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格林-巴利综合征。

Guillain-Barré syndrome.

机构信息

Neurology Unit, Department of Medicine, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia.

Department of Neurology, Faculty of Medicine and University Hospital Cologne, University of Cologne, Cologne, Germany.

出版信息

Lancet. 2021 Mar 27;397(10280):1214-1228. doi: 10.1016/S0140-6736(21)00517-1. Epub 2021 Feb 26.

Abstract

Guillain-Barré syndrome is the most common cause of acute flaccid paralysis worldwide. Most patients present with an antecedent illness, most commonly upper respiratory tract infection, before the onset of progressive motor weakness. Several microorganisms have been associated with Guillain-Barré syndrome, most notably Campylobacter jejuni, Zika virus, and in 2020, the severe acute respiratory syndrome coronavirus 2. In C jejuni-related Guillain-Barré syndrome, there is good evidence to support an autoantibody-mediated immune process that is triggered by molecular mimicry between structural components of peripheral nerves and the microorganism. Making a diagnosis of so-called classical Guillain-Barré syndrome is straightforward; however, the existing diagnostic criteria have limitations and can result in some variants of the syndrome being missed. Most patients with Guillain-Barré syndrome do well with immunotherapy, but a substantial proportion are left with disability, and death can occur. Results from the International Guillain-Barré Syndrome Outcome Study suggest that geographical variations exist in Guillain-Barré syndrome, including insufficient access to immunotherapy in low-income countries. There is a need to provide improved access to treatment for all patients with Guillain-Barré syndrome, and to develop effective disease-modifying therapies that can limit the extent of nerve injury. Clinical trials are currently underway to investigate some of the potential therapeutic candidates, including complement inhibitors, which, together with emerging data from large international collaborative studies on the syndrome, will contribute substantially to understanding the many facets of this disease.

摘要

格林-巴利综合征是全世界最常见的急性弛缓性瘫痪的病因。大多数患者在进行性肌无力发作前,通常有上呼吸道感染等前驱疾病。有几种微生物与格林-巴利综合征有关,最值得注意的是空肠弯曲菌、寨卡病毒,以及 2020 年的严重急性呼吸综合征冠状病毒 2。在空肠弯曲菌相关格林-巴利综合征中,有充分的证据支持一种自身抗体介导的免疫过程,该过程是由外周神经的结构成分与微生物之间的分子模拟引发的。诊断所谓的经典格林-巴利综合征很简单;然而,现有的诊断标准存在局限性,可能导致某些综合征变异被遗漏。大多数格林-巴利综合征患者对抗免疫疗法反应良好,但相当一部分患者仍留有残疾,并且可能导致死亡。国际格林-巴利综合征结局研究的结果表明,格林-巴利综合征存在地域差异,包括在低收入国家免疫疗法的获取不足。需要为所有格林-巴利综合征患者提供更好的治疗机会,并开发有效的疾病修正疗法,以限制神经损伤的程度。目前正在进行临床试验,以研究一些潜在的治疗候选药物,包括补体抑制剂,这些研究结果以及关于该综合征的大型国际合作研究的新数据,将大大有助于了解该疾病的许多方面。

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