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

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单位: [1]Hepatic Surgery Center, Tongji Hospital, TongjiMedical College, Huazhong University of Science and Technology, Wuhan, China. [2]Department of Hepatopancreatobiliary Surgery, the affiliated hospital ofGuilinMedical University, Guilin, China. [3]Department of Microbiology and Immunology, Children's Research Institute, Medical University of South Carolina, Charleston, SC. [4]Department of Neurology and Research center of Neurology in Second Affiliated Hospital and the Collaborative Innovation Center for Brain Science, Zhejiang University School of Medicine, Hangzhou, China. [5]Department of Neurology and Institute of Neurology, Huashan Hospital, Institutes of Brain Science and State Key Laboratory of Medical Neurobiology, Shanghai Medical College, Fudan University, Shanghai, China.
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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.

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出版当年[2014]版:
大类 | 2 区 医学
小类 | 2 区 外科 2 区 移植 3 区 免疫学
最新[2025]版:
大类 | 2 区 医学
小类 | 2 区 免疫学 2 区 外科 2 区 移植
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出版当年[2013]版:
Q1 SURGERY Q1 TRANSPLANTATION Q2 IMMUNOLOGY
最新[2024]版:
Q1 IMMUNOLOGY Q1 SURGERY Q1 TRANSPLANTATION

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第一作者单位: [1]Hepatic Surgery Center, Tongji Hospital, TongjiMedical College, Huazhong University of Science and Technology, Wuhan, China.
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