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Chronic thromboembolic pulmonary hypertension associated with venous thoracic outlet syndrome: a retrospective case series. 慢性血栓栓塞性肺动脉高压与静脉胸廓出口综合征:回顾性病例系列。
IF 21 1区 医学 Q1 RESPIRATORY SYSTEM Pub Date : 2025-12-18 Print Date: 2025-12-01 DOI: 10.1183/13993003.01610-2025
Mitja Jevnikar, Philippe Brenot, Mithum Kularatne, Alexandre Rodière, Elise Noël-Savina, Ari Chaouat, Samuel Dolidon, Athénaïs Boucly, Olivier Sitbon, Laurent Savale, David Montani, Olaf Mercier, Marc Humbert, Elie Fadel, Xavier Jaïs
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引用次数: 0
Active case finding for tuberculosis among Ukrainian soldiers by German armed forces. 在德国武装部队的乌克兰士兵中积极发现肺结核病例。
IF 21 1区 医学 Q1 RESPIRATORY SYSTEM Pub Date : 2025-12-18 Print Date: 2025-12-01 DOI: 10.1183/13993003.00728-2025
Jessica Pohl, Matthias I Gröschel, Nino Neumann, Anett Tillmann, Stephan Waldeck, Tobias Schneider, Daniel Overhoff, Stephanie G Lange, Thomas Bucksch, Rebecca Peuker, Dominic Rauschning, Gwendolyn Scheumann, Lorenz Scheit, Maximilian Schreiner, Werner Wenzel, Kai Kehe, Inna Friesen, Viola Dreyer, Stefan Niemann, Leif-Erik Sander, Martin Witzenrath, Geraldine Nouailles, Christian M Zobel
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引用次数: 0
ERJ Podcast December 2025: Year in review. ERJ播客2025年12月:年度回顾。
IF 21 1区 医学 Q1 RESPIRATORY SYSTEM Pub Date : 2025-12-18 Print Date: 2025-12-01 DOI: 10.1183/13993003.E6606-2025
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引用次数: 0
Large and small airway remodeling in human end-stage primary ciliary dyskinesia lungs. 人类终末期原发性纤毛运动障碍肺大、小气道重塑。
IF 24.3 1区 医学 Q1 RESPIRATORY SYSTEM Pub Date : 2025-12-18 DOI: 10.1183/13993003.01744-2025
Astrid Vermaut,Gitte Aerts,Lise Vanvuchelen,Vincent Geudens,Lynn Willems,Arno Vanstapel,Charlotte Hooft,Pieterjan Kerckhof,Hanne Beeckmans,Xin Jin,Marta Zapata-Ortega,Yousry Mohamady,Jan Van Slambrouck,Lucia Aversa,Janne Verhaegen,Emanuela E Cortesi,Laurens J Ceulemans,Dirk E Van Raemdonck,Robin Vos,Natalie Lorent,Birgit Weynand,Marijke Proesmans,François Vermeulen,Lieven J Dupont,Bart M Vanaudenaerde,Mieke Boon
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引用次数: 0
Emerging therapies in bronchiectasis. 支气管扩张的新疗法。
IF 21 1区 医学 Q1 RESPIRATORY SYSTEM Pub Date : 2025-12-18 Print Date: 2025-12-01 DOI: 10.1183/13993003.01309-2025
Oriol Sibila, Lidia Perea, Stefano Aliberti
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引用次数: 0
"Natural disasters and respiratory health." I. Annesi-Maesano, H. Bayram, L. Cecchi, et al. Eur Respir J 2025; 66: 2402563. “自然灾害和呼吸系统健康。”李建军,李建军,李建军,等。中华呼吸病学杂志[J];66: 2402563。
IF 24.3 1区 医学 Q1 RESPIRATORY SYSTEM Pub Date : 2025-12-18 DOI: 10.1183/13993003.52563-2024
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引用次数: 0
A Point-of-Care Ultrasound-Driven Diagnostic Pathway for Emergency Department Patients with Dyspnea: A Randomized Controlled Trial. 急诊呼吸困难患者的即时超声诊断途径:一项随机对照试验。
IF 24.3 1区 医学 Q1 RESPIRATORY SYSTEM Pub Date : 2025-12-18 DOI: 10.1183/13993003.00070-2025
Stig Holm Ovesen,Søren Helbo Skaarup,Rasmus Aagaard,Nikolaj Raaber,Gitte Boier Tygesen,Thomas Nielsen,Charlotte Møgelvang,Jesper Wamberg,Peter Biesenbach,Christina Brandhof,Danny Yu,Jakob Grønnebæk Rhode,Christian Linde Larsen,Kas Zarandi,Christina Katrin Lehmann,Søren Majgaard Pedersen,Mads Damgaard Mørkenborg,Ronja Leth,Simon Thorgaard-Rasmussen,Philip Uhd,Bo Løfgren,Stefan Posth,Michael Dan Arvig,Bo Martin Bibby,Christian B Laursen,Hans Kirkegaard,Jesper Weile
BACKGROUNDPrevious trials have suggested that point-of-care ultrasound for emergency department (ED) patients with dyspnea increases the proportion of patients discharged within 24 h. We aimed to assess whether this effect could be confirmed.METHODSThis trial was a randomized controlled trial in ten Danish EDs. Adult patients presenting to the ED with dyspnea as the chief complaint were randomized to the addition or omission of focused lung and cardiac ultrasound. The primary outcome was the proportion of patients discharged alive within 24 h. Secondary outcomes included overall hospital length of stay, chest imaging utilization, and 72 h alive and revisit-free.RESULTSAmong 674 patients who were randomized between January 25, 2023, and August 23, 2024, 663 were included in the analysis. The primary outcome occurred in 141 (42.6%) of 331 patients in the intervention group versus 151 (45.5%) of 332 in the control group (risk difference: -2.9; 95% confidence interval: -10.4-4.7; p=0.45). The overall incidence rate of hospital discharges per person-days at risk was 0.28 in the intervention group versus 0.32 in the control group (hazard ratio: 0.93; 95% confidence interval: 0.79-1.08; p=0.35).CONCLUSIONSIn adult ED patients with dyspnea as chief complaint, a point-of-care ultrasound-driven diagnostic pathway did not alter the proportion of patients discharged alive within 24 h or the overall hospital length of stay compared with standard care.
背景:先前的试验表明,急诊(ED)呼吸困难患者的即时超声可增加24小时内出院患者的比例。我们的目的是评估这种影响是否可以被证实。方法采用随机对照试验,纳入10例丹麦急诊患者。以呼吸困难为主诉的ED成年患者随机加入或遗漏聚焦肺和心脏超声。主要终点是24小时内存活出院的患者比例。次要结局包括总住院时间、胸部成像利用率、存活72小时和无再访。结果在2023年1月25日至2024年8月23日期间随机抽取的674例患者中,有663例纳入分析。干预组331例患者中有141例(42.6%)出现主要结局,对照组332例患者中有151例(45.5%)出现主要结局(风险差异:-2.9;95%可信区间:-10.4-4.7;p=0.45)。干预组每高危人日出院总发生率为0.28,对照组为0.32(风险比:0.93;95%可信区间:0.79-1.08;p=0.35)。结论在以呼吸困难为主诉的成人ED患者中,与标准治疗相比,定点超声驱动的诊断途径并没有改变24小时内存活出院的患者比例或总住院时间。
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引用次数: 0
Assessment of mTOR pathways in DIPNECH (Diffuse Idiopathic Neuroendocrine Cell Hyperplasia). 弥漫性特发性神经内分泌细胞增生(DIPNECH)中mTOR通路的评估。
IF 24.3 1区 医学 Q1 RESPIRATORY SYSTEM Pub Date : 2025-12-18 DOI: 10.1183/13993003.01692-2025
Helen O'Brien,Marissa O'Callaghan,Lisa Comerford,Janet McCormack,Amina Jouida,Niamh Boyle,Donal O'Malley,Julie Worrell,Alessandro N Franciosi,Adam J Byrne,Aurelie Fabre,Michael P Keane,Cormac McCarthy
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引用次数: 0
ERJ Podcast November 2025: Artificial intelligence in publishing. ERJ播客2025年11月:出版中的人工智能。
IF 24.3 1区 医学 Q1 RESPIRATORY SYSTEM Pub Date : 2025-12-15 DOI: 10.1183/13993003.e6605-2025
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引用次数: 0
Pulmonary hypertension in patients with Noonan syndrome. 努南综合征患者肺动脉高压的研究。
IF 24.3 1区 医学 Q1 RESPIRATORY SYSTEM Pub Date : 2025-12-11 DOI: 10.1183/13993003.01796-2025
Julien Grynblat,Mathieu Farges,Pascal Magro,Laurent Savale,Maria-Rosa Ghigna,Julia Tagmouti,Xavier Jais,Athénaïs Boucly,Marilyne Levy,Fabrice Antigny,Olivier Meyrignac,Stanislas Lyonnet,Helene Cavé,Romain Nicolle,Sylvain Marchand-Adam,Olivier Sitbon,Florence Coulet,Marc Humbert,Damien Bonnet,David Montani
BACKGROUNDNoonan syndrome (NS) is a RASopathy inherited in an autosomal dominant manner, mainly caused by gain-of-function variants activating the RAS/MAPK signalling pathway. Pulmonary hypertension (PH) may occur in NS, but its mechanisms, clinical characteristics, and outcomes remain poorly defined.METHODSWe analysed data from the French PH Network to characterize the phenotype of NS patients who develop PH, and conducted a systematic analysis of the literature.RESULTSSeven patients were identified from the French PH Network (male/female ratio: 1.1), with a median age at PH diagnosis of 9 years (range 5-21). Genetic analysis revealed five pathogenic variants in PTPN11 and one in SOS1. Associated features included facial dysmorphism, growth retardation, atrial septal defect and pulmonary valve stenosis. Hemodynamics showed severe precapillary PH without acute vasodilator response: mean pulmonary artery pressure 55 mmHg (40-78), cardiac output 3.95 L.min-1 (3.12-4.95) and pulmonary vascular resistance 13 WU (10-15.3). Computed tomography of the chest identified perivascular ground-glass opacities, mediastinal infiltration, dilated bronchial arteries, distal pulmonary vascular tortuosity and possible arteriovenous shunts. Five patients were treated with drugs approved for PAH. Three patients died and one underwent lung transplantation. Explanted lungs revealed plexiform lesions associated with diffuse lymphangiectasia. Twelve additional cases from the literature included seven with precapillary PH, four with postcapillary PH due to cardiomyopathy, and one without RHC.CONCLUSIONPrecapillary and postcapillary PH may complicate the course of NS, potentially in association with congenital heart defects and multisystem manifestations. Further studies are needed to better delineate the phenotype of PH in patients with NS.
背景noonan综合征(NS)是一种常染色体显性遗传的ras病,主要由激活RAS/MAPK信号通路的功能获得变异体引起。肺动脉高压(PH)可能发生在NS中,但其机制、临床特征和结局仍不明确。方法我们分析了法国PH网络的数据,以表征发生PH的NS患者的表型,并对文献进行了系统分析。结果7例患者来自法国PH网络(男女比例:1.1),PH诊断时的中位年龄为9岁(范围5-21岁)。遗传分析显示PTPN11有5个致病变异,SOS1有1个致病变异。相关特征包括面部畸形、生长迟缓、房间隔缺损和肺动脉瓣狭窄。血流动力学显示严重的毛细血管前PH值,无急性血管舒张反应:平均肺动脉压55 mmHg(40-78),心输出量3.95 L。min-1(3.12-4.95)和肺血管阻力13 WU(10-15.3)。胸部计算机断层扫描发现血管周围磨玻璃混浊,纵隔浸润,支气管动脉扩张,远端肺血管扭曲和可能的动静脉分流。5例患者接受批准用于PAH的药物治疗。3名患者死亡,1名患者接受肺移植。外植肺显示丛状病变伴弥漫性淋巴管扩张。文献中的另外12例包括7例毛细血管前PH, 4例由于心肌病引起的毛细血管后PH, 1例无RHC。结论毛细血管前和毛细血管后的PH值可能使NS的病程复杂化,可能与先天性心脏缺陷和多系统表现有关。需要进一步的研究来更好地描述NS患者的PH表型。
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引用次数: 0
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European Respiratory Journal
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