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Management of a High-Risk Surgery with Emicizumab and Factor VIII in a Child with a Severe Hemophilia A and Inhibitor

机译:用严重血友病A和抑制剂的儿童对emicizumab和因子VIII进行高风险手术的管理

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摘要

The recent development of a humanized, bi-specific, and monoclonal antibody mimicking the function of activated factor VIII was a revolution in the management of patients suffering from severe hemophilia A with inhibitors.1The phase III randomized studies have shown a more efficient prophylaxis of this subcutaneous administered drug in these patients compared with recombinant FVIIa (rFVIIa) and activated prothrombin complex concentrates (aPCC).23Nonetheless, there are “real life” matters that need to be explored in this new era of managing hemophilia patients, such as surgery management under emicizumab, especially in children.456Here, we report the first case, to our knowledge, of major orthopedic surgery managed with factor VIII infusions in a child with inhibitor receiving emicizumab.
机译:近期发展人源化,双抗体和单克隆抗体模仿活性因子VIII的功能是患有抑制剂的严重血友病A的患者的革命。1与重组FVIIA(RFVIIA)和活化的凝血酶复合物浓缩物(APCC)相比,III期随机研究表明,这些患者中,在这些患者中,在这些患者中的这种皮下给药药物的预防。23.尽管如此,在管理血友病患者的新时代需要探索“现实生活”问题,例如emicizumab下的手术管理,特别是在儿童。4.5.6.在这里,我们向我们的知识报告了具有因子VIII输注的主要整形外科在接受Emicizumab的抑制剂的儿童中管理的主要骨科手术。

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