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血浆置换治疗重症肌无力:病理生理基础与临床经验

Plasma exchange for treatment of myasthenia gravis: pathophysiologic basis and clinical experience.

作者信息

Qureshi A I, Suri M F

机构信息

Department of Neurosurgery, School of Biomedical Sciences and Medicine, State University of New York, Buffalo, USA.

出版信息

Ther Apher. 2000 Aug;4(4):280-6. doi: 10.1046/j.1526-0968.2000.004004280.x.

Abstract

Myasthenia gravis is an autoimmune disease characterized by production of antibodies to acetylcholine receptors located at the motor end plate in skeletal muscles. The antibodies bind and subsequently induce degeneration of these receptors. Loss of acetylcholine receptors results in inadequate contraction of muscle fibers in response to acetylcholine released from nerve terminals and clinically apparent muscle weakness. Plasma exchange removes the circulating antibodies in myasthenic patients with short-term clinical improvement. Plasma exchange may be indicated in patients with acute exacerbation of neuromuscular weakness with bulbar or respiratory compromise, preoperative optimization prior to thymectomy, and postoperative deterioration following thymectomy or other surgical procedures. Long-term, intermittent plasma exchange for patients who do not adequately respond to standard treatment is another evolving indication.

摘要

重症肌无力是一种自身免疫性疾病,其特征是产生针对位于骨骼肌运动终板处的乙酰胆碱受体的抗体。这些抗体结合并随后诱导这些受体变性。乙酰胆碱受体的丧失导致肌肉纤维对神经末梢释放的乙酰胆碱反应时收缩不足,并出现临床上明显的肌肉无力。血浆置换可清除重症肌无力患者体内的循环抗体,使临床症状得到短期改善。血浆置换适用于有球部或呼吸功能障碍的神经肌肉无力急性加重的患者、胸腺切除术前的优化处理,以及胸腺切除或其他外科手术后的病情恶化。对于对标准治疗反应不佳的患者,长期间歇性血浆置换是另一个不断发展的适应证。

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