Paediatric Respiratory Medicine Department, Birmingham Women's and Children's NHS Foundation Trust, Birmingham, UK.
Departments of Cystic Fibrosis and Paediatric Respiratory Medicine, Royal Brompton and Harefield Foundation Trust, London, UK; Cystic Fibrosis and Chronic Lung Infection Research Group, National Heart and Lung Institute, Imperial College London, London, UK.
Respir Med. 2022 Sep;201:106937. doi: 10.1016/j.rmed.2022.106937. Epub 2022 Jul 21.
Non cystic fibrosis, non primary ciliary dyskinesia bronchiectasis (nCFnPCD-BE) results in significant morbidity with few evidence-based treatments.
assessments are required to assess severity and evaluate treatment. Lung clearance index (LCI) measures ventilation inhomogeneity and is a sensitive test of disease in CF; its use in nCFnPCD-BE is unclear.
A systematic review of LCI in nCFnPCD-BE was performed using standard methodology (protocol registered on PROSPERO, University of York).
Of 276 records identified, 12 articles, describing 519 adult and paediatric patients in cross-sectional studies were included, addressing several domains. 1: What is the utility of LCI in detecting disease and severity? LCI detected disease in adults, differentiating bronchiectasis from controls (AUC 0.90 to 0.96) and mild from moderate/severe bronchiectasis on CT (AUC 0.73). 2: Does LCI correlate with spirometry and imaging? LCI correlated with spirometry in adult (r = -0.37 to -0.61) and paediatric (r = -0.6) groups, signs of bronchiectasis on CT, and CT scoring systems (modified Reiff). 3: Does LCI relate to subjective scores of severity? In adults, LCI correlated with St. George's Respiratory Questionnaire (r = 0.18) and Bronchiectasis Severity Index (r = 0.45). 4: Does LCI identify response to intervention? LCI did not change in studies examining LCI pre-post intervention (adults treated for exacerbation and undergoing physiotherapy). Overall study quality was variable.
Contrary to data in CF, the review did not identify good quality studies defining the role of LCI in children with bronchiectasis. In adults, LCI was a sensitive measure of disease severity and correlated with clinical assessment tools.
非囊性纤维化、非原发性纤毛运动障碍性支气管扩张症（nCFnPCD-BE）会导致严重的发病率，且治疗方法有限，缺乏循证医学证据。
需要评估来评估严重程度并评估治疗效果。肺清除指数（LCI）测量通气不均匀性，是 CF 疾病的敏感测试；但其在 nCFnPCD-BE 中的应用尚不清楚。
使用标准方法（约克大学 PROSPERO 注册的方案）对 nCFnPCD-BE 中的 LCI 进行系统评价。
在确定的 276 条记录中，有 12 篇文章描述了横断面研究中的 519 名成人和儿科患者，涉及多个领域。1：LCI 在检测疾病和严重程度方面的作用是什么？LCI 在成人中检测到疾病，将支气管扩张症与对照组区分开来（AUC 0.90 至 0.96），并在 CT 上区分轻度与中度/重度支气管扩张症（AUC 0.73）。2：LCI 与肺功能和影像学相关吗？LCI 与成人（r = -0.37 至-0.61）和儿科（r = -0.6）组的肺功能相关，与 CT 上的支气管扩张症征象和 CT 评分系统（改良 Reiff）相关。3：LCI 与严重程度的主观评分有关吗？在成年人中，LCI 与圣乔治呼吸问卷（r = 0.18）和支气管扩张严重指数（r = 0.45）相关。4：LCI 能识别干预反应吗？在研究 LCI 干预前后（成人因加重而接受治疗和进行物理治疗），LCI 没有变化。整体研究质量各不相同。
与 CF 中的数据相反，该综述没有发现质量良好的研究定义了 LCI 在患有支气管扩张症的儿童中的作用。在成年人中，LCI 是一种敏感的疾病严重程度衡量标准，并与临床评估工具相关。
