Factors in childhood associated with lung function decline to adolescence in cystic fibrosis |
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| Institution: | 1. Children''s Health and Environment Program, Child Health Research Centre, The University of Queensland, 62 Graham St, South Brisbane, QLD 4101, Australia;2. Starship Children''s Hospital and The University of Auckland, Auckland, New Zealand;3. Children''s Health Queensland Hospital and Health Service, Brisbane, QLD, Australia;4. The Children''s Hospital at Westmead, Westmead, NSW, Australia;5. Griffith University and Gold Coast Health, Southport, QLD, Australia;6. Royal Children''s Hospital, Melbourne, VIC, Australia;7. Erasmus MC, Sophia Children''s Hospital, Rotterdam, Netherlands;8. Child Health Research Centre, The University of Queensland, South Brisbane, QLD, Australia;9. Griffith University, Nathan, QLD, Australia;1. Imaging Department, Hopital Nord, APHM, Aix Marseille University;2. Aix Marseille Univ, LIIE, Marseille, France;3. Aix Marseille Univ, CERIMED, Marseille, France;4. Service de Pneumologie et équipe de transplantation pulmonaire, Centre de Ressources et de Compétences de la Mucoviscidose (CRCM) Adulte, AP-HM Hôpital Nord,13015, Marseille France;5. Aix Marseille Université, Service de radiologie, La Timone Hôpital, 264 rue Saint Pierre, 13005 Marseille;6. Service d''anatomie et cytologie pathologiques, Hôpital Nord, chemin des Bourrely, 13015 Marseille, France;7. U1068- CRCM, Aix Marseille université, 13015 Marseille, France;8. Service de chirurgie thoracique et Transplantation Pulmonaire, hôpital Nord, chemin des Bourrely, 13015 Marseille, France;9. Aix Marseille Univ, APHM, INSERM, IRD, SESSTIM, ISSPAM, Hop Timone, Biostatistics department, BIOSTIC, Marseille, F-13005, France;2. Slovak Cystic Fibrosis Association, Slovakia;3. Warsaw Cystic Fibrosis Centre, Dziekanow Lesny Paediatric Hospital, Institute of Mother and Child, Warsaw, Poland;4. Department of Cystic Fibrosis, National Koranyi Institute for Pulmonology, Budapest, Hungary;5. Paediatric Clinic, Paediatric Department, University Hospital Alexandrovska, Medical University Sofia, Sofia, Bulgaria;6. Institute of Hereditary Pathology of National Academy of Medical Sciences of Ukraine, Lviv, Ukraine;7. Latvian Cystic Fibrosis Society, Latvia;1. University of Texas Southwestern Medical Center, Dallas, TX, USA;2. Technion- Israel Institute of Technology and the B. Rappaport Faculty of Medicine, Haifa, Israel;3. Ophthalmology Department, Carmel Medical Center, Haifa, Israel;4. Neonatal Intensive Care Unit, Carmel Medical Center, Haifa, Israel;5. National Jewish Health, Denver, Colorado, USA;6. Pediatric Pulmonology, University of North Carolina, Chapel Hill, NC, USA;7. Pulmonology Institute and CF Center, Carmel Medical Center, Haifa, Israel;1. Department of Pediatrics, UNC School of Medicine, University of North Carolina, 450 MacNider Building, CB# 7217 321 S. Columbia Street, Chapel Hill, NC, United States;2. Department of Biostatistics, University of North Carolina, Chapel Hill, NC, United States;3. Department of Pediatrics, Columbia University Irving Medical Center, New York, NY, United States;4. Department of Infection Prevention & Control, New York-Presbyterian Hospital, New York, NY, United States;1. Department of Diagnostic and Interventional Radiology, Subdivision of Pulmonary Imaging, University Hospital Heidelberg, Im Neuenheimer Feld 420, 69120 Heidelberg, Germany;2. Translational Lung Research Center Heidelberg (TLRC), German Center for Lung Research (DZL), Im Neuenheimer Feld 156, 69120 Heidelberg, Germany;3. Department of Diagnostic and Interventional Radiology with Nuclear Medicine, Thoraxklinik at University Hospital Heidelberg, Röntgenstr. 1, 69126 Heidelberg, Germany;4. Department of Pulmonology and Respiratory Medicine, Cystic Fibrosis Center, Thoraxklinik at University Hospital Heidelberg, Röntgenstr. 1, 69126 Heidelberg, Germany;5. Department of Neuroradiology, University Hospital Heidelberg, Im Neuenheimer Feld 400, 69120 Heidelberg, Germany;6. Department of Translational Pulmonology and Division of Pediatric Pulmonology and Allergy and Cystic Fibrosis Center, University of Heidelberg, Heidelberg, Germany;7. Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany;8. German Center for Lung Research (DZL), Associated partner site, Berlin, Germany;9. Berlin Institute of Health at Charité – Universitätsmedizin Berlin, Berlin, Germany;1. Université de Paris, Institut Cochin, Inserm U1016, Paris, France;2. Respiratory Medicine and Cystic Fibrosis National Reference Center, Cochin Hospital, Assistance Publique Hôpitaux de Paris (AP-HP), Paris, France;3. ERN-Lung CF network, Frankfurt, Germany;4. Department of Respiratory Medicine and Lung Transplantation, Adult Cystic Fibrosis Center Aix Marseille Université, APHM, Hôpital Nord, Marseille, France;5. Service Hospitalo-Universitaire de Pneumologie et Physiologie, Pôle Thorax et Vaisseaux, Centre Hospitalier Universitaire de Grenoble-Alpes, La Tronche, France;6. Centre de référence Adulte de la Mucoviscidose, Service de médecine interne, Hospices Civils de Lyon, Pierre Bénite, France;8. Cystic Fibrosis Center Service de Pneumologie Pôle des Voies Respiratoires, Hôpital Larrey CHU de Toulouse, Toulouse, France;9. Service de Pneumologie, L''Institut Du Thorax, CHU Nantes, Nantes, France;10. Cystic Fibrosis Center, Hôpital Arnaud de Villeneuve, Centre Hospitalier Universitaire de Montpellier, Montpellier, France;11. Pulmonology Department, Université Côte d''Azur, Centre Hospitalier Universitaire de Nice, Fédération Hospitalo-Universitaire OncoAge, CNRS, Inserm, Institute for Research on Cancer and Aging Nice Team 3, Nice, France;12. Service de Pneumologie, Centre Hospitalier Intercommunal, FHU SENEC, Créteil, France;13. CRCM - Centre de Transplantation Pulmonaire, Service de pneumologie, Hôpital Foch, Suresnes, France;14. Hôpital Renée Sabran, Cystic Fibrosis Center, Giens, France;15. Centre de Ressources et de Compétences de la Mucoviscidose, Fondation Ildys, Roscoff, France;p. CHU Clermont-Ferrand, Centre de Référence et de Compétence Mucoviscidose, Clermont-Ferrand, France;q. Association Vaincre la Mucoviscidose, Paris, France;r. Effi-stat, Paris, France;s. URC-CIC Paris Descartes Necker Cochin, AP-HP, Hôpital Cochin, Paris, France |
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| Abstract: | BackgroundDespite improvements in general health and life expectancy in people with cystic fibrosis (CF), lung function decline continues unabated during adolescence and early adult life.MethodsWe examined factors present at age 5-years that predicted lung function decline from childhood to adolescence in a longitudinal study of Australasian children with CF followed from 1999 to 2017.ResultsLung function trajectories were calculated for 119 children with CF from childhood (median 5.0 25%-75%=5.0–5.1]) years) to early adolescence (median 12.5 25%-75%=11.4–13.8] years). Lung function fell progressively, with mean (standard deviation) annual change -0.105 (0.049) for forced vital capacity (FVC) Z-score (p<0.001), -0.135 (0.048) for forced expiratory volume in 1-second (FEV1) Z-score (p<0.001), -1.277 (0.221) for FEV1/FVC% (p<0.001), and -0.136 (0.052) for forced expiratory flow between 25% and 75% of FVC Z-score (p<0.001). Factors present in childhood predicting lung function decline to adolescence, in multivariable analyses, were hospitalisation for respiratory exacerbations in the first 5-years of life (FEV1/FVC p = 0.001, FEF25–75 p = 0.01) and bronchoalveolar lavage neutrophil elastase activity (FEV1/FVC% p = 0.001, FEV1 p = 0.05, FEF25–75 p = 0.02). No examined factor predicted a decline in the FVC Z-score.ConclusionsAction in the first 5-years of life to prevent and/or treat respiratory exacerbations and counteract neutrophilic inflammation in the lower airways may reduce lung function decline in children with CF, and these should be targets of future research. |
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| Keywords: | ACFBAL"} {"#name":"keyword" "$":{"id":"pc_h5UpOwppUL"} "$$":[{"#name":"text" "_":"Australasian cystic fibrosis bronchoalveolar lavage CF-FAB"} {"#name":"keyword" "$":{"id":"pc_nJGdLYf8nL"} "$$":[{"#name":"text" "_":"longitudinal observational follow-up study of the ACFBAL cohort |
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