Abstract
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 < -1.96). Children with CF at 6 years of age had a mean LCI of 8.0 (1.3), which was significantly higher than healthy controls (6.9 (0.7) p< 0.001), 39% had an abnormal LCI and 2% an abnormal FEV1. MP-MRI outcomes in children with CF correlated with LCI (RQ: r= 0.7, p<0.001; RV: r= 0.63, p<0.001) but not FEV1 (RQ: r= -0.13, p=0.4; RV: r=-0.15, p=0.3).
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.
Footnotes
Cite this article as: European Respiratory Journal 2020; 56: Suppl. 64, 4311.
This abstract was presented at the 2020 ERS International Congress, in session “Respiratory viruses in the "pre COVID-19" era”.
This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).
- Copyright ©the authors 2020