资源有限环境下的小儿吉兰-巴雷综合征：临床特征、诊断与管理挑战及医院结局
Pediatric Guillain-Barré Syndrome in a Resource Limited Setting: Clinical Features, Diagnostic and Management Challenges, and Hospital Outcome.
作者信息
Shibeshi Mulugeta Sitot, Mengesha Adane Alto, Gari Kefyalew Taye
机构信息
Department of Pediatrics and Child Health, Hawassa University, Hawassa, Ethiopia.
Department of Pediatrics and Child Health, Arba Minch University, Arba Minch, Ethiopia.
出版信息
Pediatric Health Med Ther. 2023 Mar 22;14:107-115. doi: 10.2147/PHMT.S401461. eCollection 2023.
BACKGROUND
Guillain-Barré syndrome (GBS) is an acute immune-mediated peripheral neuropathy with a highly variable clinical course and outcome. There remain diagnostic and treatment challenges in resource limited settings. This study aimed to describe the clinical presentation, diagnostic and management challenges, and hospital outcome of children with GBS in southern Ethiopia.
METHODS
A retrospective chart review of children aged ≤14 years who were admitted with a diagnosis of GBS to Hawassa University Comprehensive Specialized Hospital from 2017 to 2021 was done. Medical records of 102 children who fulfilled the Brighton Criteria for GBS were reviewed, and data on demographic, clinical characteristics, investigation findings, treatment, and outcome were collected. Logistic regression analysis was done to determine factors associated with mortality.
RESULTS
The mean age of the study subjects was 7.25±3.91 years and 63.7% were male. Antecedent event was present in 48% of the cases, and the most common triggering factor was upper respiratory tract infection (63.8%). The mean Hughes disability score was 4.23±0.54, 4.48±0.71, and 4.03±0.86 at admission, nadir and discharge from hospital, respectively. Cranial nerve involvement was present in 27.5% of patients and bulbar palsy was the most common finding. Dysautonomia was observed in 57.8% of the participants. Sixty-three patients (61.8%) needed ICU care but only 43 of them (68.3%) were admitted to ICU. Similarly, 31 patients (30.4%) required respiratory support but only 24 of them (77.4%) were on mechanical ventilator. No patient had nerve conduction study. Only 5.9% of patients received IVIG. Thirteen patients (12.7%) died of GBS and the presence of respiratory failure was the only determinant of mortality [AOR = 11.40 (95% CI: 1.818, 71.52), p = 0.009].
CONCLUSION
There is a gap in the diagnosis and management of children with GBS; and mortality from the disease is higher than reports from other settings.
背景
吉兰-巴雷综合征（GBS）是一种急性免疫介导的周围神经病，临床病程和结局差异很大。在资源有限的环境中，诊断和治疗仍面临挑战。本研究旨在描述埃塞俄比亚南部GBS患儿的临床表现、诊断和管理挑战以及住院结局。
方法
对2017年至2021年在哈瓦萨大学综合专科医院确诊为GBS的14岁及以下儿童进行回顾性病历审查。审查了102名符合GBS布莱顿标准的儿童的病历，并收集了人口统计学、临床特征、检查结果、治疗和结局的数据。进行逻辑回归分析以确定与死亡率相关的因素。
结果
研究对象的平均年龄为7.25±3.91岁，63.7%为男性。48%的病例有前驱事件，最常见的触发因素是上呼吸道感染（63.8%）。入院时、最低点和出院时的平均休斯残疾评分分别为4.23±0.54、4.48±0.71和4.03±0.86。27.5%的患者有颅神经受累，最常见的表现是延髓麻痹。57.8%的参与者出现自主神经功能障碍。63名患者（61.8%）需要重症监护，但其中只有43名（68.3%）被收入重症监护病房。同样，31名患者（30.4%）需要呼吸支持，但其中只有24名（77.4%）使用机械通气。没有患者进行神经传导研究。只有5.9%的患者接受了静脉注射免疫球蛋白。13名患者（12.7%）死于GBS，呼吸衰竭的存在是唯一的死亡决定因素[AOR = 11.40（95%CI：1.818，71.52），p = 0.009]。
结论
GBS患儿的诊断和管理存在差距；该疾病的死亡率高于其他地区的报告。
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