A Pilot Study of Lung Clearance Index as a Useful Outcome Marker in the Follow-Up of Pediatric Patients with Non-Cystic Fibrosis Bronchiectasis?

Wael Alkoussa; Laurence Hanssens; Valerie Sputael; Frederico De Lucia; Christine Quentin

doi:10.3390/children10050791

A Pilot Study of Lung Clearance Index as a Useful Outcome Marker in the Follow-Up of Pediatric Patients with Non-Cystic Fibrosis Bronchiectasis?

Children (Basel). 2023 Apr 27;10(5):791. doi: 10.3390/children10050791.

Authors

Wael Alkoussa¹, Laurence Hanssens¹, Valerie Sputael¹, Frederico De Lucia², Christine Quentin¹

Affiliations

¹ Department of Pulmonology and Allergology, Queen Fabiola Children's University Hospital, Université Libre de Bruxelles, 1020 Brussels, Belgium.
² Department of Radiology, Queen Fabiola Children's University Hospital, Université Libre de Bruxelles, 1020 Brussels, Belgium.

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.

Keywords: forced expiratory volume in one second; multiple breath washout; primary ciliary dyskinesia; spirometry.

Grants and funding

The authors received financial support for publication from the Queen Fabiola Children’s University Hospital, Brussels, Belgium through the research funding program of Quentin according to the billing reference 2277590.