{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,23]],"date-time":"2026-08-23T15:40:15Z","timestamp":1787499615951,"version":"build-2736575974"},"reference-count":17,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2026,3,11]],"date-time":"2026-03-11T00:00:00Z","timestamp":1773187200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,3,11]],"date-time":"2026-03-11T00:00:00Z","timestamp":1773187200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/501100021548","name":"Drottning Silvias barnsjukhus","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100021548","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Acta Paediatrica"],"published-print":{"date-parts":[[2026,6]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Aim<\/jats:title>\n                    <jats:p>Children with cystic fibrosis (CF) face substantial daily treatment burdens and the effects of transmembrane conductance regulator modulators on these have not been sufficiently described. We evaluated changes in treatment burden after elexacaftor tezacaftor ivacaftor (ETI) was initiated.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>This prospective observational study comprised children aged six to 17\u2009years from three Swedish paediatric CF centres. They were enrolled from 7 December 2022 to 30 June 2023, before ETI was initiated. Their health\u2010related quality of life, treatment burden, and adherence were assessed at baseline and at six and 12\u2009months.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      We studied 62 children who initiated ETI and were followed up to 12\u2009months. The mean standard deviation (SD) treatment burden score improved by 4.9 points (95% confidence interval (CI) \u22120.7 to 10.6,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.087), from 67.6 (\u00b120.6) at baseline to 72.5 (\u00b117.6) at 12\u2009months. The mean daily time spent on inhalation therapy and airway clearance decreased from 91.9 (\u00b137.3) to 70.4 (\u00b123.6) minutes (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001). Mean inhalation time per session fell by 7\u2009min (95% CI \u221210.5 to \u22123.3,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001) and airway clearance time by 4\u2009min (95% CI \u22126.7 to \u22121.5,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.003).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Treatment burden, particularly daily inhalation and airway clearance time, decreased after ETI initiation.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/apa.70498","type":"journal-article","created":{"date-parts":[[2026,3,12]],"date-time":"2026-03-12T05:21:06Z","timestamp":1773292866000},"page":"1283-1289","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Improved Quality of Life in Children With Cystic Fibrosis Who Received Transmembrane Conductance Regulator Modulators"],"prefix":"10.1111","volume":"115","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0194-7365","authenticated-orcid":false,"given":"Marcus","family":"Svedberg","sequence":"first","affiliation":[{"name":"Department of Paediatrics, Institute of Clinical Sciences, Sahlgrenska Academy Gothenburg University  Gothenburg Sweden"},{"name":"Department of Paediatrics Queen Silvia's Children's Hospital  Gothenburg Sweden"},{"name":"Centre for Digital Health, Sahlgrenska University Hospital  Gothenburg Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frida","family":"Lundqvist","sequence":"additional","affiliation":[{"name":"Department of Paediatrics Queen Silvia's Children's Hospital  Gothenburg Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Huda Abdulahi","family":"\u00d6strand","sequence":"additional","affiliation":[{"name":"Department of Paediatrics Sk\u00e5ne University Hospital  Lund Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christina","family":"Krantz","sequence":"additional","affiliation":[{"name":"Department of Paediatrics Uppsala University Hospital  Uppsala Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Per","family":"Ekman","sequence":"additional","affiliation":[{"name":"Statistiska Konsultgruppen Sweden  Gothenburg Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9447-663X","authenticated-orcid":false,"given":"Henrik","family":"Imberg","sequence":"additional","affiliation":[{"name":"Statistiska Konsultgruppen Sweden  Gothenburg Sweden"},{"name":"Department of Molecular and Clinical Medicine Institute of Medicine, Sahlgrenska Academy, University of Gothenburg  Gothenburg Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,3,11]]},"reference":[{"key":"e_1_2_13_2_1","doi-asserted-by":"publisher","DOI":"10.1183\/09059180.00008112"},{"key":"e_1_2_13_3_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra043184"},{"key":"e_1_2_13_4_1","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm\/151.3_Pt_2.S54"},{"key":"e_1_2_13_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2023.12.002"},{"key":"e_1_2_13_6_1","doi-asserted-by":"publisher","DOI":"10.1155\/2023\/1422319"},{"key":"e_1_2_13_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10880-014-9396-1"},{"key":"e_1_2_13_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2024.11.005"},{"key":"e_1_2_13_9_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.202102-0509OC"},{"key":"e_1_2_13_10_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1908639"},{"key":"e_1_2_13_11_1","doi-asserted-by":"publisher","DOI":"10.1097\/00063198-199811000-00003"},{"key":"e_1_2_13_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2006.03.002"},{"key":"e_1_2_13_13_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00080312"},{"key":"e_1_2_13_14_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.2006.tb02290.x"},{"key":"e_1_2_13_15_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00185-2021"},{"key":"e_1_2_13_16_1","doi-asserted-by":"publisher","DOI":"10.1136\/thorax-2023-220100"},{"key":"e_1_2_13_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.25462"},{"key":"e_1_2_13_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2021.05.005"}],"container-title":["Acta Paediatrica"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/apa.70498","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/apa.70498","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/apa.70498","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,16]],"date-time":"2026-05-16T14:52:33Z","timestamp":1778943153000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/apa.70498"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,3,11]]},"references-count":17,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2026,6]]}},"alternative-id":["10.1111\/apa.70498"],"URL":"https:\/\/doi.org\/10.1111\/apa.70498","archive":["Portico"],"relation":{},"ISSN":["0803-5253","1651-2227"],"issn-type":[{"value":"0803-5253","type":"print"},{"value":"1651-2227","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,3,11]]},"assertion":[{"value":"2025-10-21","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-03-03","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-03-11","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}