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Baseline Sinonasal Phenotypes in Cystic Fibrosis Before Elexacaftor/Tezacaftor/ Ivacaftor Therapy: A Cross-Sectional Analysis. 在elexaftor /Tezacaftor/ Ivacaftor治疗前囊性纤维化的基线鼻窦表型:一项横断面分析。
IF 3.3 3区 医学
Respiratory medicine Pub Date : 2026-10-14 DOI: 10.1016/j.rmed.2026.109185
Rebecca Gallardo, Rodrigo Delpino Gehrke, Lorenzo Silvestrin Sartorelli, Andrea Teixeira de Almeida, Bruna Santos Silva, Ligia Machado, José Dirceu Ribeiro, Eulalia Sakano
{"title":"Baseline Sinonasal Phenotypes in Cystic Fibrosis Before Elexacaftor/Tezacaftor/ Ivacaftor Therapy: A Cross-Sectional Analysis.","authors":"Rebecca Gallardo, Rodrigo Delpino Gehrke, Lorenzo Silvestrin Sartorelli, Andrea Teixeira de Almeida, Bruna Santos Silva, Ligia Machado, José Dirceu Ribeiro, Eulalia Sakano","doi":"10.1016/j.rmed.2026.109185","DOIUrl":"https://doi.org/10.1016/j.rmed.2026.109185","url":null,"abstract":"<p><strong>Background: </strong>Sinonasal disease is highly prevalent in cystic fibrosis (CF), yet pre-treatment disease burden remains incompletely characterized across CFTR genotype classes. As CFTR modulator therapy reshapes disease trajectories, establishing a genotype-stratified baseline of upper-airway disease before elexacaftor/ tezacaftor/ivacaftor (ETI) initiation is essential for contextualizing subsequent sinonasal responses.</p><p><strong>Methods: </strong>In this cross-sectional study, 45 individuals with CF underwent pre-ETI evaluation, including the SNOT-22 questionnaire, visual analog scale (VAS) for nasal obstruction, Lund-Kennedy (LK) endoscopic scoring, Lund-Mackay (LM) CT staging, and CCCRC olfactory identification testing. Disease burden was compared between heterozygous and F508del homozygous genotypes. Heterozygous patients were further stratified by the genetic class of the cystic fibrosis-causing mutation, ranging from class I to class VI, according to the type of mutation, and sinonasal outcomes were additionally compared by exocrine pancreatic status. Associations among symptoms, structural, and functional measures were assessed using Spearman correlations.</p><p><strong>Results: </strong>Mean VAS nasal obstruction was 4.15 ± 2.30 and mean SNOT-22 score 14.4 ± 11.2, indicating mild to moderate symptom burden. LK endoscopy showed mild to moderate disease (4.09 ± 1.54) and LM CT substantial opacification (14.17 ± 4.62). Olfactory scores varied widely (8.63 ± 3.08, D+E composite). No significant differences were found between genotype groups across any domain. VAS correlated strongly with SNOT-22 (ρ = 0.54, p < 0.001) and inversely with olfaction (ρ = -0.42, p = 0.02), but not with endoscopic or radiologic severity. Among heterozygotes, no domain differed between minimal-function and residual-function classes (VAS p = 0.129; SNOT-22 p = 0.177; LK p = 0.878; LM p = 0.225; olfaction p = 0.357), and none survived false-discovery-rate correction. Sinonasal scores likewise did not differ by exocrine pancreatic status, an analysis limited to 13 patients and therefore substantially underpowered.</p><p><strong>Conclusions: </strong>Individuals with CF exhibit measurable sinonasal disease across symptomatic, structural, and functional domains before ETI therapy. The absence of significant differences by genotype, by CFTR functional class, and by pancreatic status indicates that baseline upper-airway disease is largely independent of CFTR functional severity as conventionally classified. These findings establish a multidomain pre-treatment reference for future longitudinal assessment of sinonasal and olfactory responses to CFTR modulator therapy.</p>","PeriodicalId":21057,"journal":{"name":"Respiratory medicine","volume":" ","pages":"109185"},"PeriodicalIF":3.3,"publicationDate":"2026-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149184193","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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
Tofacitinib in rheumatoid arthritis-associated interstitial lung disease: immunomodulation and antifibrotic therapy as complementary strategies? 托法替尼治疗类风湿关节炎相关间质性肺疾病:免疫调节和抗纤维化治疗作为互补策略?
IF 3.3 3区 医学
Respiratory medicine Pub Date : 2026-10-14 DOI: 10.1016/j.rmed.2026.109216
Freddy Liñán-Ponce
{"title":"Tofacitinib in rheumatoid arthritis-associated interstitial lung disease: immunomodulation and antifibrotic therapy as complementary strategies?","authors":"Freddy Liñán-Ponce","doi":"10.1016/j.rmed.2026.109216","DOIUrl":"https://doi.org/10.1016/j.rmed.2026.109216","url":null,"abstract":"","PeriodicalId":21057,"journal":{"name":"Respiratory medicine","volume":" ","pages":"109216"},"PeriodicalIF":3.3,"publicationDate":"2026-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149184210","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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
Yoga Effect on Quality of Life Study Among Patients with Idiopathic Pulmonary Fibrosis (YES-IPF). 瑜伽对特发性肺纤维化患者生活质量的影响研究(YES-IPF)。
IF 3.3 3区 医学
Respiratory medicine Pub Date : 2026-10-14 DOI: 10.1016/j.rmed.2026.109194
Suha Kadura, Soumik Purkayastha, Josh Benditt, Amit Anand, Bridget Collins, Miguele De Quadros, Mafara Hobson, M J Biswas, Lawrence Ho, Cathie Spino, Ganesh Raghu
{"title":"Yoga Effect on Quality of Life Study Among Patients with Idiopathic Pulmonary Fibrosis (YES-IPF).","authors":"Suha Kadura, Soumik Purkayastha, Josh Benditt, Amit Anand, Bridget Collins, Miguele De Quadros, Mafara Hobson, M J Biswas, Lawrence Ho, Cathie Spino, Ganesh Raghu","doi":"10.1016/j.rmed.2026.109194","DOIUrl":"https://doi.org/10.1016/j.rmed.2026.109194","url":null,"abstract":"<p><strong>Rationale: </strong>Patients with idiopathic pulmonary fibrosis (IPF) have poor health-related quality of life.</p><p><strong>Objective: </strong>To explore the feasibility and potential impact of a modified yoga program on health-related quality of life in patients with IPF compared with usual care.</p><p><strong>Methods: </strong>We conducted a randomized, controlled, nonblinded, 12-week pilot clinical trial with two arms involving 63 adults with IPF diagnosed according to the 2018 ATS/ERS/JRS/ALAT clinical practice guideline. The yoga arm attended one-hour virtual group sessions twice weekly for 12 weeks, consisting of seated postures, breathing exercises, and meditation designed for patients with IPF, while the control arm continued usual care.</p><p><strong>Measure: </strong>ments: Health-related quality of life was assessed using seven patient-reported outcome measures and their associated domains.</p><p><strong>Results: </strong>Sixty of 63 randomized participants completed the week-12 assessment and comprised the analysis cohort; their mean age was 73.4 years (SD, 8.2). Statistically significant improvements favoring yoga were seen in the Living with Idiopathic Pulmonary Fibrosis (L-IPF) cough domain (-9.29 points, 95% CI -18.37 to -0.20; p=0.045; SMD=-0.36), the L-IPF total score (-7.11, 95% CI -13.15 to -1.06; p=0.022; SMD=-0.38), and the R-scale-PF cough domain (-1.18, 95% CI -2.27 to -0.10; p=0.034; SMD=-0.45).</p><p><strong>Conclusion: </strong>In this exploratory pilot trial, a modified yoga program was feasible and well tolerated and was associated with improvements in selected patient-reported outcome domains. These findings are hypothesis-generating and warrant evaluation in a larger, adequately powered trial.</p>","PeriodicalId":21057,"journal":{"name":"Respiratory medicine","volume":" ","pages":"109194"},"PeriodicalIF":3.3,"publicationDate":"2026-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149184158","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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
Treatable trait in COPD: is LVDD the potential next? 慢性阻塞性肺病的可治疗特征:下一个可能是LVDD吗?
IF 3.3 3区 医学
Respiratory medicine Pub Date : 2026-10-13 DOI: 10.1016/j.rmed.2026.109212
Pallav Bhattacharyya, Sumedha Biswas, Parthasarathi Bhattacharyya
{"title":"Treatable trait in COPD: is LVDD the potential next?","authors":"Pallav Bhattacharyya, Sumedha Biswas, Parthasarathi Bhattacharyya","doi":"10.1016/j.rmed.2026.109212","DOIUrl":"10.1016/j.rmed.2026.109212","url":null,"abstract":"","PeriodicalId":21057,"journal":{"name":"Respiratory medicine","volume":" ","pages":"109212"},"PeriodicalIF":3.3,"publicationDate":"2026-10-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149184131","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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
Association between obesity and asthma among Arab Israeli adolescents compared with Jewish Israeli peers. 阿拉伯裔以色列青少年与犹太裔以色列青少年肥胖与哮喘的关系
IF 3.3 3区 医学
Respiratory medicine Pub Date : 2026-10-12 DOI: 10.1016/j.rmed.2026.109214
Yulia Treister-Goltzman, Idan Menashe, Dan Nemet
{"title":"Association between obesity and asthma among Arab Israeli adolescents compared with Jewish Israeli peers.","authors":"Yulia Treister-Goltzman, Idan Menashe, Dan Nemet","doi":"10.1016/j.rmed.2026.109214","DOIUrl":"https://doi.org/10.1016/j.rmed.2026.109214","url":null,"abstract":"<p><strong>Background: </strong>Ethnic differences in the health implications of adolescent obesity, including asthma, are understudied in Israel. We compared associations between adolescent weight categories and asthma among Arab Israeli and Jewish Israeli adolescents.</p><p><strong>Methods: </strong>This retrospective cross-sectional study used the computerized database of Clalit Health Services (CHS). It included Arab Israeli adolescents aged 14-19 years with at least one recorded height and weight measurement between 2007 and 2022. A Jewish Israeli comparison group meeting the same eligibility criteria was selected, with a socioeconomic status distribution corresponding to that of Arab Israeli adolescents. The final sample included 313,936 Arab Israeli and 289,616 Jewish Israeli adolescents. Logistic regression assessed associations between weight categories and asthma overall and asthma with inhaled corticosteroid (ICS), inhaled bronchodilator (IBD), or systemic corticosteroid (SCS) use, separately by group.</p><p><strong>Results: </strong>Higher weight categories were associated with higher odds of asthma, with similar patterns in both groups. Among adolescents with class 3 obesity, adjusted odds ratios (95% CI) were 1.52 (1.35-1.70) for asthma overall, 2.02 (1.23-3.10) for asthma with ICS use, 2.81 (1.49-4.78) for asthma with IBD use, and 3.81 (2.16-6.19) for asthma with SCS use. Jewish Israeli adolescents had higher ICS use, whereas Arab Israeli adolescents had higher SCS use.</p><p><strong>Conclusions: </strong>Asthma management should address obesity through healthy diet and physical activity while considering lifestyle differences between Arab Israeli and Jewish Israeli adolescents. Greater ICS use and reduced SCS use among Arab Israeli adolescents may warrant attention.</p>","PeriodicalId":21057,"journal":{"name":"Respiratory medicine","volume":" ","pages":"109214"},"PeriodicalIF":3.3,"publicationDate":"2026-10-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149170471","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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
A Decade of Asthma Emergency Department Presentations in New South Wales: Temporal, Seasonal and Age-Specific Trends. 十年的哮喘急诊科报告在新南威尔士州:时间,季节和年龄特定的趋势。
IF 3.3 3区 医学
Respiratory medicine Pub Date : 2026-10-12 DOI: 10.1016/j.rmed.2026.109213
Jahidur Rahman Khan, Adam Jaffé, Nusrat Homaira
{"title":"A Decade of Asthma Emergency Department Presentations in New South Wales: Temporal, Seasonal and Age-Specific Trends.","authors":"Jahidur Rahman Khan, Adam Jaffé, Nusrat Homaira","doi":"10.1016/j.rmed.2026.109213","DOIUrl":"https://doi.org/10.1016/j.rmed.2026.109213","url":null,"abstract":"<p><p>Asthma remains a major contributor to emergency department (ED) utilisation. Although international studies have described temporal, seasonal and age-specific patterns of asthma-related ED utilisation, contemporary Australian evidence remains limited. We conducted a retrospective population-based ecological time-series study of monthly asthma-related ED presentations in New South Wales, Australia, between 2015 and 2024. A total of 231,670 presentations were recorded, averaging 1,931 per month (24.1/100,000 population). Children aged 0-15 years experienced the highest presentation rates. Rates showed marked seasonal variation, peaking in May and June (30.2 and 29.3/100,000 population respectively). These findings support targeted prevention and healthcare resource planning during peak season.</p>","PeriodicalId":21057,"journal":{"name":"Respiratory medicine","volume":" ","pages":"109213"},"PeriodicalIF":3.3,"publicationDate":"2026-10-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149166306","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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
Long-Term Psychological, Cognitive and Quality of Life Outcomes in Covid-19 Severe Acute Respiratory Failure patients managed with Extracorporeal Membrane Oxygenation. 体外膜氧合治疗Covid-19重症急性呼吸衰竭患者的长期心理、认知和生活质量
IF 3.3 3区 医学
Respiratory medicine Pub Date : 2026-10-08 DOI: 10.1016/j.rmed.2026.109205
V E R Singh, B E Garfield, S Singh, A-M Doyle
{"title":"Long-Term Psychological, Cognitive and Quality of Life Outcomes in Covid-19 Severe Acute Respiratory Failure patients managed with Extracorporeal Membrane Oxygenation.","authors":"V E R Singh, B E Garfield, S Singh, A-M Doyle","doi":"10.1016/j.rmed.2026.109205","DOIUrl":"https://doi.org/10.1016/j.rmed.2026.109205","url":null,"abstract":"<p><strong>Objectives: </strong>ICU admission with severe acute respiratory failure (SARF) can result in extended recovery and rehabilitation. We sought to evaluate the long-term psychological, cognitive and quality of life outcomes of Covid-19 SARF patients to inform our understanding of the longer-term health burden to survivors.</p><p><strong>Design: </strong>Retrospective single centre cohort study.</p><p><strong>Setting and patients: </strong>All surviving SARF Covid-19 patients admitted to a regional ECMO centre in London between March 2020 - February 2022 attending ICU follow-up clinic.</p><p><strong>Interventions: </strong>None MEASUREMENTS AND MAIN RESULTS: CORE ICU validated outcome measures alongside clinical interview. Of the surviving (n = 116) patients, 97 (83.6%) attended at least one ICU follow-up clinic. For those attending multiple reviews, despite an improvement in quality of life, depression, anxiety and PTSD were no different between 0 - 2 years and 2 - 4 years after discharge. Comprehensive long-term reviews held between 18 months and 4 years [ IQR 17.5 m - 44.8 m ] following discharge, n = 87) found prevalence of depression (44%), anxiety (25%) and PTSD (33%). Half were still receiving care from specialist healthcare teams. Cognitive impairment was seen in 63% and functional impairment in 31%. Over a quarter (26%) remained not working. Depression and PTSD scores were lower in those who had returned to work. Thematic analysis of long-term reviews revealed six key themes: a plateaued recovery, the traumatising experience, forgetfulness, family strain, appreciation and gratitude, and awareness of the fragility of life.</p><p><strong>Conclusion: </strong>Despite improvements to quality of life, impairments in psychological, cognitive and aspects of health-related quality of life can persist as long as four years after discharge in SARF ECMO survivors. Qualitative findings illustrated the enduring impact of critical illness alongside themes of gratitude an altered life perspective.</p>","PeriodicalId":21057,"journal":{"name":"Respiratory medicine","volume":" ","pages":"109205"},"PeriodicalIF":3.3,"publicationDate":"2026-10-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149138087","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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
Pleuroparenchymal fibroelastosis in a UK tertiary ILD centre: determinants of radiological progression and survival. 英国三级ILD中心的胸膜实质纤维弹性增生:放射学进展和生存的决定因素。
IF 3.3 3区 医学
Respiratory medicine Pub Date : 2026-10-03 DOI: 10.1016/j.rmed.2026.109168
S L Barratt, A Cotton, P White, M Wells, J Hardy, H Adamali, A Edey
{"title":"Pleuroparenchymal fibroelastosis in a UK tertiary ILD centre: determinants of radiological progression and survival.","authors":"S L Barratt, A Cotton, P White, M Wells, J Hardy, H Adamali, A Edey","doi":"10.1016/j.rmed.2026.109168","DOIUrl":"10.1016/j.rmed.2026.109168","url":null,"abstract":"<p><p>Pleuroparenchymal fibroelastosis (PPFE) is a rare form of interstitial lung disease (ILD). Some studies have shown that IPF patients with PPFE-like features have accelerated progression, poorer prognosis, and shorter telomeres. We examined the clinical, radiological, and physiological features of a UK PPFE cohort to identify the predictors of clinical outcomes.</p><p><strong>Methods: </strong>A retrospective analysis was performed of consecutive patients with a multidisciplinary team (MDT) diagnosis of PPFE (February 2018 - 2026). A convenience sample of 70 consecutive IPF (Idiopathic pulmonary fibrosis) patients was used as a comparator cohort. Baseline demographics, complications, pulmonary function decline at 1-year, radiological progression, and mortality were assessed. Radiological progression was determined by visual scoring of serial CT scans. Kaplan-Meier survival analysis with log-rank testing compared survival between cohorts, and multivariable Cox regression identified predictors of PPFE radiological progression and mortality.</p><p><strong>Results: </strong>Forty-three patients had an MDT diagnosis of PPFE. Compared with IPF patients, those with PPFE were younger (71 vs 75 years), more frequently female (55.8% vs 15.7%), and more likely to be never-smokers (60.5% vs 18.6%). PPFE patients had lower BMI, lower FEV1, higher TLCO, and were more likely to have a history of recurrent infections, pneumothorax, or pneumomediastinum (all p < 0.05), while FVC was comparable between groups. Co-existing ILD was present in 53% of PPFE patients, most commonly definite or probable UIP (65%). Among patients who underwent genetic testing, 88% (7/8) had a telomere biology-related gene variant. There were no significant differences in one-year lung function decline or overall survival between PPFE and IPF patients, irrespective of co-existing ILD. Baseline FVC% predicted independently predicted radiological progression, while age, baseline FVC% predicted, and change in FVC% predicted were independent predictors of mortality in PPFE.</p><p><strong>Conclusions: </strong>PPFE exhibits a distinctive phenotype characterised by younger age, female predominance, low BMI and frequent pleural complications, survival was comparable to IPF. Baseline FVC emerged as a common predictor of both mortality and radiological progression, supporting its use as a pragmatic prognostic marker while highlighting the need for prospective validation.</p>","PeriodicalId":21057,"journal":{"name":"Respiratory medicine","volume":" ","pages":"109168"},"PeriodicalIF":3.3,"publicationDate":"2026-10-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149114356","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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
Real-world efficacy, safety and heterogenicity of efficacy of elexacaftor-tezacaftor-ivacaftor in younger children (6-11 years old) with cystic fibrosis. Elexacaftor-Tezacaftor-Ivacaftor治疗6-11岁囊性纤维化儿童的实际疗效、安全性和异质性
IF 3.3 3区 医学
Respiratory medicine Pub Date : 2026-10-01 Epub Date: 2026-07-31 DOI: 10.1016/j.rmed.2026.109063
Shahid Sheikh, Melissa Holtzlander, Mariah Eisner, Courtney Gelpi, Sabrina Palacios, Eric Mull, Sehyr Imran, Kavitha Kotha, Karen S McCoy
{"title":"Real-world efficacy, safety and heterogenicity of efficacy of elexacaftor-tezacaftor-ivacaftor in younger children (6-11 years old) with cystic fibrosis.","authors":"Shahid Sheikh, Melissa Holtzlander, Mariah Eisner, Courtney Gelpi, Sabrina Palacios, Eric Mull, Sehyr Imran, Kavitha Kotha, Karen S McCoy","doi":"10.1016/j.rmed.2026.109063","DOIUrl":"10.1016/j.rmed.2026.109063","url":null,"abstract":"<p><strong>Background: </strong>Heterogenicity of efficacy of elexacaftor-tezacaftor-ivacaftor (ETI) therapy in the real world in younger children (6-11 years old) is not known.</p><p><strong>Methods: </strong>This is a longitudinal observational study. Clinical data at baseline and at one-year of therapy were compared for the total cohort and for following subgroups: genotype (homozygous vs. heterozygous for F508del), severity of lung disease at ETI initiation (ppFEV<sub>1</sub> <80 vs. ≥80) and naïve vs. previously exposed to cystic fibrosis transmembrane conductance regulator (CFTR) modulators.</p><p><strong>Results: </strong>Among the total cohort of 70 chCF (children with cystic fibrosis), median (interquartile range) age was 9.0 (6.4, 10.0) years, 40 (57%) were homozygous for F508del, 52 (80%) had ppFEV<sub>1</sub> ≥80 at baseline and 31 (44%) were naive to previous CFTR modulator therapies. Significant increases in mean ppFEV<sub>1</sub> (+4.8%, p = 0.002) and mean body mass index (BMI) (+0.68 kg/m<sup>2</sup>, p < 0.001) were observed at one year of ETI therapy, limited to homozygous and naïve subgroups and those with lower ppFEV<sub>1</sub> at baseline. After one-year on ETI therapy, significant improvements were noted in pulmonary exacerbations, hospital admissions, antibiotic courses, number of chCF receiving daily chest therapy, dornase alfa and hypertonic saline (p < 0.05 for all) in total cohort and in most of the subgroups- CONCLUSION: ETI therapy was associated with improved clinical outcomes in younger children, and heterogenicity was noted in effectiveness.</p>","PeriodicalId":21057,"journal":{"name":"Respiratory medicine","volume":" ","pages":"109063"},"PeriodicalIF":3.3,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148649269","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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
Multidisciplinary care should be the standard in referral centers for non tuberculous mycobacterial disease. 多学科治疗应成为非结核分枝杆菌疾病转诊中心的标准。
IF 3.3 3区 医学
Respiratory medicine Pub Date : 2026-10-01 Epub Date: 2026-08-11 DOI: 10.1016/j.rmed.2026.109100
Giovanni Fumagalli, Niccolò Riccardi
{"title":"Multidisciplinary care should be the standard in referral centers for non tuberculous mycobacterial disease.","authors":"Giovanni Fumagalli, Niccolò Riccardi","doi":"10.1016/j.rmed.2026.109100","DOIUrl":"10.1016/j.rmed.2026.109100","url":null,"abstract":"","PeriodicalId":21057,"journal":{"name":"Respiratory medicine","volume":" ","pages":"109100"},"PeriodicalIF":3.3,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148707576","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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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