{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T03:36:14Z","timestamp":1790739374646,"version":"4.1.0"},"reference-count":40,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2021,8,2]],"date-time":"2021-08-02T00:00:00Z","timestamp":1627862400000},"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":[[2021,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Progressive familial intrahepatic cholestasis type 1 (PFIC1) arises from biallelic variants in the <jats:italic>ATP8B1<\/jats:italic>\u00a0gene that annul FIC1 activity, resulting in progressive liver disease. Liver transplant (LT) is indicated in refractory disease; however, post\u2010LT complications including worsening diarrhea and steatohepatitis progressing to fibrosis with graft loss have been reported. We aim to describe long\u2010term outcomes of PFIC1 LT recipients at our center, focusing on the histological changes of the allografts.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We assessed 7 PFIC1 patients post\u2010LT at the Children's Hospital of Pittsburgh (CHP). All pre\u2010transplant, explant, and sequential post\u2010transplant pathology samples were reviewed. Continuous data are presented as the mean\u00a0\u00b1\u00a0SD. We compared the pre\u2010 and post\u2010transplant height and weight z\u2010scores using Wilcoxon signed\u2010rank test.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Seven (29% male) patients with PFIC1 received a LT (<jats:italic>n<\/jats:italic>\u00a0=\u00a06) or had post\u2010LT care (<jats:italic>n<\/jats:italic>\u00a0=\u00a01) at CHP. Six had confirmed or suspected identical genetic. At a mean follow\u2010up of 10.9\u00a0years, both patient survival and graft survival were 100%. Diarrhea persisted (<jats:italic>n<\/jats:italic>\u00a0=\u00a03) or newly developed (<jats:italic>n<\/jats:italic>\u00a0=\u00a04) in all patients after LT contributing to ongoing growth failure, with mean z\u2010scores \u22122.63 (weight) and \u22122.98 (height) at follow\u2010up. Histologically, allograft steatosis was common but was not accompanied by significant inflammation, ballooning, or fibrosis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>We show that extrahepatic disease persists and near\u2010universal allograft steatosis occurs. However, at a mean follow\u2010up period of over 10\u00a0years, no patients developed steatohepatitis or significant fibrosis, and both patient survival and graft survival are excellent.<\/jats:p><\/jats:sec>","DOI":"10.1111\/petr.14108","type":"journal-article","created":{"date-parts":[[2021,8,2]],"date-time":"2021-08-02T14:46:51Z","timestamp":1627915611000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Long\u2010term liver transplant outcomes for progressive familial intrahepatic cholestasis type 1: The Pittsburgh experience"],"prefix":"10.1111","volume":"25","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7189-8829","authenticated-orcid":false,"given":"Sarah A.F.","family":"Henkel","sequence":"first","affiliation":[{"name":"Department of Pediatric Gastroenterology and Hepatology University of Pittsburgh School of Medicine Children's Hospital of Pittsburgh Pittsburgh PA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claudia M.","family":"Salgado","sequence":"additional","affiliation":[{"name":"Department of Pathology University of Pittsburgh Children's Hospital of Pittsburgh Pittsburgh PA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miguel","family":"Reyes\u2010Mugica","sequence":"additional","affiliation":[{"name":"Department of Pathology University of Pittsburgh Children's Hospital of Pittsburgh Pittsburgh PA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0899-7234","authenticated-orcid":false,"given":"Kyle A.","family":"Soltys","sequence":"additional","affiliation":[{"name":"Hillman Center for Pediatric Transplantation UPMC Children's Hospital of Pittsburgh Pittsburgh PA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kevin","family":"Strauss","sequence":"additional","affiliation":[{"name":"Clinic for Special Children Strasburg PA USA"},{"name":"Department of Pediatrics Penn Medicine\u2010Lancaster General Hospital Lancaster PA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2624-8632","authenticated-orcid":false,"given":"George V.","family":"Mazariegos","sequence":"additional","affiliation":[{"name":"Hillman Center for Pediatric Transplantation UPMC Children's Hospital of Pittsburgh Pittsburgh PA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert H.","family":"Squires","sequence":"additional","affiliation":[{"name":"Department of Pediatric Gastroenterology and Hepatology University of Pittsburgh School of Medicine Children's Hospital of Pittsburgh Pittsburgh PA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8466-8504","authenticated-orcid":false,"given":"Patrick J.","family":"McKiernan","sequence":"additional","affiliation":[{"name":"Department of Pediatric Gastroenterology and Hepatology University of Pittsburgh School of Medicine Children's Hospital of Pittsburgh Pittsburgh PA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xingyu","family":"Zhang","sequence":"additional","affiliation":[{"name":"Thomas E. 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