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在儿科重症监护中免疫的先天错误:患病率,特征和预后
Fatih Celmeli1, Ayse Oz2, Hasan Serdar Kihtir3
1Department of Pediatric Allergy and Immunology, University of Health Science, Antalya Education and Research Hospital, Antalya, Turkey. fcelmeli@hotmail.com.
Journal of clinical immunology
|October 15, 2024
概括
新诊断的先天性免疫错误 (IEI) 发生在4.39%的儿科重症监护室入院患者中. 高死亡率 (58%) 强调了对这些免疫系统疾病的早期诊断和干预的迫切需要.
科学领域:
- 儿科免疫学 儿科免疫学
- 临界护理医学 临界护理医学
- 遗传性疾病 遗传性疾病
背景情况:
- 免疫的先天性错误 (IEI) 是一种影响免疫功能的遗传疾病.
- 早期发现IEI对于及时管理和改善患者治疗结果至关重要.
- 儿科重症监护室 (PICU) 管理重症儿童,其中一些患有未诊断的IEI.
研究的目的:
- 确定PICU中新诊断的IEI患者的发病率和特征.
- 分析临床过程并确定这一队列中的死亡率预测因素.
- 评估已确立的警告标志对于在重症儿童中检测IEI的有用性.
主要方法:
- 通过使用综合免疫学评估协议,在三年内对753名PICU住院患者进行前性查.
- 排除患有先前存在的疾病,并发症,创伤或手术后状态的患者.
- 详细分析诊断的IEI类型,出现症状和患者的结果,包括死亡率.
主要成果:
- 在PICU人群中 (33名患者) 观察到新诊断的IEI发生率为4.39%.
- 免疫缺陷与免疫失调 (48.5%) 和联合免疫缺陷 (24.2%) 是最常见的.
- 严重的病毒感染 (61%) 和危及生命的感染 (51.7%) 是常见的;只有31%的人遇到了杰弗里·莫德尔基金会的两个警告标志. 死亡率高达58%,死亡率很高.
结论:
- 显著比例的PICU入院患者没有诊断IEI,经常出现严重的感染.
- 对已确立的警告标志的遵守率较低表明,目前对重症儿童的查方案存在局限性.
- 高死亡率需要改进早期检测策略,包括新生儿查和家庭研究,对IEI重症监护患者.
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