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Published on: December 18, 2020
A Pilot Study of Lung Clearance Index as a Useful Outcome Marker in the Follow-Up of Pediatric Patients with
Wael Alkoussa1, Laurence Hanssens1, Valerie Sputael1
1Department of Pulmonology and Allergology, Queen Fabiola Children's University Hospital, Université Libre de Bruxelles, 1020 Brussels, Belgium.
Insights
The lung clearance index (LCI) may detect early lung function changes in children with bronchiectasis more sensitively than traditional spirometry. Patients also preferred the multiple breath washout (MBW) test, despite its longer duration.
Area of Science:
- Pediatric Pulmonology
- Respiratory Medicine
- Diagnostic Imaging
Background:
- Non-cystic fibrosis bronchiectasis (nCF-BE) follow-up typically relies on spirometry, such as forced expiratory volume in one second (FEV1).
- The lung clearance index (LCI), from multiple breath washout (MBW) testing, is a newer measure proposed to be more sensitive than FEV1 for detecting airway changes.
- Limited data exist on LCI's utility in pediatric non-cystic fibrosis bronchiectasis, unlike its established role in cystic fibrosis trials.
Purpose of the Study:
- To compare LCI with spirometry (FEV1, FVC, FEF 25-75%) and imaging (modified Reiff score) in children with primary ciliary dyskinesia (PCD-BE) and non-CF, non-PCD bronchiectasis (nCFnPCD-BE).
- To assess test duration and patient preference between LCI (MBW) and spirometry.
- To evaluate LCI's potential as a sensitive tool for early lung function assessment in pediatric bronchiectasis.
Main Methods:
- A pilot study included twenty children (9 PCD-BE, 11 nCFnPCD-BE) aged 5-18 years.
- Lung function was assessed using MBW for LCI and spirometry for FEV1, FVC, and FEF 25-75%.
- Chest imaging was evaluated using the modified Reiff score; test durations and patient preferences were recorded.
Main Results:
- Median LCI was 7, with z-scores for FEV1, FVC, and FEF 25-75% at -0.6, 0, and -0.9, respectively.
- No significant correlations were found between LCI, spirometry, or imaging scores.
- Nearly half (9/20) of children had an abnormal LCI, compared to only 10% (2/20) with an abnormal FEV1; 75% preferred MBW despite its longer duration.
Conclusions:
- LCI may be a more sensitive indicator of early lung function changes in pediatric bronchiectasis (PCD-BE and nCFnPCD-BE) than standard spirometry.
- MBW testing appears to be preferred by pediatric patients, suggesting potential for improved adherence in follow-up.
- Further research is warranted to validate LCI's role in monitoring pediatric bronchiectasis and its clinical applications.
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.

