Acute exacerbations in children's interstitial lung disease.

Autor:	Seidl E; Department of Pediatrics, Dr. von Hauner Children's Hospital, University Hospital, German Center for Lung Research, Munich, Germany., Schwerk N; Clinic for Pediatric Pneumology, Allergology, and Neonatology, Hannover Medical School, German Center for Lung Research, Hannover, Germany., Carlens J; Clinic for Pediatric Pneumology, Allergology, and Neonatology, Hannover Medical School, German Center for Lung Research, Hannover, Germany., Wetzke M; Clinic for Pediatric Pneumology, Allergology, and Neonatology, Hannover Medical School, German Center for Lung Research, Hannover, Germany., Emiralioğlu N; Division of Pediatric Pulmonology, Hacettepe University Faculty of Medicine, Ankara, Turkey, Ankara, Turkey., Kiper N; Division of Pediatric Pulmonology, Hacettepe University Faculty of Medicine, Ankara, Turkey, Ankara, Turkey., Lange J; Department of Pediatric Pneumology and Allergy, Medical University of Warsaw, Warsaw, Poland., Krenke K; Department of Pediatric Pneumology and Allergy, Medical University of Warsaw, Warsaw, Poland., Szepfalusi Z; Division of Pediatric Pulmonology, Allergy and Endocrinology, Departement of Pediatrics and Adolescent Medicine, Comprehensive Center of Pediatrics, Medical University of Vienna, Vienna, Austria., Stehling F; Department of Pediatric Pulmonology and Sleep Medicine, University Children's Hospital Essen, Essen, Germany., Baden W; Department of Pediatrics 2, Children's Hospital, Eberhard Karls University, Tuebingen, Germany., Hämmerling S; Division of Pediatric Pulmonology & Allergy and Cystic Fibrosis Center, Department of Pediatrics III, University of Heidelberg, Germany, Heidelberg, Germany., Jerkic SP; Department of Allergy, Pneumology, and Cystic Fibrosis, Goethe University Frankfurt am Main, Frankfurt, Germany., Proesmans M; Department of Pediatrics, Pediatric Pulmonology, University Hospital of Leuven, Leuven, Flanders, Belgium., Ullmann N; Pediatric Pulmonology and Respiratory Intermediate Care Unit, Sleep and Long Term Ventilation Unit, Academic Department of Pediatrics (DPUO), Pediatric Hospital 'Bambino Gesù' Research Institute, Roma, Italy., Buchvald F; Department of Paediatrics and Adolescent Medicine, Paediatric Pulmonary Service, Copenhagen University Hospital, Rigshospitalet, Danish PCD & chILD Centre, CF Centre Copenhagen, Copenhagen, Denmark., Knoflach K; Department of Pediatrics, Dr. von Hauner Children's Hospital, University Hospital, German Center for Lung Research, Munich, Germany., Kappler M; Department of Pediatrics, Dr. von Hauner Children's Hospital, University Hospital, German Center for Lung Research, Munich, Germany., Griese M; Department of Pediatrics, Dr. von Hauner Children's Hospital, University Hospital, German Center for Lung Research, Munich, Germany matthias.griese@med.uni-muenchen.de.
Jazyk:	angličtina
Zdroj:	Thorax [Thorax] 2022 Aug; Vol. 77 (8), pp. 799-804. Date of Electronic Publication: 2022 Feb 11.
DOI:	10.1136/thoraxjnl-2021-217941
Abstrakt:	Introduction: Acute exacerbations (AEs) increase morbidity and mortality of patients with chronic pulmonary diseases. Little is known about the characteristics and impact of AEs on children's interstitial lung disease (chILD). Methods: The Kids Lung Register collected data on AEs, the clinical course and quality of life (patient-reported outcomes - PRO) of rare paediatric lung diseases. Characteristics of AEs were obtained. Results: Data of 2822 AEs and 2887 register visits of 719 patients with chILD were recorded. AEs were characterised by increased levels of dyspnoea (74.1%), increased respiratory rate (58.6%) and increased oxygen demand (57.4%). Mostly, infections (94.4%) were suspected causing an AE. AEs between two register visits revealed a decline in predicted FEV1 (median -1.6%, IQR -8.0 to 3.9; p=0.001), predicted FVC (median -1.8%, IQR -7.5 to 3.9; p=0.004), chILD-specific questionnaire (median -1.3%, IQR -3.6 to 4.5; p=0.034) and the physical health summary score (median -3.1%, IQR -15.6 to 4.3; p=0.005) compared with no AEs in between visits. During the median observational period of 2.5 years (IQR 1.2-4.6), 81 patients died. For 49 of these patients (60.5%), mortality was associated with an AE. Conclusion: This is the first comprehensive study analysing the characteristics and impact on the clinical course of AEs in chILD. AEs have a significant and deleterious effect on the clinical course and health-related quality of life in chILD. Competing Interests: Competing interests: None declared. (© Author(s) (or their employer(s)) 2022. No commercial re-use. See rights and permissions. Published by BMJ.)
Databáze:	MEDLINE
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