{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,6]],"date-time":"2026-04-06T09:55:41Z","timestamp":1775469341919,"version":"3.50.1"},"reference-count":37,"publisher":"SAGE Publications","issue":"3","license":[{"start":{"date-parts":[[2020,9,8]],"date-time":"2020-09-08T00:00:00Z","timestamp":1599523200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Respir Care"],"published-print":{"date-parts":[[2021,3]]},"abstract":"<jats:sec>\n                    <jats:title>BACKGROUND:<\/jats:title>\n                    <jats:p>Noninvasive ventilation (NIV) is routinely used to treat patients with cystic fibrosis and respiratory failure. However, evidence on its use is limited, with no data on its role in disease progression and outcomes. The aim of this study was to assess the indications of NIV use and to describe the outcomes associated with NIV in adults with cystic fibrosis in a large adult tertiary center.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>METHODS:<\/jats:title>\n                    <jats:p>A retrospective analysis of data captured prospectively on the unit electronic patient records was performed. All patients with cystic fibrosis who received NIV over a 10-y period were included in the study. A priori, 2 groups were identified based on length of follow-up, with 2 subgroups identified based on duration of NIV treatment.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>RESULTS:<\/jats:title>\n                    <jats:p>\n                      NIV was initiated on 64 occasions. The duration of follow-up was categorized as &gt; 6 months or &lt; 6 months in 31 (48.4%) and 33 (51.6%) occasions, respectively. The most common indications for starting NIV were chronic (48.5%) and acute (32.8%) hypercapnic respiratory failure. Among those with a follow-up &gt; 6 months, subjects who stopped using NIV early showed a steady median (interquartile range) decline in FEV\n                      <jats:sub>1<\/jats:sub>\n                      (pre-NIV: \u20130.04 [\u20130.35 to 0.03] L\/y vs post-NIV: \u20130.07 [\u20130.35 to 0.01] L\/y,\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      = .51), while among those who continued using it had an improvement in the rate of decline (pre-NIV: \u20130.25 [\u20130.52 to \u20130.02] L\/y vs post-NIV: \u20130.07 [\u20130.13 to 0.16] L\/y,\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      = .006). No differences in intravenous antibiotic requirement or pulmonary exacerbations were noted with the use of NIV. Pneumothorax and massive hemoptysis occurred independently in 4 cases.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>CONCLUSIONS:<\/jats:title>\n                    <jats:p>NIV is being used in cystic fibrosis as adjunct therapy for the management of advanced lung disease in a similar fashion to other chronic respiratory conditions. Adherence to NIV treatment can stabilize lung function but does not reduce pulmonary exacerbations or intravenous antibiotic requirement.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.4187\/respcare.07862","type":"journal-article","created":{"date-parts":[[2020,9,8]],"date-time":"2020-09-08T20:00:20Z","timestamp":1599595220000},"page":"466-474","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":7,"title":["Noninvasive Ventilation in Cystic Fibrosis: Clinical Indications and Outcomes in a Large UK Adult Cystic Fibrosis Center"],"prefix":"10.1177","volume":"66","author":[{"given":"Giulia","family":"Spoletini","sequence":"first","affiliation":[{"name":"The Leeds Regional Adult Cystic Fibrosis Centre, St James\u2019s University Hospital, Leeds Teaching Hospital NHS Trust, Leeds, UK."},{"name":"Leeds Institute of Medical Research, University of Leeds, Leeds, UK."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kim","family":"Pollard","sequence":"additional","affiliation":[{"name":"The Leeds Regional Adult Cystic Fibrosis Centre, St James\u2019s University Hospital, Leeds Teaching Hospital NHS Trust, Leeds, UK."},{"name":"Department of Physiotherapy, Leeds Teaching Hospital NHS Trust, Leeds, UK."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ruth","family":"Watson","sequence":"additional","affiliation":[{"name":"The Leeds Regional Adult Cystic Fibrosis Centre, St James\u2019s University Hospital, Leeds Teaching Hospital NHS Trust, Leeds, UK."},{"name":"Department of Physiotherapy, Leeds Teaching Hospital NHS Trust, Leeds, UK."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael J","family":"Darby","sequence":"additional","affiliation":[{"name":"Department of Radiology, Leeds Teaching Hospital NHS Trust, Leeds, UK."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Annette","family":"Johnstone","sequence":"additional","affiliation":[{"name":"Department of Radiology, Leeds Teaching Hospital NHS Trust, Leeds, UK."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christine","family":"Etherington","sequence":"additional","affiliation":[{"name":"The Leeds Regional Adult Cystic Fibrosis Centre, St James\u2019s University Hospital, Leeds Teaching Hospital NHS Trust, Leeds, UK."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paul","family":"Whitaker","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine, Bradford Royal Infirmary, Bradford, UK."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ian J","family":"Clifton","sequence":"additional","affiliation":[{"name":"The Leeds Regional Adult Cystic Fibrosis Centre, St James\u2019s University Hospital, Leeds Teaching Hospital NHS Trust, Leeds, UK."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Daniel G","family":"Peckham","sequence":"additional","affiliation":[{"name":"The Leeds Regional Adult Cystic Fibrosis Centre, St James\u2019s University Hospital, Leeds Teaching Hospital NHS Trust, Leeds, UK."},{"name":"Leeds Institute of Medical Research, University of Leeds, Leeds, UK."}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","published-online":{"date-parts":[[2020,9,8]]},"reference":[{"key":"e_1_3_2_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(16)00576-6"},{"key":"e_1_3_2_3_2","doi-asserted-by":"publisher","DOI":"10.4187\/respcare.09540618"},{"key":"e_1_3_2_4_2","unstructured":"Cystic Fibrosis Foundation. Patient Registry Annual Data Report 2017. 2018. Available at: https:\/\/www.cff.org\/research\/researcher-resources\/patient-registry\/2017-patient-registry-annual-data-report.pdf. Accessed August 14 2020."},{"key":"e_1_3_2_5_2","doi-asserted-by":"publisher","DOI":"10.1183\/09059180.00008112"},{"key":"e_1_3_2_6_2","doi-asserted-by":"publisher","DOI":"10.1055\/s-0035-1547347"},{"key":"e_1_3_2_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2019.03.002"},{"issue":"2","key":"e_1_3_2_8_2","first-page":"CD002769","article-title":"Non\u2010invasive ventilation for cystic fibrosis","volume":"2","author":"Moran F","year":"2017","unstructured":"Moran F, , Bradley JM, , Piper AJ. Non\u2010invasive ventilation for cystic fibrosis. 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