{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T22:52:17Z","timestamp":1790376737098,"version":"4.1.0"},"reference-count":36,"publisher":"European Respiratory Society (ERS)","issue":"6","funder":[{"name":"Dutch Cystic Fibrosis Foundation"}],"content-domain":{"domain":["publications.ersnet.org"],"crossmark-restriction":true},"short-container-title":["Eur Respir J"],"accepted":{"date-parts":[[2018,4,16]]},"published-print":{"date-parts":[[2018,6]]},"abstract":"<jats:p>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 \u22655\u2005years) registered in the Dutch CF Registry (2009\u20132014).<\/jats:p><jats:p>Lung disease progression was expressed as a decline in lung function (forced expiratory volume in 1\u2005s (FEV<jats:sub>1<\/jats:sub>) % pred) and pulmonary exacerbation rate. Potential risk factors at baseline included sex, age, best FEV<jats:sub>1<\/jats:sub>% 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<jats:italic>Pseudomonas aeruginosa<\/jats:italic>.<\/jats:p><jats:p>The data of 545 children were analysed. PPI use was associated with both annual decline of FEV<jats:sub>1<\/jats:sub>% pred (p=0.017) and future pulmonary exacerbation rate (p=0.006). Moreover, lower FEV<jats:sub>1<\/jats:sub>% 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.<\/jats:p><jats:p>In a cohort of Dutch children with CF followed for 5\u2005years, 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.<\/jats:p>","DOI":"10.1183\/13993003.02509-2017","type":"journal-article","created":{"date-parts":[[2018,5,17]],"date-time":"2018-05-17T15:10:18Z","timestamp":1526569818000},"page":"1702509","update-policy":"https:\/\/doi.org\/10.1183\/ers-crossmark-policy","source":"Crossref","is-referenced-by-count":22,"title":["Risk factors for lung disease progression in children with cystic fibrosis"],"prefix":"10.1183","volume":"51","author":[{"given":"Marieke","family":"van Horck","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kim","family":"van de Kant","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6747-6228","authenticated-orcid":false,"given":"Bjorn","family":"Winkens","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Geertjan","family":"Wesseling","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincent","family":"Gulmans","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Han","family":"Hendriks","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chris","family":"van der Grinten","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Quirijn","family":"J\u00f6bsis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edward","family":"Dompeling","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"81","published-online":{"date-parts":[[2018,5,17]]},"reference":[{"key":"2024102100272004000_51.6.1702509.1","doi-asserted-by":"crossref","first-page":"533","DOI":"10.1378\/chest.14-1997","article-title":"The evolution of cystic fibrosis care","volume":"148","author":"Pittman","year":"2015","journal-title":"Chest"},{"key":"2024102100272004000_51.6.1702509.2","doi-asserted-by":"crossref","first-page":"148","DOI":"10.1016\/S2213-2600(13)70026-2","article-title":"Early lung disease in cystic fibrosis","volume":"1","author":"Grasemann","year":"2013","journal-title":"Lancet Respir Med"},{"key":"2024102100272004000_51.6.1702509.3","doi-asserted-by":"crossref","first-page":"116","DOI":"10.1016\/j.jpeds.2015.08.042","article-title":"Forced expiratory volume in 1 second variability helps identify patients with cystic fibrosis at risk of greater loss of lung function","volume":"169","author":"Morgan","year":"2016","journal-title":"J Pediatr"},{"key":"2024102100272004000_51.6.1702509.4","doi-asserted-by":"publisher","DOI":"10.1136\/thoraxjnl-2013-203262"},{"key":"2024102100272004000_51.6.1702509.5","doi-asserted-by":"crossref","first-page":"S23","DOI":"10.1016\/j.jcf.2014.03.010","article-title":"European Cystic Fibrosis Society Standards of Care: Best Practice guidelines","volume":"13","author":"Smyth","year":"2014","journal-title":"J\u00a0Cyst Fibros"},{"key":"2024102100272004000_51.6.1702509.6","doi-asserted-by":"crossref","first-page":"763","DOI":"10.1016\/j.jcf.2015.02.007","article-title":"Treatment and demographic factors affecting time to next pulmonary exacerbation in cystic fibrosis","volume":"14","author":"VanDevanter","year":"2015","journal-title":"J Cyst Fibros"},{"key":"2024102100272004000_51.6.1702509.7","doi-asserted-by":"publisher","DOI":"10.1136\/thx.2006.061366"},{"key":"2024102100272004000_51.6.1702509.8","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00166412"},{"key":"2024102100272004000_51.6.1702509.9","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.23217"},{"key":"2024102100272004000_51.6.1702509.10","doi-asserted-by":"crossref","first-page":"e0128004","DOI":"10.1371\/journal.pone.0128004","article-title":"Risk of community-acquired pneumonia with outpatient proton-pump inhibitor therapy: a systematic review and meta-analysis","volume":"10","author":"Lambert","year":"2015","journal-title":"PLoS One"},{"key":"2024102100272004000_51.6.1702509.11","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2005-1655"},{"key":"2024102100272004000_51.6.1702509.12","unstructured":"Dutch Cystic Fibrosis Foundation. 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