The effect of regular salbutamol on lung function and bronchial responsiveness in patients with primary ciliary dyskinesia

Study objective: There is growing evidence that regular beta(2)-agonist use in patients with asthma is associated with decreased airway caliber and in creased bronchial responsiveness, The aim of this study was to determine wh ether regular treatment with beta(2)-agonists induces changes in lung funct ion and bronchial responsiveness in patients with primary ciliary dyskinesi a. Design: A randomized, double-blind, placebo-controlled, crossover study. Patients: Nineteen children with primary ciliary dyskinesia. Interventions: Subjects received inhaled salbutamol or identical placebo (2 x 100 mu g qi d) for periods of 6 weeks with a wash-out period of 4 weeks. Measurements and results: FEV1 was measured before and 3 weeks and 6 weeks after salbutamol or placebo treatment. High-dose methacholine inhalation te sts were pel formed before and 6 weeks after each treatment. The provocativ e concentration of methacholine producing a 20% fall in FEV1 (PC20) and max imal airway narrowing (M Delta VFFEV1) was measured. No significant change in FEV1 was observed during the salbutamol or placebo periods. No significa nt differences in the parameters of bronchial responsiveness (PC20 and M De lta FFEV1) were noted as the result of either salbutamol or placebo treatme nt. Conclusion: Our data have shown that salbutamol, inhaled regularly for 6 we eks, did not cause either a decline in lung function or an increase in bron chial responsiveness in subjects with primary ciliary dyskinesia.