"version":"build-2736575974"},"reference-count":39,"publisher":"MDPI AG","issue":"21","license":[{"start":{"date-parts":[[2022,11,3]],"date-time":"2022-11-03T00:00:00Z","timestamp":1667433600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Robert E. Schneider Foundation"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Background: It is impossible to predict which patients with biliary atresia (BA) will fail after Kasai portoenterostomy (KPE). We evaluated the predictive nature of pre-KPE clinical and histological factors on transplant-free survival (TFS) and jaundice clearance. Methods: A retrospective review of patients who received a KPE at our institution (1997\u20132018) was performed. Primary outcomes were two-year TFS, five-year TFS, and jaundice clearance 3 months after KPE. p &lt; 0.05 was considered significant. Results: Fifty-four patients were included in this study. The two-year TFS was 35.1%, five-year TFS was 24.5%, and 37% patients reached a direct bilirubin (DB) \u2264 2.0 mg\/dL 3 months post KPE. The median age at biopsy was younger in the five-year TFS (39.0 (24.5\u201355.5) vs. 56.0 days (51.0\u201367.0), p = 0.011). Patients with DB \u2264 1.0 mg\/dL 3 months after KPE were statistically younger at biopsy (DB \u2264 1.0 44.0 (26.0\u201356.0) vs. DB &gt; 1.0 56.0 days (51.0\u201369.0), p = 0.016). Ductal plate malformation was less frequent in the five-year TFS (16\/17, 94.1%, vs. 1\/17, 5.9%, p = 0.037). Portal fibrosis (19\/23, 82.6%, vs. 4\/23, 17.4%, p = 0.028) and acute cholangitis (6\/7, 85.7%, vs. 1\/7, 14.3%, p = 0.047) occurred less frequently in two-year TFS. Conclusion: Older age at biopsy, acute cholangitis, portal fibrosis, and ductal plate malformation were associated with lower native liver survival. Evaluation in a larger study population is needed to validate these results.<\/jats:p>","DOI":"10.3390\/jcm11216523","type":"journal-article","created":{"date-parts":[[2022,11,3]],"date-time":"2022-11-03T03:53:07Z","timestamp":1667447587000},"page":"6523","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Identification of Early Clinical and Histological Factors Predictive of Kasai Portoenterostomy Failure"],"prefix":"10.3390","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0934-7495","authenticated-orcid":false,"given":"Caroline P.","family":"Lemoine","sequence":"first","affiliation":[{"name":"Division of Transplant and Advanced Hepatobiliary Surgery, Ann & Robert H. Lurie Children\u2019s Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hector","family":"Melin-Aldana","sequence":"additional","affiliation":[{"name":"Department of Pathology, Ann & Robert H. Lurie Children\u2019s Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katherine A.","family":"Brandt","sequence":"additional","affiliation":[{"name":"Division of Transplant and Advanced Hepatobiliary Surgery, Ann & Robert H. Lurie Children\u2019s Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Riccardo","family":"Superina","sequence":"additional","affiliation":[{"name":"Division of Transplant and Advanced Hepatobiliary Surgery, Ann & Robert H. Lurie Children\u2019s Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,11,3]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"493","DOI":"10.1001\/archsurg.1996.01430170039006","article-title":"Long-term results with the Kasai operation for biliary atresia","volume":"131","author":"Karrer","year":"1996","journal-title":"Arch. Surg."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"470","DOI":"10.1002\/lt.23631","article-title":"Liver transplantation for biliary atresia: Does the insurance type really make a difference?","volume":"19","author":"Superina","year":"2013","journal-title":"Liver Transplant."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.jaut.2016.06.005","article-title":"Biliary atresia: A comprehensive review","volume":"73","author":"Lakshminarayanan","year":"2016","journal-title":"J. Autoimmun."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1016\/j.jpeds.2015.11.058","article-title":"Total Serum Bilirubin within 3 Months of Hepatoportoenterostomy Predicts Short-Term Outcomes in Biliary Atresia","volume":"170","author":"Shneider","year":"2016","journal-title":"J. Pediatr."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"1354","DOI":"10.1016\/S0140-6736(04)16045-5","article-title":"Seamless management of biliary atresia in England and Wales (1999\u20132002)","volume":"363","author":"Davenport","year":"2004","journal-title":"Lancet"},{"key":"ref_6","first-page":"S41","article-title":"Sequential treatment of biliary atresia with kasai portoenterostomy and liver transplantation: A review","volume":"20","author":"Otte","year":"1994","journal-title":"Hepatology"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"1297","DOI":"10.1007\/s00383-017-4174-4","article-title":"Biliary atresia and liver transplantation: Results and thoughts for primary liver transplantation in select patients","volume":"33","author":"Superina","year":"2017","journal-title":"Pediatr. Surg. Int."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"347","DOI":"10.1016\/j.dld.2015.11.021","article-title":"Comparison of liver transplantation outcomes in biliary atresia patients with and without prior portoenterostomy: A meta-analysis","volume":"48","author":"Wang","year":"2016","journal-title":"Dig. Liver Dis."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"2184","DOI":"10.1016\/j.jpedsurg.2012.09.002","article-title":"Early surgical outcomes and pathological scoring values of older infants (\u226590 d old) with biliary atresia","volume":"47","author":"Chen","year":"2012","journal-title":"J. Pediatr. Surg."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"381","DOI":"10.1007\/s00383-018-4231-7","article-title":"Clinical and pathological features of patients with biliary atresia who survived for more than 5 years with native liver","volume":"34","author":"Sun","year":"2018","journal-title":"Pediatr. Surg. Int."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"2373","DOI":"10.1016\/j.jpedsurg.2013.08.009","article-title":"The most reliable early predictors of outcome in patients with biliary atresia after Kasai\u2019s operation","volume":"48","author":"Goda","year":"2013","journal-title":"J. Pediatr. Surg."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"461","DOI":"10.1016\/j.jpedsurg.2017.10.001","article-title":"Correlation between gamma-glutamyl transpeptidase activity and outcomes after Kasai portoenterostomy for biliary atresia","volume":"53","author":"Ihn","year":"2018","journal-title":"J. Pediatr. Surg."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"168","DOI":"10.1016\/S0022-3468(97)90173-1","article-title":"Biliary atresia: Should all patients undergo a portoenterostomy?","volume":"32","author":"Azarow","year":"1997","journal-title":"J. Pediatr. Surg."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"357","DOI":"10.1016\/j.cgh.2011.01.003","article-title":"Design and Validation of the Biliary Atresia Research Consortium Histologic Assessment System for Cholestasis in Infancy","volume":"9","author":"Russo","year":"2011","journal-title":"Clin. Gastroenterol. Hepatol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"1704","DOI":"10.1016\/S0140-6736(09)60946-6","article-title":"Biliary atresia","volume":"374","author":"Hartley","year":"2009","journal-title":"Lancet"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"577","DOI":"10.1097\/SLA.0b013e3182300950","article-title":"The Anatomic Pattern of Biliary Atresia Identified at Time of Kasai Hepatoportoenterostomy and Early Postoperative Clearance of Jaundice Are Significant Predictors of Transplant-Free Survival","volume":"254","author":"Superina","year":"2011","journal-title":"Ann. Surg."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"202","DOI":"10.1002\/hep.24023","article-title":"Effects of the infant stool color card screening program on 5-year outcome of biliary atresia in taiwan","volume":"53","author":"Lien","year":"2011","journal-title":"Hepatology"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"659","DOI":"10.1016\/j.jpeds.2007.05.051","article-title":"Biliary Atresia: The Canadian Experience","volume":"151","author":"Schreiber","year":"2007","journal-title":"J. Pediatr."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"1280","DOI":"10.1542\/peds.2008-1949","article-title":"Impact of Age at Kasai Operation on Its Results in Late Childhood and Adolescence: A Rational Basis for Biliary Atresia Screening","volume":"123","author":"Serinet","year":"2009","journal-title":"Pediatrics"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"305","DOI":"10.1177\/000992288502400601","article-title":"Guide to Early Diagnosis of Biliary Obstruction in Infancy","volume":"24","author":"Ferry","year":"1985","journal-title":"Clin. Pediatr."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"763","DOI":"10.1136\/adc.51.10.763","article-title":"Extrahepatic biliary atresia versus neonatal hepatitis. Review of 137 prospectively investigated infants","volume":"51","author":"Mowat","year":"1976","journal-title":"Arch. Dis. Child."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"753","DOI":"10.1016\/j.jpedsurg.2016.02.016","article-title":"The value of preoperative liver biopsy in the diagnosis of extrahepatic biliary atresia: A systematic review and meta-analysis","volume":"51","author":"Lee","year":"2016","journal-title":"J. Pediatr. Surg."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"154","DOI":"10.1097\/MPG.0000000000001334","article-title":"Guideline for the Evaluation of Cholestatic Jaundice in Infants: Joint Recommendations of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition","volume":"64","author":"Fawaz","year":"2017","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"184","DOI":"10.4103\/0971-9261.161040","article-title":"Correlation of pre- and post-operative liver function, duct diameter at porta hepatis, and portal fibrosis with surgical outcomes in biliary atresia","volume":"20","author":"Bhatnagar","year":"2015","journal-title":"J. Indian Assoc. Pediatr. Surg."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"902","DOI":"10.5604\/16652681.1171781","article-title":"The limited prognostic value of liver histology in children with biliary atresia","volume":"14","author":"Czubkowski","year":"2015","journal-title":"Ann. Hepatol."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"123","DOI":"10.1016\/j.jpeds.2003.09.042","article-title":"Hepatic fibrosis and survival in biliary atresia","volume":"144","author":"Weerasooriya","year":"2004","journal-title":"J. Pediatr."},{"key":"ref_27","first-page":"EC23","article-title":"Diagnostic and Prognostic Significance of Various Histopathological Features in Extrahepatic Biliary Atresia","volume":"10","author":"Muthukanagarajan","year":"2016","journal-title":"J. Clin. Diagn. Res."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"275","DOI":"10.1007\/s00383-010-2793-0","article-title":"How valuable is ductal plate malformation as a predictor of clinical course in postoperative biliary atresia patients?","volume":"27","author":"Arii","year":"2010","journal-title":"Pediatr. Surg. Int."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"1781","DOI":"10.1016\/j.transproceed.2014.05.045","article-title":"Grade IV Fibrosis Interferes in Biliary Drainage After Kasai Procedure","volume":"46","author":"Chinen","year":"2014","journal-title":"Transplant. Proc."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"1601","DOI":"10.1097\/PAS.0000000000000755","article-title":"Key Histopathologic Features of Liver Biopsies That Distinguish Biliary Atresia From Other Causes of Infantile Cholestasis and Their Correlation With Outcome","volume":"40","author":"Russo","year":"2016","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"252","DOI":"10.1111\/jpc.13371","article-title":"Clinical significance of liver histology on outcomes in biliary atresia","volume":"53","author":"Webb","year":"2017","journal-title":"J. Paediatr. Child Health"},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"1959","DOI":"10.1002\/hep.24292","article-title":"A classification of ductal plate malformations based on distinct pathogenic mechanisms of biliary dysmorphogenesis","volume":"53","author":"Raynaud","year":"2011","journal-title":"Hepatology"},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"320","DOI":"10.1067\/mpd.2001.117003","article-title":"The prognostic value of ductal plate malformation and other histologic parameters in biliary atresia: An immunohistochemical study","volume":"139","author":"Low","year":"2001","journal-title":"J. Pediatr."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"304","DOI":"10.1016\/j.jpedsurg.2007.10.023","article-title":"Significance of ductal plate malformation in the postoperative clinical course of biliary atresia","volume":"43","author":"Shimadera","year":"2008","journal-title":"J. Pediatr. Surg."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"837","DOI":"10.1007\/s00383-015-3728-6","article-title":"Impact of ductal plate malformation on survival with native liver in children with biliary atresia","volume":"31","author":"Safwan","year":"2015","journal-title":"Pediatr. Surg. Int."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"101","DOI":"10.4103\/0377-4929.59194","article-title":"A histopathological study of liver and biliary remnants with clinical outcome in cases of extrahepatic biliary atresia","volume":"53","author":"Roy","year":"2010","journal-title":"Indian J. Pathol. Microbiol."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"455","DOI":"10.1056\/NEJM199102143240705","article-title":"Syncytial Giant-Cell Hepatitis","volume":"324","author":"Phillips","year":"1991","journal-title":"N. Engl. J. Med."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"48","DOI":"10.1016\/S0022-3468(89)80300-8","article-title":"Biliary atresia: Early determination of prognosis","volume":"24","author":"Stewart","year":"1989","journal-title":"J. Pediatr. Surg."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"364","DOI":"10.1111\/ped.13816","article-title":"Histological significance of hepatitis-like findings in biliary atresia: An analysis of 34 Japanese cases","volume":"61","author":"Suda","year":"2019","journal-title":"Pediatr. Int."}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/11\/21\/6523\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T01:09:46Z","timestamp":1760144986000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/11\/21\/6523"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,11,3]]},"references-count":39,"journal-issue":{"issue":"21","published-online":{"date-parts":[[2022,11]]}},"alternative-id":["jcm11216523"],"URL":"https:\/\/doi.org\/10.3390\/jcm11216523","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,11,3]]}}}