Pulmonary Circulation最新文献

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Air travel in patients suffering from pulmonary hypertension-A prospective, multicentre study. 肺动脉高压患者的空中旅行--一项前瞻性多中心研究。
IF 2.2 4区 医学
Pulmonary Circulation Pub Date : 2024-08-05 eCollection Date: 2024-07-01 DOI: 10.1002/pul2.12397
Athiththan Yogeswaran, Jan Grimminger, Khodr Tello, Lukas Becker, Werner Seeger, Friedrich Grimminger, Natascha Sommer, Hossein A Ghofrani, Tobias J Lange, Stefan Stadler, Karen Olsson, Jan C Kamp, Stephan Rosenkranz, Felix Gerhardt, Katrin Milger, Michaela Barnikel, Silvia Ulrich, Stéphanie Saxer, Ekkehard Grünig, Satenik Harutynova, Christian Opitz, Hans Klose, Heinrike Wilkens, Michael Halank, Melanie Heberling, Henning Gall, Manuel J Richter
{"title":"Air travel in patients suffering from pulmonary hypertension-A prospective, multicentre study.","authors":"Athiththan Yogeswaran, Jan Grimminger, Khodr Tello, Lukas Becker, Werner Seeger, Friedrich Grimminger, Natascha Sommer, Hossein A Ghofrani, Tobias J Lange, Stefan Stadler, Karen Olsson, Jan C Kamp, Stephan Rosenkranz, Felix Gerhardt, Katrin Milger, Michaela Barnikel, Silvia Ulrich, Stéphanie Saxer, Ekkehard Grünig, Satenik Harutynova, Christian Opitz, Hans Klose, Heinrike Wilkens, Michael Halank, Melanie Heberling, Henning Gall, Manuel J Richter","doi":"10.1002/pul2.12397","DOIUrl":"10.1002/pul2.12397","url":null,"abstract":"<p><p>The PEGASUS study is the first multicentric and prospective assessment of the safety of air travel flying in pulmonary hypertension (PH) (NCT03051763). Data of air travel from 60 patients with PH was available. No severe adverse events occurred. Nine patients self-reported mild adverse events during flight (13%), while after landing, 12 patients reported events (20%). Solely one patient (2%) had an adverse event leading to medical consultation. In patients with PH and World Health Organization functional classes II and III, air travel was safe.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2024-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11298895/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141894204","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Clinical characteristics and anticoagulation patterns of patients with acute pulmonary thromboembolism and hemoptysis. 急性肺血栓栓塞症和咯血患者的临床特征和抗凝模式。
IF 2.2 4区 医学
Pulmonary Circulation Pub Date : 2024-07-31 eCollection Date: 2024-07-01 DOI: 10.1002/pul2.12422
Yiyao Li, Peijun Xue, Ting Zhang, Min Peng, Xuefeng Sun, Juhong Shi
{"title":"Clinical characteristics and anticoagulation patterns of patients with acute pulmonary thromboembolism and hemoptysis.","authors":"Yiyao Li, Peijun Xue, Ting Zhang, Min Peng, Xuefeng Sun, Juhong Shi","doi":"10.1002/pul2.12422","DOIUrl":"10.1002/pul2.12422","url":null,"abstract":"<p><p>Hemoptysis is a frequently encountered manifestation in cases of acute pulmonary thromboembolism (PTE), significantly impacting clinical decision-making. Despite its clinical relevance, studies focusing on patients with acute PTE and hemoptysis are notably scarce. In this retrospective study, we examined data from hospitalized patients with acute PTE at Peking Union Medical College Hospital (PUMCH) between January 2012 and October 2020. Among the 896 patients analyzed, 105 (11.7%) presented with hemoptysis. Patients with hemoptysis were younger, had higher RRs, and frequently reported chest pain, predominantly showing a negative sPESI score. A significant association with autoimmune diseases was observed (39.0% vs. 16.1%; <i>p</i> < 0.001), along with higher occurrences of pulmonary infections (29.5%), lung cancer (21.0%), and chronic heart failure (16.2%). Hemoptysis in PTE is multifactorial; 51.4% of cases were PTE-related, with 85.2% experiencing mild hemoptysis. Among patients with disease-related hemoptysis (13.3%), 90.9% with massive hemoptysis had underlying diseases, predominantly lung cancer. In 35.2% of cases, the cause of hemoptysis remained undetermined, with vasculitis accounting for 29.7%. Anticoagulation strategies varied with the severity of hemoptysis; 82.9% with mild and only 27.3% with massive hemoptysis received therapeutic-dose anticoagulation. Multivariate analysis identified massive hemoptysis as the most significant determinant of anticoagulation decisions. Patients with massive hemoptysis had the poorest outcomes, with an in-hospital mortality rate of 36.4% and 72.7% receiving reduced or no anticoagulation.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2024-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11291552/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141875788","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Editorial on "A systematic review of the impact of pulmonary thromboendarterectomy on health related quality of life". 关于 "肺血栓内膜切除术对健康相关生活质量影响的系统回顾 "的社论。
IF 2.2 4区 医学
Pulmonary Circulation Pub Date : 2024-07-29 eCollection Date: 2024-07-01 DOI: 10.1002/pul2.12420
Alison Witkin
{"title":"Editorial on \"A systematic review of the impact of pulmonary thromboendarterectomy on health related quality of life\".","authors":"Alison Witkin","doi":"10.1002/pul2.12420","DOIUrl":"10.1002/pul2.12420","url":null,"abstract":"","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2024-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11284448/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141793258","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Selexipag in patients with pulmonary arterial hypertension associated with connective tissue disease (PAH-CTD): Real-world experience from EXPOSURE. Selexipag 用于伴有结缔组织病(PAH-CTD)的肺动脉高压患者:来自 EXPOSURE 的真实世界经验。
IF 2.2 4区 医学
Pulmonary Circulation Pub Date : 2024-07-28 eCollection Date: 2024-07-01 DOI: 10.1002/pul2.12403
Sean Gaine, Pilar Escribano-Subias, Audrey Muller, Catarina C Fernandes, Martina Fontana, Tatiana Remenova, Stefan Söderberg, Tobias J Lange
{"title":"Selexipag in patients with pulmonary arterial hypertension associated with connective tissue disease (PAH-CTD): Real-world experience from EXPOSURE.","authors":"Sean Gaine, Pilar Escribano-Subias, Audrey Muller, Catarina C Fernandes, Martina Fontana, Tatiana Remenova, Stefan Söderberg, Tobias J Lange","doi":"10.1002/pul2.12403","DOIUrl":"10.1002/pul2.12403","url":null,"abstract":"<p><p>Selexipag is indicated for the treatment of pulmonary arterial hypertension (PAH), including PAH associated with connective tissue disease (CTD), and further insights into the management of selexipag-treated PAH-CTD patients in clinical settings are needed. These analyses of the ongoing, multicenter, prospective EXPOSURE (EUPAS19085) study describe characteristics, treatment patterns, tolerability, and outcomes of PAH-CTD patients initiating selexipag in Europe/Canada. All analyses were descriptive, with idiopathic PAH patients who typically display better prognosis included for context. Six hundred ninety-eight selexipag-treated patients had follow-up information; 178 (26%) had PAH-CTD. The median age was 68 years, patients were predominantly female (88%), and with WHO functional class III symptoms (63%); the median time since diagnosis was 1.7 years. There were 5% patients at low, 25% intermediate-low, 40% intermediate-high, and 30% high risk of 1-year mortality, according to the ESC/ERS 4-strata risk score. Most (80%) initiated selexipag as a triple oral therapy, and most of these (62%) remained on triple therapy 6 months post-baseline. Over a median (Q1-Q3) selexipag exposure period of 8.6 (2.5-17.2) months, 79 (44%) patients discontinued selexipag; 36 (20%) due to tolerability/adverse events. Sixty (34%) patients were hospitalized at least once; 120 hospitalizations occurred, with 49 (48%) deemed PAH-related. Survival at 1 year was 85%, and at 2 years was 71%; 29 (16%) patients died. These results describe the use of combination therapy with selexipag for patients with PAH-CTD. These findings suggest an opportunity to optimize the benefits of selexipag among patients with PAH-CTD by moving from escalating after years in response to clinical deterioration to escalating sooner to prevent clinical deterioration.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2024-07-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11284239/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141793259","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Early detection of pulmonary arterial hypertension through [18F] positron emission tomography imaging with a vascular endothelial receptor small molecule. 通过使用血管内皮受体小分子进行[18F]正电子发射断层成像,早期检测肺动脉高压。
IF 2.2 4区 医学
Pulmonary Circulation Pub Date : 2024-07-26 eCollection Date: 2024-07-01 DOI: 10.1002/pul2.12393
Zhen Yang, Feng Li, Rajarajan A Thandavarayan, Kartiga Natarajan, Diego R Martin, Zheng Li, Ashrith Guha
{"title":"Early detection of pulmonary arterial hypertension through [<sup>18</sup>F] positron emission tomography imaging with a vascular endothelial receptor small molecule.","authors":"Zhen Yang, Feng Li, Rajarajan A Thandavarayan, Kartiga Natarajan, Diego R Martin, Zheng Li, Ashrith Guha","doi":"10.1002/pul2.12393","DOIUrl":"10.1002/pul2.12393","url":null,"abstract":"<p><p>The objective of this study is to provide a positron emission tomography (PET) imaging modality targeting vascular endothelial growth factor receptors (VEGFR) for the early noninvasive detection and assessment of pulmonary arterial hypertension (PAH) severity. To validate the effectiveness of the [<sup>18</sup>F]VEGFR PET tracer, we utilized a monocrotaline (MCT)-induced PAH rat model. Molecular optical imaging, using a Cy5.5-conjugated VEGFR targeting agent, was employed to demonstrate the uptake of the agent at pulmonary arterioles, correlating with the onset and progression of PAH. Histological examinations of the MCT-PAH rat lung revealed a significant correlation between VEGFR2 expression and the pathogenesis of PAH. Molecular optical imaging demonstrated heightened uptake of the Cy5.5-conjugated VEGFR targeting agent at pulmonary arterioles, corresponding with the onset and progression of PAH. [<sup>18</sup>F]VEGFR PET showed increased lung uptake detectable in early-stage PAH before increase in pulmonary artery pressures, and this uptake correlated with increased PAH severity. Moreover, when compared to [<sup>18</sup>F]FDG PET, [<sup>18</sup>F]VEGFR PET exhibited markedly lower background cardiac signal, enhancing imaging sensitivity for lung abnormalities. Our study provides a compelling evidence for the potential utility of the innovative [<sup>18</sup>F]VEGFR PET tracer, in non-invasively detecting early signs of PAH, and monitoring its progression. The observed correlations between VEGFR2 expression, molecular optical imaging results, and [<sup>18</sup>F]VEGFR PET findings support the use of this tracer for early detection, and assessment of PAH severity. The lower background cardiac signal observed with [<sup>18</sup>F]VEGFR PET further enhances its imaging sensitivity, emphasizing its potential clinical significance.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2024-07-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11273098/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141788904","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Integrated approach of network pharmacology, molecular docking, and clinical observations in evaluating the efficacy and safety of Bufei Huoxue capsules for pulmonary hypertension associated with chronic obstructive pulmonary disease. 采用网络药理学、分子对接和临床观察相结合的方法,评价布非藿香正气胶囊治疗慢性阻塞性肺疾病相关肺动脉高压的疗效和安全性。
IF 2.2 4区 医学
Pulmonary Circulation Pub Date : 2024-07-21 eCollection Date: 2024-07-01 DOI: 10.1002/pul2.12414
Wenjun He, Chunli Liu, Xuanyi Li, Bihua Zhong, Qian Jiang, Ning Lai, Yuanhui Xiong, Weici Feng, Yilin Chen, Dansha Zhou, Defu Li, Wenju Lu, Jurjan Aman, Harm Jan Bogaard, Jian Wang, Yuqin Chen
{"title":"Integrated approach of network pharmacology, molecular docking, and clinical observations in evaluating the efficacy and safety of Bufei Huoxue capsules for pulmonary hypertension associated with chronic obstructive pulmonary disease.","authors":"Wenjun He, Chunli Liu, Xuanyi Li, Bihua Zhong, Qian Jiang, Ning Lai, Yuanhui Xiong, Weici Feng, Yilin Chen, Dansha Zhou, Defu Li, Wenju Lu, Jurjan Aman, Harm Jan Bogaard, Jian Wang, Yuqin Chen","doi":"10.1002/pul2.12414","DOIUrl":"10.1002/pul2.12414","url":null,"abstract":"<p><p>Chronic obstructive pulmonary disease (COPD) is a persistent and progressive disorder characterized by airway or alveolar abnormalities, commonly leading to pulmonary hypertension (PH). This clinical observational study investigates the therapeutic mechanisms of Bufei Huoxue capsules (BHC) in treating PH in patients with COPD-linked PH (COPD-PH) using network pharmacology and molecular docking methods, and assesses the therapeutic efficacy and safety of BHCs. The active compounds and their target proteins in BHCs were sourced from the Traditional Chinese Medicine Systems Pharmacology database, with additional target proteins derived from the GeneCards and OMIM databases. An active network was constructed using Cytoscape 3.7.1, and interaction networks were established. Intersecting targets underwent Gene Ontology (GO) enrichment analysis and Kyoto Encyclopedia of Genes and Genomes (KEGG) pathway analysis using the Metascape database. Network pharmacology and molecular docking studies demonstrated favorable binding affinities of BHC active ingredients, such as quercetin, bavachalcone, and isobavachin, for key targets including PTGS1, ESR1, and PTGS2. Gene Ontology enrichment analysis highlighted the involvement of these targets in processes such as the positive regulation of locomotion, the transmembrane receptor protein tyrosine kinase signaling pathway, and peptidyl-tyrosine phosphorylation. KEGG pathway analysis indicated their roles in pathways related to cancer, AGE-RAGE signaling in diabetic complications, and prostate cancer. BHCs exhibit therapeutic effects on COPD-PH through multi-component, multi-target, and multi-pathway interactions. This clinical observational study confirms the efficacy and safety of BHCs in improving cardiac and pulmonary functions, enhancing exercise tolerance, and elevating the quality of life in patients with COPD-PH.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2024-07-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11260391/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141734871","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
We are all in this together: Understanding organ crosstalk in the pathogenesis of acute respiratory distress syndrome. 我们同舟共济:了解急性呼吸窘迫综合征发病机制中的器官串联。
IF 2.2 4区 医学
Pulmonary Circulation Pub Date : 2024-07-19 eCollection Date: 2024-07-01 DOI: 10.1002/pul2.12418
Dustin R Fraidenburg
{"title":"We are all in this together: Understanding organ crosstalk in the pathogenesis of acute respiratory distress syndrome.","authors":"Dustin R Fraidenburg","doi":"10.1002/pul2.12418","DOIUrl":"10.1002/pul2.12418","url":null,"abstract":"","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2024-07-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11258468/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141734872","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Quantifying the impact of post-acute sequelae of coronavirus on the cardiopulmonary endurance of athletes. 量化冠状病毒急性后遗症对运动员心肺耐力的影响。
IF 2.2 4区 医学
Pulmonary Circulation Pub Date : 2024-07-17 eCollection Date: 2024-07-01 DOI: 10.1002/pul2.12413
Daniel Lubarsky, Daniel E Clark, Kimberly Crum, Ashley Karpinos, Eric D Austin, Jonathan H Soslow
{"title":"Quantifying the impact of post-acute sequelae of coronavirus on the cardiopulmonary endurance of athletes.","authors":"Daniel Lubarsky, Daniel E Clark, Kimberly Crum, Ashley Karpinos, Eric D Austin, Jonathan H Soslow","doi":"10.1002/pul2.12413","DOIUrl":"10.1002/pul2.12413","url":null,"abstract":"<p><p>Post-acute sequelae of Coronavirus (PASC), or Long COVID, has emerged as a critical health concern. The clinical manifestations of PASC have been described, but studies have not quantified the cardiopulmonary effects. The goal of this study was to quantify PASC cardiopulmonary changes among endurance athletes. Endurance athletes were recruited via social media; 45 met inclusion criteria, 32 had PASC and 13 were asymptomatic at 3 months (control). Comprehensive interviews were conducted to assess: cardiopulmonary symptoms at 3 months; quantitative and qualitative changes in cardiovascular endurance; exercise hours per week at baseline and 3 months; and Modified Oslo, Dyspnea, and EQ-5D-5L scales. All collected data was based on self-reported symptoms. Wilcoxon rank sum compared PASC with control to distinguish the effects of PASC vs effects of COVID infection/lockdown. PASC subjects were more likely to be female (Table). The most common 3-month symptoms in PASC were fatigue and shortness of breath. Based on self-reported data, subjects endorsed a median decrease of 27% in cardiopulmonary endurance levels compared with 0% in controls (<i>p</i> = 0.0019). PASC subjects exercised less hours and had worse self-reported health as compared with controls. PASC subjects also had significantly worse Modified Oslo, Dyspnea, and EQ-5D-5L scores. Of the 32 PASC patients, 10 (31%) reported a complete inability to engage in any cardiovascular endurance exercise at 3 months. PASC leads to a significant, quantifiable decrease in cardiopulmonary health and endurance.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2024-07-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11252491/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141634335","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Correction to "Cardiopulmonary exercise testing and the 2022 definition of pulmonary hypertension". 更正 "心肺运动测试和肺动脉高压的 2022 年定义"。
IF 2.2 4区 医学
Pulmonary Circulation Pub Date : 2024-07-17 eCollection Date: 2024-07-01 DOI: 10.1002/pul2.12417
{"title":"Correction to \"Cardiopulmonary exercise testing and the 2022 definition of pulmonary hypertension\".","authors":"","doi":"10.1002/pul2.12417","DOIUrl":"https://doi.org/10.1002/pul2.12417","url":null,"abstract":"<p><p>[This corrects the article DOI: 10.1002/pul2.12398.].</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2024-07-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11255375/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141724320","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
An adult patient with pulmonary arterial hypertension, a NOTCH3 mutation, and leflunomide exposure. 一名患有肺动脉高压、NOTCH3 突变和来氟米特接触的成年患者。
IF 2.2 4区 医学
Pulmonary Circulation Pub Date : 2024-07-09 eCollection Date: 2024-07-01 DOI: 10.1002/pul2.12411
Elizabeth G Fenner, Catherine E Simpson
{"title":"An adult patient with pulmonary arterial hypertension, a <i>NOTCH3</i> mutation, and leflunomide exposure.","authors":"Elizabeth G Fenner, Catherine E Simpson","doi":"10.1002/pul2.12411","DOIUrl":"10.1002/pul2.12411","url":null,"abstract":"<p><p>Pulmonary arterial hypertension (PAH) is a poorly understood disease of the small pulmonary arteries. Pulmonary vascular remodeling and progressively rising pulmonary vascular resistance are hallmarks of the disease that ultimately result in right heart failure. Several genetic mutations, most notably in bone morphogenetic protein receptor type 2, have a causal association with heritable forms of PAH. Mutations in <i>neurogenic locus notch homolog protein 3</i> (<i>NOTCH3)</i> have been reported in adults and children with PAH, but whether <i>NOTCH3</i> is causally associated with PAH is debated. With this case report, we describe the clinical characteristics, comorbidities, and exposure history of an adult patient with PAH and multiple sclerosis who was found to have a <i>NOTCH3</i> missense mutation and exposure to leflunomide.</p>","PeriodicalId":20927,"journal":{"name":"Pulmonary Circulation","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2024-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11233402/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141580722","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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