Comparison of bronchodilator responsiveness in children with primary ciliary dyskinesia and cystic fibrosis

Abstract

Introduction Short and long-acting inhaled beta-2 agonists are commonly prescribed as part of routine treatment regimens for children with cystic fibrosis (CF). Several studies have demonstrated that short and long term benefits are more likely in individuals with demonstrable bronchodilator responsiveness. Little is known about the extent to which bronchodilators might be of benefit in children with primary ciliary dyskinesia (PCD). Aim To determine the relative prevalence of bronchodilator responsiveness in a tertiary centre population of CF and PCD patients as measured when well as part of routine annual assessment. Methods 205 CF and 47 PCD children attended for annual review at our Paediatric Respiratory Centre (Southampton, UK) in 2015. Of these pre- & post- salbutamol spirometry data were available for 99 CF & 29 PCD patients. A 12% increase in FEV 1 (recommended ERS criteria) after salbutamol was considered a significant bronchodilator response. Results There were no significant differences in age, and sex between the two disease groups. Over twice as many PCD children had bronchodilator responsiveness compared with CF children (17% vs 7%) Whilst this was not significant, p=0.098, it probably reflects a lack of power. Conclusion Reversible airways obstruction is common in children with PCD. Routine assessment of bronchodilator responsiveness in this group of children is worthwhile and identifies those who might benefit from routine bronchodilator therapy. Further studies to assess the long-term benefits from bronchodilator therapy in PCD are warranted.