Respirology最新文献

{"title":"Impact of A Multidisciplinary Team Discussion for Genetic Lung Fibrosis.","authors":"Giovanni Franco, Ibrahima Ba, Nadia Nathan, Cécile Guerin, Albane Lassus, Caroline Kannengiesser, Antoine Froidure, Effrosyni Manali, Vincent Bunel, Philippe Bonniaud, Diane Bouvry, Marie Pierre Debray, Pierre Antoine Juge, Ralph Epaud, Camille Louvrier, Aurélie Plessier, Flore Sicre de Fontbrune, Lidwine Wémeau-Stervinou, Sylvain Marchand Adam, Alexandre Chabrol, Arnaud Maurac, Laurent Savale, David Montani, Caroline Raynal, Marina Konyukh, Arthur Mageau, Bruno Crestani, Vincent Cottin, Alix de Becdelièvre, Raphaël Borie","doi":"10.1111/resp.70039","DOIUrl":"https://doi.org/10.1111/resp.70039","url":null,"abstract":"<p><strong>Background and objective: </strong>Approximately 30% of individuals diagnosed with familial pulmonary fibrosis (FPF) exhibit a pathogenic variant upon genetic analysis. We established a genetic Multidisciplinary Discussion (geneMDD) aimed to enhance expertise in diagnosing and managing FPF. This study aimed at prospectively evaluating the impact of geneMDD on diagnosis and treatment in patients referred to geneMDD.</p><p><strong>Methods: </strong>In this prospective study, we enrolled all consecutive patients referred to the geneMDD. At each meeting, the impact of the meeting was questioned on the genetic conclusion, the pulmonary diagnosis, and the treatment.</p><p><strong>Results: </strong>A total of 115 patients were included. Before geneMDD, rare variants were detected in 82 out of 107 patients, among which 65 variants were classified as pathogenic/likely pathogenic. Following geneMDD, 2 pathogenic variants (3%) were reclassified as variants of uncertain significance (VUS) (n = 1) or benign (n = 1). Among the 17 variants initially classified as VUS, 2 (11.8%) were reclassified as likely pathogenic/pathogenic. The pulmonary diagnosis was confirmed for all patients (unclassifiable lung fibrosis was the more frequent diagnosis, n = 38, 33.0%). The therapeutic regimen was changed after geneMDD in 30 patients. Factors associated with therapeutic changes included the pulmonary diagnosis and presence of a pathogenic/likely pathogenic variant. In addition, the French health system allows offering whole genome sequencing (WGS) in patients with a first negative genetic analysis by NGS panel after discussion in geneMDD, but in total, since September 1st, 2021, WGS was negative for the four analysed families.</p><p><strong>Conclusion: </strong>This study suggests that geneMDD could influence the treatment of FPF patients.</p>","PeriodicalId":21129,"journal":{"name":"Respirology","volume":" ","pages":""},"PeriodicalIF":6.6,"publicationDate":"2025-03-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143711169","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":"Clinical Presentation and Diagnostic Evaluation of Vaping Related Lung Injury in Youth.","authors":"Eleanor D Muise, Rachel Gordon, Jacqueline Steiding, Keri Sullivan, Catherine A Sheils, Alicia M Casey","doi":"10.1111/resp.70034","DOIUrl":"https://doi.org/10.1111/resp.70034","url":null,"abstract":"<p><strong>Background and objectives: </strong>The vaping epidemic is a public health crisis worldwide. E-cigarette, or vaping product, use-associated lung injury (EVALI) was recognised in the summer of 2019 and resulted in more than 2800 hospitalizations and 60 deaths per the Centres for Disease Control and Prevention (CDC). Vaping refers to the use of E-cigarettes, which are electronic nicotine delivery systems (ENDS) and mimic smoking without combustion. Over 40 million people in the United States vape, of which youth and young adults represent over half. We describe the evaluation and pulmonary complications in a large cohort of adolescents and young adults referred to a Pulmonary Complications of Vaping Clinic.</p><p><strong>Methods: </strong>Youth ages 10-35 with a vaping history and respiratory symptoms underwent a comprehensive diagnostic evaluation. All patients were counselled for vaping cessation. Patients who met criteria for EVALI or probable EVALI with concurrent infection were reported to the Department of Health.</p><p><strong>Results: </strong>One hundred and thirty patients were referred, and 103 patients underwent comprehensive diagnostic evaluation. Eighty-four percent of patients reported vaping both marijuana and nicotine products. Forty-six percent of patients were diagnosed with EVALI or probable EVALI.</p><p><strong>Conclusions: </strong>The evolving vaping epidemic demonstrates a need for subject matter expertise in the evaluation and diagnosis of youth with vaping-related lung injury, including EVALI. We report a higher number of youth who met criteria for EVALI with the implementation of a standardised vaping questionnaire and evaluation inclusive of outpatients.</p>","PeriodicalId":21129,"journal":{"name":"Respirology","volume":" ","pages":""},"PeriodicalIF":6.6,"publicationDate":"2025-03-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143711167","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":"Fixed CPAP at 10 cmH₂O May Replace Manual Titration in Moderate to Severe OSA Patients: A Preliminary Randomised Controlled Trial.","authors":"Wang Lu, Yan Chen, Qishan Wei, Yingxin Wu, Cuizhen Huang, Shanfeng Liang, Joerg Steier, Peter Catcheside, Danny Eckert, Andrew Wellman, Yuanming Luo","doi":"10.1111/resp.70037","DOIUrl":"https://doi.org/10.1111/resp.70037","url":null,"abstract":"<p><strong>Background and objectives: </strong>Patient compliance with continuous positive airway pressure (CPAP) is similar using manual-titrated pressure compared to auto-titration, although auto-titration pressures are usually 2-5 cmH₂O higher than manual pressure, indicating that CPAP moderately higher than the optimal pressure will not necessarily impair compliance. We try to find the tolerable highest CPAP which does not increase respiratory effort based on changes in lung volume, diaphragm electromyography (EMG) and breathing sensations in healthy volunteers and OSA patients to simplify pressure titration.</p><p><strong>Methods: </strong>Part 1, 12 healthy subjects and 16 OSA patients were enrolled in the measurement of expiratory reserve volume, diaphragm EMG, and expiratory muscle EMG at different CPAP levels. Breathing difficulty during different CPAP levels was assessed using a customised questionnaire in 35 healthy subjects and 33 OSA patients. Part 2, a two-night randomised crossover double-blind trial using the tolerable highest CPAP (10 cmH₂O) based on the results derived from Part 1 and the manually titrated pressure was performed in 25 OSA patients.</p><p><strong>Results: </strong>End expiratory lung volume increased significantly with increasing CPAP. In general, diaphragm EMG changed little when CPAP ≤ 10 cmH₂O. Expiratory muscle activity appeared when CPAP > 12 cmH₂O. There was no significant difference in subjective sensation of breathing difficulty with CPAP ≤ 10 cmH₂O. Sleep structure, AHI, and patient preference with 10 cmH₂O CPAP were not different from those under titrated pressure.</p><p><strong>Conclusions: </strong>This study suggests that most patients with moderate to severe OSA can be effectively treated with CPAP at an initial pressure of 10 cmH₂O without pressure titration.</p><p><strong>Trial registration: </strong>ClinicalTrials.gov identifier: NCT04925466.</p>","PeriodicalId":21129,"journal":{"name":"Respirology","volume":" ","pages":""},"PeriodicalIF":6.6,"publicationDate":"2025-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143701383","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":"Sliding Into Misdiagnosis? Distinguishing Bullous Emphysema From Pneumothorax With Lung Ultrasound.","authors":"Casper Falster","doi":"10.1111/resp.70038","DOIUrl":"https://doi.org/10.1111/resp.70038","url":null,"abstract":"","PeriodicalId":21129,"journal":{"name":"Respirology","volume":" ","pages":""},"PeriodicalIF":6.6,"publicationDate":"2025-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143701386","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":"Letter From China.","authors":"Chenyang Lu, Qingling Zhang","doi":"10.1111/resp.70029","DOIUrl":"https://doi.org/10.1111/resp.70029","url":null,"abstract":"","PeriodicalId":21129,"journal":{"name":"Respirology","volume":" ","pages":""},"PeriodicalIF":6.6,"publicationDate":"2025-03-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143701384","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":"Enhancing COPD Care for Women: A Predictive Tool for Palliative Needs.","authors":"Syed Ahmar Shah","doi":"10.1111/resp.70031","DOIUrl":"https://doi.org/10.1111/resp.70031","url":null,"abstract":"","PeriodicalId":21129,"journal":{"name":"Respirology","volume":" ","pages":""},"PeriodicalIF":6.6,"publicationDate":"2025-03-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143693133","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":"Lung Function Course of Patients With Pulmonary Fibrosis After Initiation of Anti-Fibrotic Treatment: Real-World Data From the Dutch National Registry.","authors":"Mark G J P Platenburg, Gizal Nakshbandi, Catharina C Moor, Aernoud A van Batenburg, Rémy L M Mostard, Mareye Voortman, Linda Moonen, Nicolle Hekelaar, Maria J Overbeek, Brigitte Bogaarts, Henk Kramer, Emiel Marges, Bart B Boerrigter, Paul Bresser, Eveline Schakenraad, Jan van der Maten, C A van der Sloot, Stefan Walen, Pedro M Afonso, Marlies S Wijsenbeek, Jan C Grutters","doi":"10.1111/resp.70030","DOIUrl":"https://doi.org/10.1111/resp.70030","url":null,"abstract":"<p><strong>Background and objective: </strong>Real-world data on lung function course of patients with progressive pulmonary fibrosis (PPF) treated with anti-fibrotic medication are limited. We evaluated forced vital capacity (FVC) decline in patients with PPF and idiopathic pulmonary fibrosis (IPF) who started anti-fibrotic treatment.</p><p><strong>Methods: </strong>This was a nationwide multi-centre registry study in 16 hospitals throughout the Netherlands. Patients treated with anti-fibrotic medication, with at least two in-hospital pulmonary function tests before and after the initiation of anti-fibrotic treatment, were included. Linear mixed-effects modelling was used to analyse lung function trajectories 1 year before and after the start of anti-fibrotic treatment.</p><p><strong>Results: </strong>Data from 538 patients (n = 142 with PPF, n = 396 with IPF) were analysed. In PPF, the mean annualised FVC decline was 412 mL (95% confidence interval [CI]: 308-517 mL) before the initiation of anti-fibrotic treatment, and 18 mL (95% CI: 9-124 mL) in the first year after. The corresponding declines for IPF were 158 mL (95% CI: 78-239 mL) and 38 mL (95% CI: 24-101 mL). In both groups, treatment significantly slowed down FVC decline, although the change was larger in the PPF group (p = 0.0006). In the first year after treatment initiation, 28.0% of patients with PPF and 27.4% with IPF had disease progression.</p><p><strong>Conclusion: </strong>The FVC decline significantly slowed after the initiation of treatment for both IPF and PPF. Nevertheless, a significant proportion of patients exhibited disease progression, despite the start of anti-fibrotic treatment. Early identification of these patients is crucial for treatment adaptations and inclusion in clinical trials.</p>","PeriodicalId":21129,"journal":{"name":"Respirology","volume":" ","pages":""},"PeriodicalIF":6.6,"publicationDate":"2025-03-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143693135","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":"Limitations of Self-Reported Data in Evaluating COPD Phenotypes and Cardiovascular Risk.","authors":"Ibrahim Nagmeldin Hassan","doi":"10.1111/resp.70027","DOIUrl":"https://doi.org/10.1111/resp.70027","url":null,"abstract":"","PeriodicalId":21129,"journal":{"name":"Respirology","volume":" ","pages":""},"PeriodicalIF":6.6,"publicationDate":"2025-03-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143670769","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":"Turning Limitations Into Insights: The Path Forward for COPD and Cardiovascular Risk Research.","authors":"Paula Rodriguez-Miguelez, Youngdeok Kim","doi":"10.1111/resp.70028","DOIUrl":"https://doi.org/10.1111/resp.70028","url":null,"abstract":"","PeriodicalId":21129,"journal":{"name":"Respirology","volume":" ","pages":""},"PeriodicalIF":6.6,"publicationDate":"2025-03-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143664399","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":"Developments in Paediatric Interventional Pulmonology: Recent Experience in Turkiye With Broad Implications for the Field.","authors":"Aslı İmran Yılmaz, Dirk Schramm","doi":"10.1111/resp.70035","DOIUrl":"https://doi.org/10.1111/resp.70035","url":null,"abstract":"","PeriodicalId":21129,"journal":{"name":"Respirology","volume":" ","pages":""},"PeriodicalIF":6.6,"publicationDate":"2025-03-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143658440","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}