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Risk factors for bronchiectasis in children with cystic fibrosis

Peter D Sly1, Catherine L Gangell, Linping Chen

  • 1Queensland Children's Medical Research Institute, Brisbane, Australia. p.sly@uq.edu.au

The New England Journal of Medicine
|May 23, 2013
PubMed

Insights

Early life neutrophil elastase activity in cystic fibrosis patients predicts bronchiectasis. This finding highlights key risk factors for early-onset lung disease in children with CF.

Area of Science:

  • Pediatric Pulmonology
  • Cystic Fibrosis Research
  • Respiratory Medicine

Background:

  • Bronchiectasis is a common and progressive complication in cystic fibrosis (CF) that develops early in infancy.
  • Identifying risk factors for early bronchiectasis is crucial for timely intervention in CF patients.

Purpose of the Study:

  • To determine risk factors associated with the onset and persistence of bronchiectasis in infants diagnosed with cystic fibrosis.
  • To analyze data from the Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF) program.

Main Methods:

  • Longitudinal analysis of data from 127 infants diagnosed with CF via newborn screening.
  • Chest computed tomography (CT) and bronchoalveolar lavage (BAL) were performed at 3 months, 1, 2, and 3 years of age.
  • Multivariate analyses were used to identify risk factors for bronchiectasis detection.

Main Results:

  • The prevalence of bronchiectasis increased from 29.3% at 3 months to 61.5% by 3 years of age.
  • Key risk factors identified include meconium ileus, respiratory symptoms, free neutrophil elastase activity in BAL fluid, and gas trapping on expiratory CT.
  • Elevated neutrophil elastase activity at 3 months was significantly associated with persistent bronchiectasis at later ages.

Conclusions:

  • Neutrophil elastase activity in bronchoalveolar lavage fluid during early infancy is a significant predictor of early-onset bronchiectasis in children with cystic fibrosis.
  • These findings underscore the importance of monitoring neutrophil elastase activity for early detection and management of lung disease in CF.
Abstract

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