Lung clearance index and functional MRI outcomes to assess lung disease in preschool children with cystic fibrosis

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Background: Lung disease starts early in life in children with cystic fibrosis (CF). Lung clearance index (LCI) and functional matrix-pencil magnetic resonance imaging (MP-MRI) are sensitive and non-invasive tools to monitor early lung disease. Aim: Assess lung function outcomes (LCI; FEV1 from spirometry) in preschool children with CF and healthy controls and their association with MP-MRI outcomes. Methods: Children with CF from a nationwide newborn-screened cohort in Switzerland (SCILD) underwent lung function testing and MP-MRI at 4 and 6 years of age. MP-MRI outcomes included relative lung perfusion (RQ) and ventilation (RV) impairment as percentage of lung volume. Lung function outcomes at 6 years were compared to age-matched healthy controls. Results: Twenty-one children were recruited at 4 years (mean (standard deviation) 4.2y (0.4)) and 41 children at 6 years (6.7y (0.6)) along with 44 healthy controls (6.2y (0.4)). Children with CF at 4 years had a mean LCI of 8.0 (1.3); 24% had an abnormal LCI (upper limit of normal: 8.0), and 14% an abnormal FEV1 (z-score Conclusion: LCI and MP-MRI are superior to standard lung function testing in assessing early lung disease in children with CF. Functional MP-MRI allows visualization and quantification of ventilation and perfusion defects in CF lung disease.