Inflammation, Infection, and Pulmonary Function in Infants and Young Children with Cystic Fibrosis

TLDR

The study aimed to determine how lower airway infection affects clinical parameters, pulmonary function, and inflammation in clinically stable infants and young children with cystic fibrosis. A prospective cohort of 22 CF patients under 4 years underwent elective anesthesia and intubation to measure passive respiratory mechanics, lung volumes, and bronchoalveolar lavage for cytology, IL‑8, and quantitative microbiology. Lower airway pathogens were detected in 64% of subjects and were linked to prior respiratory admissions, asthma, wheeze, and correlated with higher neutrophils, IL‑8, reduced respiratory compliance, and increased air trapping, accounting for 78% of neutrophil variability.

Abstract

Our aim was to study the effect of lower airway infection on clinical parameters, pulmonary function tests, and inflammation in clinically stable infants and young children with cystic fibrosis (CF). To accomplish this goal, a prospective cohort of screened CF patients under 4 years of age were studied, using elective anesthesia and intubation for: passive respiratory mechanics (single breath occlusion passive deflation) and lung volumes (nitrogen washout), under neuromuscular blockade; and bronchoalveolar lavage (BAL) of 3 main bronchi for cytology, cytokine interleukin (IL)-8, and quantitative microbiology. There were 22 children studied, with a mean age of 23.2 months (6.7–44 months). A greater relative risk of lower airway pathogens was associated with prior respiratory admission (3.60, 95% confidence interval [CI] 2.87–4.51), history of asthma (1.75, 95% CI 1.52–2.03), and chronic symptoms (1.50, 95% CI 1.23–1.83), especially wheeze (1.88, 95% CI 1.61–2.19). Lower respiratory pathogens ( ⩾ 10 cfu/ml BAL) were found in 14 out of 22, and greater than 105 cfu/ml in 8 out of 22 subjects. The level of pathogens in BAL (log10 cfu/ml) explained 78% of the variability in percent neutrophils and 34% of the variability in IL-8 levels. Pathogen level also correlated with pulmonary function tests of specific respiratory system compliance (r − 0.49, p = 0.02) and functional residual capacity over total lung capacity (r 0.49, p = 0.03). We conclude that the presence of pathogens in the lower airways correlated with levels of inflammation, respiratory system compliance, and degree of air trapping.