Early Application of Auxiliary Partial Orthotopic Liver Transplantation in Murine Model of Wilson Disease

被引：15

作者：

Cheng, Qi ^{[1
]}

He, Song-Qing ^{[2
]}

Gao, Dan ^{[1
]}

Lei, Biao ^{[2
]}

Long, Xin ^{[1
]}

Liang, Hui-Fang ^{[1
]}

Zhu, Peng ^{[1
]}

Jin, Jun-Fei ^{[2
]}

Tang, Bo ^{[2
]}

Tomlinson, Stephen ^{[3
]}

Wu, Zhi-Ying ^{[4
,5
,6
,7
,8
,9
]}

Chen, Xiao-Ping ^{[1
]}

机构：

[1] Huazhong Univ Sci & Technol, Tongji Med Coll, Tongji Hosp, Hepat Surg Ctr, 1095 Jiefang Anvenun, Wuhan 430030, Peoples R China

[2] Guilin Med Univ, Dept Hepatopancreatobiliary Surg, Affiliated Hosp, Guilin, Peoples R China

[3] Med Univ S Carolina, Dept Microbiol & Immunol, Childrens Res Inst, Charleston, SC 29425 USA

[4] Zhejiang Univ, Sch Med, Dept Neurol, Hangzhou 310003, Zhejiang, Peoples R China

[5] Zhejiang Univ, Sch Med, Res Ctr Neurol, Affiliated Hosp 2, Hangzhou 310003, Zhejiang, Peoples R China

[6] Zhejiang Univ, Sch Med, Collaborat Innovat Ctr Brain Sci, Hangzhou 310003, Zhejiang, Peoples R China

[7] Fudan Univ, Dept Neurol, Shanghai 200433, Peoples R China

[8] Fudan Univ, Inst Neurol, Huashan Hosp, Inst Brain Sci, Shanghai 200433, Peoples R China

[9] Fudan Univ, State Key Lab Med Neurobiol, Shanghai Med Coll, Shanghai 200433, Peoples R China

来源：

TRANSPLANTATION | 2015年 / 99卷 / 11期

关键词：

COPPER ACCUMULATION; SIZE SYNDROME; LOCALIZATION; EXPERIENCE; THERAPY; ATP7B; MICE;

D O I：

10.1097/TP.0000000000000787

中图分类号：

R392 [医学免疫学]; Q939.91 [免疫学];

学科分类号：

100102 ;

摘要：

Background. Liver transplantation (LT) is the only option of treatment forWilson disease (WD) when chelation therapy fails, but it is limited due to the shortage of donor. Auxiliary partial orthotopic LT (APOLT) has been performed successfully in end-stage WD patients, which expands the donor pool. Methods. Atp7b(-/-)mice were used as experimental model of WD. Eight-and 20-week-old mice were used as different timepoints to perform APOLT. Serum copper, tissue copper, serum ceruloplasmin (CP), and liver histological examination were observed after operation. Results. Hepatic and serum copper levels in Atp7b(-/-) mice decreased after APOLT, and copper metabolism disorder of WD mice was relieved at both early and late stages. The progression of pathology in the native liver was delayed only when transplantation was performed at an early stage. Conclusions. Auxiliary partial orthotopic LT can significantly improve copper metabolism disorder in the Atp7b(-/-) mice, and early transplantation may prevent the disease progression.

引用

页码：2317 / 2324

页数：8

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