Ramsey Kathryn A, Schultz André
Wal-yan Respiratory Research Centre, Telethon Kids Institute, University of Western Australia, Perth, WA, Australia.
Respiratory Medicine, Perth Children's Hospital, Perth, WA, Australia.
Front Pediatr. 2022 Sep 16;10:1010016. doi: 10.3389/fped.2022.1010016. eCollection 2022.
Bronchiectasis (not related to cystic fibrosis) is a chronic lung disease caused by a range of etiologies but characterized by abnormal airway dilatation, recurrent respiratory symptoms, impaired quality of life and reduced life expectancy. Patients typically experience episodes of chronic wet cough and recurrent pulmonary exacerbations requiring hospitalization. Early diagnosis and management of childhood bronchiectasis are essential to prevent respiratory decline, optimize quality of life, minimize pulmonary exacerbations, and potentially reverse bronchial disease. Disease monitoring potentially allows for (1) the early detection of acute exacerbations, facilitating timely intervention, (2) tracking the rate of disease progression for prognostic purposes, and (3) quantifying the response to therapies. This narrative review article will discuss methods for monitoring disease progression in children with bronchiectasis, including lung imaging, respiratory function, patient-reported outcomes, respiratory exacerbations, sputum biomarkers, and nutritional outcomes.
支气管扩张症（与囊性纤维化无关）是一种由多种病因引起的慢性肺部疾病，其特征为气道异常扩张、反复出现呼吸道症状、生活质量受损以及预期寿命缩短。患者通常会经历慢性湿性咳嗽发作和需要住院治疗的反复肺部加重。儿童支气管扩张症的早期诊断和管理对于预防呼吸功能下降、优化生活质量、减少肺部加重以及可能逆转支气管疾病至关重要。疾病监测有可能实现以下几点：（1）早期发现急性加重，便于及时干预；（2）为预后目的跟踪疾病进展速度；（3）量化对治疗的反应。这篇叙述性综述文章将讨论监测支气管扩张症儿童疾病进展的方法，包括肺部成像、呼吸功能、患者报告结局、呼吸道加重、痰液生物标志物和营养结局。
