RT Journal Article
SR Electronic
T1 Sinonasal disease among patients with primary ciliary dyskinesia: an international study
JF ERJ Open Research
JO erjor
FD European Respiratory Society
SP 00701-2022
DO 10.1183/23120541.00701-2022
VO 9
IS 3
A1 Yin Ting Lam
A1 Jean-François Papon
A1 Mihaela Alexandru
A1 Andreas Anagiotos
A1 Miguel Armengot
A1 Mieke Boon
A1 Andrea Burgess
A1 Suzanne Crowley
A1 Sinan Ahmed D. Dheyauldeen
A1 Nagehan Emiralioglu
A1 Ela Erdem Eralp
A1 Christine van Gogh
A1 Yasemin Gokdemir
A1 Onder Gunaydın
A1 Eric G. Haarman
A1 Amanda Harris
A1 Isolde Hayn
A1 Hasnaa Ismail-Koch
A1 Bülent Karadag
A1 Céline Kempeneers
A1 Sookyung Kim
A1 Philipp Latzin
A1 Natalie Lorent
A1 Ugur Ozcelik
A1 Charlotte Pioch
A1 Anne-Lise M.L. Poirrier
A1 Ana Reula
A1 Jobst Roehmel
A1 Panayiotis Yiallouros
A1 on behalf of the EPIC-PCD team
A1 Myrofora Goutaki
A1 EPIC-PCD team:
A1 Dilber Ademhan
A1 Mihaela Alexandru
A1 Andreas Anagiotos
A1 Miguel Armengot
A1 Lionel Benchimol
A1 Achim G. Beule
A1 Irma Bon
A1 Mieke Boon
A1 Marina Bullo
A1 Andrea Burgess
A1 Doriane Calmes
A1 Carmen Casaulta
A1 Marco Caversaccio
A1 Nathalie Caversaccio
A1 Bruno Crestani
A1 Suzanne Crowley
A1 Sinan Ahmed D. Dheyauldeen
A1 Sandra Diepenhorst
A1 Nagehan Emiralioglu
A1 Ela Erdem Eralp
A1 Pinar Ergenekon
A1 Nathalie Feyaerts
A1 Gavriel Georgiou
A1 Amy Glen
A1 Christine van Gogh
A1 Yasemin Gokdemir
A1 Myrofora Goutaki
A1 Onder Gunaydın
A1 Eric G. Haarman
A1 Amanda Harris
A1 Isolde Hayn
A1 Simone Helms
A1 Sara-Lynn Hool
A1 Isabelle Honoré
A1 Hasnaa Ismail Koch
A1 Bülent Karadag
A1 Céline Kempeneers
A1 Synne Kennelly
A1 Elisabeth Kieninger
A1 Sookyung Kim
A1 Panayiotis Kouis
A1 Yin Ting Lam
A1 Philipp Latzin
A1 Marie Legendre
A1 Natalie Lorent
A1 Jane S. Lucas
A1 Bernard Maitre
A1 Alison McEvoy
A1 Rana Mitri-Frangieh
A1 David Montani
A1 Loretta Müller
A1 Noelia Muñoz
A1 Heymut Omran
A1 Ugur Ozcelik
A1 Beste Ozsezen
A1 Samantha Packham
A1 Jean-François Papon
A1 Clara Pauly
A1 Charlotte Pioch
A1 Anne-Lise M.L. Poirrier
A1 Johanna Raidt
A1 Ana Reula
A1 Rico Rinkel
A1 Jobst Roehmel
A1 Andre Schramm
A1 Guillaume Thouvenin
A1 Woolf T Walker
A1 Hannah Wilkins
A1 Panayiotis Yiallouros
A1 Ali Cemal Yumusakhuylu
A1 Niklas Ziegahn
YR 2023
UL http://openres.ersjournals.com/content/9/3/00701-2022.abstract
AB 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.This study reinforces the importance of regular sinonasal examinations for patients of all ages with PCD and the need to develop evidence-based sinonasal treatments as part of overall PCD management https://bit.ly/3lMcgbo