儿科重症监护室正在调查免疫的先天性错误吗?
Cecília Coelho Moraes de Brito1, Patrícia Gomes de Matos Bezerra1, Paula Teixeira Lyra2
1Instituto de Medicina Integral Prof. Fernando Figueira (IMIP), Departamento de Pediatria, Recife, PE, Brazil.
Jornal de pediatria
|February 8, 2025
概括
在儿科重症监护病房住院的少数患有先天性免疫错误 (IEI) 警告症状的儿童接受了诊断检查. 这种错过的机会可能会增加这些脆弱儿童的死亡风险.
科学领域:
- 儿科重症监护医药 儿科重症监护医药
- 临床免疫学 临床免疫学
- 遗传和罕见疾病是遗传和罕见疾病.
背景情况:
- 免疫的先天性错误 (IEI) 是一组影响免疫系统的遗传疾病.
- 早期诊断和治疗IEI对于改善患者的治疗结果和降低死亡率至关重要.
- 患有警告症状的儿童被送往儿科重症监护室 (PICU),可能未被诊断出IEI.
研究的目的:
- 确定在PICU住院的儿童中出现警告迹象的IEI诊断调查的频率.
- 分析年龄组,预警信号,IEI调查和临床结果 (包括死亡) 之间的关系.
主要方法:
- 在两年内接受转诊医院PICU的儿童上进行了一项回顾性横截面研究.
- 收集的数据包括社会人口统计和临床变量,IEI警告标志的存在 (按年龄分类:长达一年或以上),IEI调查,免疫学随访和出院状态 (活着或已故).
主要成果:
- 在680名儿童中,330名1岁以下,350名1岁以上.
- 在1岁以下的儿童中,32.7%的儿童有≥2个警告信号,但只有20.4%的儿童被调查IEI.
- 在一年以上的儿童中,13.7%有≥2个警告信号,43.8%被调查IEI. 死亡率在1岁以下的婴儿中最高,具有≥2个警告标志 (58.8%).
结论:
- 在PICU中出现警告迹象的儿童中,IEI的诊断调查很少发生.
- 在重症儿童中,人们错过了及时诊断IEI的机会,这可能会导致不良结果.
- 增加对高风险儿科患者IEI的警和调查是有必要的,以减少死亡率.
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