Journal of Cystic Fibrosis最新文献

{"title":"19 Microbial and metabolic ecology of expectorated sputum from people with cystic fibrosis from the STOP2 study","authors":"K. Whiteson, S. Dunham, J. Rothman, L. Carmody, L. Kalikan, L. Caverly, J. LiPuma","doi":"10.1016/S1569-1993(24)00862-2","DOIUrl":"10.1016/S1569-1993(24)00862-2","url":null,"abstract":"","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":"23 ","pages":"Page S10"},"PeriodicalIF":5.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142136103","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"52 Specific Pseudomonas aeruginosa IgG levels before and after elexacaftor/tezacaftor/ivacaftor treatment","authors":"T. Bryrup, D. Faurholt-Jepsen, M. Skov, T. Katzenstein, I. Mathiesen, J. Helweg-Larsen, H. Johansen, T. Qvist, T. Pressler","doi":"10.1016/S1569-1993(24)00895-6","DOIUrl":"10.1016/S1569-1993(24)00895-6","url":null,"abstract":"","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":"23 ","pages":"Pages S28-S29"},"PeriodicalIF":5.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142136416","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"59 Pseudomonas aeruginosa apr-type 1 secretion system is a modulator of the Toll-like receptor 5-mediated epithelial inflammatory response","authors":"M. Gaudet, R. Lévesque, D. Nguyen, S. Rousseau","doi":"10.1016/S1569-1993(24)00902-0","DOIUrl":"10.1016/S1569-1993(24)00902-0","url":null,"abstract":"","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":"23 ","pages":"Pages S32-S33"},"PeriodicalIF":5.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142136533","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Publication Information","authors":"","doi":"10.1016/S1569-1993(24)00841-5","DOIUrl":"10.1016/S1569-1993(24)00841-5","url":null,"abstract":"","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":"23 ","pages":"Page ii"},"PeriodicalIF":5.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142136759","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"66 Optimization of human DNA depletion and microbe-enrichment techniques from oropharyngeal swabs: Preparing for shotgun metagenomic analysis in the PROMISE-peds and BEGIN observational studies","authors":"D. Bouzek, C. Pope, A. Dutta, C. Miller, D. Wolter, H. Hayden, L. Hoffman","doi":"10.1016/S1569-1993(24)00909-3","DOIUrl":"10.1016/S1569-1993(24)00909-3","url":null,"abstract":"","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":"23 ","pages":"Pages S35-S36"},"PeriodicalIF":5.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142136946","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"E-learning within the European cystic fibrosis society - A multidisciplinary cross-sectional survey","authors":"","doi":"10.1016/j.jcf.2024.07.003","DOIUrl":"10.1016/j.jcf.2024.07.003","url":null,"abstract":"<div><h3>Background</h3><p>Continuing professional development (CPD) is a component of practice that spans all disciplines within cystic fibrosis (CF). E-learning resources theoretically represent flexible, low cost and time efficient methods of CPD. We aimed to explore European Cystic Fibrosis Society (ECFS) members' and CF health professional communities’ current views, experiences and perceptions of e-learning and the ECFS education platform (ECFS-EP).</p></div><div><h3>Methods</h3><p>An online cross-sectional survey was developed by the ECFS Education Committee and circulated via the ECFS conference, emails and within the society subgroups between June and September 2023.</p></div><div><h3>Results</h3><p>547 responses were received from 58 countries; 57 % of responders were ECFS members. A wide range of specialities were represented from the multidisciplinary team including 36 % clinicians. The majority of respondents (63 %) spent 6 or more hours a week on their professional education. Online platforms were used either weekly (34 %) or monthly (37 %); 54 % of respondents had used the ECFS-EP and this was rated favourably overall, specifically for content quality. Preferred formats for education were articles and medium length (15–30 min) webinars.</p></div><div><h3>Conclusions</h3><p>This multidisciplinary cohort survey illustrates contemporary practice and opinion relating to e-learning and the ECFS-EP. Strengths include the high number of responses and the wide range of countries and specialities represented. Results suggest the ECFS-EP is valued and highlights priority topics, preferred formats and opportunities to optimise awareness. Results support continued provision and oversight of high quality education via an online platform. Continued success will rely on learning from user experience and feedback to inform future practice.</p></div>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":"23 5","pages":"Pages 1020-1023"},"PeriodicalIF":5.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141599886","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Health care resource utilization preceding death or lung transplantation in people with cystic fibrosis","authors":"","doi":"10.1016/j.jcf.2024.03.001","DOIUrl":"10.1016/j.jcf.2024.03.001","url":null,"abstract":"<div><h3>Background</h3><p>We studied the health care resource utilization (HCRU) and associated costs in the year preceding LT in pwCF or death without LT, and we estimated the overall cost of LT.</p></div><div><h3>Methods</h3><p>We performed a linkage between 2006 and 2017 data from the French CF Registry (FCFR) and the French health claims database (Système National des Données de Santé; SNDS). The HCRU and associated costs were described the year before LT or before death without LT, and two years after LT.</p></div><div><h3>Results</h3><p>Among the 7,671 patients included in the FCFR, 6,187 patients (80.7 %) were successfully matched to patients in the SNDS (males (m): 51.9 %, mean±SD age at the end of follow-up: 24.6 ± 13.6). Overall, 166 patients died without LT (m: 47.6 %, age at death: 30.4 ± 14.5) and 767 patients with primary LT (m: 48.2 %, age at transplantation: 28.0 ± 9.1) were identified. HCRU was lower among patients who died without receiving LT, with marked differences in the cost of hospital stays. The mean total cost per patient was €66,759 ± 38,249 in the year before death, €149,374 ± 62,678 in the year preceding LT, €63,919 ± 35,399 in the first year following LT, and €42,813 ± 39,967 in the second year of follow-up.</p></div><div><h3>Conclusion</h3><p>Our results indicate that HCRU was two times lower in the year before death in non-transplant pwCF than in the year before LT, which may reflect inappropriate care of CF in patients who died without receiving LT. It also shows the cost associated with LT.</p></div>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":"23 5","pages":"Pages 903-909"},"PeriodicalIF":5.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140119579","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Quantifying abnormal alveolar microstructure in cystic fibrosis lung disease via hyperpolarized 129Xe diffusion MRI","authors":"","doi":"10.1016/j.jcf.2024.07.002","DOIUrl":"10.1016/j.jcf.2024.07.002","url":null,"abstract":"<div><h3>Rationale</h3><p>Cystic Fibrosis (CF) progresses through recurrent infection and inflammation, causing permanent lung function loss and airway remodeling. CT scans reveal abnormally low-density lung parenchyma in CF, but its microstructural nature remains insufficiently explored due to clinical CT limitations. To this end, diffusion-weighted ¹²⁹Xe MRI is a non-invasive and validated measure of lung microstructure. In this work, we investigate microstructural changes in people with CF (pwCF) relative to age-matched, healthy subjects using comprehensive imaging and analysis involving pulmonary-function tests (PFTs), and ¹²⁹Xe MRI.</p></div><div><h3>Methods</h3><p>38 healthy subjects (age 6–40; 17.2 ± 9.5 years) and 39 pwCF (age 6–40; 15.6 ± 8.0 years) underwent ¹²⁹<span>Xe-diffusion MRI and PFTs. The distribution of diffusion measurements (i.e., apparent diffusion coefficients (ADC) and morphometric parameters) was assessed via linear binning (LB). The resulting volume percentages of bins were compared between controls and pwCF. Mean ADC and morphometric parameters were also correlated with PFTs.</span></p></div><div><h3>Results</h3><p>Mean whole-lung ADC correlated significantly with age (<em>P</em> &lt; 0.001) for both controls and CF, and with PFTs (<em>P</em> &lt; 0.05) specifically for pwCF. Although there was no significant difference in mean ADC between controls and pwCF (<em>P</em> = 0.334), age-adjusted LB indicated significant voxel-level diffusion (i.e., ADC and morphometric parameters) differences in pwCF compared to controls (<em>P</em> &lt; 0.05).</p></div><div><h3>Conclusions</h3><p>¹²⁹Xe diffusion MRI revealed microstructural abnormalities in CF lung disease. Smaller microstructural size may reflect compression from overall higher lung density due to interstitial inflammation, fibrosis, or other pathological changes. While elevated microstructural size may indicate emphysema-like remodeling due to chronic inflammation and infection.</p></div>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":"23 5","pages":"Pages 926-935"},"PeriodicalIF":5.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141599888","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Impact of COVID-19 infection on lung function and nutritional status amongst individuals with cystic fibrosis: A global cohort study","authors":"Julie Semenchuk ,&nbsp;Yumi Naito ,&nbsp;Susan C. Charman ,&nbsp;Siobhán B Carr ,&nbsp;Stephanie Y. Cheng ,&nbsp;Bruce C. Marshall ,&nbsp;Albert Faro ,&nbsp;Alexander Elbert ,&nbsp;Hector H. Gutierrez ,&nbsp;Christopher H. Goss ,&nbsp;Bulent Karadag ,&nbsp;Pierre-Régis Burgel ,&nbsp;Carla Colombo ,&nbsp;Marco Salvatore ,&nbsp;Rita Padoan ,&nbsp;Géraldine Daneau ,&nbsp;Satenik Harutyunyan ,&nbsp;Nataliya Kashirskaya ,&nbsp;Laura Kirwan ,&nbsp;Peter G Middleton ,&nbsp;Rebecca Cosgriff","doi":"10.1016/j.jcf.2024.07.019","DOIUrl":"10.1016/j.jcf.2024.07.019","url":null,"abstract":"<div><h3>Background</h3><p>Factors associated with severe COVID-19 infection have been identified; however, the impact of infection on longer-term outcomes is unclear. The objective of this study was to examine the impact of COVID-19 infection on the trajectory of lung function and nutritional status in people with cystic fibrosis (pwCF).</p></div><div><h3>Methods</h3><p>This is a retrospective global cohort study of pwCF who had confirmed COVID-19 infection diagnosed between January 1, 2020 and December 31, 2021. Forced expiratory volume in one second percent predicted (ppFEV₁) and body mass index (BMI) twelve months prior to and following a diagnosis of COVID-19 were recorded. Change in mean ppFEV₁ and BMI were compared using a <em>t</em>-test. A linear mixed-effects model was used to estimate change over time and to compare the rate of change before and after infection.</p></div><div><h3>Results</h3><p>A total of 6,500 cases of COVID-19 in pwCF from 33 countries were included for analysis. The mean difference in ppFEV₁ pre- and post-infection was 1.4 %, (95 % CI 1.1, 1.7). In those not on modulators, the difference in rate of change pre- and post-infection was 1.34 %, (95 % CI -0.88, 3.56) per year (<em>p</em> = 0.24) and -0.74 % (-1.89, 0.41) per year (<em>p</em> = 0.21) for those on elexacaftor/tezacaftor/ivacaftor. No clinically significant change was noted in BMI or BMI percentile before and after COVID-19 infection.</p></div><div><h3>Conclusions</h3><p>No clinically meaningful impact on lung function and BMI trajectory in the year following infection with COVID-19 was identified. This work highlights the ability of the global CF community to unify and address critical issues facing pwCF.</p></div>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":"23 5","pages":"Pages 815-822"},"PeriodicalIF":5.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S156919932400804X/pdfft?md5=31a1e8960a5b2129cfb928307604f1eb&pid=1-s2.0-S156919932400804X-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142080465","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"35 The role of the type 3 secretion system on long-term intracellular survival and replication of Pseudomonas aeruginosa in airway epithelial cells","authors":"S. Jusab,&nbsp;L. Hennemann,&nbsp;K. Malet,&nbsp;H. Gnanagobal,&nbsp;D. Nguyen","doi":"10.1016/S1569-1993(24)00878-6","DOIUrl":"10.1016/S1569-1993(24)00878-6","url":null,"abstract":"","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":"23 ","pages":"Pages S18-S19"},"PeriodicalIF":5.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142135827","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}