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The negative impact of chronic rhinosinusitis on the health-related quality of life among adult patients with cystic fibrosis
Institution:1. Department of Otolaryngology-Head and Neck Surgery, St. Michael''s Hospital, Unity Health Toronto, Toronto, ON, Canada;3. Adult Cystic Fibrosis Centre, St Michael''s Hospital, Unity Health Toronto, Toronto, Ontario, Canada;5. Division of Respirology, Department of Medicine, University of Toronto, Toronto, Ontario, Canada;1. Leeds Institute of Medical Research at St James''s, University of Leeds, Leeds, United Kingdom;2. Leeds Adult Cystic Fibrosis Unit, St James''s University Hospital, Leeds, United Kingdom;1. McKusick-Nathans Department of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, MD 21287, United States;2. Berman Institute of Bioethics, Johns Hopkins University, Baltimore, MD 21287, United States;3. Eudowood Division of Pediatric Respiratory Sciences, Johns Hopkins University School of Medicine, Baltimore, MD 21287, United States;4. Child Health Evaluative Sciences, The Hospital for Sick Children, Toronto, Ontario, Canada;5. Cystic Fibrosis Foundation, Bethesda, MD 20814, United States;6. Department of Respirology, Adult Cystic Fibrosis Program, St. Michael''s Hospital, Toronto, Ontario, Canada;7. Program in Genetics and Genome Biology, The Hospital for Sick Children, Toronto, Ontario, Canada;8. IRCCS Istituto Giannina Gaslini, Cystic Fibrosis Center, Genoa, Italy;1. Department of Pediatrics, Case Western Reserve University School of Medicine, Cleveland, OH, United States;2. Department of Medicine, Johns Hopkins University, Baltimore, MD, United States;3. Department of Pediatrics, Indiana University, Indianapolis, IN, United States;4. CF Therapeutics Development Network Coordinating Center, Seattle Children''s Research Institute, Seattle, WA, United States;5. Department of Medicine, University of Washington, Seattle, WA, United States;6. Department of Pediatrics, University of Washington, Seattle, WA, United States;7. Departments of Medicine and Pediatrics, Medical University of South Carolina, Charleston, SC, United States;1. Paediatric Unit, Department of Translational Medical Sciences, Cystic Fibrosis Regional Reference Center, University of Naples Federico II, Naples, Italy;2. Cystic Fibrosis Regional Reference Center, Fondazione IRCCS Ca'' Granda Ospedale Maggiore Policlinico, University of Milan, Department of Pathophysiology and Transplantation, Milan, Italy;3. Department of Translational Medical Science, Cystic Fibrosis Center, Adult Unit, University of Naples \"Federico II\", Italy;4. Dept of Experimental Medicine, Sapienza University of Rome, Rome, Italy;5. Pasteur Institute, Cenci Bolognetti Foundation, Sapienza University of Rome, Rome, Italy;6. Cystic Fibrosis Regional Reference Center, A.O.U. Policlinico Umberto I, Rome, Italy;7. Cystic Fibrosis Regional Reference Center, Mother - Child Department, United Hospitals, Ancona, Italy;8. Cystic Fibrosis Centre, Bambino Gesù Children''s Hospital, IRCCS, Rome, Italy;9. Cystic Fibrosis Regional Support Center, Department of Pediatrics, University of Brescia, ASST Spedali Civili Brescia, Brescia, Italy;12. Meyer Children''s Hospital, Cystic Fibrosis Regional Reference Center, Department of Paediatric Medicine, Viale Gaetano Pieraccini 24, Florence, Italy;13. Diagnostic Genetics Unit, Careggi University Hospital, Florence, Italy;14. IRCCS Istituto Giannina Gaslini, Genova, Italy;15. Cystic Fibrosis Center, Hospital San Carlo, Potenza, Italy;p. Freelance Statistician, Milan, Italy;q. Department of Molecular Medicine and Medical Biotechnology, University of Naples, Naples, Italy;r. CEINGE-Advanced Biotechnology, Naples, Italy;1. CHU Nantes, Service de Pneumologie, Institut du Thorax, Nantes, France;2. Nantes Université, INSERM, Center for Research in Transplantation and Translational Immunology, UMR 1064, F-44000 Nantes, France;3. Université Paris Cité, Inserm U1016, Institut Cochin, Paris, France;4. Pulmonary Department and National Cystic Fibrosis Reference Centre, Cochin Hospital; Assistance Publique Hôpitaux de Paris, Paris, France;1. Department of Respiratory and Sleep Medicine, Royal Prince Alfred Hospital, Missenden Road, Camperdown NSW 2050, Australia;2. University of Sydney, Sydney School of Health Sciences, Faculty of Medicine and Health, NSW 2006, Australia;3. The Lung Transplant Unit, St Vincent''s Hospital, Victoria St, Darlinghurst, NSW 2010, Australia
Abstract:BackgroundWith improved survival in cystic fibrosis (CF) patients, it is crucial to evaluate the impact of chronic co-morbidities such as chronic rhinosinusitis (CRS). The objectives were 1) To determine the prevalence of CRS with a large series of CF patients 2) To evaluate the impact of CRS on the Health-Related Quality of Life (HRQoL) of CF patients and 3) To compare CRS-specific, CF-specific and general HRQoL instruments.MethodsConsecutive CF patients from the Toronto Adult Cystic Fibrosis Centre were recruited between March 2018 and January 2020. Participants completed the 22-Item Nasal Outcome Test (SNOT-22), Cystic Fibrosis Questionnaire-Revised for adolescents and adults over 14 years of age (CFQ-R), Cystic Fibrosis Quality of Life Evaluative Self-administered Test (CF-QUEST) and the 36-Item Short Form Survey (SF-36). HRQoL scores were correlated using Spearman's correlation coefficients.ResultsOut of 195 patients eligible for analysis, the prevalence of CRS with positive endoscopic findings was 42.6% (95% confidence interval: 35.5–49.8%). CRS patients reported significantly lower HRQoL with higher SNOT-22 scores and lower scores in the respiratory domain of CFQ-R and physical health domains of CF-QUEST and SF-36. The physical (ρ= ?0.63) and mental (ρ= ?0.66) domains of SF-36 and CF-QUEST (ρ= ?0.76) had a strong correlation with SNOT-22. Higher scores of SNOT-22 nasal subdomains correlated with lower scores of SF-36, CFQ-R and CF-QUEST.ConclusionCRS is a prevalent co-morbidity of CF patients, which significantly reduces HRQoL. SNOT-22, CFQ-R, CF-QUEST and SF-36 were strongly correlated. Severity of sinonasal symptoms have a strong correlation with HRQoL in CF patients.
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