Pulmonary exacerbations, airway pathogens, and long‐term course of lung clearance index in children and young adults with cystic fibrosis

摘要

Abstract Background Pulmonary exacerbations (PEx), pathogens colonizing the respiratory tract, and patients' age are associated with progressive worsening of lung function among patients with cystic fibrosis (CF). However, the effect of these factors on longitudinal changes of Lung Clearance Index?(LCI) remains unclear. Aim To assess the role of age, different types of bronchial infection, and PEx on LCI deterioration. Methods We conducted a retrospective study assessing multiple‐breath washout?(MBW) and spirometry changes among CF patients evaluated at quarterly outpatient clinic visits over 8 years. MBW and spirometry were performed at each visit, sputum samples and/or cough swabs were obtained for culture, whereas respiratory symptoms and clinical examination findings were recorded. Patients who had ≥5 serial MBW measurements, one of which coincided with a pulmonary exacerbation, were reviewed. Results Seventy‐six patients were included in the study: mean age of 10.61 years (range 1.75–23.75). A total of 1152 MBW tests and 1047 spirometry tests were performed. LCI was significantly higher among CF patients aged 11–15, 16–20, and over 20 years than those under 5 years of age; ΔLCI: 1.16 (confidence interval [CI] 0.43–1.90) and 3.25 (CI 2.33–4.17), respectively. Furthermore, LCI was significantly elevated in CF patients with positive cultures for Pseudomonas aeruginosa (0.52 LCI [CI ?0.12 to 0.71]) and Stenotrophomonas Maltophilia (1.41 LCI [CI 0.61–2.21]). Moreover, increased values of LCI in CF patients were significantly associated with increased risk of PEx (odds ratio [OR] 1.19, CI [1.14–1.25], p?