Risk factors for lung disease progression in children with cystic fibrosis
Marieke van Horck1,2, Kim van de Kant1,3, Bjorn Winkens4
1Dept of Paediatric Respiratory Medicine, Care and Public Health Research Institute (CAPHRI), Maastricht University Medical Centre (MUMC+), Maastricht, The Netherlands.
Insights
Proton pump inhibitor use in children with cystic fibrosis (CF) may worsen lung disease. Lower lung function and prior exacerbations also predict future pulmonary events in CF patients.
Area of Science:
- Pediatric Pulmonology
- Cystic Fibrosis Research
- Respiratory Medicine
Background:
- Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs, primarily the lungs.
- Lung disease progression and pulmonary exacerbations are key indicators of morbidity in pediatric CF patients.
- Identifying risk factors for CF lung disease progression is crucial for timely intervention.
Purpose of the Study:
- To identify potential risk factors associated with lung disease progression in children with cystic fibrosis.
- To analyze the relationship between baseline characteristics and future pulmonary exacerbations in pediatric CF patients.
- To investigate the impact of medication use, including proton pump inhibitors (PPIs), on CF lung disease progression.
Main Methods:
- Longitudinal data from 545 children (aged ≥5 years) with CF were analyzed from the Dutch CF Registry (2009-2014).
- Lung disease progression was assessed by decline in lung function (FEV1 % pred) and pulmonary exacerbation rate.
- Potential risk factors including demographics, clinical status, and medication use were evaluated.
Main Results:
- Proton pump inhibitor (PPI) use was significantly associated with both annual decline in FEV1 % pred (p=0.017) and increased future pulmonary exacerbation rates (p=0.006).
- Lower baseline FEV1 % pred (p=0.007), prophylactic inhaled antibiotic use (p=0.006), and prior pulmonary exacerbations (p=0.002) were linked to subsequent exacerbations.
- Several risk factors for future exacerbations were identified in this cohort of Dutch children with CF.
Conclusions:
- The study identified key risk factors for pulmonary exacerbations in children with CF.
- Proton pump inhibitor (PPI) use emerged as a significant factor associated with lung disease progression and exacerbations, warranting further investigation.
- Baseline lung function and history of exacerbations are important predictors of future respiratory events in pediatric CF patients.
Abstract:
To identify potential risk factors for lung disease progression in children with cystic fibrosis (CF), we studied the longitudinal data of all children with CF (aged ≥5 years) registered in the Dutch CF Registry (2009-2014).Lung disease progression was expressed as a decline in lung function (forced expiratory volume in 1 s (FEV1) % pred) and pulmonary exacerbation rate. Potential risk factors at baseline included sex, age, best FEV1 % pred, best forced vital capacity % pred, genotype, body mass index z-score, pancreatic insufficiency, medication use (proton pump inhibitors (PPIs), prophylactic antibiotics and inhaled corticosteroids), CF-related diabetes, allergic bronchopulmonary aspergillosis and colonisation with Pseudomonas aeruginosaThe data of 545 children were analysed. PPI use was associated with both annual decline of FEV1 % pred (p=0.017) and future pulmonary exacerbation rate (p=0.006). Moreover, lower FEV1 % pred at baseline (p=0.007), prophylactic inhaled antibiotic use (p=0.006) and pulmonary exacerbations in the baseline year (p=0.002) were related to pulmonary exacerbations in subsequent years.In a cohort of Dutch children with CF followed for 5 years, we were able to identify several risk factors for future exacerbations. In particular, the association between PPI use and lung disease progression definitely requires further investigation.
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