{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T12:43:41Z","timestamp":1753879421588,"version":"3.41.2"},"reference-count":20,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2023,7,7]],"date-time":"2023-07-07T00:00:00Z","timestamp":1688688000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Transplantation"],"published-print":{"date-parts":[[2023,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Progressive familial intrahepatic cholestasis type 1 (PFIC1) is an autosomal recessive cholestatic liver disorder caused by <jats:italic>ATP8B1<\/jats:italic> gene mutations. Although liver transplantation (LT) is indicated for progressive liver disease, postoperative complications, including severe diarrhea and graft steatohepatitis leading to graft loss, have been reported.<\/jats:p><\/jats:sec><jats:sec><jats:title>Cases<\/jats:title><jats:p>The first patient had jaundice, pruritus, diarrhea, and growth retardation (weight z\u2010score: \u22122.5; height z\u2010score: \u22123.7). She underwent LT with total internal biliary diversion (TIBD) to the colon at 2\u2009years of age. Graft biopsy at the 7\u2010year follow\u2010up examination revealed microvesicular steatosis (60%). Her diarrhea improved, and her growth failure was recovering (weight z\u2010score: \u22121.0; height z\u2010score: \u22121.7).<\/jats:p><jats:p>The second patient underwent sequential intestine\u2013liver transplantation at 8\u2009years of age due to end\u2010stage liver disease (ESLD) and short bowel syndrome caused by massive bowel resection for internal hernia after partial external biliary diversion (PEBD) at 21\u2009months of age. She developed severe pancreatitis induced by steroid\u2013bolus therapy for rejection after transplantation. She died 1.7\u2009years after intestinal transplantation due to an uncontrollable pancreatic abscess and acute respiratory distress syndrome. The third patient underwent PEBD at 15\u2009months of age and received LT with TEBD at 15\u2009years of age due to ESLD with hepatic encephalopathy. Throughout the perioperative period, she showed no abdominal symptoms, including diarrhea and pancreatitis. Graft biopsy at the 2\u2010year follow\u2010up examination revealed macrovesicular steatosis (60%) with inflammation.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>The patients showed different outcomes. Effective therapeutic options to mitigate post\u2010LT complications in patients with PFIC1 must be considered individually.<\/jats:p><\/jats:sec>","DOI":"10.1111\/petr.14566","type":"journal-article","created":{"date-parts":[[2023,7,7]],"date-time":"2023-07-07T09:22:13Z","timestamp":1688721733000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Optimal liver transplant procedure in progressive familial intrahepatic cholestasis type 1 treated with biliary diversion or intestinal transplantation: Lessons learned from three cases treated with different approaches"],"prefix":"10.1111","volume":"27","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2441-5158","authenticated-orcid":false,"given":"Hajime","family":"Uchida","sequence":"first","affiliation":[{"name":"Organ Transplantation Center, National Center for Child Health and Development  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3031-7319","authenticated-orcid":false,"given":"Seisuke","family":"Sakamoto","sequence":"additional","affiliation":[{"name":"Organ Transplantation Center, National Center for Child Health and Development  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2547-4790","authenticated-orcid":false,"given":"Ryuji","family":"Komine","sequence":"additional","affiliation":[{"name":"Organ Transplantation Center, National Center for Child Health and Development  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tasuku","family":"Kodama","sequence":"additional","affiliation":[{"name":"Organ Transplantation Center, National Center for Child Health and Development  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Toshimasa","family":"Nakao","sequence":"additional","affiliation":[{"name":"Organ Transplantation Center, National Center for Child Health and Development  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yusuke","family":"Yanagi","sequence":"additional","affiliation":[{"name":"Organ Transplantation Center, National Center for Child Health and Development  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Seiichi","family":"Shimizu","sequence":"additional","affiliation":[{"name":"Organ Transplantation Center, National Center for Child Health and Development  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Syed Hasnain","family":"Abbas","sequence":"additional","affiliation":[{"name":"Organ Transplantation Center, National Center for Child Health and Development  Tokyo Japan"},{"name":"Organ Transplantation and HPB Department Pir Abdul Qadir Shah Jeelani Institute of Medical Sciences  Gambat Sindh Pakistan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4684-0446","authenticated-orcid":false,"given":"Akinari","family":"Fukuda","sequence":"additional","affiliation":[{"name":"Organ Transplantation Center, National Center for Child Health and Development  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1651-0430","authenticated-orcid":false,"given":"Mureo","family":"Kasahara","sequence":"additional","affiliation":[{"name":"Organ Transplantation Center, National Center for Child Health and Development  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2023,7,7]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cld.2018.06.003"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/petr.14108"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPG.0000000000002983"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.12659\/AJCR.932374"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/petr.13184"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-3046.2011.01514.x"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/petr.12782"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/ajt.13909"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-6143.2012.04210.x"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cld.2015.10.011"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jceh.2021.03.008"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpedsurg.2015.07.016"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPG.0000000000001493"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpedsurg.2011.08.010"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2020.02.007"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.31787"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamainternmed.2013.2737"},{"issue":"7","key":"e_1_2_8_19_1","article-title":"Steroid\u2010induced pancreatitis: a challenging diagnosis","volume":"12","author":"Ataallah B","year":"2020","journal-title":"Cureus"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1136\/bcr-2019-234185"},{"issue":"5","key":"e_1_2_8_21_1","first-page":"669","article-title":"Ileal bile acid transporter inhibition reduces post\u2010transplant diarrhea and growth failure in FIC1 disease\u2010a case report","volume":"9","author":"Ohlendorf J","year":"2022","journal-title":"Children (Basel)"}],"container-title":["Pediatric Transplantation"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/petr.14566","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,9,6]],"date-time":"2024-09-06T13:01:47Z","timestamp":1725627707000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/petr.14566"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,7,7]]},"references-count":20,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2023,9]]}},"alternative-id":["10.1111\/petr.14566"],"URL":"https:\/\/doi.org\/10.1111\/petr.14566","archive":["Portico"],"relation":{},"ISSN":["1397-3142","1399-3046"],"issn-type":[{"type":"print","value":"1397-3142"},{"type":"electronic","value":"1399-3046"}],"subject":[],"published":{"date-parts":[[2023,7,7]]},"assertion":[{"value":"2023-05-06","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-06-28","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-07-07","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e14566"}}