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儿童发 bronchiectasis 与成人发 bronchiectasis 的成年人中较大的疾病严重程度:一个多中心 EMBARC 注册研究
Luai Khalaili1,2, Stefano Aliberti3,4, Kateryna Viligorska5
1Pulmonology institute, Carmel medical center - Haifa (Israel).
The European respiratory journal
|July 3, 2025
概括
患有儿科发作的支气管切除症 (POBE) 的年轻人显示出比成人发作的支气管切除症 (AOBE) 更严重的疾病严重程度. 这与较长的症状持续时间和先天性原因有关,这表明当前的严重程度得分可能低估了POBE.
科学领域:
- 肺部医学 肺部医学
- 呼吸系统健康 呼吸系统健康
- 临床研究 临床研究
背景情况:
- 儿科发作的支气管切除症 (POBE) 在成年人中代表性不足,了解程度不佳.
- 这项研究将POBE的特征和严重程度与成人发 bronchiectasis (AOBE) 进行比较.
研究的目的:
- 为了比较POBE和AOBE的成年人之间的临床特征和疾病严重程度.
- 为了确定与支气管切除症患者疾病严重程度相关的因素.
主要方法:
- 来自四个欧洲支气管病例登记中心 (EMBARC) 数据的分析.
- POBE是由18岁之前的症状发作定义的; AOBE是由18岁或之后的症状发作定义的.
- 多变量模型评估了肺功能,Pseudomonas aeruginosa感染,恶化和住院情况.
主要成果:
- 与AOBE患者 (n=249) 相比,POBE患者的疾病持续时间更长,肺功能更差 (FEV1% 70.8 vs 84.2),放射性程度更大,感染率更高 (72.3% vs 54.6%).
- 症状持续时间延长与P. aeruginosa感染,住院治疗,恶化和FEV1%的降低独立相关.
- 先天性原因,如原发性状动力障碍和原发性免疫缺陷加剧了疾病的严重程度.
结论:
- 与AOBE相比,POBE的成年人存在的疾病严重程度明显更大.
- 症状持续时间延长和先天性病因导致POBE严重程度增加.
- 目前的支气管切除症严重程度得分可能低估了POBE;优化过渡到成人护理对于缓解进展至关重要.
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