Magnetic Resonance Imaging Detects Progression of Lung Disease and Impact of Newborn Screening in Preschool Children with Cystic Fibrosis

Foretelling Early Lung Disease Progression in Cystic Fibrosis: The Combined Benefits of Magnetic Resonance Imaging and Newborn Screening

“…All groups were started on chronic inhaled therapies at the time of diagnosis, but the LCD group was diagnosed at a mean age of 21.6 months, compared with 2.0 months for the ECD group and 1.1 months for the NBS group. Stahl and colleagues ( 13 ) were able to show for the first time that infants identified by NBS showed milder wall thickening and bronchiectasis at all time points in the study compared with those in the ECD or LCD groups. MRI-detected changes in lung morphology and perfusion also correlated with pulmonary exacerbations.…”

“…In this issue of the Journal , Stahl and colleagues (pp. 943–953 ) elegantly demonstrate the progression of early lung disease in preschool children with CF in a longitudinal study using MRI ( 13 ). This group of experienced MR imaging researchers capitalized on the rollout of an NBS pilot program in Southwestern Germany in 2016.…”

“…The landscape of CF therapeutics is changing rapidly, and outcome measures used to detect disease must follow suit. With this well-designed observational study of radiographic abnormalities in infants with CF detected by NBS or clinical symptoms, Stahl and colleagues ( 13 ) continue to push forward to establish lung MRI as the standard of care for the detection of mild, asymptomatic CF lung disease.…”

Contrast-enhanced Magnetic Resonance Imaging Does Not Detect a Progression in Lung Morphological Score in Preschool Children with Cystic Fibrosis

“…We have read with interest the study by Stahl and colleagues about the use of lung magnetic resonance imaging (MRI) in preschool children with cystic fibrosis from 0 to 4 years of age ( 1 ). Using multiple comparison statistical tests across patients from 0 to 4 years of age, the authors concluded that MRI could allow early detection of modifications over time, which were ascribed to the progression of the lung disease.…”

Reply to Dournes et al.: Contrast-enhanced Magnetic Resonance Imaging Does Not Detect a Progression in Lung Morphological Score in Preschool Children with Cystic Fibrosis

“…We thank Dournes and colleagues for their interest in our study on the longitudinal course of early cystic fibrosis (CF) lung disease detected by proton magnetic resonance imaging (MRI) ( 1 ) and for their comment regarding the analysis of the data from multiple time points. We investigated a cohort of 96 infants and preschool children with CF over the first 4 years of life by annual chest MRI scans, demonstrating that CF lung disease is present from the first year of life and progresses over time in these infants and preschool children under symptomatic standard of care ( 1 ). Furthermore, we were able to demonstrate that CF lung disease is less pronounced in infants and preschool children after a presymptomatic diagnosis by newborn screening than in infants and preschool children diagnosed due to clinical signs of CF ( 1 ).…”