Myasthenia gravis: Management of myasthenic crisis and perioperative care
Myasthenia gravis: Management of myasthenic crisis and perioperative care
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DOI：
10.1055/s-2004-829595
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发表时间：
2004-03-01
影响因子：
2.7
通讯作者：
Juel, VC
中科院分区：
文献类型：
--
作者：
Juel, VC
肌无力危象可定义为因肌无力导致呼吸衰竭或术后拔管延迟超过 24 小时。肌无力危象是由于上呼吸道肌肉无力导致阻塞和误吸、呼吸肌无力导致潮气量减少或双肌群无力所致。大约五分之一的重症肌无力患者通常会在患病的第一年内经历危机。在过去四十年中，肌无力危象的预后已从 75% 的死亡率显着改善到目前的不到 5%。肌无力危象的常见诱发因素包括呼吸道感染、误吸、脓毒症、外科手术、快速减少免疫调节、开始皮质类固醇治疗、接触可能加重肌无力的药物以及怀孕。肌无力危象不应是致命的，只要患者得到及时的呼吸支持和适当的免疫治疗，以减轻上呼吸道和呼吸肌的肌无力。伴有口咽肌或呼吸肌无力的肌无力患者应在术前接受血浆置换或静脉注射免疫球蛋白治疗，将肌无力程度降至最低程度，以预防术后并发症。
Myasthenic crisis may be defined as respiratory failure or delayed postoperative extubation for more than 24 hours resulting from myasthenic weakness. Myasthenic crisis results from weakness of upper airway muscles leading to obstruction and aspiration, weakness of respiratory muscles leading to reduced tidal volumes, or from weakness of both muscle groups. About one-fifth of patients with myasthenia gravis experience crisis, usually within the first year of illness. Over the last four decades, prognosis from myasthenic crisis has dramatically improved from a mortality rate of 75% to the current rate of less than 5%. Common precipitating factors for myasthenic crisis include respiratory infections, aspiration, sepsis, surgical procedures, rapid tapering of immune modulation, beginning treatment with corticosteroids, exposure to drugs that may increase myasthenic weakness, and pregnancy. Myasthenic crisis should not be fatal, as long as patients receive timely respiratory support and appropriate immunotherapy to reduce myasthenic weakness of the upper airway and respiratory muscles. Myasthenic patients with oropharyngeal or respiratory muscle weakness should receive preoperative plasma exchange or intravenous immunoglobulin therapy to a minimal level of weakness to prevent postoperative complications.