{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,8]],"date-time":"2026-10-08T19:59:30Z","timestamp":1791489570330,"version":"4.3.4"},"reference-count":23,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2021,2,22]],"date-time":"2021-02-22T00:00:00Z","timestamp":1613952000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Clinical Transplantation"],"published-print":{"date-parts":[[2021,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>Crigler\u2010Najjar syndrome (CNS) is a rare inherited disorder that is characterized by high levels of non\u2010hemolytic, unconjugated hyperbilirubinemia leading to brain damage and even death. Liver transplantation (LT) can correct the metabolic defect, but there are little data regarding LT in this patient cohort. The liver parenchyma has been considered to be structurally normal in CNS, but there is growing evidence of clinically silent but histologically significant fibrosis in CNS patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Patients and methods<\/jats:title><jats:p>We included 13 patients in our retrospective study who underwent LT at our center. Patient survival, graft function, and long\u2010term complications were evaluated over a median follow\u2010up period of 10\u00a0years (range: 1\u201316\u00a0years). In addition, the prevalence of histologically relevant fibrosis was characterized.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The overall survival among our LT patients was 100%. The graft survival was only 61.5%. During the follow\u2010up period, 5 LT patients had to undergo retransplantation. More than 45% of our patients showed histological signs of fibrosis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>LT remains the only definite therapeutic option for severe CNS but needs to be considered thoroughly regarding the clinical risk\u2010benefit\u2010ratio and impact on quality of life. Furthermore, hepatic parenchymal injury needs to be considered while evaluating future therapeutic options for CNS.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ctr.14219","type":"journal-article","created":{"date-parts":[[2021,2,6]],"date-time":"2021-02-06T10:10:07Z","timestamp":1612606207000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["Outcome of liver transplantation and prevalence of liver fibrosis in Crigler\u2010Najjar syndrome"],"prefix":"10.1111","volume":"35","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8007-1110","authenticated-orcid":false,"given":"Hanna","family":"Schr\u00f6der","sequence":"first","affiliation":[{"name":"Department of Pediatrics University Medical Center Hamburg\u2010Eppendorf Hamburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Norman","family":"Junge","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University Medical Center Hanover Hanover Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Uta","family":"Herden","sequence":"additional","affiliation":[{"name":"Department of Visceral Transplant Surgery University Medical Center Hamburg\u2010Eppendorf Hamburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Deutschmann","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Medical University Hospital of Graz Graz Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S\u00f6ren Alexander","family":"Weidemann","sequence":"additional","affiliation":[{"name":"Department of Pathology University Medical Center Hamburg\u2010Eppendorf Hamburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dorothee","family":"Krebs\u2010Schmitt","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University Medical Center Hamburg\u2010Eppendorf Hamburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Enke Freya","family":"Grabhorn","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University Medical Center Hamburg\u2010Eppendorf Hamburg 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