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

被引：44

作者：

Hori, Tomohide ^{[1
,2
]}

Egawa, Hiroto ^{[1
]}

Takada, Yasutsugu ^{[1
]}

Ueda, Mikiko ^{[1
]}

Oike, Fumitaka ^{[1
]}

Ogura, Yasuhiro ^{[1
]}

Sakamoto, Seisuke ^{[1
]}

Kasahara, Mureo ^{[1
]}

Ogawa, Kohei ^{[1
]}

Miyagawa-Hayashino, Aya ^{[3
]}

Yonekawa, Yukihide ^{[4
]}

Yorifuji, Tohru ^{[5
]}

Watanabe, Ken-Ichiro ^{[5
]}

Doi, Hiraku ^{[5
]}

Nguyen, Justin H. ^{[6
]}

Chen, Feng ^{[2
]}

Baine, Ann-Marie T. ^{[2
]}

Gardner, Lindsay B. ^{[2
]}

Uemoto, Shinji ^{[1
]}

机构：

[1] Kyoto Univ Hosp, Dept Surg, Div Hepatobiliary Pancreat & Transplant Surg, Sakyo Ku, Kyoto 6068507, Japan

[2] Mayo Clin Florida, Dept Neurosci, Jacksonville, FL USA

[3] Kyoto Univ Hosp, Dept Diagnost Pathol, Kyoto 6068507, Japan

[4] Kyoto Univ Hosp, Dept Surg, Div Pediat Surg, Kyoto 6068507, Japan

[5] Kyoto Univ Hosp, Dept Pediat, Sakyo Ku, Kyoto 6068507, Japan

[6] Mayo Clin Florida, Dept Transplantat, Div Transplant Surg, Jacksonville, FL USA

来源：

CLINICAL TRANSPLANTATION | 2011年 / 25卷 / 05期

关键词：

Byler's disease; liver transplantation; living donor; progressive familial intrahepatic cholestasis; steatosis; EXTERNAL BILIARY DIVERSION; EXPORT PUMP DEFICIENCY; GENE DEFECT; DISEASE; TYPE-1; STEATOHEPATITIS; MUTATIONS; TRANSPORT; SPECTRUM; THERAPY;

D O I：

10.1111/j.1399-0012.2010.01368.x

中图分类号：

R61 [外科手术学];

学科分类号：

摘要：

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.

引用

页码：776 / 785

页数：10

共 39 条

[1] Preoperative observations and short-term outcome after partial external biliary diversion in 13 patients with progressive familial intrahepatic cholestasis [J].