Cystic Fibrosis Clinical Trial
Official title:
Exploratory Study on Link Between Dynamic Hyperinflation and Lung Clearance Index in Cystic Fibrosis (Étude du Lien Entre la Distension Dynamique et l'Indice de Clairance Pulmonaire Dans la Mucoviscidose)
Recent improvements in cystic fibrosis management have enabled an important subset of
patients to maintain normal spirometry. Nevertheless, even in these patients with normal
spirometry, chest computed tomography (CT) might identify structural lung abnormalities such
as bronchiectasis, mucus plugging, bronchiolitis and air trapping.
Lung clearance index (LCI) has been shown to correlate well with structural pulmonary
alterations seen on CT even in CF patients with well-preserved spirometry. In cystic
fibrosis, a high LCI is associated with a worse feeling of illness assessed by the Cystic
Fibrosis Questionnaire-Revised (CFQ-R). School-aged CF patients with normal spirometry also
have normal aerobic function as assessed by peak oxygen uptake (V̇O2) measured during
symptom-limited incremental cardiopulmonary exercise test. However, the ability of LCI to
predict ventilatory abnormalities appearing at exercise in CF patients has not been
investigated. The investigators therefore aimed to compare physiological parameters at
exercise between CF patients with elevated LCI (i.e., LCI above the upper limit of normal
[ULN]) and patients with normal LCI, all with preserved spirometry.
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