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Primary Ciliary Dyskinesia: Insights from a Portuguese tertiary centre cohort. 原发性纤毛运动障碍:来自葡萄牙高等教育中心队列的见解。
IF 5.3 2区 医学
Pulmonology Pub Date : 2026-12-01 Epub Date: 2026-07-21 DOI: 10.1080/25310429.2026.2704422
Sofia Teixeira-Oliveira, Inês Pais-Cunha, Susana S Lopes, Susana Seixas, Catarina Ferraz, Adelina Amorim, Inês Azevedo
{"title":"Primary Ciliary Dyskinesia: Insights from a Portuguese tertiary centre cohort.","authors":"Sofia Teixeira-Oliveira, Inês Pais-Cunha, Susana S Lopes, Susana Seixas, Catarina Ferraz, Adelina Amorim, Inês Azevedo","doi":"10.1080/25310429.2026.2704422","DOIUrl":"https://doi.org/10.1080/25310429.2026.2704422","url":null,"abstract":"<p><strong>Introduction: </strong>Primary Ciliary Dyskinesia (PCD) is a rare genetic disorder caused by defective ciliary structure and function, leading to chronic respiratory and systemic manifestations. Diagnostic pathways have evolved over time, but no single standalone test exists.</p><p><strong>Methods: </strong>Retrospective study of PCD patients followed at a Portuguese tertiary hospital, between 2001-2024.</p><p><strong>Results: </strong>Thirty-five patients with a confirmed diagnosis were included: 13 children and 22 adults. Median age at diagnosis was 7 years (0-16) in children and 38.5 years (12-64) in adults. Time to diagnosis decreased over the years, coinciding with a shift in the hierarchy of methods from high-speed video microscopy and transmission electron microscopy to genetic testing. The most frequent mutations were <i>DNAH5</i> (31.3%) and <i>DNAH11</i> (12.5%). Pulmonary function tended to be better in children (<i>p</i> = 0.085), whereas bronchiectasis were more extensive and bilateral in adults (<i>p</i> = 0.044 and <i>p</i> = 0.002, respectively). Children more frequently received treatment with hypertonic saline (<i>p</i> = 0.003) and adults with bronchodilators (<i>p</i> = 0.035). <i>Pseudomonas aeruginosa</i> was only identified in adults; inhaled antibiotics were only prescribed in this age group (18.2%).</p><p><strong>Conclusion: </strong>This represents the largest Portuguese cohort to date and provides relevant clinical and diagnostic insight into age-related differences, supporting the importance of early detection and intervention to limit lung damage.</p>","PeriodicalId":54237,"journal":{"name":"Pulmonology","volume":"32 1","pages":"2704422"},"PeriodicalIF":5.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148537102","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}
引用次数: 0
Clinical practice patterns and unmet needs in the use of GLP-1 receptor agonists in sleep medicine: A pan-European survey performed in the European sleep apnoea database network. 睡眠医学中GLP-1受体激动剂使用的临床实践模式和未满足的需求:在欧洲睡眠呼吸暂停数据库网络中进行的一项泛欧调查。
IF 5.3 2区 医学
Pulmonology Pub Date : 2026-12-01 Epub Date: 2026-08-25 DOI: 10.1080/25310429.2026.2722795
Dries Testelmans, Maria Rosaria Bonsignore, Athanasia Pataka, Francesco Fanfulla, Silke Ryan, Carolina Lombardi, Winfried Randerath, Ludger Grote, Alexandros Kalkanis
{"title":"Clinical practice patterns and unmet needs in the use of GLP-1 receptor agonists in sleep medicine: A pan-European survey performed in the European sleep apnoea database network.","authors":"Dries Testelmans, Maria Rosaria Bonsignore, Athanasia Pataka, Francesco Fanfulla, Silke Ryan, Carolina Lombardi, Winfried Randerath, Ludger Grote, Alexandros Kalkanis","doi":"10.1080/25310429.2026.2722795","DOIUrl":"10.1080/25310429.2026.2722795","url":null,"abstract":"<p><strong>Background: </strong>Glucagon-like peptide-1 receptor agonists (GLP-1RAs) and dual GIP/GLP-1 agonists have demonstrated weight loss benefits and reductions in obstructive sleep apnoea (OSA) severity. Following recent regulatory approval of tirzepatide for OSA with obesity, their integration into sleep medicine is accelerating. However, real-world clinical practice remains undefined.</p><p><strong>Methods: </strong>The European Sleep Apnoea Database (ESADA) network conducted a multinational online survey across 24 centres in 14 European countries to evaluate current practice patterns regarding GLP-1RA use in patients with obesity and suspected or established OSA.</p><p><strong>Results: </strong>Forty-two percent of centres reported that GLP-1RA therapy could be initiated by sleep physicians within the sleep clinic. Indications were consistent, primarily targeting moderate-to-severe obesity with metabolic comorbidities. Most clinicians continued to recommend diagnostic sleep studies in symptomatic (92%) and asymptomatic (81%) patients recently started on GLP-1RAs. A weight loss threshold > 10% was considered clinically meaningful for OSA reassessment by 61% of centres. Despite anticipated reductions in OSA severity, proactive positive airway pressure (PAP) re-titration or withdrawal was uncommon (14%). Monitoring focused on weight trajectory, symptoms, and PAP telemonitoring. Structured interdisciplinary pathways were largely absent.</p><p><strong>Conclusions: </strong>GLP-1RAs are increasingly incorporated into European sleep practice; however, substantial variability exists in diagnostic timing, PAP management, and follow-up strategies. Harmonised guidance and coordinated multidisciplinary care are urgently needed.</p>","PeriodicalId":54237,"journal":{"name":"Pulmonology","volume":"32 1","pages":"2722795"},"PeriodicalIF":5.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148814632","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}
引用次数: 0
Impact of sleep apnea on comorbidity burden and healthcare utilisation: Data from Beijing municipal medical insurance database. 睡眠呼吸暂停对合并症负担和医疗保健利用的影响:数据来自北京市医疗保险数据库。
IF 5.3 2区 医学
Pulmonology Pub Date : 2026-12-01 Epub Date: 2026-08-20 DOI: 10.1080/25310429.2026.2719420
Peining Zhou, Shuduo Zhou, Jing Ma, Guangfa Wang
{"title":"Impact of sleep apnea on comorbidity burden and healthcare utilisation: Data from Beijing municipal medical insurance database.","authors":"Peining Zhou, Shuduo Zhou, Jing Ma, Guangfa Wang","doi":"10.1080/25310429.2026.2719420","DOIUrl":"https://doi.org/10.1080/25310429.2026.2719420","url":null,"abstract":"<p><strong>Background: </strong>Sleep apnoea (SA) is prevalent and correlated with multiple comorbidities. Nevertheless, SA remains underdiagnosed and underrecognized in the Chinese population. This study aimed to evaluate the comorbidity burden and healthcare utilisation among SA patients from the Beijing Medical Insurance Database.</p><p><strong>Methods: </strong>This study extracted data from the Beijing Medical Insurance Database spanning 2016 to 2021. Patients with SA documented in the discharge diagnosis on the front page of medical records were enrolled as the case group, and one control subject matched by age, gender, and admission date was randomly assigned to each case. Propensity score matching (PSM) was performed to balance additional covariates. Primary indicators included comorbidity burden, hospitalisation frequency, length of stay, and medical expenditures. Generalised linear models were used to evaluate the associations.</p><p><strong>Results: </strong>A total of 50 379 pairs were initially identified, and 33 439 pairs were included in the final analysis after PSM. The comorbidity burden was significantly increased among SA patients. SA was independently associated with higher medical expenditures, including daily cost, per-visit cost, annual cost, and cumulative cost, as well as longer hospital length of stay and greater hospitalisation frequency. Subgroup analyses revealed that the increased healthcare utilisation was more pronounced among females, individuals aged ≥65 years, unemployed subjects, and patients with related comorbidities.</p><p><strong>Conclusion: </strong>The comorbidity burden and healthcare utilisation are significantly increased in SA patients. There is an urgent need to enhance awareness of SA as a prevalent disorder and to implement early screening and proactive interventions.</p>","PeriodicalId":54237,"journal":{"name":"Pulmonology","volume":"32 1","pages":"2719420"},"PeriodicalIF":5.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148801601","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}
引用次数: 0
Commentary on "prognostic implications of cluster-defined phenotypes in AECOPD patients with bronchiectasis: A multicenter study." 评论“簇定义表型在AECOPD合并支气管扩张患者中的预后意义:一项多中心研究。”
IF 5.3 2区 医学
Pulmonology Pub Date : 2026-12-01 Epub Date: 2026-09-15 DOI: 10.1080/25310429.2026.2728945
Etika Rana, Kashish Arora
{"title":"Commentary on \"prognostic implications of cluster-defined phenotypes in AECOPD patients with bronchiectasis: A multicenter study.\"","authors":"Etika Rana, Kashish Arora","doi":"10.1080/25310429.2026.2728945","DOIUrl":"https://doi.org/10.1080/25310429.2026.2728945","url":null,"abstract":"","PeriodicalId":54237,"journal":{"name":"Pulmonology","volume":"32 1","pages":"2728945"},"PeriodicalIF":5.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148971071","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}
引用次数: 0
Rescaling oxygen flow targets a distinct decision point, and its transition risk is an implementation question. 氧流量的重标是一个明确的决策点,其转换风险是一个实现问题。
IF 5.3 2区 医学
Pulmonology Pub Date : 2026-12-01 Epub Date: 2026-09-21 DOI: 10.1080/25310429.2026.2736357
Ángel Ortega-González, Juana María Machado-Gallas, Francisco Rodríguez-Argente
{"title":"Rescaling oxygen flow targets a distinct decision point, and its transition risk is an implementation question.","authors":"Ángel Ortega-González, Juana María Machado-Gallas, Francisco Rodríguez-Argente","doi":"10.1080/25310429.2026.2736357","DOIUrl":"https://doi.org/10.1080/25310429.2026.2736357","url":null,"abstract":"","PeriodicalId":54237,"journal":{"name":"Pulmonology","volume":"32 1","pages":"2736357"},"PeriodicalIF":5.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149013277","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}
引用次数: 0
Letter to the editor: Effect of exercise training on modulating the TH17/TREG imbalance in individuals with severe COPD: A randomised controlled trial. 致编辑的信:运动训练对调节重度COPD患者TH17/TREG失衡的影响:一项随机对照试验。
IF 5.3 2区 医学
Pulmonology Pub Date : 2026-12-01 Epub Date: 2026-01-22 DOI: 10.1080/25310429.2026.2620227
Anchal Thakur, Kanika Bhatia, Nikita Vaid
{"title":"Letter to the editor: Effect of exercise training on modulating the TH17/TREG imbalance in individuals with severe COPD: A randomised controlled trial.","authors":"Anchal Thakur, Kanika Bhatia, Nikita Vaid","doi":"10.1080/25310429.2026.2620227","DOIUrl":"10.1080/25310429.2026.2620227","url":null,"abstract":"","PeriodicalId":54237,"journal":{"name":"Pulmonology","volume":"32 1","pages":"2620227"},"PeriodicalIF":5.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146020361","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}
引用次数: 0
Letter to the Editor regarding: 'Statin use and its implications on survival and disease progression in the European MultiPartner idiopathic pulmonary fibrosis registry (EMPIRE)'. 致编辑的信关于:“他汀类药物的使用及其对欧洲多伙伴特发性肺纤维化登记(EMPIRE)的生存和疾病进展的影响”。
IF 5.3 2区 医学
Pulmonology Pub Date : 2026-12-01 Epub Date: 2026-05-26 DOI: 10.1080/25310429.2026.2678058
Qing-Qing Shan
{"title":"Letter to the Editor regarding: 'Statin use and its implications on survival and disease progression in the European MultiPartner idiopathic pulmonary fibrosis registry (EMPIRE)'.","authors":"Qing-Qing Shan","doi":"10.1080/25310429.2026.2678058","DOIUrl":"10.1080/25310429.2026.2678058","url":null,"abstract":"","PeriodicalId":54237,"journal":{"name":"Pulmonology","volume":"32 1","pages":"2678058"},"PeriodicalIF":5.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148037573","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}
引用次数: 0
High-flow oxygen during spontaneous breathing trials in mechanically ventilated patients: Key considerations. 机械通气患者自主呼吸试验中的高流量氧气:关键考虑因素。
IF 5.3 2区 医学
Pulmonology Pub Date : 2026-12-01 Epub Date: 2026-09-23 DOI: 10.1080/25310429.2026.2735691
Shaveta Mala, Shalom Akhai
{"title":"High-flow oxygen during spontaneous breathing trials in mechanically ventilated patients: Key considerations.","authors":"Shaveta Mala, Shalom Akhai","doi":"10.1080/25310429.2026.2735691","DOIUrl":"https://doi.org/10.1080/25310429.2026.2735691","url":null,"abstract":"","PeriodicalId":54237,"journal":{"name":"Pulmonology","volume":"32 1","pages":"2735691"},"PeriodicalIF":5.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149026882","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}
引用次数: 0
Cost efficiency or diagnostic compromise? Interpreting the economic and clinical trade-offs between UniAMP and Truenat for tuberculosis detection. 成本效益还是诊断妥协?解释结核检测中UniAMP和Truenat之间的经济和临床权衡。
IF 5.3 2区 医学
Pulmonology Pub Date : 2026-12-01 Epub Date: 2026-10-06 DOI: 10.1080/25310429.2026.2742720
Shaveta Mala, Suman Khanna, Uzma Abbas, Mahapara Abbass
{"title":"Cost efficiency or diagnostic compromise? Interpreting the economic and clinical trade-offs between UniAMP and Truenat for tuberculosis detection.","authors":"Shaveta Mala, Suman Khanna, Uzma Abbas, Mahapara Abbass","doi":"10.1080/25310429.2026.2742720","DOIUrl":"https://doi.org/10.1080/25310429.2026.2742720","url":null,"abstract":"","PeriodicalId":54237,"journal":{"name":"Pulmonology","volume":"32 1","pages":"2742720"},"PeriodicalIF":5.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149118340","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}
引用次数: 0
Health utilities in individuals with alpha-1 antitrypsin deficiency with and without COPD from the EARCO registry. 来自EARCO登记处的α -1抗胰蛋白酶缺乏症患者伴和不伴COPD的健康效用
IF 5.3 2区 医学
Pulmonology Pub Date : 2026-12-01 Epub Date: 2026-10-07 DOI: 10.1080/25310429.2026.2742798
Cristina Aljama, Ane Lopez-Gonzalez, Hanan Tanash, Maria Torres-Duran, Alice M Turner, Carlota Rodriguez-Garcia, Angelo Guido Corsico, Catarina Guimarães, José Luis Lopez-Campos, Jens-Ulrik Stæhr Jensen, Kenneth R Chapman, Christian F Clarenbach, Beatriz Lara, José María Hernandez-Perez, Juan Luis Rodriguez-Hermosa, Eva Bartošovská-Klinková, Jean-François Mornex, Vincent Bunel, Marc Miravitlles, Miriam Barrecheguren, Cristina Esquinas
{"title":"Health utilities in individuals with alpha-1 antitrypsin deficiency with and without COPD from the EARCO registry.","authors":"Cristina Aljama, Ane Lopez-Gonzalez, Hanan Tanash, Maria Torres-Duran, Alice M Turner, Carlota Rodriguez-Garcia, Angelo Guido Corsico, Catarina Guimarães, José Luis Lopez-Campos, Jens-Ulrik Stæhr Jensen, Kenneth R Chapman, Christian F Clarenbach, Beatriz Lara, José María Hernandez-Perez, Juan Luis Rodriguez-Hermosa, Eva Bartošovská-Klinková, Jean-François Mornex, Vincent Bunel, Marc Miravitlles, Miriam Barrecheguren, Cristina Esquinas","doi":"10.1080/25310429.2026.2742798","DOIUrl":"10.1080/25310429.2026.2742798","url":null,"abstract":"<p><strong>Background: </strong>Data on health utilities in Alpha-1 antitrypsin deficiency (AATD) are scarce. This study aimed to evaluate health-related quality of life (HRQoL) using the EQ-5D in a cohort of AATD Pi*ZZ individuals from the European AATD Research Collaboration (EARCO) registry and to identify factors associated with impaired health utilities.</p><p><strong>Methods: </strong>Observational, cross-sectional, multicenter study including AATD patients with Pi*ZZ genotype enrolled up to April 2025. HRQoL was assessed using the EQ-5D questionnaire (index and visual analogue scale (VAS)). Sociodemographic data, smoking history, respiratory symptoms, comorbidities, lung function and exacerbations were collected. Multivariable generalised linear models were used to identify factors independently associated with EQ-5D index scores in the overall population and in patients with AATD-COPD.</p><p><strong>Results: </strong>A total of 1710 Pi*ZZ individuals were analysed with a mean age 55.2 ± 13.8 years, 51% were male and 49.6% had COPD. Mean EQ-5D index and VAS were 0.82 ± 0.22 and 62.9 ± 25.9, respectively. Patients with COPD had significantly worse EQ-5D index (0.75 ± 0.24 vs 0.90 ± 0.16) and VAS scores (56.3 ± 24.4 vs 70.4 ± 26.8; both <i>p</i> < 0.001). In multivariable analyses, higher symptom burden (CAT, mMRC), long-term oxygen therapy, being unemployed, exacerbations, COPD, bronchiectasis and lower educational level were independently associated with worse EQ-5D scores in Pi*ZZ subjects.</p><p><strong>Conclusions: </strong>HRQoL was substantially impaired in Pi*ZZ individuals, particularly in those with COPD. Symptom burden and exacerbations were stronger determinants of EQ-5D than lung function alone in all Pi*ZZ patients. Patients with AATD-associated COPD exhibited an HRQoL impairment comparable to that observed in usual COPD populations, but at a younger age.</p>","PeriodicalId":54237,"journal":{"name":"Pulmonology","volume":"32 1","pages":"2742798"},"PeriodicalIF":5.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149129179","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}
引用次数: 0
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