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格林-巴利综合征患儿血清免疫球蛋白G水平系列及其与预后的相关性

Serial Serum Immunoglobulin G Levels and Correlation with Outcomes in Children with Guillain Barre Syndrome.

作者信息

Khan Dilsad Ali, Randhawa Manjinder Singh, Jayashree Muralidharan, Rawat Amit, Suthar Renu, Bansal Arun

机构信息

Division of Pediatric Critical Care, Department of Pediatrics, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012, India.

Division of Pediatric Immunology and Rheumatology, Department of Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India.

出版信息

Indian J Pediatr. 2025 Jun 26. doi: 10.1007/s12098-025-05633-4.

Abstract

OBJECTIVES

Guillain-Barré Syndrome (GBS) has variable severity, inconsistent therapy response, and uncertain recovery. Intravenous immunoglobulins (IVIG) are used to treat this immune-mediated disorder. The authors investigated serial IgG levels and outcomes in IVIG-treated GBS patients.

METHODS

This prospective observational study from July 2019 and December 2021 in a tertiary pediatric referral hospital included children ≤12 y with GBS diagnosis. Children with recurrent GBS or chronic neuromuscular disorders were excluded. All patients received IVIG (2 g/kg). Serum IgG levels were measured at enrolment, 2 wk, 4 wk, and 3 mo. IgG levels and change in IgG levels from baseline were correlated with outcome. The authors hypothesize that IgG levels before and after therapy may affect functional status outcomes [Medical Research Council (MRC) sum score, GBS disability score].

RESULTS

Seventy patients, median age 6.5 y, were enrolled. Acute motor axonal neuropathy predominated in nerve conduction studies (n = 46, 66%). Admission, 2 wk, 4 wk, and 3 mo median IgG levels were 10.1, 17.9, 13.0, and 9.9 g/L. At 2 wk, higher IgG levels and increments were associated with better functional status. Higher ΔIgG at 2 wk correlated with a shorter hospital stay (r = -0.381, p = 0.001). Lower levels of IgG (16.01 vs. 20.56, p = 0.006) and ΔIgG (6.83 vs. 10.03, p = 0.041) at 2 wk were associated with inability to walk independently at 3 mo.

CONCLUSIONS

The increase in serum IgG after IVIG therapy weakly correlates with GBS outcomes in children. A second IVIG dose for non-responders did not improve outcomes at 3 mo, suggesting unknown factors causing severe disease and poor recovery that need further studies.

摘要

目的

吉兰 - 巴雷综合征(GBS)病情严重程度不一,治疗反应不一致,恢复情况也不确定。静脉注射免疫球蛋白(IVIG)用于治疗这种免疫介导的疾病。作者研究了接受IVIG治疗的GBS患者的系列IgG水平及预后情况。

方法

这项于2019年7月至2021年12月在一家三级儿科转诊医院开展的前瞻性观察性研究纳入了年龄≤12岁且诊断为GBS的儿童。复发性GBS或慢性神经肌肉疾病患儿被排除。所有患者均接受IVIG(2 g/kg)治疗。在入组时、2周、4周和3个月时测量血清IgG水平。IgG水平以及IgG水平相对于基线的变化与预后相关。作者推测治疗前后的IgG水平可能会影响功能状态预后[医学研究委员会(MRC)总分、GBS残疾评分]。

结果

共纳入70例患者,中位年龄6.5岁。神经传导研究中急性运动轴索性神经病占主导(n = 46,66%)。入组时、2周、4周和3个月时的中位IgG水平分别为10.1、17.9、13.0和9.9 g/L。在2周时,较高的IgG水平及升高幅度与更好的功能状态相关。2周时较高的ΔIgG与较短的住院时间相关(r = -0.381,p = 0.001)。2周时较低的IgG水平(16.01 vs. 20.56,p = 0.006)和ΔIgG水平(

6.83 vs. 10.03,p = 0.041)与3个月时无法独立行走相关。

结论

IVIG治疗后血清IgG的升高与儿童GBS的预后弱相关。对无反应者给予第二剂IVIG并未改善3个月时的预后,这表明存在导致严重疾病和恢复不佳的未知因素,需要进一步研究。

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