Advanced but not mild liver disease is a predictor of decreased survival in children with cystic fibrosis,with far greater impact in females: A 27-year real-life cohort study |
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| Institution: | 1. Pontifícia Universidade Católica do Rio Grande do Sul, Porto Alegre, RS, Brazil;2. Department of Gastroenterology and Hepatology, Queensland Children''s Hospital, Brisbane, QLD, Australia;3. Faculty of Medicine, The University of Queensland, Brisbane, QLD, Australia;4. Hepatic Fibrosis Group, Department of Cellular and Molecular Biology, QIMR Berghofer Medical Research Institute, Brisbane, QLD, Australia;5. QIMR Berghofer Statistics Unit, QIMR Berghofer Medical Research Institute, QLD, Australia;1. Evidence Based Child Health Group, University of Nottingham, Nottingham, United Kingdom;2. Population, Policy and Practice Research and Teaching Department, UCL Great Ormond Street Institute of Child Health, London, United Kingdom;3. School of Medicine, University of Nottingham, Nottingham, United Kingdom;4. Person with CF, Plymouth, United Kingdom;5. Parent of children with CF, Nottingham, United Kingdom;6. York Hull Adult CF Centre, York Teaching Hospital NHS Foundation Trust, York, United Kingdom;1. Wessex Adult Cystic Fibrosis Service, University Hospital Southampton NHS Foundation Trust, Southampton, UK;2. School of Health and Related Research (ScHARR), University of Sheffield, Sheffield, UK;3. Sheffield Adult Cystic Fibrosis Centre, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK;4. Centre for Health Informatics, University of Manchester, Manchester, UK;5. Newcastle Adult Cystic Fibrosis Centre, The Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK;6. Bristol Adult Cystic Fibrosis Centre, University Hospitals Bristol and Weston NHS Foundation Trust, Bristol, UK;7. North West Midlands Cystic Fibrosis Centre, University Hospitals of North Midlands NHS Trust, Stoke-on-Trent, UK;8. York Hull Adult Cystic Fibrosis Centre, Hull University Teaching Hospitals NHS Trust, Hull, UK;9. York Hull Adult Cystic Fibrosis Centre, York Teaching Hospital NHS Foundation Trust, York, UK;10. Oxford Adult Cystic Fibrosis Centre, Oxford University Hospitals NHS Foundation Trust, Oxford, UK;11. Respiratory Biomedical Research Centre, University of Southampton, Southampton, UK;12. Leicester Adult Cystic Fibrosis Centre, University Hospitals of Leicester NHS Trust, Leicester, UK;13. Wolfson Adult Cystic Fibrosis Centre, Nottingham University Hospitals NHS Trust, Nottingham, UK;14. Adult Cystic Fibrosis Clinic, Norfolk and Norwich University Hospitals NHS Foundation Trust, Norwich, UK;15. Derriford Hospital Adult Cystic Fibrosis Centre, University Hospitals Plymouth NHS Trust, Plymouth, UK;p. Cystic Fibrosis Unit, Frimley Health NHS Foundation Trust, Frimley, UK;1. Department of Paediatric Cardiology and Intensive Care Medicine, Paediatric Pulmonary and Allergology Outpatient Clinic, Universitätsmedizin Göttingen, Göttingen, Germany;2. Clinical Research Group ''Molecular Pathology of Cystic Fibrosis'', Department of Paediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany;3. Department of Paediatric and Adolescent Medicine, Paediatric Gastroenterology Outpatient Clinic, Universitätsmedizin Göttingen, Göttingen, Germany;4. Biomedical Research in Endstage and Obstructive Lung Disease Hannover (BREATH), Member of the German Center for Lung Research, Hannover, Germany;1. Université Paris Saclay, UVSQ, Inserm, Infection et Inflammation, Montigny-le-Bretonneux, France;2. AP-HP, GHU Paris Saclay, Hôpital Ambroise Paré, Service de Microbiologie, Boulogne-Billancourt, France;3. AP-HP, GHU Paris, Hôpital Necker-Enfants Malades, Service de Microbiologie, Paris 15e, France;4. Hospices Civils de Lyon, Hôpital de la Croix Rousse-Centre de Biologie Nord, Institut des Agents Infectieux, Laboratoire de Bactériologie, Grande Rue de la Croix Rousse, 69004, Lyon, France;5. Centre International de Recherche en Infectiologie, INSERM U1111, Université de Lyon, Lyon, France;6. CHU, Service de Parasitologie-Mycologie, Groupe d''Etude des Interactions Hôte-Pathogène (GEIHP, EA 3142), UNIV Angers, UNIV Brest, SFR 4208 ICAT, Angers, France;7. Laboratoire de Bactériologie, CHRU de Besançon, UMR CNRS 6249 Chrono-Environnement, Faculté de Médecine-Pharmacie, Université de Bourgogne Franche-Comté, Besançon, France;8. Université Aix-Marseille, CNRS, LISM, IMM FR3479, Marseille, France;9. Département de bactériologie-virologie, hygiène et parasitologie-mycologie, centre hospitalier régional universitaire (CHRU) de Brest, Brest, France; Inserm, EFS, UMR 1078 France « génétique, génomique fonctionnelle et biotechnologies », GGB, université Brest, 29200 Brest, France;10. AP-HP, GHU Paris Saclay, Hôpital Raymond Poincaré, Service de Microbiologie, Garches, France;1. Cystic Fibrosis Therapeutics Development Network Coordinating Center, Seattle Children''s Hospital, Seattle, WA, United States of America;2. Department of Pediatrics, University of Washington, Seattle, WA, United States of America;3. Department of Biostatistics, University of Washington, Seattle, WA, United States of America;4. Department of Epidemiology, University of Washington, Seattle, WA, United States of America;5. Department of Pediatrics, University of North Carolina at Chapel Hill, Chapel Hill, NC, United States of America |
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| Abstract: | BackgroundImproved survival of children with CF has increased our need to understand the relevance of cystic fibrosis-associated liver disease (CFLD). We assessed the impact of liver disease and disease severity on the survival of children with cystic fibrosis.MethodsA real life, single center cohort study with 27 years follow up was conducted. Mild CFLD was diagnosed as children with abnormal serum liver function tests and abnormal ultrasound. Advanced CFLD was established by detection of cirrhosis or portal hypertension. A directed acyclic graph, Kaplan-Meier (KM) and Cox regression analysis were used to model survival.Results290 patients were enrolled, 48 (16.5%) had mild CFLD and 55 (19%) had advanced CFLD. Ten children with advanced CFLD and 1 with mild CFLD died. Based on the KM analysis, the mean (SE) overall survival age of all CF children was 29.1 years (0.50). The mean (SE) survival among females with advanced CFLD was 24.7 years (1.58) compared to 30.4 years (0.66) for females without advanced CFLD (p = 0.0027). Advanced CFLD was a predictor of decreased survival when adjusted for sex and diabetes (HR 2.54, 95%CI 1.05–6.15, p = 0.039). Mild CFLD was not associated with decreased survival. The effect of advanced CFLD on survival was mainly borne by females (HR = 6.37, 95%CI 1.62–25.06 vs. males, HR = 1.00, 95%CI 0.25–4.01).ConclusionAdvanced but not mild CFLD was associated with an increased risk of death when adjusted for sex and diabetes, and resulted in premature death in females with cystic fibrosis by approximately 6 years. |
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