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Blood remembers the lung: epigenetic signatures of severe bronchiectasis. 血液记得肺:严重支气管扩张的表观遗传特征。
IF 4.4 3区 医学
ERJ Open Research Pub Date : 2026-10-05 eCollection Date: 2026-09-01 DOI: 10.1183/23120541.01085-2026
Raphael Ewen, Felix C Ringshausen, Pieter C Goeminne
{"title":"Blood remembers the lung: epigenetic signatures of severe bronchiectasis.","authors":"Raphael Ewen, Felix C Ringshausen, Pieter C Goeminne","doi":"10.1183/23120541.01085-2026","DOIUrl":"https://doi.org/10.1183/23120541.01085-2026","url":null,"abstract":"<p><p>This editorial discusses the article on DNA methylation in bronchiectasis by Perea<i>et al</i>. showing that severe bronchiectasis is associated with a distinct whole-blood DNA methylation signature, and discusses the importance of the findings https://bit.ly/3SGjvSw.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"12 5","pages":""},"PeriodicalIF":4.4,"publicationDate":"2026-10-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13662812/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149192006","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Ever-smokers in Norway and lung cancer: median time from self-reported smoking debut to lung cancer diagnosis in 11 prospective population studies (CONOR). 挪威吸烟者与肺癌:11项前瞻性人群研究(CONOR)中,从自我报告首次吸烟到肺癌诊断的中位时间。
IF 4.4 3区 医学
ERJ Open Research Pub Date : 2026-10-05 eCollection Date: 2026-09-01 DOI: 10.1183/23120541.00351-2026
Olav Toai Duc Nguyen, Ioannis Fotopoulos, Ioannis Tsamardinos, Vincenzo Lagani, Oluf Dimitri Røe
{"title":"Ever-smokers in Norway and lung cancer: median time from self-reported smoking debut to lung cancer diagnosis in 11 prospective population studies (CONOR).","authors":"Olav Toai Duc Nguyen, Ioannis Fotopoulos, Ioannis Tsamardinos, Vincenzo Lagani, Oluf Dimitri Røe","doi":"10.1183/23120541.00351-2026","DOIUrl":"https://doi.org/10.1183/23120541.00351-2026","url":null,"abstract":"<p><strong>Background: </strong>Tobacco smoking patterns and lung cancer incidence are closely related, but with years of lag. Most smokers start smoking as teenagers, and lung cancer is diagnosed at a median age of 70 years. However, the median lag time between smoking consumption and lung cancer incidence and smoking-related mortality is commonly reported to be up to 40 years. Here we investigate the median lag time from smoking debut to lung cancer diagnosis in Norway.</p><p><strong>Methods: </strong>We used the Cohort of Norway (CONOR), consisting of linked data from 11 Norwegian prospective population-based cohorts (n=180 534). The study included 80 761 ever-smokers, where 1336 developed lung cancer. Median follow-up time from inclusion in the CONOR study was 11.9 years (0.0-20.8 years) and follow-up from self-reported smoking debut was 44.2 years (2.7-85.4 years). The time from smoking initiation to lung cancer diagnosis was calculated for all cases.</p><p><strong>Results: </strong>The median age of lung cancer diagnosis is 72.5 years. The mean and median time from smoking debut to lung cancer diagnosis are 51.9 (30.2-69.1) and 53.3 years, respectively. The median time from smoking debut until lung cancer diagnosis is >40 years (p<0.001). There is a shorter median lag time in women compared to men and in current smokers compared to former smokers, 48.6 <i>versus</i> 55.5 years and 50.7 <i>versus</i> 54.2 years, respectively (p<0.001).</p><p><strong>Conclusion: </strong>The latency between smoking debut and lung cancer diagnosis in Norwegian ever-smokers is much longer than what has previously been reported. Women and current smokers have a shorter time to diagnosis than men and former smokers, respectively. This is important knowledge in the planning of future healthcare, lung cancer screening, and prevention measures.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"12 5","pages":""},"PeriodicalIF":4.4,"publicationDate":"2026-10-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13636103/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149105528","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Sputum cathepsin G activity predicts exacerbations and hospitalisations in bronchiectasis. 痰组织蛋白酶G活性预测支气管扩张的恶化和住院。
IF 4.4 3区 医学
ERJ Open Research Pub Date : 2026-09-28 eCollection Date: 2026-09-01 DOI: 10.1183/23120541.01667-2025
Mattia Nigro, Andrea Gramegna, Emma Johnson, Lidia Perea, Martina Contarini, Sofia Misuraca, Morven Shuttleworth, Amelia Shoemark, Belen Solarat, Rosa Faner, Edoardo Simonetta, Paola Scarano, Oriol Sibila, Francesco Blasi, James D Chalmers, Stefano Aliberti
{"title":"Sputum cathepsin G activity predicts exacerbations and hospitalisations in bronchiectasis.","authors":"Mattia Nigro, Andrea Gramegna, Emma Johnson, Lidia Perea, Martina Contarini, Sofia Misuraca, Morven Shuttleworth, Amelia Shoemark, Belen Solarat, Rosa Faner, Edoardo Simonetta, Paola Scarano, Oriol Sibila, Francesco Blasi, James D Chalmers, Stefano Aliberti","doi":"10.1183/23120541.01667-2025","DOIUrl":"10.1183/23120541.01667-2025","url":null,"abstract":"<p><strong>Background: </strong>Bronchiectasis is a neutrophil-driven inflammatory chronic respiratory disease. While neutrophil elastase has been deeply investigated, other neutrophil serine proteases are less studied. The association between airway cathepsin G and clinical outcomes in bronchiectasis has not been described. Is cathepsin G sputum activity associated with clinical outcomes in bronchiectasis?</p><p><strong>Methods: </strong>This international, multicentre, prospective cohort study enrolled bronchiectasis patients from three bronchiectasis programmes in Milan (Italy), Dundee (UK) and Barcelona (Spain). Participants were stratified into three groups based on sputum cathepsin G activity measured using immunoassays. Data on clinical features, sputum characteristics, exacerbations, hospitalisations and mortality were collected over a 3-year follow-up period.</p><p><strong>Results: </strong>The median (interquartile range) activity of cathepsin G in sputum was 45.1 ng·mL<sup>-1</sup> (1.3-128.8 ng·mL<sup>-1</sup>). Patients with higher sputum cathepsin G activity demonstrated significantly worse clinical outcomes in terms of disease severity (measured by the Bronchiectasis Severity Index, E-FACED, the modified Reiff score, % predicted forced expiratory volume in 1 s and the prevalence of chronic bacterial infections, particularly <i>Pseudomonas aeruginosa</i>) and disease activity (daily produced sputum volume and quality of life scores). Additionally, high cathepsin G activity was linked to more frequent exacerbations during the 3-year follow-up (incidence rate ratio 1.62, 95% CI 1.29-2.07) and a greater risk of hospitalisation due to severe exacerbations (incidence rate ratio 2.32, 95% CI 1.03-5.21). A strong correlation was found between sputum cathepsin G and neutrophil elastase activities.</p><p><strong>Conclusion: </strong>Sputum cathepsin G activity relates to disease severity, disease activity and prognosis in bronchiectasis. High cathepsin G levels are associated with more severe clinical outcomes and could serve as a target for future therapeutic interventions, including dipeptidyl peptidase 1 inhibitors.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"12 5","pages":""},"PeriodicalIF":4.4,"publicationDate":"2026-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13617600/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149073804","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Is full inhalation during spirometry inhibited by COPD pathophysiology? 肺活量测定时的完全吸入是否受到COPD病理生理的抑制?
IF 4.4 3区 医学
ERJ Open Research Pub Date : 2026-09-28 eCollection Date: 2026-09-01 DOI: 10.1183/23120541.00256-2026
James Dean, Anthony Dean, Augusta Beech, Dave Singh
{"title":"Is full inhalation during spirometry inhibited by COPD pathophysiology?","authors":"James Dean, Anthony Dean, Augusta Beech, Dave Singh","doi":"10.1183/23120541.00256-2026","DOIUrl":"10.1183/23120541.00256-2026","url":null,"abstract":"<p><p><b>The FIVC-FVC value is not entirely dependent on technique in COPD patients, and may be influenced by muscle weakness and certain lung function characteristics</b> https://bit.ly/3RLFHtE.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"12 5","pages":""},"PeriodicalIF":4.4,"publicationDate":"2026-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13617602/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149073806","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Improving paediatric asthma outcomes in rural settings: clinical effectiveness of a multicomponent asthma care model. 改善农村儿童哮喘结局:多成分哮喘护理模式的临床效果。
IF 4.4 3区 医学
ERJ Open Research Pub Date : 2026-09-28 eCollection Date: 2026-09-01 DOI: 10.1183/23120541.00389-2026
Ryan Mackle, Melinda Gray, Mei Chan, Nicole Campbell, Trudie Campbell, Dominque Howard, Lisa Williams, Tracey Tyler, Prue McNamara, Nan Hu, Carmen Crespo-Gonzalez, Michael Hodgins, Blake Angell, Louisa Owens, Jeffery Fletcher, Timothy McCrossin, Susie Piper, Hong Du, Stuart Haggie, Aunty Kerrie Doyle, Bronwyn Gould, Sue Woolfenden, Flic Ward, Anthony Flynn, Raghu Lingam, Adam Jaffe, Nusrat Homaira
{"title":"Improving paediatric asthma outcomes in rural settings: clinical effectiveness of a multicomponent asthma care model.","authors":"Ryan Mackle, Melinda Gray, Mei Chan, Nicole Campbell, Trudie Campbell, Dominque Howard, Lisa Williams, Tracey Tyler, Prue McNamara, Nan Hu, Carmen Crespo-Gonzalez, Michael Hodgins, Blake Angell, Louisa Owens, Jeffery Fletcher, Timothy McCrossin, Susie Piper, Hong Du, Stuart Haggie, Aunty Kerrie Doyle, Bronwyn Gould, Sue Woolfenden, Flic Ward, Anthony Flynn, Raghu Lingam, Adam Jaffe, Nusrat Homaira","doi":"10.1183/23120541.00389-2026","DOIUrl":"10.1183/23120541.00389-2026","url":null,"abstract":"<p><strong>Introduction: </strong>Asthma is a leading cause of childhood hospitalisation and emergency department (ED) presentations in Australia. Barriers persist in accessing paediatric asthma care, particularly in rural areas. We implemented and evaluated a multicomponent model to reduce preventable hospital presentations across rural New South Wales.</p><p><strong>Methods: </strong>We conducted pre-post evaluation of children aged 5-12 years with asthma exacerbations across 13 hospitals in four rural NSW health districts. Children received: 1) a standardised discharge pack, 2) general practitioners (GP) and school linkage, 3) text reminders, and 4) nurse-led virtual home visits for uncontrolled asthma. Primary outcome was healthcare utilisation (ED presentations, admissions and GP visits) from electronic records, comparing matched pre-post periods at 1, 3, 6 and 12 months. Secondary outcomes included asthma control and quality of life at 6 and 12 months. Analyses used generalised estimating equations and mixed-effects models to calculate adjusted rate ratios (aRRs) and mean differences.</p><p><strong>Results: </strong>188 children participated (62% male, mean age 7.6 years). ED presentations decreased 93% at 1 month (aRR 0.07, 95% confidence interval 0.04-0.12), 85% at 3 months (aRR 0.15, 0.10-0.22), 78% at 6 months (aRR 0.22, 0.16-0.30) and 69% at 12 months (aRR 0.31, 0.25-0.40). Hospital admissions decreased 97%, 95%, 90% and 81%. Uncontrolled asthma decreased from 73% to 37% (6 months) and 30% (12 months). Asthma control and quality of life improved significantly at both timepoints.</p><p><strong>Conclusion: </strong>This multisite study achieved sustained reductions in preventable hospitalisations and meaningful improvements in asthma control and quality of life. This model offers a replicable approach for underserved populations.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"12 5","pages":""},"PeriodicalIF":4.4,"publicationDate":"2026-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13617586/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149073843","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Prognostic validation of the 2019 International Society for Heart and Lung Transplantation chronic lung allograft dysfunction states in lung transplant recipients. 2019年国际心肺移植学会对肺移植受者慢性同种异体肺功能障碍状态的预后验证。
IF 4.4 3区 医学
ERJ Open Research Pub Date : 2026-09-28 eCollection Date: 2026-09-01 DOI: 10.1183/23120541.01642-2025
Michael Gerckens, Gülümser Hale Alkan, Gamze Kasman, Paola Arnold, Jürgen Barton, Tobias Veit, Teresa Kauke, Christian Schneider, Sebastian Michel, Michael Zoller, Ali Önder Yildirim, Jürgen Behr, Nikolaus Kneidinger, Carlo Mümmler
{"title":"Prognostic validation of the 2019 International Society for Heart and Lung Transplantation chronic lung allograft dysfunction states in lung transplant recipients.","authors":"Michael Gerckens, Gülümser Hale Alkan, Gamze Kasman, Paola Arnold, Jürgen Barton, Tobias Veit, Teresa Kauke, Christian Schneider, Sebastian Michel, Michael Zoller, Ali Önder Yildirim, Jürgen Behr, Nikolaus Kneidinger, Carlo Mümmler","doi":"10.1183/23120541.01642-2025","DOIUrl":"10.1183/23120541.01642-2025","url":null,"abstract":"<p><strong>Background: </strong>The 2019 ISHLT consensus chronic lung allograft dysfunction (CLAD) definition introduced a standardised classification for CLAD into Potential, Possible, Probable and Definite stages, based on the degree and persistence of forced expiratory volume in 1 s (FEV<sub>1</sub>) decline. While Probable and Definite CLAD have been associated with graft loss, the significance of earlier stages remains unclear. This study aimed to validate the 2019 ISHLT CLAD classification system under simulated prospective conditions and to define their prognostic and clinical relevance.</p><p><strong>Methods: </strong>In 482 lung allograft recipients with measurable baseline FEV<sub>1</sub>, CLAD states were assigned longitudinally for 10 312 pulmonary function tests according to ISHLT 2019 criteria. Each CLAD state was modelled as a time-dependent covariate to assess mortality risk in multivariable Cox regression analysis. Transitions between CLAD stages were evaluated by Kaplan-Meier and Cox regression models.</p><p><strong>Results: </strong>Possible CLAD was the earliest stage independently associated with increased mortality (HR 2.8, 95% CI 1.7-4.5), similar to Probable and Definite CLAD (HR 3.5 (1.8-7.6) and 3.5 (2.4-5.2), respectively). Most lung allograft recipients with Possible CLAD progressed to Probable/Definite CLAD, confirming its clinical importance. In contrast, Potential CLAD frequently resolved and was not associated with increased mortality, identifying it as an early but reversible warning signal.</p><p><strong>Conclusion: </strong>Potential CLAD shows uncertain prognostic significance, underscoring the need for biomarkers to identify patients at risk of progression. Possible CLAD is independently linked to increased mortality and defines the earliest actionable stage of chronic allograft dysfunction, for timely intervention.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"12 5","pages":""},"PeriodicalIF":4.4,"publicationDate":"2026-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13617585/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149073829","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Event duration, endotypes and hypoxic burden in obstructive sleep apnoea. 阻塞性睡眠呼吸暂停的事件持续时间、内源性和缺氧负担。
IF 4.4 3区 医学
ERJ Open Research Pub Date : 2026-09-28 eCollection Date: 2026-09-01 DOI: 10.1183/23120541.01792-2025
Tsu-Hui Shiao, Kun-Ta Chou, Liang-Wen Hang, Eysteinn Finnsson, Jón S Ágústsson, Scott A Sands, Wan-Ju Cheng
{"title":"Event duration, endotypes and hypoxic burden in obstructive sleep apnoea.","authors":"Tsu-Hui Shiao, Kun-Ta Chou, Liang-Wen Hang, Eysteinn Finnsson, Jón S Ágústsson, Scott A Sands, Wan-Ju Cheng","doi":"10.1183/23120541.01792-2025","DOIUrl":"10.1183/23120541.01792-2025","url":null,"abstract":"<p><strong>Background: </strong>Respiratory event duration is an observable phenotype of obstructive sleep apnoea (OSA) in routine polysomnographic studies and is relatively longer in Asian patients. This study examined the association of respiratory event duration with OSA severity and endotypes in Asian patients with moderate-to-severe OSA.</p><p><strong>Methods: </strong>We recruited 1008 patients who underwent in-laboratory full-night polysomnographic studies. Event durations were averaged during non-rapid eye movement (NREM) and rapid eye movement (REM) sleep, respectively. The associations of event duration with endotypes (including arousal threshold, loop gain, circulatory delay, upper airway collapsibility and compensation) and OSA severity (apnoea-hypopnoea index (AHI) and hypoxic burden) were examined.</p><p><strong>Results: </strong>Participants demonstrated a mean event duration of 25.9 s in NREM sleep. Event duration exhibited an inverse U-shaped association with AHI during NREM sleep (peaking at 26.8 s) and a negative association with AHI during REM sleep, whereas hypoxic burden consistently increased with longer event durations. After adjusting for AHI, a 1-s increase in event duration during NREM sleep was associated with a 3.46 %eupnoea (95% CI 3.20-3.73) higher arousal threshold, 1.26 %eupnoea (95% CI 1.13-1.39) greater collapsibility (<i>V'</i> <sub>passive</sub>), 0.61 %eupnoea (95% CI 0.40-0.83) lower compensation, 0.07 s (95% CI 0.05-0.08) higher delay and 7.43 %·min·h<sup>-1</sup> (95% CI: 6.56-8.30) higher hypoxic burden. In REM sleep, event duration was positively associated with arousal threshold, loop gain and hypoxic burden, but negatively associated with <i>V'</i> <sub>passive</sub> and compensation.</p><p><strong>Conclusions: </strong>A high arousal threshold, a collapsible upper airway and poor compensatory function characterise long event durations in patients with OSA. AHI does not adequately reflect the disease burden associated with hypoxia caused by prolonged respiratory events.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"12 5","pages":""},"PeriodicalIF":4.4,"publicationDate":"2026-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13617597/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149073789","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Erratum: "A simple score to identify the sickest normotensive patients with acute pulmonary embolism" Winnifer Briceño, Ana Castillo, Ignacio Jara, Laura Lago, Edwin Yong, Alfonso Muriel, Luis Jara-Palomares, Simone Vanni, Ginevra Fabiani, Manuel Monreal, Behnood Bikdeli and David Jiménez. ERJ Open Res 2026; 12: 01203-2025. 更正:“一个简单的分数来识别急性肺栓塞最严重的正常血压患者”Winnifer Briceño, Ana Castillo, Ignacio Jara, Laura Lago, Edwin Yong, Alfonso Muriel, Luis Jara- palomares, Simone Vanni, Ginevra Fabiani, Manuel Monreal, Behnood Bikdeli和David jimsamunez。ERJ Open Res 2026;12: 01203 - 2025。
IF 4.4 3区 医学
ERJ Open Research Pub Date : 2026-09-28 eCollection Date: 2026-09-01 DOI: 10.1183/23120541.51203-2025
{"title":"Erratum: \"A simple score to identify the sickest normotensive patients with acute pulmonary embolism\" Winnifer Briceño, Ana Castillo, Ignacio Jara, Laura Lago, Edwin Yong, Alfonso Muriel, Luis Jara-Palomares, Simone Vanni, Ginevra Fabiani, Manuel Monreal, Behnood Bikdeli and David Jiménez. <i>ERJ Open Res</i> 2026; 12: 01203-2025.","authors":"","doi":"10.1183/23120541.51203-2025","DOIUrl":"https://doi.org/10.1183/23120541.51203-2025","url":null,"abstract":"<p><p>[This corrects the article DOI: 10.1183/23120541.01203-2025.].</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"12 5","pages":""},"PeriodicalIF":4.4,"publicationDate":"2026-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13617589/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149073783","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Mapping challenges in the delivery of care for primary ciliary dyskinesia: an international survey on barriers and priorities. 在提供初级纤毛运动障碍护理方面的挑战:一项关于障碍和优先事项的国际调查。
IF 4.4 3区 医学
ERJ Open Research Pub Date : 2026-09-28 eCollection Date: 2026-09-01 DOI: 10.1183/23120541.01624-2025
Bruna Rubbo, Laura Behan, Lydia M Newman, Janice Coles, John Goodwin, Robert A Hirst, Claire L Jackson, Corine Driessens, Myrofora Goutaki, Claudia E Kuehni, Jane S Lucas
{"title":"Mapping challenges in the delivery of care for primary ciliary dyskinesia: an international survey on barriers and priorities.","authors":"Bruna Rubbo, Laura Behan, Lydia M Newman, Janice Coles, John Goodwin, Robert A Hirst, Claire L Jackson, Corine Driessens, Myrofora Goutaki, Claudia E Kuehni, Jane S Lucas","doi":"10.1183/23120541.01624-2025","DOIUrl":"10.1183/23120541.01624-2025","url":null,"abstract":"<p><strong>Background: </strong>Primary ciliary dyskinesia (PCD) is a rare genetic disorder causing chronic multisystemic symptoms due to impaired motile ciliary function. Diagnosis and care of PCD requires specialised, resource-intensive laboratory and clinical services, which are often unavailable in settings with limited resources. We compared the opinions of PCD healthcare professionals on the barriers to and priorities of delivering care for people with PCD to inform strategies for improvement.</p><p><strong>Methods: </strong>We conducted a mixed-methods study comprising semi-structured interviews with PCD healthcare professionals, followed by an electronic survey guided by interview findings. We classified participants' countries by gross national income (GNI) to enable comparisons across settings.</p><p><strong>Results: </strong>We conducted 15 interviews across 13 countries, identifying key barriers and priorities through content analysis. 128 participants from 35 countries completed the survey. Common barriers included lack of PCD awareness among non-specialist clinicians, underestimation of disease severity, insufficient funding and limited time for clinical and research activities. Priorities were raising awareness, disseminating referral guidelines and enhancing collaboration between centres. Participants from countries with a high GNI highlighted the lack of evidence-based guidelines for clinical management as an important barrier, and the need for multidisciplinary teams as a priority. Participants from countries with a low GNI emphasised limited prioritisation of PCD by adult clinicians and high diagnostic cost as barriers, and access to experienced diagnostic personnel and tests as priorities.</p><p><strong>Conclusion: </strong>Our findings inform a shared roadmap for improving PCD care. We highlight the need for future research to identify systemic, contextual and modifiable factors influencing access, management and funding for PCD across service delivery models.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"12 5","pages":""},"PeriodicalIF":4.4,"publicationDate":"2026-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13617590/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149073781","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Reduced vascular function, ventilatory efficiency and exercise capacity in young adult electronic cigarette users. 青少年电子烟使用者血管功能、通气效率和运动能力降低。
IF 4.4 3区 医学
ERJ Open Research Pub Date : 2026-09-28 eCollection Date: 2026-09-01 DOI: 10.1183/23120541.00425-2026
Azmy Faisal, Agata Giles, Sarah Jones, Christopher I Morse, Hans Degens, Caroline J Jolley, Steven K Rogers, Rashid Ahmed, Amany F Elbehairy, Rehan T Junejo
{"title":"Reduced vascular function, ventilatory efficiency and exercise capacity in young adult electronic cigarette users.","authors":"Azmy Faisal, Agata Giles, Sarah Jones, Christopher I Morse, Hans Degens, Caroline J Jolley, Steven K Rogers, Rashid Ahmed, Amany F Elbehairy, Rehan T Junejo","doi":"10.1183/23120541.00425-2026","DOIUrl":"10.1183/23120541.00425-2026","url":null,"abstract":"<p><strong>Background: </strong>Acute electronic cigarette (E-cigarette) use has shown adverse cardiorespiratory impacts similar to conventional cigarette (C-cigarette) smoking; however, its early effects remain unclear. We aimed to examine the early effects of E-cigarette use on systemic vascular function, cardiorespiratory responses to exercise and exertional symptoms in young adults.</p><p><strong>Methods: </strong>75 healthy active young adults (18-30 years), divided into three age- and sex-matched groups (E-cigarette users, C-cigarette smokers and never-smoker controls; n=25 each, E-cigarette use or smoking duration ≥18 months), underwent an incremental cycle exercise test with standard metabolic, cardiorespiratory, blood lactate and sensory measurements. Brachial artery flow-mediated dilatation (FMD) was assessed using Doppler ultrasound. Blood samples were collected to assess vascular inflammatory markers.</p><p><strong>Results: </strong>Cotinine (nicotine metabolite) levels were not different between E-cigarette users and C-cigarette smokers (80.7±33.0 <i>versus</i> 65.5±27.6 pg·mL<sup>-1</sup>, p=0.2). At peak exercise, both E-cigarette users and C-cigarette smokers had lower exercise capacity, oxygen uptake, and ventilation compared with controls (182±31, 178±27 <i>versus</i> 219±43 watts; 35.1±6.4, 35.1±5.3 <i>versus</i> 41.2±5.9 mL·kg<sup>-1</sup>·min<sup>-1</sup>; 97.8±24.7, 98.9±21.6 <i>versus</i> 116.1±29.8 L·min<sup>-1</sup>, respectively; all p<0.05). Furthermore, E-cigarette users and C-cigarette smokers demonstrated decreased exercise ventilatory efficiency, with higher ventilation, blood lactate, dyspnoea ratings and leg discomfort during submaximal exercise compared with controls. E-cigarette users and C-cigarette smokers showed lower FMD compared with never-smokers (4.7±3.0, 4.5±2.9 <i>versus</i> 8.1±3.5%, p<0.05), which was inversely associated with vascular cell adhesion molecule-1 and thrombospondin-2.</p><p><strong>Conclusion: </strong>E-cigarette use and C-cigarette smoking were both associated with early detrimental effects on exercise capacity, ventilatory efficiency, systemic vascular function and amplified exertional symptoms.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"12 5","pages":""},"PeriodicalIF":4.4,"publicationDate":"2026-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13617591/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149073825","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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