Respiration最新文献

{"title":"DIAGNOSTIC MANAGEMENT OF PEDIATRIC BRONCHIECTASIS: A LITERATURE REVIEW AND CLINICAL EXAMPLES.","authors":"Paola Faverio, Giovanni Franco, Valentina Landoni, Marta Nadalin, Davide Negri, Alessandro Tagliabue, Federica Acone, Francesca Cattaneo, Filippo Cipolla, Chiara Vimercati, Stefano Aliberti, Giovanna Lucchini, Adriana Cristina Balduzzi, Fabrizio Luppi","doi":"10.1159/000546030","DOIUrl":"https://doi.org/10.1159/000546030","url":null,"abstract":"<p><strong>Background: </strong>bronchiectasis is an often underdiagnosed chronic respiratory disease in children and adolescents. Recent international guidelines highlighted the management of bronchiectasis in pediatric patients in comparison to adults. The purpose of this manuscript is to review the diagnostic, etiological work-up and follow-up in children and adolescents with bronchiectasis, using real-life clinical cases that highlight the multidisciplinary approach required for this complex condition.</p><p><strong>Summary: </strong>the diagnostic process requires both thoracic imaging and a clinical evaluation with the addition of pulmonary function tests and microbiological exams, when possible. Specific work-up should include past medical history (e.g. recurrent pneumonia, otitis or extra-respiratory infections), including family history, testing for genetic diseases (e.g. cystic fibrosis), evaluation of airways abnormalities and obstruction (e.g. bronchial foreign body), exclusion of concomitant immunodeficiencies and conditions associated to impaired mucociliary clearance. Main comorbidities and possible etiological conditions include chronic pulmonary aspiration and gastro-esophageal reflux, upper respiratory tract and otologic diseases, particularly rhinosinusitis, otitis, and asthma. Patients should be followed up every 3 to 6 months, with closer monitoring for those with severe disease; transition to adult care should be individualized, with emphasis on patient education, treatment adherence and multidisciplinary support.</p><p><strong>Key messages: </strong>the diagnostic work-up for children and adolescents with bronchiectasis is challenging and requires input from multiple experts. Early detection of bronchiectasis is crucial for establishing effective diagnostic and therapeutic strategies and may help prevent further progression.</p>","PeriodicalId":21048,"journal":{"name":"Respiration","volume":" ","pages":"1-22"},"PeriodicalIF":3.5,"publicationDate":"2025-04-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143981193","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}

{"title":"Effect of Eccentric Cycling on Oxygen Uptake and Hemodynamics in Patients with Chronic Obstructive Pulmonary Disease: A Randomized Controlled Crossover Trial.","authors":"Aldo Kammerlander, Simon Raphael Schneider, Michael Furian, Esther Irene Schwarz, Mona Lichtblau, Silvia Ulrich, Julian Müller","doi":"10.1159/000545787","DOIUrl":"10.1159/000545787","url":null,"abstract":"<p><strong>Introduction: </strong>Chronic obstructive pulmonary disease (COPD) is a leading cause of morbidity and mortality worldwide and contributes significantly to reduced quality of life due to symptoms such as dyspnea and exercise intolerance. Eccentric cycling exercise (ECC) has shown potential as an alternative to conventional concentric cycling exercise (CON) in cardiopulmonary disease, including COPD, as it has a lower metabolic demand and potentially allows for higher exercise intensity with less perceived exertion. We aimed to compare ventilatory and circulatory responses of COPD patients between ECC and CON at identical submaximal workloads.</p><p><strong>Methods: </strong>In a randomized-controlled crossover trial, 17 COPD patients (6 female, mean ± SD age 67 ± 7 years) completed identical submaximal stepwise incremental cycling tests using ECC and CON, each step increasing by 10 W. The main outcome was oxygen uptake (<inline-formula><mml:math id=\"m1\" xmlns:mml=\"http://www.w3.org/1998/Math/MathML\"><mml:mrow><mml:mover accent=\"true\"><mml:mi mathvariant=\"normal\">V</mml:mi><mml:mo>˙</mml:mo></mml:mover><mml:msub><mml:mi mathvariant=\"normal\">O</mml:mi><mml:mn>2</mml:mn></mml:msub></mml:mrow></mml:math></inline-formula>). Additional outcomes were breath-by-breath ergospirometric measurements including minute ventilation (<inline-formula><mml:math id=\"m2\" xmlns:mml=\"http://www.w3.org/1998/Math/MathML\"><mml:mrow><mml:mover accent=\"true\"><mml:mi mathvariant=\"normal\">V</mml:mi><mml:mo>˙</mml:mo></mml:mover><mml:mi mathvariant=\"normal\">E</mml:mi></mml:mrow></mml:math></inline-formula>) and hemodynamics by echocardiography at each step.</p><p><strong>Results: </strong>At a mean end-exercise intensity of 41.3 ± 3.5 W, ECC lowered <inline-formula><mml:math id=\"m3\" xmlns:mml=\"http://www.w3.org/1998/Math/MathML\"><mml:mrow><mml:mover accent=\"true\"><mml:mi mathvariant=\"normal\">V</mml:mi><mml:mo>˙</mml:mo></mml:mover><mml:msub><mml:mi mathvariant=\"normal\">O</mml:mi><mml:mn>2</mml:mn></mml:msub></mml:mrow></mml:math></inline-formula> by -122 mL/min (-25%, 95% CI: -213 to -47, p = 0.005) and <inline-formula><mml:math id=\"m4\" xmlns:mml=\"http://www.w3.org/1998/Math/MathML\"><mml:mrow><mml:mover accent=\"true\"><mml:mi mathvariant=\"normal\">V</mml:mi><mml:mo>˙</mml:mo></mml:mover><mml:mi mathvariant=\"normal\">E</mml:mi></mml:mrow></mml:math></inline-formula> by -5.7 L/min (-29%, 95% CI: -10.0 to -1.6, p = 0.012) compared to CON. Perceived dyspnea and leg fatigue did not differ. A trend toward reduced strain on the right ventricle was observed in ECC (37 ± 13 mm Hg ECC vs. 48 ± 7 mm Hg CON), but this was not significant (p = 0.063). No adverse events occurred.</p><p><strong>Conclusion: </strong>ECC allowed COPD patients to exercise at the same workload but with a lower metabolic and ventilatory demand compared to CON, suggesting it has the potential to further improve exercise capacity in pulmonary rehabilitation.</p>","PeriodicalId":21048,"journal":{"name":"Respiration","volume":" ","pages":"1-11"},"PeriodicalIF":3.5,"publicationDate":"2025-04-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12101805/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144042064","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":"Anti-Inflammatory Activity of Ensifentrine: A Novel, Selective Dual Inhibitor of Phosphodiesterase 3 and Phosphodiesterase 4.","authors":"Domenico Spina, Lui Franciosi, Radhakrishnan Venkatasamy, David A Saint, Margot MacDonald-Berko, Tara Rheault","doi":"10.1159/000545645","DOIUrl":"10.1159/000545645","url":null,"abstract":"<p><p>Ensifentrine is a novel, low-molecular-weight molecule that is a selective, dual inhibitor of phosphodiesterase (PDE)3 and PDE4. Inhibition of PDE3 has been shown to relax airway smooth muscle and inhibition of PDE4 to inhibit inflammatory responses and to stimulate the cystic fibrosis transmembrane conductance regulator in human airway epithelial cells through accumulation of intracellular cyclic adenosine monophosphate. Additionally, the dual inhibition of PDE3 and PDE4 demonstrates enhanced or synergistic effects compared with inhibition of either PDE3 or PDE4 alone on contraction of airway smooth muscle and suppression of inflammatory responses. Ensifentrine inhalation suspension 3 mg was recently approved in the USA for the maintenance treatment of chronic obstructive pulmonary disease in adult patients and is marketed under the trade name Ohtuvayre™. This manuscript describes further evidence that ensifentrine is a selective dual inhibitor of both human PDE3 and PDE4 enzymes and that this drug has significant anti-inflammatory activity in vivo in both allergic guinea pigs and non-human primates. This dual bronchodilator and anti-inflammatory activity of ensifentrine makes it a promising strategy as a novel inhaled \"bifunctional\" drug for the treatment of obstructive and inflammatory diseases of the respiratory tract.</p>","PeriodicalId":21048,"journal":{"name":"Respiration","volume":" ","pages":"1-10"},"PeriodicalIF":3.5,"publicationDate":"2025-04-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12101800/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144014611","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":"COPD Exacerbations and Airflow Obstruction Severity Pre- and Post-Pneumococcal Vaccination: A post hoc Analysis of the RETRIEVE Real-World Study.","authors":"Stavros Tryfon, Efthymia Papadopoulou, Polyanthi Papanastasiou, Alexandros Ginis, Konstantinos Kostikas","doi":"10.1159/000545232","DOIUrl":"10.1159/000545232","url":null,"abstract":"<p><strong>Introduction: </strong>Chronic obstructive pulmonary disease (COPD) is a major cause of mortality and morbidity worldwide. Prevention of exacerbations is fundamental to COPD management.</p><p><strong>Methods: </strong>The RETRIEVE study was a multicenter, 7-year retrospective study (NCT03858348) of patients with spirometry confirmation of COPD, who received LABA/ICS for at least 1 year and either continued or escalated to open triple therapy, according to their physicians' judgment. In this post hoc analysis, we explored exacerbation rate and lung function pre- and post-pneumococcal vaccination in a real-world setting.</p><p><strong>Results: </strong>Among 466 COPD patients, 48.1% received LABA/ICS for a mean 32 months, while 51.9% received LABA/ICS for a mean 21 months and then escalated to open triple therapy. Patients treated solely with LABA/ICS presented a significant reduction in COPD exacerbations (incidence rate ratio 0.66, 95% confidence interval [0.56, 0.78]) and related hospitalizations (0.43 [0.25, 0.72]) after pneumococcal vaccination compared to pre-vaccination. High-risk patients, who ultimately escalated to triple therapy, presented no reduction in exacerbations post-vaccination compared to pre-vaccination, and their airflow obstruction tended to deteriorate over time post-vaccination while they received LABA/ICS. However, after escalation to open triple therapy, there was significant reduction in exacerbations (0.78 [0.66, 0.94]) and related hospitalizations (0.41 [0.26, 0.66]) post-vaccination compared to pre-vaccination. Pneumococcal vaccination rates increased from 2012 to 2018 in our study, exceeding 90% from 2015 onward. Preserved lung function potentially delayed vaccination.</p><p><strong>Conclusion: </strong>This post hoc analysis of the RETRIEVE real-world study highlights the importance of appropriate, personalized maintenance COPD treatment, along with pneumococcal vaccination, for the prevention of COPD exacerbations.</p>","PeriodicalId":21048,"journal":{"name":"Respiration","volume":" ","pages":"1-11"},"PeriodicalIF":3.5,"publicationDate":"2025-04-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144004700","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}

{"title":"Systemic Immune-Inflammation Index Is Associated with Adverse Outcomes in Patients Hospitalized for AECOPD: A Multicenter Cohort Study.","authors":"Xueqing Chen, Shiman Liu, Yuanming Luo, Hailong Wei, Huiqing Ge, Huiguo Liu, Jianchu Zhang, Xianhua Li, Pinhua Pan, Xiufang Xie, Mengqiu Yi, Lina Cheng, Hui Zhou, Jiarui Zhang, Lige Peng, Jiaqi Pu, Jiaxin Zeng, Qun Yi, Haixia Zhou","doi":"10.1159/000545267","DOIUrl":"10.1159/000545267","url":null,"abstract":"<p><strong>Introduction: </strong>Acute exacerbations of chronic obstructive pulmonary disease (AECOPDs) are associated with increased morbidity and mortality. The novel inflammatory biomarker, systemic immune-inflammation index (SII), may have prognostic value. This study aimed to assess the association between SII and short-term and long-term adverse outcomes among AECOPD inpatients.</p><p><strong>Methods: </strong>This was a multicenter, retrospective analysis of a prospectively collected cohort of AECOPD inpatients. We initially compared SII and other clinical characteristics between survivors and non-survivors during hospitalization, adjusting for primary comorbidities using propensity score matching (PSM). We assessed the short-term and long-term adverse outcomes, particularly focusing on in-hospital mortality and 2-year all-cause mortality, across different levels of SII. Multivariate Cox analysis was employed to evaluate the associations of SII, neutrophil-to-lymphocyte ratio (NLR), and platelet-to-lymphocyte ratio (PLR) with in-hospital mortality of AECOPD patients. Restricted cubic spline (RCS) models investigated the nonlinear relationships between these biomarkers and in-hospital mortality. To compare the predictive values of SII, NLR, and PLR for in-hospital mortality, receiver operating characteristic (ROC) curve analysis was performed. Subgroup analysis was carried out to further determine the predictive capacity of SII among diverse subgroups.</p><p><strong>Results: </strong>The study included 12,551 AECOPD inpatients, among whom 180 (1.4%) died in hospital. Whether before or after PSM adjusting for comorbidities, the levels of SII, NLR, and PLR in non-survivors were significantly higher than those in survivors (all p < 0.001). Elevated SII levels (divided into quartiles) were associated with increased in-hospital mortality (Q1 vs. Q2 vs. Q3 vs. Q4: 0.6% vs. 0.8% vs. 1.5% vs. 2.8%) and 2-year all-cause mortality (15.4% vs. 22.6% vs. 22.2% vs. 27.8%), as well as other adverse outcomes (all p < 0.05). After adjusting for covariates, higher levels of SII and NLR consistently remained associated with increased in-hospital mortality. RCS analysis revealed a consistent linear relationship between SII and in-hospital mortality, while NLR and PLR exhibited nonlinear relationships. Furthermore, ROC curve analysis indicated that SII showed inferiority to NLR but superiority to PLR in predicting in-hospital mortality among AECOPD patients (area under the curve for SII vs. NLR vs. PLR: 0.670 vs. 0.731 vs. 0.609). Subgroup analysis revealed that the association between SII and in-hospital mortality varied across different subgroups.</p><p><strong>Conclusion: </strong>Elevated SII is associated with increased risks of short-term and long-term adverse outcomes in AECOPD inpatients, making it potential prognostic factor used to identify high-risk patients and guide the management of AECOPD.</p>","PeriodicalId":21048,"journal":{"name":"Respiration","volume":" ","pages":"1-19"},"PeriodicalIF":3.5,"publicationDate":"2025-04-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144029459","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}

{"title":"Predictive Factors of Clinical Success of Therapeutic Bronchoscopy in Malignant Central Airway Obstruction: Results from the EpiGETIF Registry.","authors":"Pascalin Roy, Lyria Amari, Sophie Laroumagne, Julien Legodec, Clément Fournier, Laurent Cellerin, Christine Lorut, François Gonin, Jean-Michel Vergnon, Thomas Egenod, Nicolas Favrolt, Pascal Schlossmacher, Valerian Bourinet, Loïc Perrot, Samy Lachkar, Juliette Camuset, Amandine Briault, Tristan Degot, Christophe Gut-Gobert, Gilles Mangiapan, Jean-Yves Jasnot, Eric Briens, Adrian Crutu, Armelle Marceau, Bénédicte Toublanc, Maxime Dewolf, Julien Dutilh, Nathalie Germain, Julie Tronchetti, Philippe Astoul, Nicolas Guibert, Hervé Dutau","doi":"10.1159/000545568","DOIUrl":"10.1159/000545568","url":null,"abstract":"<p><strong>Introduction: </strong>Therapeutic bronchoscopy (TB) is considered a safe and effective treatment for patients with malignant central airway obstruction (MCAO). While many factors have been associated with technical success, it does not always translate in clinical success. Few factors to predict clinical response have been described. The objective of this study was to determine predictive factors of clinical success for patients with MCAO undergoing TB.</p><p><strong>Methods: </strong>We used the multicenter prospective registry EpiGETIF to collect data from patients with MCAO undergoing TB from January 2019 to June 2021. The criterion for clinical success was dyspnea measured on the Borg scale. Patients were classified as super responders if they had an improvement of 4 points after the procedure. Uni- and multivariate analyses were performed to highlight an association between preprocedural features and clinical success.</p><p><strong>Results: </strong>A total of 496 patients from 24 centers met inclusion criteria. The mean preprocedural Borg score was 6.5 ± 2.0 versus 2.2 ± 1.7 postprocedural (mean difference 4.3 ± 2.3). Overall, 302 patients (60.9%) were considered super responders. The only factor associated with super responders in multivariate analysis was a higher baseline Borg score. The only factor associated with non-super responders was a poor performance status and mechanical ventilation.</p><p><strong>Conclusion: </strong>Patients show good clinical results following TB for MCAO, influenced positively by a worse pre-procedure dyspnea and negatively by a worse performance status. No other data could help predict the effectiveness of TB, confirming the complexity of the process and heterogeneity of the target population.</p>","PeriodicalId":21048,"journal":{"name":"Respiration","volume":" ","pages":"1-11"},"PeriodicalIF":3.5,"publicationDate":"2025-04-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143796204","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}

{"title":"Endobronchial Seeding of Tuberculous Granulomas after EBUS-TBNA of Mediastinal Lymph Nodes: Case Report.","authors":"Ales Rozman, Izidor Kern","doi":"10.1159/000545506","DOIUrl":"10.1159/000545506","url":null,"abstract":"","PeriodicalId":21048,"journal":{"name":"Respiration","volume":" ","pages":"1-3"},"PeriodicalIF":3.5,"publicationDate":"2025-04-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143773155","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}

{"title":"Machine Learning-Based Model for Predicting Severe Exacerbations in Adult-Onset Type 2 Inflammatory Asthma.","authors":"JunJie Dai, Huaxiang Ling, Yaqin Liu, Rongchang Chen, Fei Shi","doi":"10.1159/000545039","DOIUrl":"10.1159/000545039","url":null,"abstract":"<p><strong>Introduction: </strong>Currently, scholars have applied machine learning to the clinical prediction of acute asthma exacerbations. However, given the heterogeneity of inflammatory phenotypes in asthma, it is imperative to develop machine learning models tailored to specific asthma inflammatory phenotypes. The aim of this study was to develop predictive models to identify risk factors for the severe exacerbations in adult-onset type 2 inflammatory asthma, which could help facilitate early diagnosis and intervention, potentially reducing healthcare costs.</p><p><strong>Methods: </strong>This was a retrospective analysis of patients with acute exacerbations of type 2 inflammatory asthma at Shenzhen People's Hospital from May 2017 to September 2022. Patients were categorized into mild-to-moderate exacerbation (n = 300) and severe exacerbation groups (n = 209). We collected clinical data from all participants, including demographic characteristics, laboratory results, pulmonary function test results, comorbidities, and asthma medication use. We tested four models: decision trees, logistic regression, random forests, and LightGBM. For each model, 80% of the dataset was used for training and 20% was used to validate the models. The area under (AUC) the receiver-operating characteristic (ROC) curve was calculated for each model.</p><p><strong>Results: </strong>Multivariate logistic regression revealed that low ACT scores, low FEV1/FVC ratio, a history of diabetes, high absolute neutrophil count, and a family history of asthma were independent risk factors for severe exacerbations of type 2 inflammatory asthma. LightGBM outperformed all other models, achieving the highest AUC of 0.9344, with sensitivity = 0.8293, specificity = 0.9180, PPV = 0.8718, and NPV = 0.8889. The accuracy stood at 0.8824, with an F1 score of 0.8500. The top 10 clinical variables impacting the prediction outcome in the LightGBM model were ACT score, FEV1/FVC ratio, age, lactate dehydrogenase, FEV1% predicted, fasting blood glucose, angiotensin-converting enzyme, duration of disease, neutrophil-to-lymphocyte ratio, and platelet-to-lymphocyte ratio. Finally, through DCA, the clinical decision-support value of the LightGBM model was confirmed, demonstrating its maximum net benefit for type 2 asthma patients across a threshold probability range of 20%-80%.</p><p><strong>Conclusions: </strong>We have developed and established a prediction model for severe exacerbations of adult-onset type 2 inflammatory asthma using the LightGBM machine learning approach, which exhibits good predictive performance. This model can aid in the early prediction and prevention of severe exacerbations of adult-onset type 2 inflammatory asthma.</p>","PeriodicalId":21048,"journal":{"name":"Respiration","volume":" ","pages":"1-20"},"PeriodicalIF":3.5,"publicationDate":"2025-03-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143754248","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}

{"title":"Lung Function and Symptoms in Idiopathic Pulmonary Fibrosis Treated with High-Flow Nasal Therapy for 1 Year.","authors":"Francesca Simioli, Anna Annunziata, Maurizia Lanza, Maria Cardone, Antonietta Coppola, Antonella Marotta, Cecilia Calabrese, Giuseppe Fiorentino","doi":"10.1159/000545165","DOIUrl":"10.1159/000545165","url":null,"abstract":"<p><strong>Introduction: </strong>Idiopathic pulmonary fibrosis (IPF) is a chronic progressive disease that subverts the normal structure of the lungs and finally causes respiratory failure. High-flow nasal therapy (HFNT) is currently used in the acute setting for IPF with acute respiratory failure. Also, acute exacerbation of IPF and end-stage disease are common indications. Chronic cough is often an unmet need in IPF because it is partially responsive to common pharmacological treatment. Moreover, opioids have known adverse events. The aim of this paper was to investigate the effects and safety of chronic HFNT on lung function and symptoms of IPF.</p><p><strong>Methods: </strong>This is a single-center case-control study including patients affected by IPF. We included 35 adult patients with a consistent radiological diagnosis of IPF, clinical history of lung function decline, and high prevalence of symptoms. All patients received the standard of treatment, particularly including antifibrotic drugs and conventional oxygen therapy (COT). Eighteen subjects were assigned to additional treatment with HFNT for 12 months.</p><p><strong>Results: </strong>No significant differences were observed after the follow-up with HFNT in terms of lung function. The mean forced vital capacity (FVC) was 1.89 ± 0.73 L with HFNT and 2.43 ± 0.87 L without HFNT (p = 0.09). The mean FVC decline per year was 190 mL with HFNT versus 200 mL with standard of care. The mean DLCO % of predicted was 28.86 ± 14.51% with HFNT and 36.03 ± 19.18% with COT (p = 0.276). No significant impact was observed on dyspnea; the mean Borg scale value was 6.72 ± 2.22 after HFNT and 7.14 with COT (p = 0.56). The score for cough significantly improved after treatment with a mean score in the HFNT group being 46.67 ± 10.85 versus 73.8 ± 18.43 (p < 0.0001) with standard of care.</p><p><strong>Conclusions: </strong>Long-term HFNT significantly reduces chronic cough in patients affected by IPF compared to COT. Lung function including FVC and DLCO is not significatively influenced.</p>","PeriodicalId":21048,"journal":{"name":"Respiration","volume":" ","pages":"1-7"},"PeriodicalIF":3.5,"publicationDate":"2025-03-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143754334","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}

{"title":"A Unique Combination of Heterozygous CFTR Gene Variants in a Person with Cystic Fibrosis and Mycobacterium abscessus Infection.","authors":"Arthur Lemson, Cedric Bosteels, Jakko van Ingen, Monique Reijers, Dineke Westra, Wouter Hoefsloot","doi":"10.1159/000545552","DOIUrl":"10.1159/000545552","url":null,"abstract":"<p><strong>Introduction: </strong>Cystic fibrosis (CF) is a genetic disorder caused by mutations in the CFTR gene. A minority of people with CF carry two heterozygous CFTR mutations other than the common Phe508del, complicating diagnosis and treatment.</p><p><strong>Case presentation: </strong>We report the case of a 25-year-old South American male diagnosed with CF respiratory disease, characterized by a history of recurrent infections, pulmonary Mycobacterium abscessus infection, airway disease on high-resolution CT, and an elevated sweat chloride level (74 mmol/L). Exome sequencing identified a unique combination of CFTR mutations: a pathogenic frameshift variant (c.2052dup) and a variant of unknown clinical significance (c.710A>C). Notably, there were no signs of pancreatic insufficiency. Rectal mucosal organoid cultures demonstrated residual CFTR function with responsiveness to ivacaftor and the combination of elexacaftor, tezacaftor, and ivacaftor.</p><p><strong>Conclusion: </strong>This case highlights a unique combination of heterozygous CFTR variants in a person with CF respiratory disease, which may be amendable to CFTR modulation therapy.</p>","PeriodicalId":21048,"journal":{"name":"Respiration","volume":" ","pages":"1-5"},"PeriodicalIF":3.5,"publicationDate":"2025-03-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143754331","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}