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A review of myasthenia gravis: Pathogenesis, clinical features and treatment

机译:重症肌无力的回顾:发病机制,临床特征和治疗

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Myasthenia gravis is an organ-specific autoimmune disease characterised by fatigable weakness of voluntary muscles. It is associated with antibodies to the post-synaptic nicotinic acetylcholine receptor in the neuromuscular junction. It peaks in the 20s especially in women and in the 50s in men. Patients may present with a wide range of neurological symptoms but usually develop ptosis and/or diplopia at some point in their illness. Some patients may present with neuromuscular respiratory failure from the onset. Management is aimed at firstly treating the symptoms with acetylcholine esterase inhibitors, such as pyridostig-mine, secondly treating the underlying disease process with immunosuppressants and thirdly considering thymectomy in patients with a thymoma and patients with generalised myasthenia. In this review, the important aspects of pathogenesis, diagnosis, investigation and management are discussed.
机译:重症肌无力是一种器官特异性自身免疫性疾病,其特征是自愿性肌肉可察觉的虚弱。它与神经肌肉接头中突触后烟碱乙酰胆碱受体的抗体有关。它在20年代达到顶峰,尤其是女性,而在50年代则达到男性。患者可能表现出广泛的神经系统症状,但通常在其疾病的某个时候出现上睑下垂和/或复视。有些患者从发病开始就可能出现神经肌肉呼吸衰竭。管理的目标是首先使用乙酰胆碱酯酶抑制剂(例如吡啶并斯的明)治疗症状,其次使用免疫抑制剂治疗潜在的疾病过程,其次考虑在胸腺瘤患者和广泛性肌无力患者中进行胸腺切除术。在这篇综述中,讨论了发病机理,诊断,研究和管理的重要方面。

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