Current treatment for primary ciliary dyskinesia conditions

Abstract

Primary ciliary dyskinesia (PCD) is a heterogeneous group of conditions characterised by ultrastructural defects of the cilia, which result in impaired mucociliary clearance. Although the incidence of PCD is low, early recognition and prompt management are important in order to prevent unnecessary morbidity, the progression of bronchiectasis and the deterioration of lung function. As the underlying defect in PCD cannot be corrected, the mainstay of therapy remains effective clearance of airway secretions and antibiotic therapy of respiratory tract infections. This paper highlights new developments in the field that have implications for the future management of PCD. These include β-adrenergic agonists, arginine, uridine-5’-triphosphate, hypertonic saline and recombinant human DNase. It is to be hoped that these treatment modalities will have a therapeutic role in PCD.

• arginine
• β-adrenergic agonists
• current treatment
• hypertonic saline
• mucociliary clearance
• primary ciliary dyskinesia
• recombinant human DNase
• uridine-5’-triphosphate