{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T14:53:28Z","timestamp":1786978408000,"version":"build-2736575974"},"reference-count":34,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2023,6,6]],"date-time":"2023-06-06T00:00:00Z","timestamp":1686009600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2023,9]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Cystic fibrosis (CF) is an autosomal recessive disorder caused by CF\u00a0transmembrane conductance regulator (CFTR) genetic variants. CFTR modulators improve pulmonary function and reduce respiratory infections in CF. This study investigated the clinical and laboratory follow\u2010up parameters over 1\u00a0year in patients with CF who could not receive this treatment.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>This retrospective cohort study included 2018 and 2019 CF patient data from the CF registry of Turkey. Demographic and clinical characteristics of 294 patients were assessed, who had modulator treatment indications in 2018 but could not reach the treatment.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      In 2019, patients younger than 18 years had significantly lower BMI\n                      <jats:italic>z<\/jats:italic>\n                      \u2010scores than in 2018. During the 1\u2010year follow\u2010up, forced expiratory volumes\u00a0(FEV1) and FEV1\n                      <jats:italic>z<\/jats:italic>\n                      \u2010scores a trend toward a decrease. In 2019, chronic\n                      <jats:italic>Staphylococcus aureus<\/jats:italic>\n                      colonization, inhaled antipseudomonal antibiotic use for more than 3 months, oral nutritional supplement requirements, and oxygen support need increased.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Patients who had indications for modulator treatments but were unable to obtain them worsened even after a year of follow\u2010up. This study emphasized the importance of using modulator treatments for patients with CF in our country, as well as in many countries worldwide.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ppul.26535","type":"journal-article","created":{"date-parts":[[2023,6,7]],"date-time":"2023-06-07T22:12:23Z","timestamp":1686175943000},"page":"2505-2512","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Patients with cystic fibrosis who could not receive the CFTR modulator treatment: What did they lose in 1\u00a0year?"],"prefix":"10.1002","volume":"58","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7301-3692","authenticated-orcid":false,"given":"Salih","family":"Uytun","sequence":"first","affiliation":[{"name":"Division of Pediatric Pulmonology Ankara City Hospital Ankara 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