{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,23]],"date-time":"2026-08-23T14:30:05Z","timestamp":1787495405184,"version":"build-2736575974"},"reference-count":49,"publisher":"SAGE Publications","license":[{"start":{"date-parts":[[2024,1,1]],"date-time":"2024-01-01T00:00:00Z","timestamp":1704067200000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100003793","name":"Hj\u00e4rt-Lungfonden","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003793","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100009235","name":"Stiftelsen Riksf\u00f6rbundet Cystisk Fibros Forskningsfond","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100009235","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Ther Adv Respir Dis"],"published-print":{"date-parts":[[2024,1]]},"abstract":"<jats:sec>\n                    <jats:title>Background:<\/jats:title>\n                    <jats:p>A significant decline in pulmonary exacerbation rates has been reported in CF patients homozygous for F508del treated with lumacaftor\/ivacaftor. However, it is still unclear whether this reduction reflects a diminished microbiological burden.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objectives:<\/jats:title>\n                    <jats:p>The aim of this study was to determine the impact of lumacaftor\/ivacaftor on the bacterial and fungal burden.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Design:<\/jats:title>\n                    <jats:p>The study is a prospective multicenter cohort study including 132 CF patients homozygous for F508del treated with lumacaftor\/ivacaftor.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods:<\/jats:title>\n                    <jats:p>Clinical parameters as well as bacterial and fungal outcomes 1\u2009year after initiation of lumacaftor\/ivacaftor were compared to data from 2\u2009years prior to initiation of the treatment. Changes in the slope of the outcomes before and after the onset of treatment were assessed.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>Lung function measured as ppFEV1 ( p\u2009&lt;\u20090.001), body mass index (BMI) in adults ( p\u2009&lt;\u20090.001), and BMI z-score in children ( p\u2009=\u20090.007) were improved after initiation of lumacaftor\/ivacaftor. In addition, the slope of the prevalence of Streptococcus pneumoniae ( p\u2009=\u20090.007) and Stenotrophomonas maltophilia ( p\u2009&lt;\u20090.001) shifted from positive to negative, that is, became less prevalent, 1\u2009year after treatment, while the slope for Candida albicans ( p\u2009=\u20090.009), Penicillium spp ( p\u2009=\u20090.026), and Scedosporium apiospermum ( p\u2009&lt;\u20090.001) shifted from negative to positive.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion:<\/jats:title>\n                    <jats:p>The current study showed a significant improvement in clinical parameters and a reduction of some of CF respiratory microorganisms 1\u2009year after starting with lumacaftor\/ivacaftor. However, no significant changes were observed for Pseudomonas aeruginosa, Staphylococcus aureus, or Aspergillus fumigatus, key pathogens in the CF context.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1177\/17534666241254090","type":"journal-article","created":{"date-parts":[[2024,5,23]],"date-time":"2024-05-23T08:41:45Z","timestamp":1716453705000},"update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":8,"title":["Impact of lumacaftor\/ivacaftor on the bacterial and fungal respiratory pathogens in cystic fibrosis: a prospective multicenter cohort study in Sweden"],"prefix":"10.1177","volume":"18","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1443-6369","authenticated-orcid":false,"given":"Mahasin","family":"Al Shakirchi","sequence":"first","affiliation":[{"name":"Stockholm CF Centre, Karolinska University Hospital Huddinge, Stockholm, Department of Clinical Science, Intervention and Technology, 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