格林-巴利综合征患儿的临床流行病学特征及预后预测。

IF 3.2 3区 医学 Q1 PEDIATRICS
Debashree Priyadarshini, Velaga Anuhya, Anuspandana Mahapatra
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引用次数: 0

摘要

背景:格林-巴勒综合征(GBS)是一种罕见但严重的免疫介导的神经病变,以急性发作的运动无力和潜在的呼吸衰竭为特征。其发病机制通常涉及先前感染引发的分子模仿,导致周围神经的自身免疫靶向。流行病学数据表明,传染性暴发与GBS发病率增加之间存在相关性,2025年印度浦那与空肠弯曲杆菌肠炎相关的病例激增就是例证。方法:这项前瞻性观察研究在一家三级医院的儿科重症监护病房(PICU)进行了超过24个月的研究。获得伦理批准。连续入组包括2-14岁诊断为GBS并在两周内出现症状的儿童。收集了全面的临床、电生理和治疗数据。患者前瞻性随访6个月,使用GBS残疾评分评估结果。采用改良的Erasmus GBS结局评分(mEGOS)和Erasmus GBS呼吸功能不全评分(EGRIS)进行预后评估。结果:研究队列包括27名儿童,5-9岁的男性最常受影响。平均(±SD)年龄为6.56(±3.00)岁。所有参与者都报告了先前的疾病,主要是肠胃炎。临床上,81.5%的患者出现对称性运动无力,77.8%的患者表现为感觉受累。11.1%的患者出现需要机械通气的呼吸损害。电生理学研究发现急性运动轴索神经病(AMAN)是主要的变体,急性运动感觉轴索神经病(AMSAN)表现出更严重的临床病程,包括更高的通气要求和更差的功能结果。预后评估显示入院时mEGOS中位(IQR)评分为5分(4,6分),入院后1周为4分(3,6分)。这些分数显著预测了4周、3个月和6个月的结果,分数越高,残疾程度越高。该队列的平均EGRIS评分为5.67,较高的评分预示着机械通气需求的增加。值得注意的是,所有患者都取得了良好的结果,没有死亡,突出了实施管理方案的有效性。结论:我们的研究结果表明,mEGOS和EGRIS是儿童GBS的有效预后工具。mEGOS可靠地预测多个恢复阶段的功能结果,而EGRIS在早期识别有呼吸衰竭风险需要机械通气的患者方面特别有用。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Clinico-epidemiological profile and prediction of outcome in children with Guillain-Barre syndrome.

Background: Guillain-Barré Syndrome (GBS) is a rare but serious immune-mediated neuropathy characterized by acute-onset motor weakness and potential respiratory failure. Its pathogenesis often involves molecular mimicry triggered by antecedent infections, leading to autoimmune targeting of peripheral nerves. Epidemiological data suggest a correlation between infectious outbreaks and increased GBS incidence, as exemplified by the 2025 surge in cases associated with Campylobacter jejuni enteritis in Pune, India.

Methods: This prospective observational study was conducted over 24 months in the Pediatric Intensive Care Unit (PICU) of a tertiary care hospital. Ethical approval was obtained. Consecutive enrolment included children aged 2-14 years diagnosed with GBS who presented within two weeks of symptom onset. Comprehensive clinical, electrophysiological, and treatment data were collected. Patients were prospectively followed for six months, and outcomes were assessed using the GBS Disability Score. The modified Erasmus GBS outcome Score (mEGOS) and Erasmus GBS Respiratory Insufficiency Score (EGRIS) were applied for prognostic evaluation.

Results: The study cohort comprised 27 children, with males aged 5-9 years being the most commonly affected. The mean (± SD) age was 6.56 (± 3.00) years. All participants reported antecedent illnesses, predominantly gastroenteritis. Clinically, symmetric motor weakness was observed in 81.5%, and 77.8% exhibited sensory involvement. Respiratory compromise requiring mechanical ventilation occurred in 11.1% of patients. Electrophysiological studies identified acute motor axonal neuropathy (AMAN) as the predominant variant, with acute motor sensory axonal neuropathy (AMSAN) demonstrating more severe clinical courses, including higher ventilation requirements and poorer functional outcomes. Prognostic assessment revealed median (IQR) mEGOS scores of 5 (4,6) at admission and 4 (3,6) at 1-week post-admission. These scores significantly predicted outcomes at 4 weeks, 3 months, and 6 months, with higher scores correlating with greater disability. The cohort's mean EGRIS score was 5.67, with higher scores predictive of increased mechanical ventilation requirements.Notably, all patients achieved favourable outcomes with no mortality, highlighting the effectiveness of the implemented management protocol.

Conclusion: Our findings demonstrate that mEGOS and EGRIS are effective prognostic tools in pediatric GBS. The mEGOS reliably predicts functional outcomes at multiple recovery stages, while the EGRIS is particularly useful in early identification of patients at risk for respiratory failure requiring mechanical ventilation.

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来源期刊
CiteScore
6.10
自引率
13.90%
发文量
192
审稿时长
6-12 weeks
期刊介绍: Italian Journal of Pediatrics is an open access peer-reviewed journal that includes all aspects of pediatric medicine. The journal also covers health service and public health research that addresses primary care issues. The journal provides a high-quality forum for pediatricians and other healthcare professionals to report and discuss up-to-the-minute research and expert reviews in the field of pediatric medicine. The journal will continue to develop the range of articles published to enable this invaluable resource to stay at the forefront of the field. Italian Journal of Pediatrics, which commenced in 1975 as Rivista Italiana di Pediatria, provides a high-quality forum for pediatricians and other healthcare professionals to report and discuss up-to-the-minute research and expert reviews in the field of pediatric medicine. The journal will continue to develop the range of articles published to enable this invaluable resource to stay at the forefront of the field.
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