Abstract
Objective: Up to now, little is known about the impact of the date of diagnosis on the clinical course of children with primary ciliary dyskinesia (pcd).
Methods: Over a ten year period all paediatric patients with confirmed pcd in a tertiary centre who could be followed up for more than 12 months were retrospectively included in the study.
Results: Out of 19 patients 63% had a situs inversus, the median age a diagnosis was 95 months. There was a significant correlation between the date of diagnosis and development of bronchiectasis (p=0,04), but no effect on lung function. 12 months after diagnosis 75% of patients received professional physiotherapy, 88% were on continuous antibiotic therapy, 50% on inhaled β2 agonists and 13% on inhaled corticosteroids.
After starting the treatment, all patients improved clinically, the number of pulmonary complications decreased, especially pneumonias (p=0,03) and bronchitis (p=0,01). Chronic wet cough and rhinitis often persisted and lung function measurements did not improve permanently.
Conclusions: Late diagnosis of pcd carries an increased risk for developing bronchiectasis. Pulmonary exacerbations decrease with appropriate treatment whereas there is no significant effect on lung function parameters.
- © 2011 ERS
