Respirology最新文献

{"title":"Molecular Investigation in Early-Onset Interstitial Lung Disease: Results From 699 Unrelated Patients.","authors":"Camille Louvrier, Nadia Nathan, Vincent Cottin, Tifenn Desroziers, Valérie Nau, Yohan Soreze, Florence Dastot-Le Moal, Philippe Reix, Diane Bouvry, Caroline Thumerelle, Martine Reynaud-Gaubert, Alice Hadchouel, Grégoire Prévot, Effrosyni Manali, Caroline Kannengiesser, Ibrahima Ba, Serge Amselem, Véronique Houdouin, Raphaël Borie, Marie Legendre","doi":"10.1111/resp.70132","DOIUrl":"https://doi.org/10.1111/resp.70132","url":null,"abstract":"<p><strong>Background and objective: </strong>Interstitial lung diseases (ILDs) are rare and severe respiratory conditions that may ultimately result in pulmonary fibrosis (PF). The objective of this study was to present the results of molecular diagnosis of early-onset ILD (from neonates to young adults < 50 years) in a reference genetic diagnostic laboratory.</p><p><strong>Methods: </strong>DNAs from 699 index cases and 190 relatives were studied over 6 years by Sanger and/or targeted next generation sequencing of surfactant-related genes and other genes involved in early-onset ILD.</p><p><strong>Results: </strong>Pathogenic/likely pathogenic variants were evidenced for 62 patients (8.9%). The genes most frequently involved were SFTPA2 (13/62), followed by ABCA3 (12/62) and SFTPC (10/62). Among index cases for whom precise clinical data were available (n = 542), indications associated with a high molecular diagnostic yield were pulmonary alveolar proteinosis (61.5%, 8/13; p < 0.0007); family history of ILD/PF and lung cancer (36.8%, 7/19; p = 0.0132) and newborns > 32 weeks gestation with neonatal respiratory distress (14.8%, 9/61). The proportion of positive molecular investigations culminated in two age groups over the lifespan: 23.3% (7/30) in children aged 1 to 10 years, and 18.3% (15/82) in adults aged 30 to 40 years. Over the 6-year period, 190 relatives were subjected to testing in order to perform segregation studies (n = 123) and/or predictive testing (n = 79).</p><p><strong>Conclusion: </strong>This study highlights the specific patient's characteristics associated with a high or low molecular diagnostic yield in clinical practice. Furthermore, it emphasises the importance of establishing a molecular diagnosis in order to provide genetic counselling to the family.</p>","PeriodicalId":21129,"journal":{"name":"Respirology","volume":" ","pages":""},"PeriodicalIF":6.3,"publicationDate":"2025-10-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145213545","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":"Lobe-Specific Variability in Postoperative Pulmonary Function in Lung Cancer Patients: A Longitudinal Analysis and Comparison With Traditional Predictive Models.","authors":"Yeong Jeong Jeon, Sumin Shin, Sunga Kong, Seongwoo Yang, Jong Ho Cho, Hong Kwan Kim, Young Mog Shim, Danbee Kang, Hye Yun Park","doi":"10.1111/resp.70133","DOIUrl":"https://doi.org/10.1111/resp.70133","url":null,"abstract":"<p><strong>Background and objective: </strong>Lobectomy is the primary treatment for early-stage non-small cell lung cancer. However, pulmonary function declines postoperatively, presenting a significant challenge. This study investigated pulmonary function following lobectomy and compared these changes with predicted lung function based on the traditional segmental method, focusing on resected lobe-specific variability.</p><p><strong>Methods: </strong>This prospective cohort study included 419 patients who underwent lobectomies for NSCLC. Pulmonary function tests measuring forced vital capacity (FVC), forced expiratory volume in one second (FEV₁), and diffusion capacity for carbon monoxide (DLCO) were conducted preoperatively and at 1, 3, 6, and 12 months postoperatively. We compared the observed pulmonary function values to the predicted postoperative (PPO) values based on the traditional segmental method and analysed lobe-specific differences using mixed-effects models.</p><p><strong>Results: </strong>Left upper and right lower lobectomies demonstrated observed pulmonary function values similar to their PPOs as early as 2 weeks postoperatively. However, the other lobes declined significantly, with an approximately 25% reduction in FVC, FEV1, and DLCO, regardless of the number of segments in the lobe. Three months postoperatively, observed pulmonary function values exceeded PPO values across all lobes. Surgical approach (open vs. video-assisted thoracoscopic surgery), right upper lobectomy in the reference to right lower lobectomy, and postoperative decreased physical activity were significant factors related to the non-recovery of lung function to their PPOs at 6 months after surgery.</p><p><strong>Conclusion: </strong>Postoperative pulmonary function recovery varied significantly by resected lobe, with most lobes showing better recovery than predicted postoperative values. Accurate outcome prediction requires accounting for lobe-specific characteristics and compensatory mechanisms.</p>","PeriodicalId":21129,"journal":{"name":"Respirology","volume":" ","pages":""},"PeriodicalIF":6.3,"publicationDate":"2025-10-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145207501","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":"Inter-Night Variability of Nocturnal Pulse Oximetry in People Living With Motor Neuron Disease: A Retrospective Observational Study.","authors":"Steven Luu, Olivia McGuiness, Collette Menadue, Amanda J Piper, Keith Wong, Brendon J Yee, Emma L Gray","doi":"10.1111/resp.70072","DOIUrl":"10.1111/resp.70072","url":null,"abstract":"<p><strong>Background and objective: </strong>Nocturnal pulse oximetry (NPO) is a simple and inexpensive assessment tool that has previously been shown to correlate with prognosis and timing of non-invasive ventilation (NIV) initiation in people living with motor neuron disease (plwMND). However, the optimal number of nights for measuring NPO has not been defined for this population, with other respiratory conditions exhibiting both low and high night-to-night variability in NPO parameters. This study aims to determine the inter-night variability in NPO data over three nights in plwMND.</p><p><strong>Methods: </strong>We conducted a retrospective analysis of 132 studies in which plwMND underwent three consecutive nights of NPO. Intraclass correlation coefficients (ICC) were used to assess the reliability of key NPO parameters, including mean percentage of total recording time with oxygen saturation (SpO₂) < 90% (T90), oxygen desaturation index (ODI), basal SpO₂ and nadir SpO₂. The proportion of plwMND meeting NIV criteria based on single-night versus multi-night assessments was also compared.</p><p><strong>Results: </strong>Excellent reliability was observed for T90 (ICC(1) = 0.940) and ODI (ICC(1) = 0.901), while basal SpO₂ (ICC(1) = 0.845) and nadir SpO₂ (ICC(1) = 0.768) demonstrated good reliability. However, relying on a single-night NPO assessment failed to identify 12% of plwMND who met NIV criteria when evaluated over three nights.</p><p><strong>Conclusion: </strong>Despite good to excellent inter-night variability of NPO data in plwMND, multi-night NPO monitoring improves the accuracy of identifying plwMND requiring NIV. These findings support the need for multi-night assessments to enhance clinical decision-making in MND management.</p>","PeriodicalId":21129,"journal":{"name":"Respirology","volume":" ","pages":"995-1002"},"PeriodicalIF":6.3,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12486346/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144267145","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":"Recommendations From the Medical Education Editor.","authors":"Mark Lavercombe","doi":"10.1111/resp.70126","DOIUrl":"10.1111/resp.70126","url":null,"abstract":"","PeriodicalId":21129,"journal":{"name":"Respirology","volume":" ","pages":"914-916"},"PeriodicalIF":6.3,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145001481","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":"Cheap, Convenient, and Quality Healthcare-Pick Any Two.","authors":"Robert J Hancox","doi":"10.1111/resp.70094","DOIUrl":"10.1111/resp.70094","url":null,"abstract":"","PeriodicalId":21129,"journal":{"name":"Respirology","volume":" ","pages":"1003-1004"},"PeriodicalIF":6.3,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144626984","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":"Hospital-At-Home for Respiratory Diseases-Opportunities and Challenges.","authors":"Stephanie Q Ko, Adrian Kee","doi":"10.1111/resp.70131","DOIUrl":"https://doi.org/10.1111/resp.70131","url":null,"abstract":"","PeriodicalId":21129,"journal":{"name":"Respirology","volume":" ","pages":""},"PeriodicalIF":6.3,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145207454","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":"Melioidosis in Asia-Pacific Nations: Expanding Boundaries but Unknowns Remain.","authors":"Bart J Currie, Ella M Meumann","doi":"10.1111/resp.70098","DOIUrl":"10.1111/resp.70098","url":null,"abstract":"","PeriodicalId":21129,"journal":{"name":"Respirology","volume":" ","pages":"917-919"},"PeriodicalIF":6.3,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12486359/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144744558","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":"Non-Intubated VATS for Interstitial Lung Disease Diagnosis: A Safer Technique or Too Soon to Tell?","authors":"Nathan J Mortimer, Lauren K Troy","doi":"10.1111/resp.70085","DOIUrl":"10.1111/resp.70085","url":null,"abstract":"","PeriodicalId":21129,"journal":{"name":"Respirology","volume":" ","pages":"910-911"},"PeriodicalIF":6.3,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144507981","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":"Effectiveness of Perioperative Radiotherapy With Systemic Therapy on Survival of Squamous Cell Lung Cancer Patients With Pathologic N2.","authors":"Yongzheng Yao, Chang Liu, Fei Pei, Changqiang Zhang, Xiaoqing Cao","doi":"10.1111/resp.70078","DOIUrl":"10.1111/resp.70078","url":null,"abstract":"<p><strong>Background and objective: </strong>The role of perioperative radiotherapy in squamous cell lung cancer patients with pathologic N2 remains unclear. The purpose of this study was to explore the effectiveness of preoperative radiotherapy (PRRT) and postoperative radiotherapy (PORT) on survival.</p><p><strong>Methods: </strong>Patients with pathologic N2 stage III squamous cell lung cancer who received PRRT combined with preoperative systemic therapy (PRST) or PORT combined with postoperative systemic therapy (POST) were identified in the Surveillance, Epidemiology, and End Results database from 2000 through 2021. Propensity score matching (PSM) was used for the matching of patients (1:1 ratio). Overall survival (OS) was compared among the different groups by using Kaplan-Meier analysis.</p><p><strong>Results: </strong>A total of 2132 patients were included and 344 patients were excluded. After PSM, 105 patients received PRST combined with PRRT while 105 patients received PRST alone; 446 patients received POST combined with PORT while 446 patients received POST alone. For PRST combined with/without PRRT, median OS for radiotherapy was 80 months (95% confidence interval [CI], 60 to 121), which was significantly longer than that without radiotherapy (40 months [95% CI, 30 to 70]; p = 0.016); and the hazard ratio for radiotherapy was 0.65 (95% CI, 0.46 to 0.92) compared with no radiotherapy. For POST combined with/without PORT, there was no significant difference between radiotherapy and no radiotherapy for median OS (34 months [95% CI, 30 to 42] for radiotherapy versus 41 months [95% CI, 34 to 51] for no radiotherapy; p = 0.17).</p><p><strong>Conclusion: </strong>Our results revealed that PRST combined with PRRT significantly improved OS.</p>","PeriodicalId":21129,"journal":{"name":"Respirology","volume":" ","pages":"987-994"},"PeriodicalIF":6.3,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144529458","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":"Exploring Benefits of Non-Intubated Surgery for Lung Biopsy in Interstitial Lung Disease.","authors":"Aude Nguyen, Timothée Jouitteau, Arnaud Bourdin, Anne-Sophie Gamez, Laurence Solovei, José Molina, Kheira Hireche","doi":"10.1111/resp.70067","DOIUrl":"10.1111/resp.70067","url":null,"abstract":"<p><strong>Background and objectives: </strong>General anaesthesia with intubation is the standard technique for video-assisted thoracic surgery (VATS). However, non-intubated VATS (NIVATS) is emerging as a less invasive alternative, particularly for lung biopsies in patients with interstitial lung disease (ILD) and impaired respiratory function. This study aims to investigate the benefits of non-intubated surgery on postoperative respiratory function and to compare operative outcomes between intubated VATS (IVATS) and NIVATS.</p><p><strong>Methods: </strong>We conducted a single-centre retrospective study of patients who underwent lung biopsy for ILD diagnosis via VATS between January 2020 and September 2023 at Montpellier University Hospital. Primary outcomes included the variation in pulmonary function tests after surgery (FEV₁, FVC, DLCO). Secondly, this study compares clinical postoperative outcomes and diagnostic yield between the two groups.</p><p><strong>Results: </strong>The study included 61 patients, with 42% (n = 26) undergoing NIVATS. The NIVATS group showed a significantly smaller decrease in postoperative FEV₁ and FVC compared to the IVATS group (p = 0.001 for both variables). Additionally, NIVATS was associated with shorter operating room time (247 min vs. 288 min, p = 0.013), reduced hospital stay duration (47.5 h vs. 65.2 h, p = 0.018), and decreased need for additional analgesics (3% vs. 15%, p = 0.001). Diagnostic yield was at least similar to IVATS. There were no significant differences in DLCO or chest tube duration between the two groups.</p><p><strong>Conclusions: </strong>NIVATS offers better preservation of respiratory function, a good diagnostic yield, and improved overall outcomes following surgical lung biopsy in patients with ILD and could be a preferred technique for diagnosis.</p>","PeriodicalId":21129,"journal":{"name":"Respirology","volume":" ","pages":"949-956"},"PeriodicalIF":6.3,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12486366/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144226453","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}