Progressive familial intrahepatic cholestasis: A single-center experience of living-donor liver transplantation during two decades in Japan

Tomohide Hori, Hiroto Egawa, Yasutsugu Takada, Mikiko Ueda, Fumitaka Oike, Yasuhiro Ogura, Seisuke Sakamoto, Mureo Kasahara, Kohei Ogawa, Aya Miyagawa-Hayashino, Yukihide Yonekawa, Tohru Yorifuji, Ken Ichiro Watanabe, Hiraku Doi, Justin H. Nguyen, Feng Chen, Ann Marie T. Baine, Lindsay B. Gardner, Shinji Uemoto

Research output: Contribution to journalArticlepeer-review

23 Scopus citations

Abstract

Background: Progressive familial intrahepatic cholestasis (PFIC) results in liver cirrhosis. Therefore, some PFIC patients require liver transplantation (LT). Although three types of PFIC have been identified, their etiologies include unknown mechanisms. Patients: A total of 717 recipients who underwent living-donor LT (LDLT) at <20yr old were enrolled in this study. Among these recipients, 14 PFIC recipients comprising 11 PFIC type 1 (PFIC1) and three PFIC type 2 (PFIC2) were evaluated. Results: Three of 11 PFIC1 recipients died, while all three PFIC2 recipients survived. Eight of 11 PFIC1 recipients showed steatosis after LDLT. Among the eight steatosis-positive PFIC1 recipients, seven showed severe steatosis and seven were complicated with steatohepatitis. Nine of 11 PFIC1 recipients showed fibrosis after LDLT, and eight of the nine fibrosis-positive PFIC1 recipients showed severe fibrosis. In contrast to the PFIC1 recipients, the PFIC2 recipients did not show any steatosis or fibrosis after LDLT. Conclusions: The clinical courses and outcomes of PFIC1 recipients after LDLT are still not sufficient owing to steatosis/fibrosis, unlike the case for PFIC2 recipients. As PFIC1 patients will require LT during the long-term progression of the disease, further strategy improvements are required for PFIC1 patients.

Original languageEnglish (US)
Pages (from-to)776-785
Number of pages10
JournalClinical Transplantation
Volume25
Issue number5
DOIs
StatePublished - Sep 2011

Keywords

  • Byler's disease
  • Liver transplantation
  • Living donor
  • Progressive familial intrahepatic cholestasis
  • Steatosis

ASJC Scopus subject areas

  • Transplantation

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