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吉兰-巴雷综合征的自然病史及治疗效果:一项多中心研究。

Natural history and treatment effects in Guillain-Barré syndrome: a multicentre study.

作者信息

Korinthenberg R, Mönting J S

机构信息

Department of Neuropaediatrics and Muscular Disorders, Albert-Ludwigs-University, Freiburg, Germany.

出版信息

Arch Dis Child. 1996 Apr;74(4):281-7. doi: 10.1136/adc.74.4.281.

Abstract

A retrospective multicentre study was performed to investigate the natural history and treatment effects in childhood Guillain-Barré syndrome in a large number of patients. Structured questionnaires were sent to 155 paediatric hospitals for details of patients who conformed to internationally accepted diagnostic criteria and who were treated from spring 1989 to summer 1994. Sixty nine hospitals reported data of 175 patients aged 11 months to 17.7 years. At the height of the disease 26% of the patients remained able to walk, but 16% had to be artificially ventilated. The median time from onset of symptoms to first recovery was 17 days, to walk unaided 37 days, and to be free of symptoms 66 days. There was a large group with a benign and a smaller one with a more protracted course. At long term follow up, 98/106 patients were free of symptoms and the remainder were able to walk unaided. Maximum disability grade was the most powerful prognostic factor. In children unable to walk but not yet tetraplegic, immunoglobulins were able to accelerate recovery. Corticosteroids were less potent. Plasmapheresis could not be evaluated because it was administered only in the most severe cases. The natural history of Guillain-Barré syndrome in children is extremely variable and more benign than in adults. Treatment with immunoglobulins should be considered in patients unable to walk. Corticosteroids are not as effective and should be withheld except when, in protracted courses, suspicion of chronic inflammatory demyelinating polyneuropathy arises.

摘要

开展了一项回顾性多中心研究,以调查大量儿童吉兰-巴雷综合征的自然病史和治疗效果。向155家儿科医院发送了结构化问卷,以获取符合国际公认诊断标准且在1989年春季至1994年夏季接受治疗的患者的详细信息。69家医院报告了175例年龄在11个月至17.7岁之间患者的数据。在疾病高峰期,26%的患者仍能行走,但16%的患者需要人工通气。从症状出现到首次恢复的中位时间为17天,独立行走为37天,症状消失为66天。有一大组患者病程良性,一小组患者病程较长。长期随访时,106例患者中有98例无症状,其余患者能够独立行走。最大残疾等级是最有力的预后因素。在不能行走但尚未四肢瘫痪的儿童中,免疫球蛋白能够加速恢复。皮质类固醇的效果较差。由于血浆置换仅在最严重的病例中使用,因此无法进行评估。儿童吉兰-巴雷综合征的自然病史差异极大,且比成人更具良性。对于不能行走的患者,应考虑使用免疫球蛋白进行治疗。皮质类固醇效果不佳,除非在病程较长且怀疑为慢性炎症性脱髓鞘性多发性神经病时,应避免使用。

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