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

Overview

Journal J Clin Med

Specialty General Medicine

Date 2019 May 10

PMID 31067752

Citations 25

Authors

Emilie Bequignon

Laurence Dupuy

Francoise Zerah-Lancner

Laurence Bassinet

Isabelle Honore

Marie Legendre

Marie Devars du Mayne

Virginie Escabasse

Bruno Crestani

Bernard Maitre

Estelle Escudier

Andre Coste

Jean-Francois Papon

Affiliations

Soon will be listed here.

Abstract

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 , and . 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.

Citing Articles

Definition of sinonasal and otological exacerbation in patients with primary ciliary dyskinesia: an expert consensus.

Goutaki M, Lam Y, Anagiotos A, Armengot M, Burgess A, Campbell R ERJ Open Res. 2024; 10(6).

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Primary Ciliary Dyskinesia: A Clinical Review.

Despotes K, Zariwala M, Davis S, Ferkol T Cells. 2024; 13(11.

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[Primary ciliary dyskinesia].

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Association between upper and lower respiratory disease among patients with primary ciliary dyskinesia: an international study.

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PMID: 38444659 PMC: 10910353. DOI: 10.1183/23120541.00932-2023.

Molecular defects in primary ciliary dyskinesia are associated with agenesis of the frontal and sphenoid paranasal sinuses and chronic rhinosinusitis.

Schramm A, Raidt J, Gross A, Bohmer M, Beule A, Omran H Front Mol Biosci. 2023; 10:1258374.

PMID: 37860582 PMC: 10584328. DOI: 10.3389/fmolb.2023.1258374.

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