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Critical Evaluation of Sinonasal Disease in 64 Adults with Primary Ciliary Dyskinesia

AP-HP, Hôpital Henri-Mondor–A Chenevier et Hôpital intercommunal, Services d’ORL et de Pneumologie, Créteil 94010, France
INSERM, U955, Créteil 94010, France
Université Paris-Est, Faculté de Médecine, Créteil F-94010, France
CNRS, ERL 7000, Créteil 94010, France
AP-HP, Hôpital Henri-Mondor–A Chenevier, Service De Physiologie et d’explorations Fonctionnelles, Créteil 94010, France
Assistance Publique-Hôpitaux de Paris, Hôpital Cochin, Service de Pneumologie, Paris 75014, France
Université Paris Descartes, Sorbonne Paris Cité, Paris 75006 France
Service de Génétique et Embryologie Médicales, Hôpital Armand-Trousseau, Assistance Publique-Hôpitaux de Paris, Paris 75012, France
INSERM, Unité Mixte de Recherche S933, Université Pierre et Marie Curie, Paris 75005, France
Maladies pulmonaires Rares (DHU FIRE) Service de Pneumologie A, Assistance Publique-Hôpitaux de Paris (AP-HP), Hôpital Bichat, Paris 75877, France
Laboratoire d’Excellence Inflamex, Paris 75013, France
Université Paris Diderot, Sorbonne Paris Cité, Paris 75013, France
INSERM, Unité 1552, Paris 75013, France
AP-HP, Hôpital Kremlin-Bicetre, Service d’ORL et de Chirurgie Cervico-Faciale, Le Kremlin-Bicêtre 94275, France
Université Paris-Sud, Faculté de Médecine, Kremlin-Bicêtre F-94070, France
Author to whom correspondence should be addressed.
These authors contributed equally to this manuscript.
J. Clin. Med. 2019, 8(5), 619;
Received: 14 March 2019 / Revised: 23 April 2019 / Accepted: 1 May 2019 / Published: 7 May 2019
(This article belongs to the Special Issue Prevention, Diagnosis and Management of Chronic Rhinosinusitis)
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To date, no study precisely described ear, nose and throat (ENT) disease in adults with primary ciliary dyskinesia (PCD) and its relationship with ciliary function/ultrastructure. A retrospective study of standardized ENT data (exam, audiogram, sinus Computed tomography (CT), and bacteriology) was conducted in 64 adults with confirmed PCD who were followed in two ENT reference centers. Rhinorrhoea and hearing loss were the main symptoms. Symptom scores were higher in older patients. Nasal endoscopy was abnormal in all patients except one, showing nasal polyps in one-third of the patients and stagnant nasal mucus secretions in 87.5% of the patients. Sinus CT opacities were mainly incomplete and showed one-third of the patients with sinus hypoplasia and/or agenesis. Middle meatus mainly grew Haemophilus influenzae, Streptoccocus pneumoniae and Pseudomonas aeruginosa. Otitis media with effusion (OME), which is constant in childhood, was diagnosed in less than one-quarter of the patients. In two-thirds of the patients, audiogram showed hearing loss that was sensorineural in half of the patients. ENT disease severity was not correlated with ciliary function and ultrastructure, but the presence of OME was significantly associated with a forced expiratory volume (FEV1) < 70%. Rhinosinusitis is the most common clinical feature of PCD in adults, while OME is less frequent. The presence of active OME in adults with PCD could be a severity marker of lung function and lead to closer monitoring. View Full-Text
Keywords: airway mucociliary clearance; chronic rhinosinusitis; Kartagener syndrome; otitis media with effusion; primary ciliary dyskinesia airway mucociliary clearance; chronic rhinosinusitis; Kartagener syndrome; otitis media with effusion; primary ciliary dyskinesia

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Bequignon, E.; Dupuy, L.; Zerah-Lancner, F.; Bassinet, L.; Honoré, I.; Legendre, M.; Devars du Mayne, M.; Escabasse, V.; Crestani, B.; Maître, B.; Escudier, E.; Coste, A.; Papon, J.-F. Critical Evaluation of Sinonasal Disease in 64 Adults with Primary Ciliary Dyskinesia. J. Clin. Med. 2019, 8, 619.

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