{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,22]],"date-time":"2026-04-22T10:13:02Z","timestamp":1776852782304,"version":"3.51.2"},"reference-count":33,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2024,11,28]],"date-time":"2024-11-28T00:00:00Z","timestamp":1732752000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2024,11,28]],"date-time":"2024-11-28T00:00:00Z","timestamp":1732752000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2025,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>Cystic fibrosis transmembrane conductance regulator modulator therapies (CFTR\u2010MT) have altered management, reducing exacerbations and slowing pulmonary function decline. Nevertheless, it is still uncertain if the benefits of CFTR\u2010MTs last when they are stopped. This study aimed to assess pulmonary function changes, and exacerbation rates during and after CFTR\u2010MT use in adult cystic fibrosis patients.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Between 2018 and 2022, we conducted a study involving adult CF patients who initially used CFTR\u2010MTs but later discontinued them due to reimbursement issues. The study was divided into three phases: predrug (T1), in\u2010drug (T2), and postdrug (T3). We recorded pulmonary function tests, laboratory and culture results, and the number of exacerbations.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      The study involved 33 patients, with 28 (84.8%) receiving Elexacaftor\/Tezacaftor\/Ivacaftor and 5 (15.2%) receiving Ivacaftor. The median treatment and interruption durations were 3.1 (IQR\u2009=\u20092.9\u20135.7), and 2.5 (IQR\u2009=\u20091.5\u20134.0) months, respectively. The mean FEV1% was 54.3% (\u00b1\u200926.6), 70.4% (\u00b1\u200927.4), and 60.2% (\u00b1\u200926.5) during T1, T2, and T3, respectively (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001). The mean FVC% was 65.5% (\u00b1\u200923.9) in T1, increased to 81.5% (\u00b1\u200924.5) in T2, and decreased to 71.6% (\u00b1\u200925.9) in T3 (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001). The number of\n                      <jats:italic>Psedomonas aeruginosa<\/jats:italic>\n                      , and\n                      <jats:italic>Aspergillus<\/jats:italic>\n                      positive sputum cultures decreased significantly with drug use (T1: 72.7%, 39.4%; T2: 48.5%, 9.1%; T3: 45.5%, 18.2%;\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.014,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.004, respectively). The median number of hospitalizations was 1.0 (0\u20135.0) in T1, 0 (0\u20130) in T2, and 0 (0\u20131.0) in T3.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>This study revealed that CFTR\u2010MTs are effective even in the short term for adult CF patients, but their beneficial effects quickly diminish after discontinuation. Real\u2010life data obtained as a result of discontinuation of drugs due to reimbursement problems has highlighted the significance of regular and uninterrupted use of modulators.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ppul.27416","type":"journal-article","created":{"date-parts":[[2024,11,28]],"date-time":"2024-11-28T09:14:21Z","timestamp":1732785261000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Outcomes of Modulator Therapy Discontinued After Short\u2010Term Use in Adult Cystic Fibrosis"],"prefix":"10.1002","volume":"60","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6071-0851","authenticated-orcid":false,"given":"Oguz","family":"Karcioglu","sequence":"first","affiliation":[{"name":"Department of Chest Diseases, Faculty of Medicine Hacettepe University Ankara Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5750-1223","authenticated-orcid":false,"given":"Aytekin","family":"Idikut","sequence":"additional","affiliation":[{"name":"Department of Chest Diseases, Faculty of Medicine Hacettepe University Ankara Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9206-6856","authenticated-orcid":false,"given":"Ebru","family":"Ozturk","sequence":"additional","affiliation":[{"name":"School of Medicine, Division of Biostatistics, Hacettepe University Ankara Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6250-2100","authenticated-orcid":false,"given":"Ebru","family":"Damadoglu","sequence":"additional","affiliation":[{"name":"Department of Chest Diseases, Division of Allergy and Clinical Immunology, Faculty of Medicine Hacettepe University Ankara Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2024,11,28]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.isci.2024.108835"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2020.04.008"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.21526"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.chest.2020.09.084"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(19)30337-6"},{"key":"e_1_2_10_7_1","unstructured":"Cystic Fibrosis Foundation (CFF) \u201cCFF 2022 Patient Registry Annual Data Report \u201d accessed April 07 2024 https:\/\/wwwcfforg\/about-us\/annual-report."},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.coph.2022.102239"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1097\/MOP.0000000000000892"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2023.11.008"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2020.06.012"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2016.09.064"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1183\/23120541.00204-2022"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(17)30215-1"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(21)00454-9"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1105185"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2022.12.009"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2020.11.008"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.1136\/thx.2011.161117"},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2023.03.002"},{"key":"e_1_2_10_21_1","doi-asserted-by":"publisher","DOI":"10.1136\/thoraxjnl-2017-210394"},{"key":"e_1_2_10_22_1","doi-asserted-by":"publisher","DOI":"10.3390\/nu13092907"},{"key":"e_1_2_10_23_1","doi-asserted-by":"publisher","DOI":"10.1159\/000538606"},{"key":"e_1_2_10_24_1","doi-asserted-by":"publisher","DOI":"10.3390\/nu16121811"},{"key":"e_1_2_10_25_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00408-017-0024-3"},{"key":"e_1_2_10_26_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.26261"},{"key":"e_1_2_10_27_1","doi-asserted-by":"publisher","DOI":"10.1093\/infdis\/jiae051"},{"key":"e_1_2_10_28_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11046-021-00541-5"},{"key":"e_1_2_10_29_1","doi-asserted-by":"publisher","DOI":"10.3390\/ijms23073513"},{"key":"e_1_2_10_30_1","doi-asserted-by":"publisher","DOI":"10.3389\/fcimb.2020.00372"},{"key":"e_1_2_10_31_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2023.09.003"},{"key":"e_1_2_10_32_1","doi-asserted-by":"publisher","DOI":"10.3389\/fphar.2023.1163391"},{"key":"e_1_2_10_33_1","unstructured":"S.Robbins \u201cMiracle Cystic Fibrosis DDrug Kept out of Reach in Developing Countries \u201dNew York Times accessed February 07 2023 https:\/\/www.nytimes.com\/2023\/02\/07\/health\/cystic-fibrosis-drug-trikafta.html?smid=url-share."},{"key":"e_1_2_10_34_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.202307-1200VP"}],"container-title":["Pediatric Pulmonology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.27416","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/ppul.27416","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.27416","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,2,18]],"date-time":"2026-02-18T15:29:11Z","timestamp":1771428551000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ppul.27416"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,11,28]]},"references-count":33,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2025,1]]}},"alternative-id":["10.1002\/ppul.27416"],"URL":"https:\/\/doi.org\/10.1002\/ppul.27416","archive":["Portico"],"relation":{},"ISSN":["8755-6863","1099-0496"],"issn-type":[{"value":"8755-6863","type":"print"},{"value":"1099-0496","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,11,28]]},"assertion":[{"value":"2024-07-11","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-11-12","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-11-28","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e27416"}}