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Abstract：
Background: Guillain-Barre syndrome is the most common cause of acute flaccid paralysis worldwide since the eradication of poliomyelitis. Severe cases may require intensive care and mechanical ventilation. Purpose: was to study pediatric patients with severe GBS requiring intensive care unit (ICU) admission, to assess their course and response to initial treatment modality plasma exchange (PE) or intravenous immunoglobulins (IVIg) and their final outcome. Methods: children with severe GBS who had either actual or impending respiratory failure, bulbar involvement or rapid progression of acute flaccid paralysis with trunk, upper limb and neck involvement within 24 h of the onset of weakness were enrolled. Results: 40 children were included. Following the initial treatment (33 subjects had 5 PE sessions each and IVIg in 7), 16 patients improved (40%), two died and 22 (55%) showed initial treatment failure. Axonal neuropathy, rapid progression and severe motor weakness significantly predicted poor response to therapy. At discharge, favorable outcomes (patient can walk unaided) were present in 22 cases (58%). Conclusion: Despite relatively low mortality, critically ill children with severe GBS have increased prevalence of axonal neuropathy and guarded response to initial therapy with PE or IVIg.
摘要：
背景：自根除脊髓灰质炎以来，格林-巴利综合征是全球范围内急性弛缓性麻痹的最常见原因。严重病例可能需要重症监护和机械通气。目的：研究需要重症监护病房（ICU）入院的重症GBS患儿，评估他们的病程和对初始治疗模式血浆置换（PE）或静脉免疫球蛋白（IVIg）的反应及其最终结果。方法：患有严重GBS的儿童，有实际或即将发生的呼吸衰竭，延髓受累或躯干急性弛缓性麻痹快速进展，纳入了无力发作24h内的上肢和颈部受累。结果：纳入40名儿童。初始治疗后（33名受试者各5次PE，7次IVIg），16例患者改善（40％），2例死亡,22例(55%)初始治疗失败.轴突神经病,快速进展和严重的运动无力显著预测治疗反应不佳.出院时,22例(58%)出现了良好的结局(患者可以无辅助行走).结论：尽管死亡率相对较低，患有严重GBS的危重患儿轴索神经病的患病率增加，对PE或IVIg初始治疗的反应受到保护。
