Exercise capacity and ventilation inhomogeneity in cystic fibrosis: A cross-sectional study

Simone Gambazza, Riccardo Guarise, Federica Carta, Federico Ambrogi, Marina Mirabella, Anna Brivio, Carla Colombo

Research output: Contribution to journalArticlepeer-review


Background: Lung clearance index (LCI2.5) is a marker of overall lung ventilation inhomogeneity and has proven to be able to detect early peripheral damage in subjects with cystic fibrosis (CF), with greater sensitivity than conventional spirometry. Combining its sensitivity with the output of an incremental exercise testing, we hypothesized that any sign of ventilation inhomogeneity in subjects without severe airflow obstruction and with a normal exercise tolerance could be relevant for the CF team, tracking early lung disease and potential exercise limiting factors. Methods: Patients with CF in clinical stable conditions were recruited between 2015 and 2017. Available spirometry, nitrogen multiple-breath washout test and symptoms-limited exercise testing performed as parts of patients' annual routine evaluation were considered for this cross-sectional study. To describe the relationship between exercise intensity and ventilation inhomogeneity, a linear regression analysis was performed using backward elimination based on Akaike information criteria. Results: Seventy-seven patients (38 females) were included. Sacin and LCI2.5 were significantly higher in patients with an overall reduced exercise tolerance. Peak work developed during exercise was associated with body mass index (b = 5.25; 95% confidence interval [CI] = 1.53-8.98), forced expiratory volume in 1 second (FEV1; b = 3.71; 95% CI = 1.96-5.46), Pseudomonas aeruginosa chronic infection (b = −8.84; 95% CI = −15.84 to −1.84) but not with LCI2.5. Conclusion: Exercise capacity and airflow obstruction are associated in this Italian CF cohort. Considering the greater discriminatory power of LCI2.5 over FEV1 and peak work, the Godfrey protocol without gas analysis cannot provide detailed information about lung function or efficiency. However, this incremental protocol without gas exchange measures can still provide the CF team with information about exercise tolerance and disability.

Original languageEnglish
Pages (from-to)394-400
Number of pages7
JournalPediatric Pulmonology
Issue number2
Publication statusPublished - Feb 1 2020


  • cystic fibrosis
  • exercise tolerance
  • lung clearance index
  • multiple breath washout
  • ventilation inhomogeneity

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Pulmonary and Respiratory Medicine


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