Previous cross-sectional studies demonstrated that chest magnetic resonance imaging (MRI) is sensitive to detect early lung disease in infants and preschool children with cystic fibrosis (CF) without radiation exposure. However, the ability of MRI to detect progression of lung disease and the impact of early diagnosis in preschool children with CF remains unknown.
To investigate the potential of MRI to detect progression of early lung disease and impact of early diagnosis by CF newborn screening (NBS) in preschool children with CF.
Annual MRI was performed from diagnosis over four years in a cohort of 96 preschool children with CF (age 0-4 yr) that were concurrently diagnosed based on NBS (n=28) or clinical symptoms (n=68). MRI scans were evaluated using a dedicated morphofunctional score and the relationship between longitudinal MRI scores and respiratory symptoms, pulmonary exacerbations, upper airway microbiology and mode of diagnosis were determined.
The MRI global score increased in the total cohort of children with CF during preschool years (P<0.001) which was associated with cough, pulmonary exacerbations (P<0.0001), and detection of Staphylococcus aureus and Haemophilus influenzae (P<0.05). MRI-defined abnormalities in lung morphology, especially airway wall thickening/bronchiectasis, were lower in NBS compared to clinically diagnosed children with CF throughout the observation period (P<0.01).
MRI detected progression of early lung disease and benefits of early diagnosis by NBS in preschool children with CF. These findings support MRI as sensitive outcome measure for diagnostic monitoring and early intervention trials in preschool children with CF.