TY - JOUR
T1 - Progressive familial intrahepatic cholestasis
T2 - A single-center experience of living-donor liver transplantation during two decades in Japan
AU - Hori, Tomohide
AU - Egawa, Hiroto
AU - Takada, Yasutsugu
AU - Ueda, Mikiko
AU - Oike, Fumitaka
AU - Ogura, Yasuhiro
AU - Sakamoto, Seisuke
AU - Kasahara, Mureo
AU - Ogawa, Kohei
AU - Miyagawa-Hayashino, Aya
AU - Yonekawa, Yukihide
AU - Yorifuji, Tohru
AU - Watanabe, Ken Ichiro
AU - Doi, Hiraku
AU - Nguyen, Justin H.
AU - Chen, Feng
AU - Baine, Ann Marie T.
AU - Gardner, Lindsay B.
AU - Uemoto, Shinji
PY - 2011/9
Y1 - 2011/9
N2 - 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.
AB - 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.
KW - Byler's disease
KW - Liver transplantation
KW - Living donor
KW - Progressive familial intrahepatic cholestasis
KW - Steatosis
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U2 - 10.1111/j.1399-0012.2010.01368.x
DO - 10.1111/j.1399-0012.2010.01368.x
M3 - Article
C2 - 21158920
AN - SCOPUS:80054123164
SN - 0902-0063
VL - 25
SP - 776
EP - 785
JO - Clinical Transplantation
JF - Clinical Transplantation
IS - 5
ER -