TY - JOUR
T1 - Risk factors for lung disease progression in children with cystic fibrosis
AU - van Horck, Marieke
AU - van de Kant, Kim
AU - Winkens, Bjorn
AU - Wesseling, Geertjan
AU - Gulmans, Vincent
AU - Hendriks, Han
AU - van der Grinten, Chris
AU - Jobsis, Quirijn
AU - Dompeling, Edward
PY - 2018/6/1
Y1 - 2018/6/1
N2 - 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 aeruginosa. The 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.
AB - 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 aeruginosa. The 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.
KW - FORCED EXPIRATORY VOLUME
KW - PULMONARY EXACERBATIONS
KW - YOUNG-CHILDREN
KW - PSEUDOMONAS-AERUGINOSA
KW - DECLINE
KW - ADULTS
KW - FEV1
KW - PREDICTORS
KW - EQUATIONS
KW - CARE
U2 - 10.1183/13993003.02509-2017
DO - 10.1183/13993003.02509-2017
M3 - Article
C2 - 29773689
SN - 0903-1936
VL - 51
JO - European Respiratory Journal
JF - European Respiratory Journal
IS - 6
M1 - 1702509
ER -