An update on multiple breath washout in children with cystic fibrosis.

Abstract

Introduction: Cystic fibrosis (CF) is an autosomal recessive disorder caused by mutations in the CF transmembrane regulator (CFTR) gene, leading to progressive lung disease and systemic complications. Lung disease remains the primary cause of morbidity and mortality, making early detection of lung function decline crucial. The Lung Clearance Index (LCI), derived from the multiple breath washout (MBW) test, has emerged as a sensitive measure for identifying early airway disease.

Areas covered: This review examines the technical aspects and clinical relevance of LCI, its advantages over traditional lung function tests, and its application in CF clinical trials. A focused literature review highlights LCI's utility in evaluating treatment efficacy and its potential integration into routine CF care.

Expert opinion: LCI is more sensitive than spirometry for detecting early lung function decline and is predominantly used in pediatric settings. Its use is expanding in adult CF populations as advances in treatment allow adults to maintain stable lung function. In clinical trials, LCI is widely recognized as an outcome measure. While implemented into clinical care in many centers in Europe, this is not yet the case in North America. Faster testing protocols and point-of-care interpretation tools will support LCI's integration into routine CF monitoring.