[The respiratory mucociliary system and its exploration in primary ciliary dyskinesia].

BACKGROUND

We analyzed the main characteristic features of the respiratory epithelium mucociliary system and the different tests of ciliary beat and mucociliary transport (mucociliary clearance). This knowledge is necessary for an often interdisciplinary diagnosis and treatment of primary ciliary dyskinesia.

METHODS

Review of the literature and personal experience of the different tests of ciliary structure and function.

RESULTS

This disease is characterized by abnormalities in ciliary structure/function. The genetic mechanisms and the ultrastructural abnormalities that are involved are heterogenous compared to the relative homogeneity of the clinical presentation.

CONCLUSION

The diagnostic criteria are compatible clinical features (chronic upper airway and bronchopulmonary infections, situs inversus...) coupled with tests of ciliary structure and function.