Positive expiratory pressure in the physiotherapeutic management of primary ciliary dyskinesia in paediatric age.

The clinical features of primary ciliary dyskinesia are related to congenital impairment of mucociliary clearance, which represents the common aetiopathogenetic denominator. If untreated, the disease evolves with progressive, slowly developing, structural lung damage. Chest physiotherapy may play a relevant role in the therapeutic management of this pathological condition, owing to the improvement in mucus transport and the recruitment of the obstructed lung periphery. This may lead to modification of the natural history of the disease, if early intervention is implemented. The rationale behind this intervention and the advantages/disadvantages of the two main positive expiratory pressure techniques used are discussed here.