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静脉注射免疫球蛋白在印度吉兰-巴雷综合征患儿中的作用。

Role of IV Immunoglobulin in Indian Children With Guillain-Barré Syndrome.

机构信息

All authors: Department of Neurology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India.

出版信息

Pediatr Crit Care Med. 2019 Jul;20(7):652-659. doi: 10.1097/PCC.0000000000001935.

Abstract

OBJECTIVES

To evaluate the outcome of Indian children with Guillain-Barré syndrome who received IV immunoglobulin compared with those who did not receive any specific therapy.

DESIGN

Single center, prospective cross-sectional study.

SETTING

Tertiary care neurology teaching hospital.

PATIENTS

Children (≤ 18 yr old) with Guillain-Barré syndrome were included from a prospectively maintained Guillain-Barré syndrome registry from January 2008 to April 2017. Children were classified into acute inflammatory demyelinating polyradiculoneuropathy, acute motor axonal neuropathy, acute motor-sensory axonal neuropathy, and inexcitable motor nerves based on nerve conduction study.

INTERVENTIONS

Out of 138 pediatric Guillain-Barré syndrome, 50 received IV immunoglobulin and another 50 age and peak disability matched controls (who did not receive IV immunoglobulin or plasmapheresis) were selected from the same registry for comparison.

MEASUREMENTS AND MAIN RESULTS

Outcome at 3 and 6 months was defined on the basis of a 0-10 Clinical Grading Scale into complete (Clinical Grading Scale < 3), partial (Clinical Grading Scale 3-5), and poor (Clinical Grading Scale > 5) recovery. The primary outcome was proportion of patients with complete recovery at 3 and 6 months in IV immunoglobulin and non-IV immunoglobulin groups. Secondary outcomes included in-hospital deaths, duration of mechanical ventilation, and hospital stay. Subgroup analysis was done in acute motor axonal neuropathy and acute inflammatory demyelinating polyradiculoneuropathy groups. The baseline characteristics were similar except for shorter duration of illness and higher proportion of facial palsy in IV immunoglobulin group. Hospital deaths, duration of mechanical ventilation, hospital stay, and outcome at 3 and 6 months were not different between the two groups. Children with acute motor axonal neuropathy had better recovery at 6 months on IV immunoglobulin (58.3% vs 11.1%; p = 0.03), but not those with acute inflammatory demyelinating polyradiculoneuropathy (58.3% vs 72.2%; p = 0.22). In nonambulatory Guillain-Barré syndrome children, complete recovery at 6 months was similar in IV immunoglobulin and non-IV immunoglobulin group (57.4% vs 57.1%; p = 0.98).

CONCLUSIONS

In Indian children with Guillain-Barré syndrome, the outcome at 6 months in IV immunoglobulin treated group was similar to non-IV immunoglobulin group. Children with acute motor axonal neuropathy responded better to IV immunoglobulin.

摘要

目的

评估印度吉兰-巴雷综合征(Guillain-Barré syndrome,GBS)患儿接受静脉注射免疫球蛋白(intravenous immunoglobulin,IVIg)治疗与未接受任何特定治疗的患儿的结局。

设计

单中心、前瞻性横断面研究。

地点

三级神经科教学医院。

患者

2008 年 1 月至 2017 年 4 月,从前瞻性维持的吉兰-巴雷综合征登记处纳入≤18 岁的吉兰-巴雷综合征患儿。根据神经传导研究,患儿分为急性炎症性脱髓鞘性多发性神经病、急性运动轴索性神经病、急性运动感觉轴索性神经病和不可兴奋运动神经。

干预措施

在 138 例儿科吉兰-巴雷综合征患儿中,50 例接受 IVIg 治疗,另从同一登记处选择 50 例年龄和高峰残疾相匹配的对照(未接受 IVIg 或血浆置换)进行比较。

测量和主要结果

根据 0-10 临床分级量表,将 3 个月和 6 个月的结局定义为完全(临床分级量表<3)、部分(临床分级量表 3-5)和不良(临床分级量表>5)恢复。主要结局为 3 个月和 6 个月时 IVIg 组和非-IVIg 组完全恢复的患者比例。次要结局包括院内死亡、机械通气时间和住院时间。在急性运动轴索性神经病和急性炎症性脱髓鞘性多发性神经病组进行了亚组分析。除 IVIg 组疾病持续时间较短和面部瘫痪比例较高外,两组的基线特征相似。两组之间的院内死亡、机械通气时间、住院时间以及 3 个月和 6 个月的结局均无差异。急性运动轴索性神经病患儿接受 IVIg 治疗 6 个月时的恢复情况更好(58.3%比 11.1%;p=0.03),但急性炎症性脱髓鞘性多发性神经病患儿则不然(58.3%比 72.2%;p=0.22)。在不能行走的吉兰-巴雷综合征患儿中,6 个月时 IVIg 组和非-IVIg 组的完全恢复情况相似(57.4%比 57.1%;p=0.98)。

结论

在印度吉兰-巴雷综合征患儿中,6 个月时 IVIg 治疗组的结局与非-IVIg 组相似。急性运动轴索性神经病患儿对 IVIg 反应更好。

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