Lung clearance index and exercise capacity among children with CF and non-CF bronchiectasis over a two year period

Aim: To compare progression of lung disease assessed with LCI and exercise capacity among children with bronchiectasis. Method: Fourteen stable CF patients and 13 stable children with non-CF bronchiectasis and comparable FEV 1 were assessed with HRCT, maximal incremental cardiopulmonary exercise testing and Multiple Breath Washout (MBW) over a two-year period. Results: Fourteen patients with CF (mean age 13.5 years, mean FEV 1 =80.1%) and 13 patients with non-CF bronchiectasis (mean age =13.05 years, mean FEV1=76.6%), participated in the study. In both groups, there was evidence of exercise limitation, with mean Peak Aerobic Capacity (VOpeak) 69.2 % predicted (CF group) and 76.4 % (non CF group), (p=0.321). VE/VO2 and VE/VCO2 were significantly impaired in the CF group (p 1 , VD/CT, VE/VO2 and VE/VCO2 showed significantly improvement over the two year period among patients with non-CF bronchiectasis (p Conclusions: Despite comparable FEV1 values, patients with CF seem to have more impaired exercise capacity and ventilation inhomogeneity, compared to patients with non-CF bronchiectasis. Aggressive treatment may improve lung function and exercise capacity among patients with non-CF bronchiectasis, while exercise capacity and ventilation inhomogeneity deteriorate among patients with CF in a two year period.