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Abstract
Primary ciliary dyskinesia (PCD) comprises a wide range of phenotypes related to the impaired function of epithelial cilia. Histologically altered or absent cilia lead to multiple and variable consequences at the clinical level. Several research and clinical interest have surrounded respiratory ciliated epithelium because of its key role in clearing mucus from the ear, nose, and respiratory tract. Our aim was to provide a current state of the art on the ENT signs and symptoms of primary ciliary dyskinesia and their practical management. We systematically searched the following databases from 2011 until 2021: Cochrane Central Register of Controlled Trials, PubMed, and ScienceDirect. The searches were performed by 2 independent investigators. After removing duplications, articles were selected after the evaluation of the publications by reading their titles and abstracts. Eventually, full-text reading took place. Early onset of upper and lower respiratory symptoms in a full-term born child is suggestive of ciliary dyskinesia, especially in the absence of a usual triggering factor (passive smoking, allergy). The cornerstone of care is improving mucociliary clearance, using nasal and sinus irrigations, autoinflaction devices for middle ear effusion, physiotherapy and/or physical exercise for upper airway recovery. Decongestants, mucolytics, steroids, and antihistamines are part of the therapeutic arsenal with a low level of evidence. Early eradication of airway infections should be based on bacteriological analysis. Surgical interventions are common and mainly aim at restoring drainage. in summary, PCD is associated with ENT manifestations from the first days of life. The key to management is restoring adequate drainage of the upper airway and ENT cavities, using medical and surgical interventions.
Cite this article as: Benchimol L, Dubois S, Bricmont N, et al. Clinical manifestations and management of primary ciliary dyskinesia in ENT practice. B-ENT. Published online August 26, 2024. doi:10.5152/B-ENT.2024.221028