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作 者:周星 余泽波[1] ZHOU Xing;YU Zeb(Department of Blood Transfusion,The first Affiliated Hospital of Chongqing Medical University,Chongqing 400016,China)
机构地区:[1]重庆医科大学附属第一医院输血科,重庆400016
出 处:《中国输血杂志》2024年第8期946-952,共7页Chinese Journal of Blood Transfusion
摘 要:重症肌无力(myasthenia gravis,MG)是主要由自身抗体介导的骨骼肌神经肌接头处兴奋传递障碍的自身免疫性疾病。其首发临床症状为肌肉波动性无力和易疲劳性,绝大部分MG患者发病早期仅轻微累及眼外肌和四肢骨骼肌,少部分患者在短期内会出现病情的迅速进展,累及咽喉肌和呼吸肌等重要肌群,发生肌无力危象,从而严重威胁患者生命。临床上常采用治疗性血浆置换(therapeutic plasma exchange,TPE)或静脉注射免疫球蛋白(intravenous immunoglobulins,IVIG)对这类患者进行救治,可以快速缓解肌无力症状、改善呼吸功能。近年来,关于TPE的临床研究大量涌现,TPE在改善MG症状、发挥早期疗效、缩短ICU住院时间及安全性方面的临床价值被逐渐阐明。我们通过检索近5年国内外TPE应用于MG治疗的临床研究文章,从MG和TPE的概括,TPE在MG治疗临床有效性及安全性,TPE延伸技术在MG治疗中的应用等方面,总结当前TPE在MG治疗中的临床研究进展,为TPE更好地应用于MG提供参考价值。Myasthenia gravis(MG)is an autoimmune disease mainly mediated by autoantibodies that disorders the transmission of excitation at the neuromuscular junction.The predominant clinical manifestation is the muscle fluctuant weakness and fatigability,and the extraocular and skeletal muscles are slightly affected at the early onset for the majority of MG patients.However,there is a rapid progression in a short period of time for a small group,involving the laryngeal muscles and respiratory muscles and developing the myasthenia crisis,which severely threatens their lives.It’s commonly used for such patients to be rescued by therapeutic plasma exchange(TPE)or intravenous immunoglobulins(IVIG)that rapidly relieves their symptoms of muscle weakness and improves the respiratory function.In recent years,plentiful clinical studies about TPE have emerged,and the clinical value of TPE in improving MG symptoms,exerting early efficacy,reducing the length of ICU hospital stay and its safety has been gradually clarified.This review aims to summarize the clinical research advancement of TPE in the treatment of MG in terms of basic conditions of MG and TPE,the therapeutic efficiency and safety of TPE,and the application of TPE extension technology in the treatment of MG by searching for clinical research articles at home and abroad in the past 5 years,which can provide reference for better clinical application of MG.
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