{"title":"Is There a Role for Lumacaftor/Ivacaftor in Young Children with Cystic Fibrosis?","authors":"Isaac Martin, Hartmut Grasemann","doi":"10.1513/AnnalsATS.202410-1070LE","DOIUrl":"10.1513/AnnalsATS.202410-1070LE","url":null,"abstract":"","PeriodicalId":93876,"journal":{"name":"Annals of the American Thoracic Society","volume":" ","pages":"468-469"},"PeriodicalIF":0.0,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11892668/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143018090","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Coralynn Sack, Daniel M Wojdyla, Maeve G MacMurdo, Amanda Gassett, Joel D Kaufman, Ganesh Raghu, Carrie A Redlich, Peide Li, Amy L Olson, Thomas B Leonard, Jamie L Todd, Megan L Neely, Laurie D Snyder, Mridu Gulati
{"title":"Long-Term Air Pollution Exposure and Severity of Idiopathic Pulmonary Fibrosis: Data from the Idiopathic Pulmonary Fibrosis Prospective Outcomes (IPF-PRO) Registry.","authors":"Coralynn Sack, Daniel M Wojdyla, Maeve G MacMurdo, Amanda Gassett, Joel D Kaufman, Ganesh Raghu, Carrie A Redlich, Peide Li, Amy L Olson, Thomas B Leonard, Jamie L Todd, Megan L Neely, Laurie D Snyder, Mridu Gulati","doi":"10.1513/AnnalsATS.202404-382OC","DOIUrl":"10.1513/AnnalsATS.202404-382OC","url":null,"abstract":"<p><p><b>Rationale:</b> Although exposure to air pollution is a known risk factor for adverse pulmonary outcomes, its impact in individuals with idiopathic pulmonary fibrosis (IPF) is less well understood. <b>Objectives:</b> To investigate the effects of long-term exposure to air pollution on disease severity and progression in patients with IPF and to determine whether genomic factors, such as MUC5B promoter polymorphism or telomere length, modify these associations. <b>Methods:</b> We performed analyses at enrollment and after 1 year of follow-up in the IPF-PRO (Idiopathic Pulmonary Fibrosis Prospective Outcomes) Registry, a prospective observational registry that enrolled individuals with IPF at 46 U.S. sites from June 2014 to October 2018. Five-year average pollution exposures (particulate matter ≤2.5 μm in aerodynamic diameter [PM<sub>2.5</sub>], nitrogen dioxide, ozone) before the enrollment date were estimated at participants' residential addresses with validated national spatiotemporal models. Multivariable regression models estimated associations between pollution exposure and physiologic measurements (forced vital capacity [FVC], diffusing capacity of the lung for carbon monoxide, supplemental oxygen use at rest) and quality-of-life measurements (St. George's Respiratory Questionnaire, EuroQoL, Cough and Sputum Assessment Questionnaire) at enrollment. Cox proportional hazards models estimated associations between pollutants and a composite outcome of death, lung transplant, or >10% absolute decline in FVC percent predicted in the year after enrollment. Models were adjusted for individual-level and spatial confounders, including proxies for disease onset. Gene-environment interactions with MUC5B and telomere length were assessed. <b>Results:</b> Of 835 participants, 94% were non-Hispanic White individuals, 76% were male, and the mean (standard deviation) age was 70 (7.7) years. In fully adjusted analyses, higher PM<sub>2.5</sub> exposure was associated with worse quality of life per St. George's Respiratory Questionnaire activity score (3.48 [95% confidence interval (CI), 0.64, 6.32] per 2 μg/m<sup>3</sup> PM<sub>2.5</sub>) and EuroQoL scores (-0.04 [95% CI, -0.06, -0.01] per 2 μg/m<sup>3</sup> PM<sub>2.5</sub>), as well as lower FVC percent predicted and lower diffusing capacity of the lung for carbon monoxide percent predicted at enrollment<sub>.</sub> Each 3 parts per billion difference in O<sub>3</sub> exposure was associated with a 1.57% (95% CI, 0.15, 2.98) higher FVC percent predicted at enrollment, although this effect was attenuated in multipollutant models. There was no association between nitrogen dioxide and enrollment measures or between pollution exposure and 1-year outcomes and no evidence for gene-environment interactions. <b>Conclusions:</b> In the IPF-PRO Registry, long-term exposure to PM<sub>2.5</sub> was associated with worse quality of life and lung function at enrollment, but not with short-term disease progression","PeriodicalId":93876,"journal":{"name":"Annals of the American Thoracic Society","volume":" ","pages":"378-386"},"PeriodicalIF":0.0,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142633362","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Erika Matsumura, Gustavo F Grad, Fernanda Madeiro, Pedro R Genta, Geraldo Lorenzi-Filho
{"title":"Mouth Leak Is Associated with Sleep Fragmentation During Nasal Continuous Positive Airway Pressure Treatment of Obstructive Sleep Apnea and May Be Detected by Leak Waveform Analysis.","authors":"Erika Matsumura, Gustavo F Grad, Fernanda Madeiro, Pedro R Genta, Geraldo Lorenzi-Filho","doi":"10.1513/AnnalsATS.202404-425OC","DOIUrl":"10.1513/AnnalsATS.202404-425OC","url":null,"abstract":"<p><p><b>Rationale:</b> Mouth air leak is a major cause of low adherence to nasal continuous positive airway pressure (CPAP) in patients with obstructive sleep apnea (OSA). However, CPAP reports do not distinguish mouth leak from mask leak. We hypothesized that mouth air leak is terminated abruptly by an arousal from sleep and mouth closing that can be detected by CPAP leak waveform analysis. <b>Objectives:</b> To describe patterns of mouth air leak waveform during polysomnography (PSG) in patients with OSA treated with well-fitted nasal CPAP. <b>Methods:</b> PSG recordings with a jaw motion sensor to detect mouth opening were performed in patients with OSA treated with nasal CPAP with suspected mouth air leak. Careful mask fitting and visual inspection excluded mask leak. Mouth leak episodes were characterized by an increase (⩾20%) above the intentional leak. Leak episodes were classified as intermittent (<5 minutes) or continuous (⩾5 minutes). <b>Results:</b> Twenty patients (80% men; age, 63 ± 11 years; body mass index, 29.9 ± 6 kg/m<sup>2</sup>; baseline apnea-hypopnea index, 46.9 ± 19 events per hour) treated with nasal CPAP and documented mouth leak completed the study. All but one patient experienced an overlap of intermittent and continuous mouth leak. Most mouth leak episodes ended with mouth closure (97.7%) and an arousal (52.7%) or awakening (38.6%). Only 34.9% of the leak episodes were associated with respiratory events. Intermittent mouth air leak was more common in sleep stages N1 and N2 (<i>P</i> < 0.01), whereas continuous leak was more common in sleep stage N3 (<i>P</i> < 0.01). Continuous episodes of air leak were associated with a higher amplitude of mouth opening. The CPAP report waveform was able to detect only 29.6% of the leak episodes detected by PSG. Only 10 patients (50%) had a high unintentional leak according to the criteria adopted by ResMed, and only 2 patients (10%) presented large leak according to the Philips criteria. <b>Conclusions:</b> Intermittent and continuous mouth leak during nasal CPAP frequently coexist and contribute to sleep fragmentation. Identification of leak waveform patterns may help detect mouth air leak, which, in turn, is an important cause of poor CPAP adherence.</p>","PeriodicalId":93876,"journal":{"name":"Annals of the American Thoracic Society","volume":" ","pages":"430-437"},"PeriodicalIF":0.0,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142585078","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Nadine Al-Naamani, Melanie Prakash, Steven Pugliese, Jason S Fritz, K Akaya Smith, Harold I Palevsky, Charlene Compher
{"title":"Nutrition and Diet Quality in Pulmonary Arterial Hypertension.","authors":"Nadine Al-Naamani, Melanie Prakash, Steven Pugliese, Jason S Fritz, K Akaya Smith, Harold I Palevsky, Charlene Compher","doi":"10.1513/AnnalsATS.202405-563RL","DOIUrl":"10.1513/AnnalsATS.202405-563RL","url":null,"abstract":"","PeriodicalId":93876,"journal":{"name":"Annals of the American Thoracic Society","volume":" ","pages":"462-466"},"PeriodicalIF":0.0,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11892655/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142606768","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Surya P Bhatt, Chaoqi Wu, Yifei Sun, Pallavi P Balte, Joseph E Schwartz, Miguel J Divo, Byron C Jaeger, Paulo H Chaves, David Couper, David R Jacobs, Donald Lloyd-Jones, Ravi Kalhan, Anne B Newman, George T O'Connor, Jason G Umans, Wendy B White, Sachin Yende, Elizabeth C Oelsner
{"title":"Low Lung Function Is Associated with High Population Attributable Fraction for Cardiovascular Mortality.","authors":"Surya P Bhatt, Chaoqi Wu, Yifei Sun, Pallavi P Balte, Joseph E Schwartz, Miguel J Divo, Byron C Jaeger, Paulo H Chaves, David Couper, David R Jacobs, Donald Lloyd-Jones, Ravi Kalhan, Anne B Newman, George T O'Connor, Jason G Umans, Wendy B White, Sachin Yende, Elizabeth C Oelsner","doi":"10.1513/AnnalsATS.202407-715OC","DOIUrl":"10.1513/AnnalsATS.202407-715OC","url":null,"abstract":"<p><p><b>Rationale:</b> Chronic lung diseases are associated with increased risk of mortality due to coronary heart disease (CHD). Nonetheless, the population attributable fraction (PAF) of lung function impairment relative to other established cardiovascular risk factors is unclear. <b>Objectives:</b> To evaluate the PAF of low lung function for CHD mortality <b>Methods:</b> We harmonized and pooled lung function and clinical data across eight U.S. general population cohorts. Impaired lung function was defined as forced expiratory volume in 1 second (FEV<sub>1</sub>) and/or forced vital capacity ≤ 95% predicted on baseline spirometry. The association between CHD mortality and risk factors was assessed using cause-specific proportional hazards and Fine-Gray proportional subdistribution hazard models, treating non-CHD mortality as a competing risk. Models were adjusted for lung function as well as age, sex, race/ethnicity, educational attainment, body mass index, smoking status, pack-years of smoking, diabetes mellitus, high-density lipoprotein, and high low-density lipoprotein (≥130 mg/dl). PAF was calculated as the relative change in the average absolute risk of 10-year CHD mortality by elimination of lung function lower than 95% predicted. <b>Results:</b> Among 35,143 participants, 1,844 of 13,174 (14.0%) deaths were due to CHD. Compared with percentage predicted FEV<sub>1</sub> (FEV<sub>1</sub>pp) > 95%, the subdistribution adjusted hazard ratio for low FEV<sub>1</sub>pp was 1.30 (95% confidence interval, 1.18-1.44). The PAF for FEV<sub>1</sub>pp ≤ 95% was 12%, ranking low FEV<sub>1</sub> third on the list of PAF for CHD mortality, after hypertension and diabetes. Low FEV<sub>1</sub>pp ranked second in the subgroup of active smokers (PAF 14%), after hypertension. <b>Conclusions:</b> Low lung function, even in the range considered clinically normal, ranks high on the list of attributable risk factors for CHD mortality and should be considered in cardiovascular risk stratification.</p>","PeriodicalId":93876,"journal":{"name":"Annals of the American Thoracic Society","volume":" ","pages":"359-366"},"PeriodicalIF":0.0,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11892675/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142514522","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Alan Hamilton, Nancy Kline Leidy, Ashley I Duenas, Soren E Skovlund, Bruce E Miller
{"title":"A Unified Set of Patient-inspired Health Concepts for Chronic Obstructive Pulmonary Disease: A North Star for Understanding Treatment Benefit.","authors":"Alan Hamilton, Nancy Kline Leidy, Ashley I Duenas, Soren E Skovlund, Bruce E Miller","doi":"10.1513/AnnalsATS.202408-864IP","DOIUrl":"10.1513/AnnalsATS.202408-864IP","url":null,"abstract":"","PeriodicalId":93876,"journal":{"name":"Annals of the American Thoracic Society","volume":" ","pages":"317-321"},"PeriodicalIF":0.0,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11892663/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142585058","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Alexander T Moffett, Scott D Halpern, Gary E Weissman
{"title":"Sex Differences in the Diagnosis of Chronic Obstructive Pulmonary Disease after Spirometry.","authors":"Alexander T Moffett, Scott D Halpern, Gary E Weissman","doi":"10.1513/AnnalsATS.202404-402RL","DOIUrl":"10.1513/AnnalsATS.202404-402RL","url":null,"abstract":"","PeriodicalId":93876,"journal":{"name":"Annals of the American Thoracic Society","volume":" ","pages":"466-468"},"PeriodicalIF":0.0,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11892671/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142717969","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Theodore J Iwashyna, Katrina E Hauschildt, Vincent Ni, Julia Lynch, Cecília Tomori
{"title":"Lest a Smoky Haze of Doubt Suffocate Progress toward Better Pulse Oximeters.","authors":"Theodore J Iwashyna, Katrina E Hauschildt, Vincent Ni, Julia Lynch, Cecília Tomori","doi":"10.1513/AnnalsATS.202411-1213ED","DOIUrl":"10.1513/AnnalsATS.202411-1213ED","url":null,"abstract":"","PeriodicalId":93876,"journal":{"name":"Annals of the American Thoracic Society","volume":" ","pages":"322-324"},"PeriodicalIF":0.0,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11892665/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142866608","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ellis Cerrone, Abdul G Hameed, David G Kiely, Robin Condliffe, Andrew J Swift, Smitha Rajaram, Ian Smith, Judith A Hurdman, Charlie A Elliot, A A Roger Thompson, Alexander M K Rothman, Athanasios Charalampopoulos
{"title":"Do Hemodynamic Definitions of Chronic Thromboembolic Pulmonary Hypertension Distinguish between Distinct Phenotypes of Chronic Thromboembolic Pulmonary Disease?","authors":"Ellis Cerrone, Abdul G Hameed, David G Kiely, Robin Condliffe, Andrew J Swift, Smitha Rajaram, Ian Smith, Judith A Hurdman, Charlie A Elliot, A A Roger Thompson, Alexander M K Rothman, Athanasios Charalampopoulos","doi":"10.1513/AnnalsATS.202405-524OC","DOIUrl":"10.1513/AnnalsATS.202405-524OC","url":null,"abstract":"<p><p><b>Rationale:</b> Chronic thromboembolic pulmonary disease (CTEPD) is defined by chronic organized thrombi in the pulmonary circulation without or with pulmonary hypertension. The current definition of chronic thromboembolic pulmonary hypertension (CTEPH) has adopted lower mean pulmonary arterial pressure (mPAP) and pulmonary vascular resistance (PVR) thresholds. <b>Objectives:</b> Our aim was to identify its impact on the characterization of patients with CTEPD. <b>Methods:</b> All consecutive patients with CTEPD referred for cardiopulmonary exercise testing (CPET) in a pulmonary hypertension center were divided into four groups on the basis of pulmonary hemodynamics: group A, mPAP ≤ 20 mm Hg; group B, mPAP > 20 mm Hg with PVR > 2 and ≤3 Wood units (WU); group C, mPAP > 20 mm Hg with PVR > 3 WU; and group D, mPAP > 20 mm Hg with PVR < 2 WU (\"unclassified\"). We compared CPET, computed tomography pulmonary angiography, and cardiac magnetic resonance imaging data across the groups. <b>Results:</b> There was mild aerobic capacity impairment, mild/moderate ventilatory inefficiency, and no significant cardiac limitation on CPET in all groups. However, patients in groups A and D had better ventilatory efficiency and less oxygen desaturation on exercise because of lower dead-space ventilation. There was no difference in chronic pulmonary embolus burden and distribution or resting right ventricular function among the groups. Seventeen patients were reclassified as having \"CTEPH\" on the basis of the current definition. No functional deterioration was noted within a median period of 13 months on repeat CPET. <b>Conclusions:</b> CTEPD patients with similar clot burden and right ventricular function without or with mild/moderate pulmonary hypertension displayed a similar pattern of cardiopulmonary limitation, except for ventilatory efficiency. The current definition of CTEPH may lead to the reclassification of CTEPH in a considerable number of patients.</p>","PeriodicalId":93876,"journal":{"name":"Annals of the American Thoracic Society","volume":" ","pages":"331-338"},"PeriodicalIF":0.0,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7617186/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142482819","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A 76-Year-Old Man with New Unilateral Pleural Effusion.","authors":"Jared M Stuart, Laura J Hinkle","doi":"10.1513/AnnalsATS.202403-320CC","DOIUrl":"https://doi.org/10.1513/AnnalsATS.202403-320CC","url":null,"abstract":"","PeriodicalId":93876,"journal":{"name":"Annals of the American Thoracic Society","volume":"22 3","pages":"456-459"},"PeriodicalIF":0.0,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143525507","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}