Lack of Correlation of Sinonasal and Otologic Reported Symptoms With Objective Measurements Among Patients With Primary Ciliary Dyskinesia: An International Study.
[en] Sinonasal and otologic symptoms are common among patients with primary ciliary dyskinesia (PCD) of all ages. We used baseline data from the ENT Prospective International Cohort of PCD patients (EPIC-PCD), the first PCD cohort focused on ENT disease manifestations. We assessed agreement between patient- or parent-reported symptoms and relevant examination findings, and calculated unweighted Cohen’s kappa to adjust for agreement by chance. We included 404 participants, from 12 centres. We found no correlation between patient-reported sinonasal symptoms and relevant clinical examination findings. Otologic symptoms correlated poorly or weakly with otoscopy and audiometry findings, with age and centre identified as determinants of agreement.
Disciplines :
Otolaryngology
Author, co-author :
Lam, Yin Ting; Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland ; Graduate School for Cellular and Biomedical Sciences, University of Bern, Bern, Switzerland
Papon, Jean-François; Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris-Saclay, Service d'ORL, Hôpital Bicêtre, Le Kremlin-Bicêtre, France ; Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
Alexandru, Mihaela; Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris-Saclay, Service d'ORL, Hôpital Bicêtre, Le Kremlin-Bicêtre, France ; Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France ; Département de Génétique Médicale, Sorbonne Université, Inserm UMR_S933, Maladies Génétiques D'expression Pédiatrique, Hôpital Armand Trousseau, Paris, France
Anagiotos, Andreas; Department of Otolaryngology, Nicosia General Hospital, Nicosia, Cyprus
Armengot, Miguel; Department of Otorhinolaryngology, and Primary Ciliary Dyskinesia Unit, La Fe University and Polytechnic Hospital, Valencia, Spain ; Medical School, Valencia University, Valencia, Spain ; Molecular, Cellular and Genomic Biomedicine Group, IIS La Fe, Valencia, Spain
Boon, Mieke; Department of Paediatrics, University Hospitals Leuven, Leuven, Belgium
Burgess, Andrea; Primary Ciliary Dyskinesia Centre, Southampton Children's Hospital, Southampton NHS Foundation Trust, Southampton, UK
Caversaccio, Nathalie; Department of Otorhinolaryngology, Head and Neck Surgery, University Hospital of Bern, University of Bern, Bern, Switzerland
Crowley, Suzanne; Paediatric Department of Allergy and Lung Diseases, Oslo University Hospital, Oslo, Norway
Dheyauldeen, Sinan Ahmed D; Department of Otorhinolaryngology, Head and Neck Surgery, Oslo University Hospital, Oslo, Norway ; Faculty of Medicine, University of Oslo, Oslo, Norway
Emiralioglu, Nagehan; Department of Pediatric Pulmonology, Hacettepe University, School of Medicine, Ankara, Turkey
Erdem, Ela; Department of Pediatric Pulmonology, Marmara University, School of Medicine, Istanbul, Turkey
Gogh, Christine van; Department of Otorhinolaryngology-Head and Neck Surgery, Amsterdam UMC, Amsterdam, The Netherlands
Gokdemir, Yasemin; Department of Pediatric Pulmonology, Marmara University, School of Medicine, Istanbul, Turkey
Gunaydın, Onder; Department of Otorhinolaryngology, Hacettepe University School of Medicine, Ankara, Turkey
Haarman, Eric G; Department of Pediatric Pulmonology, Emma Children's Hospital, Amsterdam UMC, Vrije Universiteit, Amsterdam, The Netherlands
Harris, Amanda; Southampton Children's Hospital, University of Southampton, Southampton, UK ; Primary Ciliary Dyskinesia Centre, NIHR Respiratory Biomedical Research Centre, University of Southampton, Southampton, UK
Hayn, Isolde; Department of Otorhinolaryngology, Head and Neck Surgery, Charité-Universitätsmedizin Berlin, Berlin, Germany
Ismail-Koch, Hasnaa; Primary Ciliary Dyskinesia Centre, Southampton Children's Hospital, Southampton NHS Foundation Trust, Southampton, UK
Karadag, Bulent; Department of Pediatric Pulmonology, Marmara University, School of Medicine, Istanbul, Turkey
Kempeneers, Céline ; Centre Hospitalier Universitaire de Liège - CHU > > Service de pédiatrie
Kim, Sookyung; Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris-Saclay, Service d'ORL, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
Lorent, Natalie; Department of Respiratory Diseases, University Hospitals Leuven, Leuven, Belgium
Ozcelik, Ugur; Department of Pediatric Pulmonology, Hacettepe University, School of Medicine, Ankara, Turkey
Pioch, Charlotte; Department of Pediatric Pulmonology, Immunology and Critical Care Medicine, Charité-Universitätsmedizin Berlin, Berlin, Germany
Poirrier, Anne-Lise ; Centre Hospitalier Universitaire de Liège - CHU > > Service d'ORL, d'audiophonologie et de chir. cervico-faciale
Roehmel, Jobst; Department of Pediatric Pulmonology, Immunology and Critical Care Medicine, Charité-Universitätsmedizin Berlin, Berlin, Germany
Yiallouros, Panayiotis; Medical School, University of Cyprus, Nicosia, Cyprus ; Pediatric Pulmonology Unit, Hospital 'Archbishop Makarios III', Nicosia, Cyprus
Goutaki, Myrofora; Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland ; Paediatric Respiratory Medicine, Children's University Hospital of Bern, University of Bern, Bern, Switzerland
Lack of Correlation of Sinonasal and Otologic Reported Symptoms With Objective Measurements Among Patients With Primary Ciliary Dyskinesia: An International Study.
JFP reports personal fees from Sanofi, GSK, Medtronic and ALK outside the submitted work. MB reports grants from Forton grant (King Baudouin Foundation) 2020-J1810150-217926 Cystic fibrosis research and personal fees from Vertex outside the submitted work. JR received grants and clinical study rec-ompensations from Vertex, INSMED, Medical Research Council/ UK, BMBF, Mukoviszidose Institut outside the submitted work. ALMLP received speaker honorarium from GSK and Sanofi outside the submitted work. NL received honoraria to her institution from GSK, INSMED, AN2 Therapeutics outside the submitted work and a travel grant from Pfizer.The study was supported by a Swiss National Science Foundation Ambizione fellowship (PZ00P3_185923) granted to M Goutaki. The authors participate in the BEAT-PCD (Better Experimental Approaches to Treat Primary Ciliary Dyskinesia) clinical research collaboration, supported by the European Respiratory Society, and most centers participate in the ERN-LUNG (European Reference Network on rare respiratory diseases) PCD core.
Lam YT, Papon JF, Alexandru M, Anagiotos A, Armengot M, Boon M, et al. Sinonasal disease among patients with primary ciliary dyskine-sia: an international study. ERJ Open Res. 2023 May;9(3):00701-2022.
Goutaki M, Lam YT, Alexandru M, Anagiotos A, Armengot M, Boon M, et al. Characteristics of otologic disease among patients with primary ciliary dyskinesia. JAMA Otolaryngol Head Neck Surg. 2023 Jul;149(7):587-96.
Goutaki M, Meier AB, Halbeisen FS, Lucas JS, Dell SD, Maurer E, et al. Clinical manifestations in primary ciliary dyskinesia: systematic review and meta-analysis. Eur Respir J. 2016 Oct;48(4):1081-95.
Goutaki M, Lam YT, Alexandru M, Anagiotos A, Armengot M, Be-quignon E, et al. Study protocol: the ear-nose-throat (ENT) prospective international cohort of patients with primary ciliary dyskinesia (EPIC-PCD). BMJ Open. 2021 Oct;11(10):e051433.
Lucas JS, Barbato A, Collins SA, Goutaki M, Behan L, Caudri D, et al. European Respiratory Society guidelines for the diagnosis of primary ciliary dyskinesia. Eur Respir J. 2017 Jan;49(1):1601090.
Goutaki M, Papon JF, Boon M, Casaulta C, Eber E, Escudier E, et al. Standardised clinical data from patients with primary ciliary dyski-nesia: FOLLOW-PCD. ERJ Open Res. 2020 Feb;6(1):00237-2019.
Harris PA, Taylor R, Thielke R, Payne J, Gonzalez N, Conde JG. Research electronic data capture (REDCap): a metadata-driven meth-odology and workflow process for providing translational research informatics support. J Biomed Inform. 2009 Apr;42(2):377-81.
McHugh ML. Interrater reliability: the kappa statistic. Biochem Med (Zagreb). 2012;22(3):276-82.
Reichenheim ME. Confidence intervals for the kappa statistic. Stata J. 2004 Dec;4(4):421-8.
Cohen J. A coefficient of agreement for nominal scales. Educ Psy-chol Meas. 1960 Apr;20(1):37-46.
Campbell R. Managing upper respiratory tract complications of primary ciliary dyskinesia in children. Curr Opin Allergy Clin Immunol. 2012 Feb;12(1):32-8.
Majithia A, Fong J, Hariri M, Harcourt J. Hearing outcomes in children with primary ciliary dyskinesia: a longitudinal study. Int J Pedi-atr Otorhinolaryngol. 2005 Aug;69(8):1061-4.
Racette SD, Wijewickrama RC, Jayaprakash V, Sherris DA, Santos C, Kita H, et al. Correlation of symptoms, clinical signs, and biomarkers of inflammation in postsurgical chronic rhinosinusitis. Ann Otol Rhi-nol Laryngol. 2017 Jun;126(6):455-62.
Park DY, Lee EJ, Kim JH, Kim YS, Jung CM, Kim KS. Correlation between symptoms and objective findings may improve the symp-tom-based diagnosis of chronic rhinosinusitis for primary care and epidemiological studies. BMJ Open. 2015 Dec;5(12):e009541.
Cornford CS. Why patients consult when they cough: a comparison of consulting and non-consulting patients. Br J Gen Pract. 1998 Nov; 48(436):1751-4.
Muramatsu K, Miyaoka H, Muramatsu Y, Fuse K, Yoshimine F, Ka-mijima K, et al. The amplification of somatic symptoms in upper respiratory tract infections. Gen Hosp Psychiatry. 2002 May-Jun;24(3): 172-5.
Mozun R, Ardura-Garcia C, Pedersen ES, Goutaki M, Usemann J, Singer F, et al. Agreement of parent-and child-reported wheeze and its association with measurable asthma traits. Pediatr Pulmonol. 2021 Dec;56(12):3813-21.
Swierniak W, Gos E, Skarzynski PH, Czajka N, Skarzynski H. The accuracy of parental suspicion of hearing loss in children. Int J Pedi-atr Otorhinolaryngol. 2021 Feb;141:110552.
