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Progressive familial intrahepatic cholestasis: a single‐center experience of living‐donor liver transplantation during two decades in Japan
Author(s) -
Hori Tomohide,
Egawa Hiroto,
Takada Yasutsugu,
Ueda Mikiko,
Oike Fumitaka,
Ogura Yasuhiro,
Sakamoto Seisuke,
Kasahara Mureo,
Ogawa Kohei,
MiyagawaHayashino Aya,
Yonekawa Yukihide,
Yorifuji Tohru,
Watanabe KenIchiro,
Doi Hiraku,
Nguyen Justin H.,
Chen Feng,
Baine AnnMarie T.,
Gardner Lindsay B.,
Uemoto Shinji
Publication year - 2010
Publication title -
clinical transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.918
H-Index - 76
eISSN - 1399-0012
pISSN - 0902-0063
DOI - 10.1111/j.1399-0012.2010.01368.x
Subject(s) - medicine , progressive familial intrahepatic cholestasis , steatosis , cirrhosis , liver transplantation , gastroenterology , steatohepatitis , cholestasis , fibrosis , single center , transplantation , fatty liver , surgery , disease
Hori T, Egawa H, Takada Y, Ueda M, Oike F, Ogura Y, Sakamoto S, Kasahara M, Ogawa K, Miyagawa‐Hayashino A, Yonekawa Y, Yorifuji T, Watanabe K‐Ichiro, Doi H, Nguyen JH, Chen F, Baine A‐MT, Gardner LB, Uemoto S. Progressive familial intrahepatic cholestasis: a single‐center experience of living‐donor liver transplantation during two decades in Japan.
Clin Transplant 2011: 25: 776–785. © 2010 John Wiley & Sons A/S. 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 <20 yr 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.