{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,5]],"date-time":"2026-07-05T01:58:27Z","timestamp":1783216707768,"version":"3.54.6"},"reference-count":43,"publisher":"European Respiratory Society (ERS)","issue":"3","license":[{"start":{"date-parts":[[2023,2,23]],"date-time":"2023-02-23T00:00:00Z","timestamp":1677110400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["publications.ersnet.org"],"crossmark-restriction":true},"short-container-title":["ERJ Open Res"],"accepted":{"date-parts":[[2023,2,2]]},"published-print":{"date-parts":[[2023,5]]},"abstract":"<jats:sec><jats:title>Aim<\/jats:title><jats:p>To examine the trajectory of forced expiratory volume in 1\u2005s (FEV<jats:sub>1<\/jats:sub>) using data from the European Cystic Fibrosis Society patient registry (ECFPR) collected from 2008 to 2016,<jats:italic>i.e.<\/jats:italic>the era before highly effective modulator therapy (HEMT). We evaluated risk factors for FEV<jats:sub>1<\/jats:sub>decline.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>The study population included patients with a confirmed diagnosis of cystic fibrosis recorded in the ECFPR (2008\u20132016). The evolution of FEV<jats:sub>1<\/jats:sub>% predicted (%FEV<jats:sub>1<\/jats:sub>) with age, and the yearly change in %FEV<jats:sub>1<\/jats:sub>were evaluated. Risk factors considered were cystic fibrosis transmembrane conductance regulator (<jats:italic>\u00adCFTR<\/jats:italic>) mutation class, gender, age at diagnosis, neonatal screening, meconium ileus, sweat chloride concentration at diagnosis and country's income level.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We used 199\u2009604 FEV<jats:sub>1<\/jats:sub>recordings from 38\u2009734 patients. The fastest decline was seen during puberty and in patients diagnosed before the age of 10\u2005years. Males had a higher %FEV<jats:sub>1<\/jats:sub>, but a higher yearly %FEV<jats:sub>1<\/jats:sub>loss between the ages of 15 and 25 years. We showed stabilisation and even improvement in %FEV<jats:sub>1<\/jats:sub>over age in adults with a class III<jats:italic>CFTR<\/jats:italic>mutation, but a steady decline in patients homozygous for F508del or with both mutations of classes I\/II. A faster decline in %FEV<jats:sub>1<\/jats:sub>was found in patients from low-income countries compared to a similar %FEV<jats:sub>1<\/jats:sub>evolution in patients from middle- and high-income countries.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>These longitudinal FEV<jats:sub>1<\/jats:sub>data reflect the reality of cystic fibrosis across Europe in the era pre-HEMT, and can serve as baseline for comparison with the post-HEMT era. The similar evolution in middle- and high-income countries underlines opportunities for low-income countries.<\/jats:p><\/jats:sec>","DOI":"10.1183\/23120541.00449-2022","type":"journal-article","created":{"date-parts":[[2023,2,23]],"date-time":"2023-02-23T17:05:54Z","timestamp":1677171954000},"page":"00449-2022","update-policy":"https:\/\/doi.org\/10.1183\/ers-crossmark-policy","source":"Crossref","is-referenced-by-count":10,"title":["Risk factors for forced expiratory volume in 1 s decline in European patients with cystic fibrosis: data from the European Cystic Fibrosis Society Patient 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