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Structural determinants of long-term functional outcomes in young children with cystic fibrosis

Lidija Turkovic1, Daan Caudri2,3,4, Tim Rosenow2,5

  • 1Telethon Kids Institute, Perth, Australia lidijat@gmail.com.

The European Respiratory Journal
|March 7, 2020
PubMed

Insights

Early chest CT scans in children with cystic fibrosis (CF) can predict future lung function decline. Identifying structural lung disease early with CT scans is crucial for improving CF patient outcomes.

Area of Science:

  • Pediatric Pulmonology
  • Radiology
  • Cystic Fibrosis Research

Background:

  • Accelerated lung function decline in cystic fibrosis (CF) begins in adolescence.
  • Respiratory complications are the primary cause of mortality in later life.
  • The relationship between early childhood structural lung disease and lung function decline is not well understood.

Purpose of the Study:

  • To investigate the association between early childhood structural lung disease detected by chest CT and long-term lung function in CF patients.
  • To determine if chest CT findings in early childhood can predict adverse long-term outcomes in CF.
  • To assess the utility of chest CT in monitoring disease progression and therapeutic response in CF.

Main Methods:

  • Utilized annual chest computed tomography (CT) scans from the AREST CF cohort (2005-2016) for children aged 3 months to 6 years.
  • Collected annual spirometry data from age 5 to 15 years for 167 children (89.77% of the cohort).
  • Analyzed CT scores and spirometry measurements (forced expiratory volume in 1 second - FEV1) to identify correlations.

Main Results:

  • Children with CT scores above the median at age 5-6 years were significantly more likely to have abnormal FEV1 in the subsequent 10 years (adjusted hazard ratio 2.67, p=0.037).
  • Structural abnormalities on CT, excluding bronchial wall thickening, correlated with lower FEV1 Z-scores.
  • Mucus plugging and air trapping were the most predictive CT sub-scores for reduced FEV1 (p<0.001 for both).

Conclusions:

  • Chest CT scans in early childhood can identify children with cystic fibrosis at risk for adverse long-term outcomes.
  • Preventing structural lung damage through early intervention, assessed by CT, is a key goal for improving CF patient prognosis.
  • Chest CT serves as an early indicator of potential long-term treatment success in the evolving therapeutic landscape for CF.
Abstract

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