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.
journal_name
Clin Transplantjournal_title
Clinical transplantationauthors
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,Doi H,Nguyen JH,Chen F,Baine AM,Gardner LB,Uemoto Sdoi
10.1111/j.1399-0012.2010.01368.xsubject
Has Abstractpub_date
2011-09-01 00:00:00pages
776-85issue
5eissn
0902-0063issn
1399-0012journal_volume
25pub_type
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更新日期:1992-06-01 00:00:00
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pub_type: 临床试验,杂志文章
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更新日期:2012-11-01 00:00:00
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pub_type: 杂志文章
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pub_type: 杂志文章
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更新日期:1995-12-01 00:00:00
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更新日期:2011-01-01 00:00:00
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pub_type: 杂志文章,评审
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更新日期:2018-06-01 00:00:00
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journal_title:Clinical transplantation
pub_type: 临床试验,杂志文章,随机对照试验
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更新日期:2011-07-01 00:00:00
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pub_type: 杂志文章
doi:10.1111/j.1399-0012.2009.01003.x
更新日期:2009-08-01 00:00:00
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journal_title:Clinical transplantation
pub_type: 临床试验,杂志文章
doi:10.1111/j.1399-0012.2005.00309.x
更新日期:2005-06-01 00:00:00
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pub_type: 杂志文章
doi:10.1111/ctr.13063
更新日期:2017-10-01 00:00:00