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Myasthenia gravis: a clinical-immunological update

机译:重症肌无力:临床免疫学更新

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Myasthenia gravis (MG) is the archetypic disorder of both the neuromuscular junction and autoantibody-mediated disease. In most patients, IgG1-dominant antibodies to acetylcholine receptors cause fatigable weakness of skeletal muscles. In the rest, a variable proportion possesses antibodies to muscle-specific tyrosine kinase while the remainder of seronegative MG is being explained through cell-based assays using a receptor-clustering technique and, to a lesser extent, proposed new antigenic targets. The incidence and prevalence of MG are increasing, particularly in the elderly. New treatments are being developed, and results from the randomised controlled trial of thymectomy in non-thymomatous MG, due for release in early 2016, will be of particular clinical value. To help navigate an evidence base of varying quality, practising clinicians may consult new MG guidelines in the fields of pregnancy, ocular and generalised MG (GMG). This review focuses on updates in epidemiology, immunology, therapeutic and clinical aspects of GMG in adults.
机译:重症肌无力(MG)是神经肌肉接头和自身抗体介导的疾病的原型疾病。在大多数患者中,针对乙酰胆碱受体的IgG1优势抗体会引起骨骼肌的明显疲劳。在其余部分中,可变比例的人具有针对肌肉特异性酪氨酸激酶的抗体,而血清阴性的MG的其余部分则通过使用受体聚类技术的基于细胞的测定方法进行了解释,并在较小程度上提出了新的抗原性靶标。 MG的发病率和患病率正在增加,尤其是在老年人中。正在开发新的治疗方法,预计将于2016年初发布的非胸腺性MG胸腺切除术的随机对照试验结果将具有特殊的临床价值。为了帮助找到质量各异的证据基础,执业临床医生可以咨询怀孕,眼科和广义MG(GMG)领域的新MG指南。这篇综述的重点是成人GMG在流行病学,免疫学,治疗和临床方面的更新。

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