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Progression of early structural lung disease in young children with cystic fibrosis assessed using CT

Lauren S Mott1, Judy Park, Conor P Murray

  • 1Telethon Institute for Child Health Research and Centre for Child Health Research, University of Western Australia, Perth, Australia.

Thorax
|December 28, 2011
PubMed

Insights

Structural lung disease in infants with cystic fibrosis (CF) persists and progresses over one year. Worsening inflammation and infection are linked to this progression, highlighting the need for early intervention in CF patients.

Area of Science:

  • Pediatric Pulmonology
  • Radiology
  • Genetics

Background:

  • Cross-sectional studies suggest neutrophilic inflammation and infection are risk factors for early lung disease in cystic fibrosis (CF).
  • Longitudinal progression of lung disease in newborn screened CF populations remains understudied.

Purpose of the Study:

  • To assess the persistence and progression of early structural lung disease in CF infants over one year.
  • To identify factors associated with the radiological persistence and progression of lung disease.

Main Methods:

  • Analysis of 444 annual chest CT scans from 143 CF children (aged 0.2-6.5 years) over one year.
  • Scoring for bronchiectasis and air trapping, with logistic and linear regression models used to identify associated factors.
  • Inclusion of age, sex, CFTR genotype, pancreatic sufficiency, symptoms, and bronchoalveolar lavage findings in the analysis.

Main Results:

  • Bronchiectasis persisted in 74% and air trapping in 81% of paired scans.
  • The extent of bronchiectasis increased in 63% and air trapping in 47% of paired scans.
  • Radiological progression was associated with severe CFTR genotype, increased neutrophilic inflammation, and pulmonary infection.

Conclusions:

  • CT-detected structural lung disease in young CF patients is persistent and progressive over one year.
  • Deteriorating lung disease is linked to worsening inflammation and infection.
  • Early intervention is crucial to prevent or halt structural lung disease progression in young children with CF.
Abstract

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