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Plasmapheresis Combined with Continuous Plasma Filtration Adsorption Rescues Severe Acute Liver Failure in Wilson's Disease before Liver Transplantation

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单位: [1]Huazhong Univ Sci & Technol, Tongji Med Coll, Tongji Hosp, Dept Pediat, 1095 Jiefang Ave, Wuhan 430030, Hubei, Peoples R China
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关键词: Wilsonian crisis Hemolytic crisis Acute liver failure Plasmapheresis Continuous plasma filtration adsorption

摘要:
Wilsonian crisis (hemolytic crisis and acute liver failure [ALF] in Wilson's disease) is fatal and almost all patients ultimately need a timely liver transplantation to save their lives. How to safely pass the critical period to liver transplantation is still a big challenge to doctors especially to pediatricians facing more difficult situations in blood purification. Here, we report about a 7-year-old child (weight 21 kg) presenting with severe hemolysis and impending ALF that made a rapid recovery with prompt initiation of plasmapheresis combined with continuous plasma filtration adsorption (CPFA) and chelation therapy. Rapidly efficient removal of copper, bilirubin, and albumin-binding toxins by hybrid blood purification alleviated hemolysis and liver injury and successfully bridged the patient over the critical period to late liver transplantation. Moreover, a review of the literature was performed examining the use of plasmapheresis, molecular adsorbent recirculation system, single-pass albumin dialysis, and continuous veno-venous hemodiafiltration in Wilson disease. The experience of our case points to that plasmapheresis combined with CPFA treatment can improve clinical symptoms and bridge children over through Wilsonian crisis to late live transplantation, and CPFA treatment is feasible and safe in children weighing more than 20 kg. (c) 2018 S. Karger AG, Basel

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出版当年[2018]版:
大类 | 4 区 医学
小类 | 4 区 血液学 4 区 泌尿学与肾脏学
最新[2025]版:
大类 | 4 区 医学
小类 | 4 区 血液学 4 区 泌尿学与肾脏学
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出版当年[2017]版:
Q3 HEMATOLOGY Q3 UROLOGY & NEPHROLOGY
最新[2023]版:
Q2 UROLOGY & NEPHROLOGY Q3 HEMATOLOGY

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第一作者单位: [1]Huazhong Univ Sci & Technol, Tongji Med Coll, Tongji Hosp, Dept Pediat, 1095 Jiefang Ave, Wuhan 430030, Hubei, Peoples R China
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