儿科新发超耐火状态 (NOSRSE):一个病例系列
Hyungjin Chin1, Soo Yeon Kim2, Byung Chan Lim1
1Department of Pediatrics, Seoul National University Children's Hospital, Seoul, South Korea.
概括
在儿童中,新发性超耐火状态 (NOSRSE) 通常呈现为密码性发烧性感染相关综合征 (FIRES),导致不良结果. 初始脑脊液蛋白质升高表明儿科NOSRSE的严重程度.
科学领域:
- 儿科神经学 儿科神经学
- 发病学 (Epileptology) 是一个专业的学科.
- 关键护理医学 关键护理医学
背景情况:
- 新发性超耐火状态 (NOSRSE) 是一种严重的神经疾病,与显著的功能缺陷和持续性有关.
- 了解儿科NOSRSE的临床特征对于改善患者的治疗结果和管理策略至关重要.
研究的目的:
- 描述一个儿科队列的临床特征,其中包括新发性超耐火状态 (NOSRSE).
- 确定与儿科NOSRSE结果相关的因素.
主要方法:
- 两家三级医院在2013年至2024年期间诊断出NOSRSE的儿科患者的回顾性审查.
- 收集和分析临床数据,包括神经成像 (MRI),脑脊液 (CSF) 档案,脑电图 (EEG) 和治疗干预措施.
- 使用修改的兰金尺度 (mRS) 评估功能结局在发作后3个月.
主要成果:
- 包括23名患有NOSRSE的儿科患者,平均年龄为7.9岁; 91%患有发烧性感染相关综合征 (FIRES).
- 最初的脑脊液分析显示,77%的患者有异常 (39%的pleocytosis,68%的蛋白质升高),而61%的初始脑MRI正常.
- 在61%的患者中观察到功能结果差 (mRS ≥4),所有患者都经历了残留. 增加的初始CSF蛋白质,延长的输管/气管切除术和延迟的免疫疗法与较差的结果有关.
结论:
- 儿科NOSRSE,主要是密码性FIRES,与不利的长期神经结果有关.
- 最初的脑脊液 (CSF) 蛋白质水平可以作为儿童NOSRSE疾病严重程度的客观标记.
- 虽然MRI变化很常见,但它们并不总是与功能结果相关.
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