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Through the MIRRA and what we found there. 通过 MIRRA 和我们在那里发现的东西。
IF 4.3 3区 医学 Q1 RESPIRATORY SYSTEM Pub Date : 2024-01-08 eCollection Date: 2024-01-01 DOI: 10.1183/23120541.00825-2023
Shigeharu Ueki

A series of post hoc MIRRA studies have illuminated eosinophilic granulomatosis with polyangiitis as an eosinophil-driven disease from various perspectives https://bit.ly/468pj86.

一系列事后MIRRA研究从不同角度揭示了嗜酸性粒细胞肉芽肿伴多血管炎是一种嗜酸性粒细胞驱动的疾病https://bit.ly/468pj86。
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引用次数: 0
Vocal cord dysfunction/inducible laryngeal obstruction cannot be diagnosed from symptoms. 声带功能障碍/诱发性喉阻塞不能从症状中诊断出来。
IF 4.6 3区 医学 Q1 RESPIRATORY SYSTEM Pub Date : 2023-12-27 eCollection Date: 2023-11-01 DOI: 10.1183/23120541.00707-2023
Grace Yap, Laurence E Ruane, Kais Hamza, Elizabeth Leahy, Adriana Avram, Malcolm Baxter, Joo Koh, Philip G Bardin, Paul Leong

Vocal cord dysfunction/inducible laryngeal obstruction is highly variable. Standard clinical symptoms and questionnaires cannot predict laryngoscopic diagnosis in a "lung disease" population. https://bit.ly/3QUtsbB.

声带功能障碍/诱发性喉阻塞的情况千变万化。标准临床症状和问卷调查无法预测 "肺病 "人群的喉镜诊断结果。https://bit.ly/3QUtsbB。
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引用次数: 0
Using an electronic diary and wristband accelerometer to detect exacerbations and activity levels in COPD: a feasibility study. 使用电子日记和腕带加速度计检测慢性阻塞性肺病的病情加重和活动量:一项可行性研究。
IF 4.6 3区 医学 Q1 RESPIRATORY SYSTEM Pub Date : 2023-12-27 eCollection Date: 2023-11-01 DOI: 10.1183/23120541.00366-2023
Lydia J Finney, Stefan Avey, Dexter Wiseman, Anthony Rowe, Matthew J Loza, Patrick Branigan, Christopher S Stevenson, Frédéric Baribaud, Jadwiga A Wedzicha, Ioannis Pandis, Gavin C Donaldson

Background: Early and accurate identification of acute exacerbations of COPD may lead to earlier treatment and prevent hospital admission. Electronic diaries have been developed for symptom monitoring and accelerometers to monitor activity. However, it is unclear whether this technology is usable in the COPD population. This study aimed to assess the feasibility of an electronic diary (eDiary) for symptom reporting using the MoreCare app and activity monitoring with the Garmin Vivofit 2 in COPD.

Methods: Participants were recruited from the London COPD Cohort. Participants were provided a Garmin Vivofit 2 activity monitor and an android tablet with the MoreCare app for a period of 3 months.

Results: 25 COPD patients were recruited (mean±sd age 70.8±7.1 years, forced expiratory volume in 1 s (FEV1) 49.8±14.8% predicted). Age, gender, disease severity and exacerbation frequency had no impact on eDiary compliance. There was a moderate positive correlation between median daily very active minutes and FEV1 % pred (ρ=0.62, p=0.005). Daily step counts decreased during the initial 7 days of exacerbation and recovery compared to a pre-exacerbation baseline. A decision-tree model identified change in sputum colour, change in step count, severity of cold, exacerbation history and use of rescue medication as the most important predictors of acute exacerbations of COPD in this cohort.

Conclusions: Symptom and activity monitoring using digital technology is feasible in COPD. Further large-scale digital health studies are needed to assess whether eDiaries can be used to identify patients at risk of exacerbation and guide early intervention.

背景:及早准确地识别慢性阻塞性肺病的急性加重,可使患者更早接受治疗,避免入院。目前已开发出用于监测症状的电子日记和监测活动的加速度计。然而,目前还不清楚这种技术是否适用于慢性阻塞性肺病患者。本研究旨在评估使用 MoreCare 应用程序报告症状的电子日记(eDiary)和使用 Garmin Vivofit 2 监测慢性阻塞性肺病患者活动的可行性:从伦敦慢性阻塞性肺病队列中招募参与者。结果:共招募了 25 名慢性阻塞性肺病患者(平均年龄(±sd)为 70.8±7.1 岁,1 秒内用力呼气容积(FEV1)预测值为 49.8±14.8%)。年龄、性别、疾病严重程度和恶化频率对电子日记依从性没有影响。每日非常活跃分钟数中位数与预测值 FEV1 % 之间存在中度正相关(ρ=0.62,p=0.005)。在病情加重和恢复的最初 7 天内,与病情加重前的基线相比,每日步数有所减少。通过决策树模型确定,痰液颜色的变化、步数的变化、感冒的严重程度、病情恶化史和抢救药物的使用是该队列中慢性阻塞性肺病急性加重最重要的预测因素:结论:使用数字技术监测慢性阻塞性肺病患者的症状和活动是可行的。需要进一步开展大规模的数字健康研究,以评估电子日记是否可用于识别有病情加重风险的患者并指导早期干预。
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引用次数: 0
Feasibility and value of a domiciliary spirometry programme in the assessment of severe asthma: a real-world evaluation. 家庭肺活量测量计划在评估严重哮喘中的可行性和价值:真实世界评估。
IF 4.6 3区 医学 Q1 RESPIRATORY SYSTEM Pub Date : 2023-12-27 eCollection Date: 2023-11-01 DOI: 10.1183/23120541.00635-2023
Zander Williams, James H Hull, Yueqi Ge, Jo Ming, Cara Roberts, Serena Rhamie, Pujan H Patel

Background: Domiciliary spirometry (DS) is a novel tool that is widely employed in the assessment of respiratory disease. We assessed real-world feasibility, effectiveness and value of a physiologist-led home spirometry programme in patients with treatment-refractory severe asthma.

Methods: Patients were referred and provided with a hand-held DS device. Patients completed baseline measurements in a physiologist-led virtual clinic and were instructed to provide further values during any periods of respiratory symptoms. Outcome measures included prevalence of new obstructed events, DS adherence and uptake of this approach.

Results: 112 patients were enrolled from November 2020 to January 2023. 102 individuals, mean±sd age 44±13 years (86% female) with median (IQR) forced expiratory volume in 1 s % predicted 88% (77-97%), successfully recorded baseline spirometry values. During follow-up (24 months), 11 (11%) were identified with new obstructive spirometry and were subsequently able to be commenced on biologic therapy. Patient engagement was poor with median (IQR) of 4 (2-6) attempts of contact made before baseline values were recorded, and 2 (1-3) attempts required to record technically acceptable values. Continued DS use was suboptimal; 34% failed to use their device after baseline and only 10% continued at the end of the study period. The cost of DS measurements was greater than a single hospital-based visit but enables multiple event capture.

Conclusion: Overall, DS measurement uptake was poor, with a minority of patients continuing to use the device at the end of the study period. However, for those that engage, DS provides an alternative approach to traditional hospital-based spirometry measurements that can alter clinical management.

背景:家庭肺活量测定(DS)是一种新型工具,被广泛用于呼吸系统疾病的评估。我们评估了由生理学家主导的家庭肺活量测量项目在治疗难治性重症哮喘患者中的实际可行性、有效性和价值:方法:患者被转诊并获得手持式 DS 设备。患者在生理学家主导的虚拟诊所完成基线测量,并被要求在出现呼吸道症状期间提供进一步的测量值。结果测量包括新阻塞事件的发生率、DS的坚持率和对这种方法的接受率:112 名患者于 2020 年 11 月至 2023 年 1 月期间入组。102人,平均年龄(±sd)为44±13岁(86%为女性),1秒内用力呼气容积中位数(IQR)预测值为88%(77-97%),成功记录了基线肺活量值。在随访期间(24 个月),发现 11 例(11%)患者出现新的阻塞性肺活量,随后开始接受生物治疗。患者参与度较低,在记录基线值之前,患者尝试联系的次数中位数(IQR)为 4 次(2-6 次),记录技术上可接受的数值需要 2 次(1-3 次)。继续使用 DS 的情况并不理想;34% 的人在基线后没有使用设备,只有 10% 的人在研究结束时继续使用。DS 测量的成本高于单次医院就诊,但可以捕捉多个事件:总体而言,DS 测量的使用率较低,只有少数患者在研究结束时继续使用该设备。然而,对于那些参与的患者,DS 提供了一种替代传统医院肺活量测量的方法,可以改变临床管理。
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引用次数: 0
Reflecting real-world patients with mesothelioma in research: an interim report of baseline characteristics from the ASSESS-meso cohort 在研究中反映间皮瘤患者的真实情况:ASSESS-meso 队列基线特征中期报告
IF 4.6 3区 医学 Q1 RESPIRATORY SYSTEM Pub Date : 2023-12-21 DOI: 10.1183/23120541.00467-2023
R. Conway, Natalie Smith, William Cooper, Geraldine Lynch, S. Patole, J. Symonds, Anthony Edey, Nick A Maskell, A. Bibby
Mesothelioma varies in clinical phenotype and survival. Clinical trials are unavoidably affected by selection bias, reducing generalisability. ASSESS-meso is a UK, multi-centre, prospective, mesothelioma cohort study (ISRCTN61861764). This pre-specified interim analysis, conducted when recruitment reached 25% of target, summarised participant characteristics and evaluated external validity through comparison with real-world and clinical trial cohorts.The study took place at 14 hospitals across the UK. People diagnosed with mesothelioma, at any anatomical site, were eligible. Clinical, radiological and biochemical data were collected at enrolment. In this interim report, the external validity of the cohort was investigated through comparison of baseline demographic data with populations included in the 2020 UK National Mesothelioma Audit (real-world cohort), and CHECKMATE-743 and MAPS trials (clinical trial cohorts).Between 07/04/2017–01/03/2022, 244 patients were enrolled. The cohort was predominantly male (195/244; 80%) with median age of 74 years. Pleural disease and epithelioid sub-types were most prevalent. ASSESS-meso participants were more similar to the real-world population with regards age, performance status, disease site and stage than the clinical trial population. ASSESS-meso participants were more likely to be formally staged and less likely to have undifferentiated histology compared with the real-world cohort, possibly reflecting high rates of discussion of ASSESS-meso participants at regional mesothelioma MDTs. As expected, poorer performance status, non-epithelioid histology and neutrophil-lymphocyte ratio were associated with shorter survival in the adjusted analysis.ASSESS-meso is representative of the UK mesothelioma population. Future outputs from the cohort will help characterise different mesothelioma phenotypes with high external validity.
间皮瘤的临床表型和存活率各不相同。临床试验不可避免地会受到选择偏差的影响,从而降低普适性。ASSESS-meso 是英国一项多中心、前瞻性间皮瘤队列研究(ISRCTN61861764)。这项预先指定的中期分析是在招募人数达到目标的25%时进行的,它总结了参与者的特征,并通过与真实世界和临床试验队列的比较评估了外部有效性。该研究在英国 14 家医院进行,任何解剖部位的间皮瘤患者均符合条件。临床、放射和生化数据均在注册时收集。在这份中期报告中,通过将基线人口统计学数据与2020年英国国家间皮瘤审计(真实世界队列)、CHECKMATE-743和MAPS试验(临床试验队列)中的人群进行比较,研究了队列的外部有效性。在 2017 年 4 月 7 日至 2022 年 3 月 1 日期间,244 名患者入组。入组患者主要为男性(195/244;80%),中位年龄为 74 岁。胸膜疾病和上皮样亚型最为常见。与临床试验人群相比,ASSESS-meso 参与者在年龄、表现状态、发病部位和分期方面与实际人群更为相似。与真实世界人群相比,ASSESS-meso参与者更有可能被正式分期,而未分化组织学的可能性较小,这可能反映了ASSESS-meso参与者在地区间皮瘤MDT中的高讨论率。正如预期的那样,在调整后的分析中,较差的表现状态、非上皮样组织学和中性粒细胞-淋巴细胞比率与较短的生存期有关。ASSESS-meso具有英国间皮瘤人群的代表性,该群组的未来产出将有助于描述不同间皮瘤表型的特征,具有很高的外部有效性。
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引用次数: 0
Elevated transaminases in congenital central hypoventilation syndrome (CCHS) 先天性中枢通气不足综合征(CCHS)转氨酶升高
IF 4.6 3区 医学 Q1 RESPIRATORY SYSTEM Pub Date : 2023-12-21 DOI: 10.1183/23120541.00658-2023
Rachel Y. Wang, Victoria S. Wang, Thomas G. Keens, Yan Chai, Nisreen Soufi, Iris A. Perez
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引用次数: 0
The many faces of copd in real life: a longitudinal analysis of the novelty cohort 现实生活中的多面 copd:对新奇队列的纵向分析
IF 4.6 3区 医学 Q1 RESPIRATORY SYSTEM Pub Date : 2023-12-21 DOI: 10.1183/23120541.00895-2023
Alvar Agustí, R. Hughes, Eleni Rapsomaki, B. Make, Ricardo del Olmo, Alberto Papi, David Price, Laura Benton, Stefan Franzén, Jørgen Vestbo, Hana Mullerova
The diagnosis of Chronic Obstructive Pulmonary Disease (COPD) requires the demonstration of non-fully reversible airflow limitation by spirometry in the appropriate clinical context. Yet, there are patients with symptoms and relevant exposures suggestive of COPD with either normal spirometry (pre-COPD) or Preserved Ratio but Impaired Spirometry (PRISm). Their prevalence, clinical characteristics and associated outcomes in a real-life setting are unclear.To investigate them, we studied 3183 patients diagnosed of COPD by their attending physician included in the NOVELTY study (NCT02760329), a global, 3-year, observational, real-life cohort that included patients recruited from both primary and specialist care clinics in 18 countries.We found that:(1)about a quarter of patients diagnosed with (and treated for) COPD in real-life did not fulfil the spirometric diagnostic criteria recommended by GOLD, and could be instead categorized as pre-COPD (13%) or PRISm (14%);(2)disease burden (symptoms and exacerbations) was highest in GOLD 3–4 patients (exacerbations per person-year [PPY]: 0.82) and lower but similar in those in GOLD 1–2, pre-COPD and PRISm (exacerbations PPY range: 0.27-0.43);(3)lung function decline was highest in pre-COPD and GOLD 1–2, and much less pronounced in PRISm and GOLD 3-4;(4); PRISm and pre-COPD were not stable diagnostic categories and change substantially over time; and,(5)all-cause mortality was highest in GOLD 3–4, lowest in pre-COPD, and intermediate and similar in GOLD 1–2 and PRISm.Patients diagnosed COPD in a real-life clinical setting present great diversity in symptom burden, progression, and survival, warranting medical attention.
慢性阻塞性肺病(COPD)的诊断需要在适当的临床环境下通过肺活量测定证明气流受限是非完全可逆的。然而,有些患者有慢性阻塞性肺病的症状和相关暴露,但肺活量正常(慢性阻塞性肺病前期)或肺活量保留比率但受损(PRISm)。为了对这些患者进行调查,我们对 NOVELTY 研究(NCT02760329)中由主治医生诊断为慢性阻塞性肺病的 3183 名患者进行了研究,该研究是一项为期 3 年的全球性观察性现实生活队列,包括从 18 个国家的初级和专科诊所招募的患者。我们发现:(1)在现实生活中被诊断为慢性阻塞性肺病(并接受治疗)的患者中,约有四分之一不符合 GOLD 推荐的肺活量诊断标准,可被归类为慢性阻塞性肺病前期(13%)或 PRISm(14%);(2)GOLD 3-4 患者的疾病负担(症状和病情恶化)最高(每人年病情恶化次数 [PPY]:0.82),而GOLD 1-2、COPD前期和PRISm患者的疾病负担较低但相似(每人年加重次数范围:0.27-0.43);(3)COPD前期和GOLD 1-2患者的肺功能下降幅度最大,而PRISm和GOLD 3-4患者的肺功能下降幅度较小;(4)PRISm和COPD前期并非稳定的诊断类别,随着时间的推移会发生很大变化;(5)GOLD 3-4患者的全因死亡率最高,COPD前期最低,而GOLD 1-2和PRISm患者的全因死亡率居中且相似。在真实的临床环境中,被诊断为慢性阻塞性肺病的患者在症状负担、病情发展和存活率方面表现出很大的差异,值得引起医疗关注。
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引用次数: 0
Endothelial to mesenchymal transition is an active process in smokers and patients with early COPD contributing to pulmonary arterial pathology 内皮向间充质转化是吸烟者和早期慢性阻塞性肺疾病患者的一个活跃过程,可导致肺动脉病变
IF 4.6 3区 医学 Q1 RESPIRATORY SYSTEM Pub Date : 2023-12-21 DOI: 10.1183/23120541.00767-2023
P. Bhattarai, Wenying Lu, A. Hardikar, Surajit Dey, A. Gaikwad, Affan Mahmood Shahzad, C. Chia, Andrew Williams, G. Singhera, T. Hackett, M. Eapen, S. Sohal
We have previously reported pulmonary arterial remodelling in smokers and patients with early COPD, which can be attributed to EndMT. In this study, we aimed to evaluate if EndMT is an active mechanism in smokers and COPD.Immunohistochemical staining for EndMT biomarkers, CD-31, N-cadherin, Vimentin and S100A4, was done on lung resections from 49 subjects. Fifteen were non-smoker-controls (NC), six normal lung function smokers (NLFS), nine patients with small-airway diseases (SAD), nine mild-moderate COPD-current smokers (COPD-CS) and ten COPD-ex-smokers (COPD-ES). Pulmonary arteries were analysed using Image ProPlus software v7.0.We noted reduced junctional CD31-positive endothelial cells (p<0.05) in intimal layer of all smoking groups compared to NC. Compared to NC, increased abundance of mesenchymal markers N-cadherin (p<0.05) and Vimentin (p<0.001) was observed in all smoking groups and across all arterial sizes, except for N-Cadherin in large arterial size for COPD-CS. Abundance of S100A4 correlated with arterial thickness (r= 0.29, 0.33, 0.35; p=0.05, 0.03, 0.02 respectively for small, medium, and large arteries). Vimentin in the small arterial wall negatively correlated with FEV1/ FVC and FEF25–75% (r= −0.35, −0.34; p= 0.02,0.03, respectively), while increased cytoplasmic CD-31 abundance in the intimal layer of medium and large arteries negatively correlated with DLCO-predicted (r= −0.35, −0.39; p=0.04, 0.03 respectively).This is the first study showing the acquisition of mesenchymal traits by pulmonary endothelial cells from NLFS, SAD and mild-moderate COPD patients through EndMT. This informs the potential early origins of pulmonary hypertension in smokers and patients with early COPD.
我们以前曾报道过吸烟者和早期慢性阻塞性肺病患者的肺动脉重塑,这可能是由于 EndMT 引起的。在这项研究中,我们旨在评估 EndMT 是否是吸烟者和慢性阻塞性肺病患者的一种活跃机制。我们对 49 名受试者的肺部切除物进行了免疫组化染色,以检测 EndMT 的生物标记物:CD-31、N-粘连蛋白、Vimentin 和 S100A4。其中 15 人为非吸烟控制者(NC),6 人为肺功能正常的吸烟者(NLFS),9 人为小气道疾病患者(SAD),9 人为轻中度慢性阻塞性肺疾病-当前吸烟者(COPD-CS),10 人为慢性阻塞性肺疾病-戒烟者(COPD-ES)。使用 Image ProPlus 软件 v7.0 对肺动脉进行分析。与 NC 相比,我们发现所有吸烟组内膜层的交界处 CD31 阳性内皮细胞减少(p<0.05)。与NC相比,除了COPD-CS大动脉中的N-Cadherin外,所有吸烟组和所有尺寸的动脉中均观察到间质标记物N-cadherin(p<0.05)和Vimentin(p<0.001)的丰度增加。S100A4 的丰度与动脉厚度相关(r= 0.29、0.33、0.35;小动脉、中动脉和大动脉的 p=0.05、0.03、0.02)。小动脉壁中的波形蛋白与 FEV1/ FVC 和 FEF25-75% 呈负相关(r= -0.35, -0.34; p= 0.02,0.03),而中动脉和大动脉内膜层中细胞质 CD-31 丰度的增加与 DLCO 预测值呈负相关(r= -0.这是第一项显示 NLFS、SAD 和轻中度 COPD 患者的肺内皮细胞通过 EndMT 获得间充质特征的研究。这是首次研究表明,NLFS、SAD 和轻中度慢性阻塞性肺病患者的肺内皮细胞通过 EndMT 获得了间充质特质。
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引用次数: 0
Amyloid-β and caspase-1 are indicators of sepsis and organ injury 淀粉样蛋白-β和 Caspase-1 是败血症和器官损伤的指标
IF 4.6 3区 医学 Q1 RESPIRATORY SYSTEM Pub Date : 2023-12-21 DOI: 10.1183/23120541.00572-2023
Amanda N Tuckey, Arcole Brandon, Y. Eslaamizaad, Waqar Siddiqui, Talha Nawaz, Christopher Clarke, Erica Sutherland, Veronica Williams, Domenico Spadafora, Robert A. Barrington, Diego F. Alvarez, Madhuri S. Mulekar, Jon D. Simmons, Brian W. Fouty, J. Audia
Sepsis is a life-threatening condition that results from a dysregulated host response to infection, leading to organ dysfunction. Despite the prevalence and associated socioeconomic costs, treatment of sepsis remains limited to antibiotics and supportive care, and a majority of intensive care unit (ICU) survivors develop long-term cognitive complications post- discharge. The present study identifies a novel regulatory relationship between amyloid-β (Aβ) and the inflammasome-caspase-1 axis as key innate immune mediators that define sepsis outcomes.Medical ICU patients and healthy individuals were consented for blood and clinical data collection. Plasma cytokine, caspase-1, and Aβ levels were measured. Data were compared against indices of multi-organ injury and other clinical parameters. Additionally, recombinant proteins were testedin vitroto examine the effect of caspase-1 on a functional hallmark of Aβ, namely aggregation.Plasma caspase-1 levels displayed the best predictive value in discriminating ICU patients with sepsis from non-infected ICU patients (AUROC=0.7080). Plasma caspase-1 and the 40 amino acid Aβ isoform (Aβx−40) showed a significant positive correlation and Aβx-40associated with organ injury. Additionally, Aβ plasma levels continued to rise from time of ICU admission to 7-days post-admission.In silico, Aβ harbors a predicted caspase-1 cleavage site, andin vitrostudies demonstrated that caspase-1 cleaved Aβ to inhibit its auto-aggregation, suggesting a novel regulatory relationship.Aβx-40and caspase-1 are potentially useful early indicators of sepsis and its attendant organ injury. Additionally, Aβx-40has emerged as a potential culprit in the ensuing development of post-ICU-syndrome.
败血症是一种危及生命的疾病,是由于宿主对感染的反应失调而导致器官功能障碍。尽管脓毒症的发病率很高,而且相关的社会经济成本也很高,但对脓毒症的治疗仍然仅限于抗生素和支持性护理,而且大多数重症监护室(ICU)幸存者在出院后会出现长期的认知并发症。本研究发现了淀粉样蛋白-β(Aβ)与炎性体-caspase-1轴之间的新型调节关系,它们是决定败血症结局的关键先天性免疫介质。对血浆细胞因子、caspase-1和Aβ水平进行了测量。将数据与多器官损伤指数和其他临床参数进行比较。此外,还对重组蛋白进行了体外测试,以检验 caspase-1 对 Aβ 功能标志(即聚集)的影响。血浆 caspase-1 水平在鉴别 ICU 败血症患者与非感染 ICU 患者方面显示出最佳预测价值(AUROC=0.7080)。血浆 caspase-1 和 40 个氨基酸的 Aβ 异构体(Aβx-40)呈显著正相关,Aβx-40 与器官损伤有关。Aβx-40 和 caspase-1 是脓毒症及其伴随的器官损伤的潜在有用的早期指标。Aβx-40和caspase-1是脓毒症及其伴随的器官损伤的潜在有用的早期指标。此外,Aβx-40已成为导致ICU后综合症的潜在罪魁祸首。
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引用次数: 0
Phenotypes and treatment outcomes in idiopathic pulmonary arterial hypertension (IPAH) patients with comorbidities 有合并症的特发性肺动脉高压 (IPAH) 患者的表型和治疗效果
IF 4.6 3区 医学 Q1 RESPIRATORY SYSTEM Pub Date : 2023-12-21 DOI: 10.1183/23120541.00668-2023
Dirk Skowasch, Hans Klose, Ralf Ewert, Heinrike Wilkens, Manuel Richter, Stephan Rosenkranz, Gesine Setzer, Ekkehard Grünig, M. Halank
IPAH is often diagnosed in elderly patients with frequent comorbidities. Whereas a clear treatment strategy and risk assessment is recommended for patients with rare classical IPAH, monotherapy with phos­phodi­esterase type 5 inhibitors (PDE5i) or endothelin receptor antagonists (ERA) followed by regular follow-up and individualized therapy should be used for frequent patients with cardiopulmonary comorbidities. Here, we focus on these patients with IPAH and comorbidities, present a review of the literature with a focus on recently published work, and summarize factors that may help to provide guidance for individualized treatment approaches in such patients.
IPAH 通常在合并症较多的老年患者中确诊。对于罕见的典型 IPAH 患者,建议采取明确的治疗策略并进行风险评估;而对于经常合并心肺疾病的患者,则应使用 5 型磷酸二酯酶抑制剂(PDE5i)或内皮素受体拮抗剂(ERA)进行单药治疗,然后进行定期随访和个体化治疗。在此,我们将重点关注这些患有 IPAH 和合并症的患者,对文献进行综述,重点关注近期发表的研究成果,并总结有助于为此类患者的个体化治疗方法提供指导的因素。
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引用次数: 0
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