Sinonasal disease among patients with primary ciliary dyskinesia: an international study.


Journal

ERJ open research
ISSN: 2312-0541
Titre abrégé: ERJ Open Res
Pays: England
ID NLM: 101671641

Informations de publication

Date de publication:
May 2023
Historique:
received: 12 12 2022
accepted: 21 03 2023
medline: 25 5 2023
pubmed: 25 5 2023
entrez: 25 5 2023
Statut: epublish

Résumé

Sinonasal symptoms are a common feature of primary ciliary dyskinesia (PCD); however, literature about their severity and frequency, particularly during the life course, is scarce. Using baseline data from the Ear, nose and throat (ENT) Prospective International Cohort of PCD patients, we describe sinonasal disease in PCD. We included participants who had a routine sinonasal examination during which they completed a symptoms questionnaire. We compared frequency of reported symptoms and examination findings among children and adults, and identified characteristics potentially associated with higher risk of sinonasal disease using ordinal regression. 12 centres contributed 384 participants; median age was 16 years (IQR 9-22), and 54% were male. Chronic nasal problems were the most common feature, reported by 341 (89%). More adults (33; 24%) than children (10; 4%) described hyposmia. Quality of life was moderately affected by rhinosinusitis among 136 participants with completed SNOT-22 questionnaires (median score 31; IQR 23-45). Examinations revealed nasal polyps among 51 of 345 participants (15%) and hypertrophic inferior nasal turbinates among 127 of 341 participants (37%). Facial pain was detected in 50 of 342 participants (15%). Nasal polyps, hypertrophic turbinates, deviated septum and facial pain were found more commonly in adults than children. The only characteristic associated with higher risk of sinonasal disease was age 10 years and older. Based on our findings, regular sinonasal examinations are relevant for patients with PCD of all ages. There is a need for improved management of sinonasal disease supported by evidence-based guidelines.

Sections du résumé

Background UNASSIGNED
Sinonasal symptoms are a common feature of primary ciliary dyskinesia (PCD); however, literature about their severity and frequency, particularly during the life course, is scarce. Using baseline data from the Ear, nose and throat (ENT) Prospective International Cohort of PCD patients, we describe sinonasal disease in PCD.
Methods UNASSIGNED
We included participants who had a routine sinonasal examination during which they completed a symptoms questionnaire. We compared frequency of reported symptoms and examination findings among children and adults, and identified characteristics potentially associated with higher risk of sinonasal disease using ordinal regression.
Results UNASSIGNED
12 centres contributed 384 participants; median age was 16 years (IQR 9-22), and 54% were male. Chronic nasal problems were the most common feature, reported by 341 (89%). More adults (33; 24%) than children (10; 4%) described hyposmia. Quality of life was moderately affected by rhinosinusitis among 136 participants with completed SNOT-22 questionnaires (median score 31; IQR 23-45). Examinations revealed nasal polyps among 51 of 345 participants (15%) and hypertrophic inferior nasal turbinates among 127 of 341 participants (37%). Facial pain was detected in 50 of 342 participants (15%). Nasal polyps, hypertrophic turbinates, deviated septum and facial pain were found more commonly in adults than children. The only characteristic associated with higher risk of sinonasal disease was age 10 years and older.
Conclusions UNASSIGNED
Based on our findings, regular sinonasal examinations are relevant for patients with PCD of all ages. There is a need for improved management of sinonasal disease supported by evidence-based guidelines.

Identifiants

pubmed: 37228283
doi: 10.1183/23120541.00701-2022
pii: 00701-2022
pmc: PMC10204851
pii:
doi:

Types de publication

Journal Article

Langues

eng

Informations de copyright

Copyright ©The authors 2023.

Déclaration de conflit d'intérêts

Conflict of interest: P. Latzin received grants or honoraria for participation in data safety monitoring boards or advisory boards from Vertex, Vifor, OM Pharma, Polyphor, Santhera (DMC) and Sanofi Aventis within the last 36 months. J. Roehmel received grants and clinical study remuneration from Vertex, INSMED, Medical Research Council/UK, BMBF and Mukoviszidose Institut. All other authors have nothing to declare.

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Auteurs

Yin Ting Lam (YT)

Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland.
Graduate School for Cellular and Biomedical Sciences, University of Bern, Bern, Switzerland.

Jean-François Papon (JF)

Assistance Publique-Hôpitaux de Paris, Université Paris-Saclay, Hôpital Bicêtre, Service d'ORL, Le Kremlin-Bicêtre, France.
Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France.

Mihaela Alexandru (M)

Assistance Publique-Hôpitaux de Paris, Université Paris-Saclay, Hôpital Bicêtre, Service d'ORL, Le Kremlin-Bicêtre, France.
Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France.

Andreas Anagiotos (A)

Department of Otorhinolaryngology, Nicosia General Hospital, Nicosia, Cyprus.

Miguel Armengot (M)

Department of Otorhinolaryngology, and Primary Ciliary Dyskinesia Unit, La Fe University and Polytechnic Hospital, Valencia, Spain.
Medical School, Valencia University, Valencia, Spain.

Mieke Boon (M)

Department of Paediatrics, University Hospital, Leuven, Belgium.

Andrea Burgess (A)

Primary Ciliary Dyskinesia Centre, Southampton Children's Hospital, Southampton NHS Foundation Trust, Southampton, UK.

Suzanne Crowley (S)

Paediatric Department of Allergy and Lung Diseases, Oslo University Hospital, Oslo, Norway.

Sinan Ahmed D Dheyauldeen (SAD)

Department of Otorhinolaryngology, Head and Neck Surgery, Oslo University Hospital, Oslo, Norway.
Faculty of Medicine, University of Oslo, Oslo, Norway.

Nagehan Emiralioglu (N)

Department of Pediatric Pulmonology, Hacettepe University, School of Medicine, Ankara, Turkey.

Ela Erdem Eralp (E)

Department of Pediatric Pulmonology, Marmara University, School of Medicine, Istanbul, Turkey.

Christine van Gogh (C)

Department of Otorhinolaryngology - Head and Neck Surgery, Amsterdam UMC, Amsterdam, The Netherlands.

Yasemin Gokdemir (Y)

Department of Pediatric Pulmonology, Marmara University, School of Medicine, Istanbul, Turkey.

Onder Gunaydın (O)

Department of Otorhinolaryngology, Hacettepe University, School of Medicine, Ankara, Turkey.

Eric G Haarman (EG)

Department of Pediatric Pulmonology, Emma Children's Hospital, Amsterdam UMC, Vrije Universiteit, Amsterdam, The Netherlands.

Amanda Harris (A)

Southampton Children's Hospital, University of Southampton, Southampton, UK.
Primary Ciliary Dyskinesia Centre, NIHR Respiratory Biomedical Research Centre, University of Southampton, Southampton, UK.

Isolde Hayn (I)

Department of Otorhinolaryngology, Head and Neck Surgery, Charité-Universitätsmedizin Berlin, Berlin, Germany.

Hasnaa Ismail-Koch (H)

Primary Ciliary Dyskinesia Centre, Southampton Children's Hospital, Southampton NHS Foundation Trust, Southampton, UK.

Bülent Karadag (B)

Department of Pediatric Pulmonology, Marmara University, School of Medicine, Istanbul, Turkey.

Céline Kempeneers (C)

Division of Respirology, Department of Pediatrics, University Hospital Liège, Liège, Belgium.

Sookyung Kim (S)

Assistance Publique-Hôpitaux de Paris, Université Paris-Saclay, Hôpital Bicêtre, Service d'ORL, Le Kremlin-Bicêtre, France.

Philipp Latzin (P)

Division of Paediatric Respiratory Medicine and Allergology, Department of Paediatrics, Inselspital, University Hospital, University of Bern, Bern, Switzerland.

Natalie Lorent (N)

Department of Respiratory Diseases, University Hospital, Leuven, Belgium.

Ugur Ozcelik (U)

Department of Pediatric Pulmonology, Hacettepe University, School of Medicine, Ankara, Turkey.

Charlotte Pioch (C)

Department of Pediatric Pulmonology, Immunology and Critical Care Medicine, Charité-Universitätsmedizin Berlin, Berlin, Germany.

Anne-Lise M L Poirrier (AML)

Department of Otorhinolaryngology, University Hospital Liège, Liège, Belgium.

Ana Reula (A)

Biomedical Sciences Department, CEU-Cardenal Herrera University, Castellón, Spain.
Molecular, Cellular and Genomic Biomedicine Group, IIS La Fe, Valencia, Spain.

Jobst Roehmel (J)

Department of Pediatric Pulmonology, Immunology and Critical Care Medicine, Charité-Universitätsmedizin Berlin, Berlin, Germany.

Panayiotis Yiallouros (P)

Medical School, University of Cyprus, Nicosia, Cyprus.
Pediatric Pulmonology Unit, Hospital "Archbishop Makarios III", Nicosia, Cyprus.

Myrofora Goutaki (M)

Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland.
Division of Paediatric Respiratory Medicine and Allergology, Department of Paediatrics, Inselspital, University Hospital, University of Bern, Bern, Switzerland.

Classifications MeSH