Related Experiment Video
Updated: Oct 4, 2025
Author Spotlight: Evaluating the Therapeutic Efficacy of Moving Cupping Along Meridians for Acute Exacerbation of COPD
Published on: September 27, 2024
Acute exacerbations in children's interstitial lung disease
Elias Seidl1, Nicolaus Schwerk2, Julia Carlens2
1Department of Pediatrics, Dr. von Hauner Children's Hospital, University Hospital, German Center for Lung Research, Munich, Germany.
Insights
Acute exacerbations (AEs) in children with interstitial lung disease (chILD) significantly worsen lung function and quality of life. These events, often caused by infections, are linked to a substantial portion of chILD-related deaths.
Area of Science:
- Pediatric Pulmonology
- Rare Diseases
- Interstitial Lung Disease
Background:
- Acute exacerbations (AEs) are known to increase morbidity and mortality in chronic pulmonary diseases.
- The specific characteristics and impact of AEs on children's interstitial lung disease (chILD) remain poorly understood.
Purpose of the Study:
- To characterize the features of AEs in pediatric patients with chILD.
- To evaluate the impact of AEs on the clinical course and quality of life in chILD patients.
Main Methods:
- Utilized data from the Kids Lung Register, collecting information on AEs, clinical course, and patient-reported outcomes (PROs).
- Analyzed data from 719 patients with chILD, encompassing 2822 AEs and 2887 register visits.
Main Results:
- The most common AE symptoms included increased dyspnea (74.1%), respiratory rate (58.6%), and oxygen demand (57.4%), with infections suspected in 94.4%.
- AEs were associated with significant declines in predicted FEV1, FVC, chILD-specific quality of life, and physical health scores compared to periods without AEs.
- Mortality was associated with AEs in 60.5% of the 81 patient deaths during the study period.
Conclusions:
- This study provides the first comprehensive analysis of AEs in chILD, highlighting their significant negative impact.
- AEs have a detrimental effect on the clinical trajectory and health-related quality of life for children with interstitial lung disease.
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.
More Related Videos
Related Concept Videos
01:17Pulmonary Cycle: Exhalation
01:29Asthma-I: Introduction
01:30Asthma-IV: Diagnostic and Management
Clinical Assessment for Asthma:
This is the first step in diagnosing and managing asthma. It includes:
01:24Asthma-III: Symptoms and Complications
Classification of Asthma
01:27Chronic Obstructive Pulmonary Disease-IV: Assessement and Diagnostic Studies
Medical History
01:26COPD: Management Using Bronchodilators and Corticosteroids