MRI detects early lung abnormality progression in young children with cystic fibrosis

In preschool children with cystic fibrosis, MRI detected progression of early lung disease, researchers reported.

“Early diagnosis by cystic fibrosis newborn screening has created a unique opportunity for early initiation of specialized interdisciplinary cystic fibrosis care,” Mirjam Stahl, MD, from the department of pediatric respiratory medicine, immunology and critical care medicine at Charité - Universitätsmedizin Berlin, and colleagues wrote in the American Journal of Respiratory and Critical Care Medicine. “While it was shown that early diagnosis and therapy results in nutritional benefits, the impact of cystic fibrosis newborn screening on trajectories of early cystic fibrosis lung disease remains less clear.”

Researchers performed annual MRIs from the first year of life to age 4 years in 96 children with cystic fibrosis (mean age at diagnosis, 8 months). All children received their cystic fibrosis diagnosis based on newborn screening (n = 28) or clinical symptoms (n = 68).

The MRI global score showed an increase from baseline to age 4 years and was associated with cough and pulmonary exacerbations (P < .001 for both), as well as the detection of Staphylococcus aureus and Haemophilus influenzae (P < .05 for both). In addition, the MRI morphology score and perfusion score increased from baseline to age 4 years (P < .001 for both).

The researchers reported fewer MRI-defined abnormalities in lung morphology, specifically airway wall thickening/bronchiectasis, among children diagnosed based on newborn screening compared with children diagnosed by clinical symptoms (P < .01).

Children with clinical symptoms and/or MRI score deterioration initiated inhalation therapy with dornase alfa (Pulmozyme, Genentech); this therapy was more frequently administered to children diagnosed by early and late clinical diagnosis compared with children diagnosed based on newborn screening (P < .001 and P < .05, respectively).

“Collectively, our results support MRI as a quantitative radiation-free outcome measure for longitudinal surveillance of early lung disease, and as a potential endpoint in early intervention trials testing novel therapies that may slow disease progression or even prevent lung damage in infants and preschool children with cystic fibrosis,” the researchers wrote.