Abstract
Background: Respiratory therapy in cystic fibrosis (CF) consists of airway clearance,
infection control, and reduction of airway inflammation. It is well recognized that physical activity as
well as daily chest physiotherapy, enhance airway clearance. We investigated the effects of
pulmonary rehabilitation, including physical activity and chest physiotherapy, on airway
inflammation in children with CF. Methods: Eighteen children with stable CF (six females), aged
8.2–16.2 years, participating in a 3-week multidisciplinary inpatient rehabilitation program were
recruited. Assessment at the beginning and the end of the program included clinical score,
pulmonary function test, exhaled breath condensate (EBC) and sputum analysis. Sputum
supernatant and EBC were analyzed for interleukin (IL)-1b, 6, 8, 10, 12, tumor necrosis factoralpha
(TNF-a) and LTB4. Results: Median (IQR) symptom scores decreased from 19 [23] to 16
[21], p =0.005. Vital capacity and FVC increased significantly (P<0.05). However no difference
was found for the total sputum cells and sputum as well as EBC cytokines between the two visits.
Significant correlations were found for sputum IL-1 (+), IL-6 (-), and IL-8 (+) to total sputum cell
count and neutrophils and for IL-8 to TNF-a. Conclusions: We have shown that a short-term
inpatient rehabilitation for children with stable CF with intensive physical activity mainly improve
subjective clinical symptoms and measures of lung function such as VC and FVC but does not
influence airflow obstruction and airway inflammation as assessed by sputum and EBC analysis.