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

Background 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...

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Main Authors: Yin Ting Lam, Jean-François Papon, Mihaela Alexandru, Andreas Anagiotos, Miguel Armengot, Mieke Boon, Andrea Burgess, Suzanne Crowley, Sinan Ahmed D. Dheyauldeen, Nagehan Emiralioglu, Ela Erdem Eralp, Christine van Gogh, Yasemin Gokdemir, Onder Gunaydın, Eric G. Haarman, Amanda Harris, Isolde Hayn, Hasnaa Ismail-Koch, Bülent Karadag, Céline Kempeneers, Sookyung Kim, Philipp Latzin, Natalie Lorent, Ugur Ozcelik, Charlotte Pioch, Anne-Lise M.L. Poirrier, Ana Reula, Jobst Roehmel, Panayiotis Yiallouros, on behalf of the EPIC-PCD team, Myrofora Goutaki
Format: Article
Language:English
Published: European Respiratory Society 2023-05-01
Series:ERJ Open Research
Online Access:http://openres.ersjournals.com/content/9/3/00701-2022.full
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author Yin Ting Lam
Jean-François Papon
Mihaela Alexandru
Andreas Anagiotos
Miguel Armengot
Mieke Boon
Andrea Burgess
Suzanne Crowley
Sinan Ahmed D. Dheyauldeen
Nagehan Emiralioglu
Ela Erdem Eralp
Christine van Gogh
Yasemin Gokdemir
Onder Gunaydın
Eric G. Haarman
Amanda Harris
Isolde Hayn
Hasnaa Ismail-Koch
Bülent Karadag
Céline Kempeneers
Sookyung Kim
Philipp Latzin
Natalie Lorent
Ugur Ozcelik
Charlotte Pioch
Anne-Lise M.L. Poirrier
Ana Reula
Jobst Roehmel
Panayiotis Yiallouros
on behalf of the EPIC-PCD team
Myrofora Goutaki
author_facet Yin Ting Lam
Jean-François Papon
Mihaela Alexandru
Andreas Anagiotos
Miguel Armengot
Mieke Boon
Andrea Burgess
Suzanne Crowley
Sinan Ahmed D. Dheyauldeen
Nagehan Emiralioglu
Ela Erdem Eralp
Christine van Gogh
Yasemin Gokdemir
Onder Gunaydın
Eric G. Haarman
Amanda Harris
Isolde Hayn
Hasnaa Ismail-Koch
Bülent Karadag
Céline Kempeneers
Sookyung Kim
Philipp Latzin
Natalie Lorent
Ugur Ozcelik
Charlotte Pioch
Anne-Lise M.L. Poirrier
Ana Reula
Jobst Roehmel
Panayiotis Yiallouros
on behalf of the EPIC-PCD team
Myrofora Goutaki
author_sort Yin Ting Lam
collection DOAJ
description Background 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 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 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 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.
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spelling doaj.art-af2659ccbe26408ba0bf123c0d1ed6c32023-09-09T13:53:54ZengEuropean Respiratory SocietyERJ Open Research2312-05412023-05-019310.1183/23120541.00701-202200701-2022Sinonasal disease among patients with primary ciliary dyskinesia: an international studyYin Ting Lam0Jean-François Papon1Mihaela Alexandru2Andreas Anagiotos3Miguel Armengot4Mieke Boon5Andrea Burgess6Suzanne Crowley7Sinan Ahmed D. Dheyauldeen8Nagehan Emiralioglu9Ela Erdem Eralp10Christine van Gogh11Yasemin Gokdemir12Onder Gunaydın13Eric G. Haarman14Amanda Harris15Isolde Hayn16Hasnaa Ismail-Koch17Bülent Karadag18Céline Kempeneers19Sookyung Kim20Philipp Latzin21Natalie Lorent22Ugur Ozcelik23Charlotte Pioch24Anne-Lise M.L. Poirrier25Ana Reula26Jobst Roehmel27Panayiotis Yiallouros28on behalf of the EPIC-PCD team29Myrofora Goutaki30 Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland Assistance Publique-Hôpitaux de Paris, Université Paris-Saclay, Hôpital Bicêtre, Service d'ORL, Le Kremlin-Bicêtre, France Assistance Publique-Hôpitaux de Paris, Université Paris-Saclay, Hôpital Bicêtre, Service d'ORL, Le Kremlin-Bicêtre, France Department of Otorhinolaryngology, Nicosia General Hospital, Nicosia, Cyprus Department of Otorhinolaryngology, and Primary Ciliary Dyskinesia Unit, La Fe University and Polytechnic Hospital, Valencia, Spain Department of Paediatrics, University Hospital, Leuven, Belgium Primary Ciliary Dyskinesia Centre, Southampton Children's Hospital, Southampton NHS Foundation Trust, Southampton, UK Paediatric Department of Allergy and Lung Diseases, Oslo University Hospital, Oslo, Norway Department of Otorhinolaryngology, Head and Neck Surgery, Oslo University Hospital, Oslo, Norway Department of Pediatric Pulmonology, Hacettepe University, School of Medicine, Ankara, Turkey Department of Pediatric Pulmonology, Marmara University, School of Medicine, Istanbul, Turkey Department of Otorhinolaryngology – Head and Neck Surgery, Amsterdam UMC, Amsterdam, The Netherlands Department of Pediatric Pulmonology, Marmara University, School of Medicine, Istanbul, Turkey Department of Otorhinolaryngology, Hacettepe University, School of Medicine, Ankara, Turkey Department of Pediatric Pulmonology, Emma Children's Hospital, Amsterdam UMC, Vrije Universiteit, Amsterdam, The Netherlands Southampton Children's Hospital, University of Southampton, Southampton, UK Department of Otorhinolaryngology, Head and Neck Surgery, Charité-Universitätsmedizin Berlin, Berlin, Germany Primary Ciliary Dyskinesia Centre, Southampton Children's Hospital, Southampton NHS Foundation Trust, Southampton, UK Department of Pediatric Pulmonology, Marmara University, School of Medicine, Istanbul, Turkey Division of Respirology, Department of Pediatrics, University Hospital Liège, Liège, Belgium Assistance Publique-Hôpitaux de Paris, Université Paris-Saclay, Hôpital Bicêtre, Service d'ORL, Le Kremlin-Bicêtre, France Division of Paediatric Respiratory Medicine and Allergology, Department of Paediatrics, Inselspital, University Hospital, University of Bern, Bern, Switzerland Department of Respiratory Diseases, University Hospital, Leuven, Belgium Department of Pediatric Pulmonology, Hacettepe University, School of Medicine, Ankara, Turkey Department of Pediatric Pulmonology, Immunology and Critical Care Medicine, Charité-Universitätsmedizin Berlin, Berlin, Germany Department of Otorhinolaryngology, University Hospital Liège, Liège, Belgium Biomedical Sciences Department, CEU-Cardenal Herrera University, Castellón, Spain Department of Pediatric Pulmonology, Immunology and Critical Care Medicine, Charité-Universitätsmedizin Berlin, Berlin, Germany Medical School, University of Cyprus, Nicosia, Cyprus For a list of the EPIC-PCD team members see the Acknowledgements Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland Background 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 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 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 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.http://openres.ersjournals.com/content/9/3/00701-2022.full
spellingShingle Yin Ting Lam
Jean-François Papon
Mihaela Alexandru
Andreas Anagiotos
Miguel Armengot
Mieke Boon
Andrea Burgess
Suzanne Crowley
Sinan Ahmed D. Dheyauldeen
Nagehan Emiralioglu
Ela Erdem Eralp
Christine van Gogh
Yasemin Gokdemir
Onder Gunaydın
Eric G. Haarman
Amanda Harris
Isolde Hayn
Hasnaa Ismail-Koch
Bülent Karadag
Céline Kempeneers
Sookyung Kim
Philipp Latzin
Natalie Lorent
Ugur Ozcelik
Charlotte Pioch
Anne-Lise M.L. Poirrier
Ana Reula
Jobst Roehmel
Panayiotis Yiallouros
on behalf of the EPIC-PCD team
Myrofora Goutaki
Sinonasal disease among patients with primary ciliary dyskinesia: an international study
ERJ Open Research
title Sinonasal disease among patients with primary ciliary dyskinesia: an international study
title_full Sinonasal disease among patients with primary ciliary dyskinesia: an international study
title_fullStr Sinonasal disease among patients with primary ciliary dyskinesia: an international study
title_full_unstemmed Sinonasal disease among patients with primary ciliary dyskinesia: an international study
title_short Sinonasal disease among patients with primary ciliary dyskinesia: an international study
title_sort sinonasal disease among patients with primary ciliary dyskinesia an international study
url http://openres.ersjournals.com/content/9/3/00701-2022.full
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