Abstract
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.
Dokumententyp: | Zeitschriftenartikel |
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Fakultät: | Medizin > Klinikum der LMU München > Kinderklinik und Kinderpoliklinik im Dr. von Haunerschen Kinderspital |
Themengebiete: | 600 Technik, Medizin, angewandte Wissenschaften > 610 Medizin und Gesundheit |
URN: | urn:nbn:de:bvb:19-epub-107192-9 |
ISSN: | 0040-6376 |
Sprache: | Englisch |
Dokumenten ID: | 107192 |
Datum der Veröffentlichung auf Open Access LMU: | 20. Sep. 2023, 08:40 |
Letzte Änderungen: | 20. Sep. 2023, 08:40 |