PDF(Pubmed)
Abstract：
The forced expiratory volume in one second (FEV1) is regularly used for the follow-up of patients with non-cystic fibrosis bronchiectasis (nCF-BE). The lung clearance index (LCI), measured by the multiple breath washout test, has been recently proposed as a lung function measure and a potential tool more sensitive than the FEV1 measured by spirometry in assessing airway changes seen on imaging. While several data have been endorsed as a useful endpoint in clinical trials of patients with early or mild CF lung disease and as the main outcome measure in clinical trials with CFTR modulators in children and adolescents with CF, few data are available in the context of non-CF bronchiectasis. The aim of this pilot study was to compare the LCI with the FEV1 as well as the forced vital capacity (FVC), the forced expiratory flow at 25-75% of the FVC (FEF 25-75%), and chest imaging based on the modified Reiff score in patients with primary ciliary dyskinesia (PCD) and non-CF, non-PCD bronchiectasis (PCD-BE and nCFnPCD-BE). Additionally, we compared each test\'s duration and the preferred technique. Twenty children were included; nine had PCD-BE and eleven had nCFnPCD-BE. The median age was twelve years (ages ranging between five and eighteen years). The median LCI was seven while the median z-scores of the FEV1, FVC, and FEF 25-75% were -0.6, 0, and -0.9, respectively. No significant associations or correlations were observed between LCI, spirometric parameters, or the modified Reiff score. However, nearly half of the population (n = 9) had an abnormal LCI, while only 10% had an abnormal FEV1. A total of 75% of children preferred MBW, despite it lasting five times longer than spirometry. In this paper, the authors suggest that LCI might be useful in a cohort of pediatric patients with PCD-BE and nCFnPCD-BE for detecting early lung function changes during their follow-up. Additionally, MBW seems to be preferred by patients. These data may encourage further studies on this topic.
摘要：
一秒钟用力呼气量（FEV1）通常用于非囊性纤维化支气管扩张症（nCF-BE）患者的随访。肺清除指数(LCI),通过多次呼气冲洗测试测量，最近被提出作为一种肺功能测量和潜在的工具，在评估成像上看到的气道变化时，比肺活量测定法测量的FEV1更敏感。虽然一些数据已被认可为早期或轻度CF肺病患者的临床试验中的有用终点，并且在儿童和青少年CF患者中使用CFTR调节剂的临床试验中作为主要结果指标，在非CF支气管扩张的背景下，很少有数据可用。这项初步研究的目的是将LCI与FEV1以及强制肺活量（FVC）进行比较，在FVC的25-75％（FEF25-75％）的强制呼气流量，和基于改良Reiff评分的原发性纤毛运动障碍（PCD）和非CF患者的胸部影像学，非PCD支气管扩张（PCD-BE和nCFnPCD-BE）。此外,我们比较了每个测试的持续时间和首选技术。包括20名儿童；9名患有PCD-BE，11名患有nCFnPCD-BE。中位年龄为12岁（年龄在5至18岁之间）。LCI中位数为7，而FEV1、FVC、和FEF25-75%分别为-0.6、0和-0.9。LCI、肺活量测定参数、或修改后的Reiff分数。然而,近一半的人群(n=9)有异常的LCI,而只有10%的人群有异常的FEV1.总共75%的儿童更喜欢MBW，尽管它的持续时间比肺活量测定法长五倍。在本文中,作者认为,在PCD-BE和nCFnPCD-BE的儿科患者队列中,LCI可能有助于检测随访期间的早期肺功能变化.此外,MBW似乎是患者的首选。这些数据可能会鼓励对此主题的进一步研究。
