Servizio di Allergo-Pneumologia, Clinica Pediatrica, Università di Udine
Key words: Primary ciliary dyskinesia, Respiratory disease
Primary ciliary dyskinesia (PCD) is a rare condition, which may appear to paediatricians, general practitioners, ENT specialists as well as in the infertility clinic. The Authors provide a review of the morphology and function of respiratory cilia and emphasis is placed on the importance of muco-ciliary clearance as the most important defence mechanism of the upper and lower airways. A description of changes of the microtubular pattern is given, which can influence ciliary activity and muco-ciliary transport. Since the diagnosis of PCD leads to modifying the management of the upper respiratory infections as well as the course of the disease and particularly the perspectives for lung damage, it is important to consider it among the differential diagnoses of recurrent and chronic respiratory disease and refer the patient to specialized centres. Specialized management must be started and continued lifelong.
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