57 stable children with CF (mean age, 9.5±6.2 years) had chest MRI scans performed. All children with CF and 106 healthy children (mean age, 8.9±4.8 years) underwent age-adapted MBW testing to assess LCI and to calculate LCI z-scores. 36 children (mean age, 13.9±4.4 years) were investigated at time of pulmonary exacerbation either via MRI (n = 10) or MBW (n = 26).
Mean LCI z-score in healthy children was 0.1±1.2). LCI z-score was significantly higher in stable children with CF (5.4±5.5, P<0.001). Morphological, perfusion and global MRI chest score were elevated in stable children with CF and were positively correlated with a higher LCI z-score (R 0.682; 0.669; 0.711). Concordance between abnormal findings in MBW and MRI was above 70%. At exacerbation, LCI increased in children with CF from baseline (P<0.001) and decreased after antibiotic therapy (P<0.001). The MRI chest scores also decreased after iv antibiotic treatment (P<0.05).
LCI z-score and MRI chest score are elevated in CF and show a high correlation. Both methods are feasible and sensitive non-invasive markers of CF lung disease in children with CF under stable conditions and at time of exacerbation.