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Published on: May 8, 2017
Infection, inflammation, and lung function decline in infants with cystic fibrosis
Naveen Pillarisetti1, Elizabeth Williamson, Barry Linnane
1Department of Respiratory Medicine, Royal Children's Hospital Melbourne, Flemington Road, Parkville, 3052, Australia.
Insights
In infants with cystic fibrosis (CF), pulmonary inflammation is linked to reduced lung function, while infections accelerate lung function decline. Early interventions targeting inflammation and infection are vital for preserving lung health in CF infants.
Area of Science:
- Pediatric Pulmonology
- Cystic Fibrosis Research
- Respiratory Medicine
Background:
- Understanding the evolution of lung function in infants with cystic fibrosis (CF) is critical for developing early intervention strategies.
- Pulmonary inflammation and infection significantly impact lung damage and outcomes in infants with CF.
- Lung function measurements are essential outcome measures in studies of early CF lung disease.
Purpose of the Study:
- To longitudinally track changes in lung function among infants diagnosed with cystic fibrosis.
- To investigate the association between pulmonary infection, inflammation, and lung function trajectories in this population.
- To inform the development of targeted therapies for early-stage CF lung disease.
Main Methods:
- Prospective recruitment of infants diagnosed with CF via newborn screening or clinical presentation.
- Measurement of lung function parameters including FVC, FEV(0.5), and FEF(75) using the raised-volume technique with z-score calculations.
- Assessment of pulmonary infection and inflammation via bronchoalveolar lavage performed within 48 hours of lung function testing.
Main Results:
- Thirty-seven infants completed at least two lung function tests; significant declines in FVC and FEV(0.5) z-scores were observed over two years.
- Presence of free neutrophil elastase was associated with lower FVC and FEV(0.5) z-scores (P=0.003 and P=0.001, respectively).
- Infection with Staphylococcus aureus or Pseudomonas aeruginosa correlated with a greater decline in FEV(0.5) z-scores (P=0.018 and P=0.021, respectively).
Conclusions:
- Pulmonary inflammation is directly associated with diminished lung function in infants with CF.
- Pulmonary infections contribute to an accelerated rate of lung function decline in this vulnerable group.
- Targeting both pulmonary inflammation and infection is crucial to prevent early lung function deterioration in infants with CF.
Rationale:
Better understanding of evolution of lung function in infants with cystic fibrosis (CF) and its association with pulmonary inflammation and infection is crucial in informing both early intervention studies aimed at limiting lung damage and the role of lung function as outcomes in such studies.
Objectives:
To describe longitudinal change in lung function in infants with CF and its association with pulmonary infection and inflammation.
Methods:
Infants diagnosed after newborn screening or clinical presentation were recruited prospectively. FVC, forced expiratory volume in 0.5 seconds (FEV(0.5)), and forced expiratory flows at 75% of exhaled vital capacity (FEF(75)) were measured using the raised-volume technique, and z-scores were calculated from published reference equations. Pulmonary infection and inflammation were measured in bronchoalveolar lavage within 48 hours of lung function testing.
Measurements And Main Results:
Thirty-seven infants had at least two successful repeat lung function measurements. Mean (SD) z-scores for FVC were -0.8 (1.0), -0.9 (1.1), and -1.7 (1.2) when measured at the first visit, 1-year visit, or 2-year visit, respectively. Mean (SD) z-scores for FEV(0.5) were -1.4 (1.2), -2.4 (1.1), and -4.3 (1.6), respectively. In those infants in whom free neutrophil elastase was detected, FVC z-scores were 0.81 lower (P=0.003), and FEV(0.5) z-scores 0.96 lower (P=0.001), respectively. Significantly greater decline in FEV(0.5) z-scores occurred in those infected with Staphylococcus aureus (P=0.018) or Pseudomonas aeruginosa (P=0.021).
Conclusions:
In infants with CF, pulmonary inflammation is associated with lower lung function, whereas pulmonary infection is associated with a greater rate of decline in lung function. Strategies targeting pulmonary inflammation and infection are required to prevent early decline in lung function in infants with CF.
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