ERJ Open Research最新文献

{"title":"Rare variants in <i>STAB2</i> in patients with chronic thromboembolic pulmonary hypertension.","authors":"Mark W Dodson, Kristina Allen-Brady, Jeffrey Stevens, Meghan M Cirulis, Mona Alotaibi, Timothy M Fernandes, Nick H Kim, Kim M Kerr, Demosthenes G Papamatheakis, David S Poch, Julianna Desmarais, D Hunter Best, Nathan D Hatton, John J Ryan, C Gregory Elliott, Lisa A Cannon-Albright","doi":"10.1183/23120541.01322-2024","DOIUrl":"10.1183/23120541.01322-2024","url":null,"abstract":"<p><strong>Background: </strong>The pathogenesis of chronic thromboembolic pulmonary hypertension (CTEPH) is poorly understood. Studies of the genetic risk factors for CTEPH are likely to improve our understanding of CTEPH pathogenesis and may lead to novel treatment and prevention strategies. Genetic analysis focused on shared gene variants in high-risk disease pedigrees can aid in the identification of rare variants with a strong effect on disease risk.</p><p><strong>Methods: </strong>We identified 13 CTEPH high-risk pedigrees and performed whole-exome sequencing in 22 CTEPH cases from these pedigrees, focusing on rare and deleterious variants that were shared between related CTEPH cases. We validated CTEPH candidate gene variants in two independent CTEPH cohorts, one from Utah (n=78) and one from the University of California San Diego (n=238), and compared them to controls from the UK Biobank.</p><p><strong>Results: </strong>A rare and predicted deleterious missense variant in <i>STAB2</i> was identified in two related CTEPH cases. Qualifying <i>STAB2</i> variant alleles were observed more frequently in both CTEPH cohorts (pooled allele frequency 4.6%) than in subjects from the UK Biobank with a history of pulmonary embolism (PE) (allele frequency 2.2%, p=0.0002) or without a history of PE (allele frequency 1.9%, p<0.0001). CTEPH subjects with qualifying <i>STAB2</i> variants had elevated levels of plasma von Willebrand Factor (vWF) and factor VIII, consistent with the known role of <i>STAB2</i> as a genetic regulator of circulating vWF levels.</p><p><strong>Conclusions: </strong>Rare variants in <i>STAB2</i> are identified in a CTEPH high-risk pedigree and are over-represented in nonrelated CTEPH cases compared to controls with PE. These data suggest that <i>STAB2</i> is a CTEPH risk gene.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"11 4","pages":""},"PeriodicalIF":4.0,"publicationDate":"2025-07-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12301891/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144728871","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}

{"title":"The genetics of chronic thromboembolic pulmonary hypertension: no longer a stab in the dark.","authors":"David Montani, Mark Toshner","doi":"10.1183/23120541.00551-2025","DOIUrl":"10.1183/23120541.00551-2025","url":null,"abstract":"<p><p><b>As our understanding of the genetic basis of CTEPH deepens, we are moving closer to predicting which PE survivors are at greatest risk and to intervening before irreversible vascular remodelling occurs</b> https://bit.ly/3RTcE4k.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"11 4","pages":""},"PeriodicalIF":4.0,"publicationDate":"2025-07-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12301890/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144728872","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}

{"title":"Low-molecular-weight hyaluronic acid improves regeneration of cystic fibrosis airway epithelium.","authors":"Damien Adam, Emilie Luczka-Majérus, Julie Cellier, Charline Dos Santos-Dietz, Claire Kileztky, Arnaud Bonnomet, Christophe Ruaux, Edouard Sage, Myriam Polette, Michel Abély, Christelle Coraux","doi":"10.1183/23120541.00799-2024","DOIUrl":"10.1183/23120541.00799-2024","url":null,"abstract":"<p><strong>Background: </strong>Cystic fibrosis is characterised by defective mucociliary clearance, chronic lung infection and exaggerated neutrophilic inflammation. Airway epithelium damage and remodelling affect lung defence functions and are therefore important components of lung pathology progression in cystic fibrosis. Identifying compounds that favour mucociliary clearance by improving airway epithelial structure and regeneration is therefore crucial for patients with cystic fibrosis.</p><p><strong>Materials and methods: </strong>Using air-liquid interface culture of human airway epithelial cells obtained from patients with cystic fibrosis, we examined the influence of low-molecular-weight hyaluronic acid (LMW-HA) (∼40 kDa) on the regeneration and remodelling of cystic fibrosis human airway epithelial cells.</p><p><strong>Results: </strong>Our results show that LMW-HA normalises cystic fibrosis epithelial regeneration, even in an inflamed environment, by preventing remodelling in terms of epithelial height and basal cell hyperplasia, by avoiding inflammation-related goblet cell hyperplasia and by stimulating multiciliated cell differentiation. Because remodelling is mainly due to either intrinsic inflammation of cystic fibrosis human airway epithelial cells or an extrinsic inflammatory environment, we examined the impact of LMW‑HA on epithelial interleukin 8 pro-inflammatory chemokine and found that it exerts an anti-inflammatory effect, evidenced by reduced epithelial interleukin 8 expression and secretion.</p><p><strong>Conclusion: </strong>We report here that LMW-HA prevents cystic fibrosis human airway epithelial cell remodelling and normalises its cell structure, probably through the epithelial cell inflammatory phenotype modulation, and improves multiciliated cell differentiation by a mechanism that is independent of its anti-inflammatory effect. These results demonstrate that LMW-HA should be considered as a therapeutic candidate for the treatment of cystic fibrosis lung disease.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"11 4","pages":""},"PeriodicalIF":4.0,"publicationDate":"2025-07-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12301892/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144728936","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}

{"title":"6 min of walking to capture aerobic capacity.","authors":"Gael Deboeck, Gabriel Dias Rodrigues, Marco Vicenzi","doi":"10.1183/23120541.01354-2024","DOIUrl":"10.1183/23120541.01354-2024","url":null,"abstract":"<p><p><b>6MWD lower than 440 m in PAH patients probaly indicates very limited haemodynamic adpatation to exercise, and is linearly associated with higher <i>V̇</i> _E/<i>V̇</i> _CO₂ , suggesting more altered chemo-, baro- or metabo-reflex sensitivity in those patients</b> https://bit.ly/3QKQ51b.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"11 4","pages":""},"PeriodicalIF":4.3,"publicationDate":"2025-07-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12278315/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144682227","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}

{"title":"Implications of reference equations for interpretation of spirometry in three African countries: a cross-sectional study.","authors":"Denise Banze, Claire J Calderwood, Celina Nhamuave, Edson T Marambire, Alfred Mfinanga, Leyla Larsson, Akanksha Mimi Malhotra, Lilian T Minja, Olena Ivanova, Lindsay Zurba, Norbert Heinrich, Rashida A Ferrand, Katherine Fielding, Katharina Kranzer, Andrea Rachow, John R Hurst, Celso Khosa","doi":"10.1183/23120541.00932-2024","DOIUrl":"10.1183/23120541.00932-2024","url":null,"abstract":"<p><strong>Background: </strong>The Global Lung Function Initiative (GLI) and American Thoracic Society recently endorsed a race-composite spirometry reference equation (\"GLI Global\"). Africa (outside North Africa) is not represented in the underlying dataset; GLI Global has not been evaluated in the region. We evaluated the fit and diagnostic implications of GLI and African (identified by scoping review) reference equations in three East/Southern African countries.</p><p><strong>Methods: </strong>Among healthy participants from a tuberculosis household contact cohort study in Mozambique, Tanzania and Zimbabwe (age ≥10 years) with post-bronchodilator spirometry we calculated forced expiratory volume in 1 s (FEV₁), forced vital capacity (FVC) and FEV₁/FVC z-scores using different equations, the proportion of people with obstructive airways disease or preserved-ratio-impaired spirometry by different equations. We compared these measures across reference equations.</p><p><strong>Results: </strong>In total, 806 healthy people had good-quality post-bronchodilator spirometry. Across GLI equations, \"African American\" fitted best (mean±sd FEV₁ z-score -0.12±1.20, mean FVC z-score -0.35±1.19). Compared with \"African American\", GLI Global resulted in twice as many people being identified as having preserved-ratio impaired spirometry (22% <i>versus</i> 11%) with a similar proportion having obstruction (4.2% <i>versus</i> 3.8%). Reference equations developed in Africa conferred similar fit compared with the GLI African American equation.</p><p><strong>Conclusions: </strong>Reference equations have clinical and public health implications that demand careful consideration, particularly in resource-constrained environments. Use of GLI Global may result more people being identified as having lung function impairment. Further work that includes clinical outcomes is needed to ensure that GLI Global is globally representative. The key limitation of this work is the potential for people with undiagnosed respiratory disease to have been included in the analysis.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"11 4","pages":""},"PeriodicalIF":4.0,"publicationDate":"2025-07-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12278320/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144682232","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}

{"title":"Deupirfenidone (LYT-100) in post-acute sequelae of SARS-CoV-2 with respiratory complications.","authors":"Tejaswini Kulkarni, Joel Santiaguel, Raminder Aul, Mark Harnett, Julie Krop, Michael C Chen, Camilla S Graham, Toby M Maher","doi":"10.1183/23120541.01142-2024","DOIUrl":"10.1183/23120541.01142-2024","url":null,"abstract":"<p><strong>Introduction: </strong>The pathophysiology of respiratory complications in post-acute sequelae of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection (PASC) is poorly understood, but a high incidence of progressive pulmonary fibrosis was anticipated. Deupirfenidone (LYT-100) is a selectively deuterated form of pirfenidone that retains antifibrotic and anti-inflammatory activity but with improved tolerability. This study evaluated the safety and efficacy of deupirfenidone in PASC patients with respiratory complications.</p><p><strong>Methods: </strong>Global, double-blind, randomised placebo-controlled trial evaluating 750 mg deupirfenidone twice daily <i>versus</i> placebo for 3 months in PASC patients with respiratory complications following hospitalisation for acute COVID-19 infection severe enough to necessitate supplemental oxygen (NCT04652518).</p><p><strong>Results: </strong>185 patients were randomised and treated (95 with deupirfenidone, 90 with placebo), with 177 included in the modified intention-to-treat population. The mean age was 54.5 years, 62.7% were male and 10.7% had prior mechanical ventilation. The 6-min walk distance improved across both arms between baseline and day 91 (deupirfenidone 44.3 m (95% CI 24.8-63.8 m) <i>versus</i> placebo 48.8 m (95% CI 29.2-68.4 m); p=0.70). The most common treatment-emergent adverse events (TEAEs) for deupirfenidone <i>versus</i> placebo were nausea (9.5% <i>versus</i> 1.1%), upper abdominal discomfort (5.3% <i>versus</i> 2.2%) and dyspepsia (6.3% <i>versus</i> 1.1%). TEAEs leading to trial drug discontinuation were 11.6% for deupirfenidone and 4.4% for placebo. The proportion of discontinuations considered at least possibly related to treatment was 8.6% for deupirfenidone and 2.4% for placebo.</p><p><strong>Discussion: </strong>Most patients with PASC and respiratory complications showed significant improvement over 91 days irrespective of treatment assignment. Deupirfenidone was well tolerated, with low rates of TEAEs, which supports further investigation in patients with idiopathic pulmonary fibrosis.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"11 4","pages":""},"PeriodicalIF":4.3,"publicationDate":"2025-07-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12278312/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144682230","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}

{"title":"Long-term PM_2.5 exposure impairs lung growth and increases airway inflammation in Taiwanese school children.","authors":"Yi-Giien Tsai, Jiu-Yao Wang, Kuender D Yang, Hsiao-Yu Yang, Yen-Po Yeh, Yu-Jun Chang, Jui Huan Lee, Shu-Li Wang, Shau-Ku Huang, Chang-Chuan Chan","doi":"10.1183/23120541.00972-2024","DOIUrl":"10.1183/23120541.00972-2024","url":null,"abstract":"<p><strong>Rationale: </strong>Prolonged exposure to particulate matter with aerodynamic diameter <2.5 µm (PM_2.5) may aggravate asthma, impair lung development and increase airway inflammation. This study investigated the impact of long-term PM_2.5 exposure on respiratory health, lung function growth and fractional exhaled nitric oxide (<i>F</i> _ENO) in a large longitudinal cohort of school children.</p><p><strong>Methods: </strong>A total of 6120 elementary school children residing in townships near coal-fired power plants in Taiwan were prospectively enrolled from 2016 to 2018. Baseline and follow-up data on asthmatic symptoms, spirometry, <i>F</i> _ENO and environmental factors were collected. Annual PM_2.5 exposure was estimated using land-use regression models based on school and home addresses, and associations were adjusted for SO₂ and NO₂.</p><p><strong>Results: </strong>The final analysis included 5364 children and revealed that a 1 μg·m^-3 increase in annual PM_2.5 exposure was associated with higher odds of current wheezing (OR 1.07), \"ever\" wheeze (OR 1.03), diagnosed asthma (OR 1.03) and exercise-induced wheeze (OR 1.04) (p<0.05). Each unit increase in PM_2.5 exposure was associated with a decrease of 7 mL in forced expiratory volume in 1 s (FEV₁), 5 mL in forced vital capacity (FVC) and a 0.479-ppb rise in <i>F</i> _ENO after adjusting for potential confounders (p<0.05). Among 207 new-onset wheezing patients, increased PM_2.5 exposure significantly decreased FEV₁ by 13 mL and FVC by 15 mL, while increasing <i>F</i> _ENO levels by 0.847 ppb (p<0.05).</p><p><strong>Conclusions: </strong>Long-term PM_2.5 exposure significantly increases the risk of asthma symptoms, impedes lung growth and triggers airway inflammation, particularly affecting children with new-onset wheezing in community settings.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"11 4","pages":""},"PeriodicalIF":4.3,"publicationDate":"2025-07-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12278304/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144682233","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}

{"title":"Outcomes and measures in studies of techniques to promote secretion clearance in individuals with neuromuscular conditions: a scoping review.","authors":"Neeraj M Shah, Chloe Apps, Reshma Amin, Georgios Kaltsakas, Nicholas Hart, Patrick B Murphy, Louise Rose","doi":"10.1183/23120541.01007-2024","DOIUrl":"10.1183/23120541.01007-2024","url":null,"abstract":"<p><strong>Background: </strong>Techniques to support secretion clearance for individuals with neuromuscular conditions and respiratory muscle weakness include mechanical insufflation-exsufflation and chest wall vibrations. Assessing the comparative efficacy of these techniques is challenging due to the absence of a core outcome set. We sought to describe outcomes and measurement instruments reported in studies of airway clearance techniques for individuals with neuromuscular conditions living in the community.</p><p><strong>Methods: </strong>We conducted a scoping review of primary research studies. We searched six databases from inception to 22 February 2024. Two reviewers independently screened citations against the inclusion criteria and extracted data on outcomes and measurement characteristics. Outcomes were categorised according to the Core Outcome Measures in Effectiveness Trials (COMET) 38-domain taxonomy.</p><p><strong>Results: </strong>We identified 75 eligible studies describing 55 outcomes. We grouped outcomes deemed overlapping and categorised them using the COMET 38-domain taxonomy, resulting in 34 distinct outcomes. Common physiological/clinical outcomes were cough strength (n=48 studies, 64%), lung volume (n=48, 64%) and insufflation capacity (n=22, 29%). The most common measurement tools for these outcomes were spirometer (n=38, 51%), peak flow meter (n=24, 32%) and pneumotachograph (n=20, 27%). The most common resource-use outcome was hospitalisation due to respiratory illness (n=13, 17%). Few studies reported on life impact outcomes, with the most common being comfort (n=6, 8%) and patient satisfaction (n=4, 5%).</p><p><strong>Conclusion: </strong>We identified 34 outcomes from 75 studies, which were most commonly physiological/clinical, with resource-use and life impact outcomes being seldom reported. The number and range of outcomes and measures demonstrates the need for a core outcome set.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"11 4","pages":""},"PeriodicalIF":4.3,"publicationDate":"2025-07-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12278321/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144682234","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}

{"title":"Erratum: \"External validation of the LENT and PROMISE prognostic scores for malignant pleural effusion\" Craig A. Mounsey, Nikolaos I. Kanellakis, Dinesh N. Addala, Jamie A. Mawhinney, Nick Freemantle and Najib M. Rahman. <i>ERJ Open Res</i> 2025; 11: 01019-2024.","authors":"","doi":"10.1183/23120541.51019-2024","DOIUrl":"https://doi.org/10.1183/23120541.51019-2024","url":null,"abstract":"<p><p>[This corrects the article DOI: 10.1183/23120541.01019-2024.].</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"11 4","pages":""},"PeriodicalIF":4.3,"publicationDate":"2025-07-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12278302/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144682231","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}

{"title":"Classification and course of pulmonary hypertension associated with end-stage COPD.","authors":"Michaela Barnikel, Katrin Milger, Pontus Mertsch, Paola Arnold, Gabriela Leuschner, Tobias Veit, Michael Gerckens, Carlo Mümmler, Jürgen Barton, Alessandro Ghiani, Ali Önder Yildirim, Julien Dinkel, Claus Neurohr, Jürgen Behr, Nikolaus Kneidinger","doi":"10.1183/23120541.01141-2024","DOIUrl":"10.1183/23120541.01141-2024","url":null,"abstract":"<p><strong>Background: </strong>Pulmonary hypertension (PH) associated with COPD contributes to morbidity and mortality. Further characterisation to improve management is warranted. The aim of the study was to apply the recently proposed PH classification and to assess the association of lung volume involvement and PH over the course of disease in patients with advanced COPD.</p><p><strong>Methods: </strong>Patients with COPD undergoing transplant evaluation, including right heart catheterisation were included irrespective of the likelihood of having PH. Spirometry, plethysmography and computed tomography were used to assess the degree of parenchymal and vascular involvement. Follow-up investigation was performed for 18±12 months. The 2022 European Society of Cardiology/European Respiratory Society guidelines were used for classification of PH.</p><p><strong>Results: </strong>In total, 340 patients were included and 639 right heart catheters were assessed. The majority of patients were classified as no PH (n=131, 38%) or nonsevere PH (n=133, 39%), whereas severe COPD-PH was present in 26 patients (8%). Patients with severe COPD-PH had similar degrees of airflow obstruction but lower lung volumes. Further, pulmonary vascular resistance (PVR) correlated negatively with residual volume. Interstitial lung abnormalities were present in 11 patients (3%) and scattered across all PH groups. Follow-up (n=141, 41.5%) demonstrated a low rate of deterioration to severe COPD-PH (4%). However, an increase of PVR was common and was associated with a decrease of total lung capacity.</p><p><strong>Conclusion: </strong>Unbiased longitudinal invasive follow-up and assessment of lung volumes by plethysmography provided evidence of an association of lung volume and PVR.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"11 4","pages":""},"PeriodicalIF":4.3,"publicationDate":"2025-07-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12278318/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144682229","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}