Neurology Team, Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, Hong Kong Special Administrative Region; Neurology Team, Department of Paediatrics and Adolescent Medicine, The University of Hong Kong, Hong Kong Special Administrative Region.
Neurology Team, Department of Paediatrics and Adolescent Medicine, Princess Margaret Hospital, Hong Kong Special Administrative Region.
Brain Dev. 2022 Nov;44(10):715-724. doi: 10.1016/j.braindev.2022.07.003. Epub 2022 Jul 26.
Guillain-Barré syndrome (GBS) is a rare acquired immune-mediated polyneuropathy. Updated population-based data concerning paediatric GBS is needed.
Paediatric patients aged below 18 years diagnosed with GBS between 2009 and 2018 in all 11 paediatric departments in Hong Kong were identified from the Hong Kong Hospital Authority Clinical Data Analysis and Reporting System. The collected data from medical health records were reviewed by paediatric neurologist from each department. Estimated incidence of paediatric GBS was calculated. We also compared our findings with other paediatric GBS studies in Asia.
63 subjects of paediatric GBS were identified, giving an estimated annual incidence of 0.62 per 100,000 population. Half of the subjects had acute inflammatory demyelinating polyneuropathy (AIDP) (n = 31; 49.2%), one quarter had Miller Fisher Syndrome (MFS) (n = 16; 25.4%), one-fifth had axonal types of GBS (n = 12; 19.0%), and four were unclassified. Paediatric subjects with axonal subtypes of GBS compared to the other 2 subtypes, had significantly higher intensive care unit (ICU) admission rates (p = 0.001) and longest length of stay (p = 0.009). With immunomodulating therapy, complete recovery was highest in those with MFS (100%), followed by AIDP (87.1%) and axonal GBS (75%). Our study also confirms a higher MFS rate for paediatric GBS in East Asia region and our study has the highest MFS rate (25.4%).
Our population-based 10-year paediatric GBS study provides updated evidence on estimated incidence, healthcare burden and motor outcome of each subtype of paediatric GBS and confirmed a higher occurrence of paediatric MFS in East Asia.
吉兰-巴雷综合征（GBS）是一种罕见的获得性免疫介导的多神经病。需要更新有关儿科 GBS 的基于人群的数据。
从香港医院管理局临床数据分析和报告系统中，确定了 2009 年至 2018 年间在香港所有 11 个儿科部门诊断为 GBS 的 18 岁以下儿科患者。从每个部门的儿科神经科医生那里审查了从医疗记录中收集的数据。计算了儿科 GBS 的估计发病率。我们还将我们的发现与亚洲其他儿科 GBS 研究进行了比较。
确定了 63 例儿科 GBS 患者，估计发病率为每 100,000 人口 0.62 例。一半的患者为急性炎症性脱髓鞘性多发性神经病（AIDP）（n=31；49.2%），四分之一为米勒费舍尔综合征（MFS）（n=16；25.4%），五分之一为轴索型 GBS（n=12；19.0%），还有四个未分类。与其他 2 个亚型相比，患有轴索型 GBS 的儿科患者入住重症监护病房（ICU）的比例明显更高（p=0.001），住院时间最长（p=0.009）。接受免疫调节治疗后，MFS 患者的完全恢复率最高（100%），其次是 AIDP（87.1%）和轴索 GBS（75%）。我们的研究还证实东亚地区儿科 GBS 的 MFS 发生率较高，我们的研究 MFS 发生率最高（25.4%）。
我们的基于人群的 10 年儿科 GBS 研究提供了有关儿科 GBS 每种亚型的估计发病率、医疗负担和运动结果的最新证据，并证实东亚地区儿科 MFS 的发生率较高。
