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Grey zones in the supportive treatments of cardiac amyloidosis 心脏淀粉样变性支持治疗的灰色地带
Pub Date : 2022-01-01 DOI: 10.20517/2574-1209.2021.134
A. Porcari, L. Pagura, G. Varrà, M. Rossi, F. Longo, R. Saro, D. Barbisan, M. Cittar, C. Rapezzi, M. Merlo
Recent advances in the diagnosis and treatment of cardiac amyloidosis (CA) have translated into a longer life expectancy of patients and more challenging clinical scenarios. Compared to the past, patients with CA and heart failure (HF) currently encountered in clinical practice are a more heterogeneous population and require tailored strategies. The perception of CA as a treatable disease has opened new possibilities for the management of these patients, but many grey areas remain to be explored. The aim of this review is to provide practical suggestions for daily clinical activity in the management of challenging scenarios in CA, including the effectiveness and tolerability of evidence-based HF medication; rate vs. rhythm control in atrial fibrillation, thromboembolic risk, and anticoagulation therapies; replacement of severe aortic valve stenosis; the impact of implantable cardioverter defibrillator on survival; and the usefulness of cardiac resynchronization therapy.
心脏淀粉样变性(CA)的诊断和治疗的最新进展已经转化为更长的患者预期寿命和更具挑战性的临床情况。与过去相比,目前在临床实践中遇到的CA和心力衰竭(HF)患者是一个更加异质性的人群,需要量身定制的策略。认为CA是一种可治疗的疾病,为这些患者的治疗开辟了新的可能性,但仍有许多灰色地带有待探索。本综述的目的是为CA中具有挑战性情况的日常临床活动管理提供实用建议,包括循证HF药物的有效性和耐受性;心房颤动的频率与节律控制、血栓栓塞风险和抗凝治疗重度主动脉瓣狭窄置换术;植入式心律转复除颤器对生存率的影响以及心脏再同步化治疗的有效性。
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引用次数: 1
Pregnancy with congenital heart disease 患有先天性心脏病的孕妇
Pub Date : 2022-01-01 DOI: 10.20517/2574-1209.2021.96
A. Saxena, J. Relan
Pregnancy is complicated by maternal cardiovascular disease in 1%-4% of cases. With advances in management of congenital heart diseases (CHDs), the survival to adulthood and childbearing age is increasing all over the world. The physiological adaptation during pregnancy adds to the hemodynamic burden of CHD, and, hence, many women are diagnosed with CHD for the first time during pregnancy, more so in developing countries. The type of underlying CHD and pre-pregnancy hemodynamics determine the risk of developing complications during pregnancy. Hence, pre-pregnancy risk stratification and counseling are a crucial part of management plan. Some of the serious CHDs are best treated in the preconception stage. The maximum chance of developing complications is between 28 and 32 weeks of gestation, during labor, and up to two weeks after delivery. Common complications in women with CHD during pregnancy and labor include heart failure, arrhythmias, bleeding/thrombosis, infective endocarditis, and rarely maternal death. Fetal complications include abortion, stillbirth, prematurity, low birth weight, and CHD. Comprehensive knowledge of these complications and their management is very important as an experienced multidisciplinary team is critical for improving outcome of these patients. Special care is required for pregnant women who have pulmonary hypertension, due to either Eisenmenger syndrome or other causes, severe valve stenosis, aortopathy associated with bicuspid aortic valve/coarctation, or severe cyanotic CHDs. Most women with CHD are at low risk, and successful pregnancy is feasible in the majority with optimal management.
妊娠合并产妇心血管疾病的病例占1%-4%。随着先天性心脏病(CHDs)治疗的进步,全世界先天性心脏病患者的成人期和育龄期存活率都在不断提高。怀孕期间的生理适应增加了冠心病的血流动力学负担,因此,许多妇女在怀孕期间首次被诊断为冠心病,在发展中国家更是如此。潜在冠心病的类型和孕前血流动力学决定了妊娠期间发生并发症的风险。因此,孕前风险分层和咨询是管理计划的重要组成部分。一些严重的冠心病最好在孕前阶段治疗。发生并发症的最大机会是在妊娠28至32周之间,分娩期间和分娩后两周。妊娠和分娩期间冠心病患者的常见并发症包括心力衰竭、心律失常、出血/血栓形成、感染性心内膜炎,以及罕见的产妇死亡。胎儿并发症包括流产、死产、早产、低出生体重和冠心病。全面了解这些并发症及其管理是非常重要的,因为一个经验丰富的多学科团队对改善这些患者的预后至关重要。对于因艾森曼格综合征或其他原因、严重瓣膜狭窄、二尖瓣主动脉瓣缩窄相关的主动脉病变或严重紫绀型冠心病而患有肺动脉高压的孕妇,需要特别护理。大多数患有冠心病的妇女是低风险的,通过最佳的管理,大多数妇女是可以成功怀孕的。
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引用次数: 0
A literature review: pre-/post-operative atrial fibrillation for thoracic aortic aneurysm procedures 文献综述:胸主动脉瘤手术前/术后房颤
Pub Date : 2022-01-01 DOI: 10.20517/2574-1209.2021.122
Annet S. Kuruvilla, So Agha, Ashutosh Yaligar, H. Tannous, A. Mclarty, A. L. Shroyer, Thomas V. Bilfinge
Atrial fibrillation (AF) is among the most frequent cardiac surgical arrhythmias documented. The global AF prevalence is estimated at over 33 million cases, with estimates ranging up to 6.1 million cases in the United States. Among cardiac surgical patients, the risk factors for new-onset post-operative AF (POAF) include Caucasian race with increased prevalence documented in older men. Due to trends of earlier thoracic aortic aneurysm (TAA) detection and treatment, it is timely to review the AF association with adverse TAA clinical outcomes. Towards this goal, a comprehensive PubMed literature review was performed. For this initial Medline literature search, the MeSH search strategy included “thoracic aortic aneurysm” and “atrial fibrillation”. Based on the pertinent articles identified, the limited literature available for preoperative TAA AF and the predictors of POAF following TAA procedures were reviewed. Given only a handful of publications addressing these pre-/post-operative AF topics were identified using this very broad initial search approach, a knowledge chasm exists–as very little is known about TAA patients with pre-operative or new-onset post-operative AF. Given the paucity of evidence-based information available, clinically relevant TAA-specific research questions have been raised to guide future TAA AF-related investigations.
心房颤动(AF)是最常见的心脏外科心律失常之一。全球房颤患病率估计超过3300万例,美国估计高达610万例。在心脏手术患者中,新发术后房颤(POAF)的危险因素包括高加索人种,在老年男性中患病率增加。鉴于胸主动脉瘤(TAA)早期发现和治疗的趋势,回顾AF与TAA不良临床结果的关系是及时的。为了实现这一目标,我们进行了全面的PubMed文献综述。对于最初的Medline文献检索,MeSH检索策略包括“胸主动脉瘤”和“心房颤动”。基于相关文章,我们回顾了术前TAA AF和TAA手术后POAF预测因素的有限文献。使用这种非常广泛的初始搜索方法,只有少数出版物涉及这些术前/术后房颤主题,因此存在知识缺口,因为对术前或术后新发房颤的TAA患者知之甚少。鉴于缺乏循证信息,提出了临床相关的TAA特异性研究问题,以指导未来TAA房颤相关研究。
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引用次数: 0
Preoperative atrial fibrillation/flutter impact on risk-adjusted repeat aortic intervention patients 术前房颤/扑动对经风险调整的重复主动脉介入治疗患者的影响
Pub Date : 2022-01-01 DOI: 10.20517/2574-1209.2021.139
S. Novotny, Julia Dokko, Xiaoyue Zhang, So Agha, Ashutosh Yaligar, Natalie K. Kolba, Vineet Tummala, P. Parikh, A. Pryor, H. Tannous, A. L. Shroyer, Thomas Bilfinger
Aim: Impacts of pre-operative atrial fibrillation or flutter (AF/AFL) upon repeat aortic valve replacement (r-AVR) patients’ risk-adjusted short-term outcomes is unknown. Methods: From 2005-2018, New York State AF/AFL versus non-AF/AFL adults’ risk-adjusted r-AVR outcomes were compared. Primary endpoints included the Society of Thoracic Surgeons’ 30-day operative mortality or major morbidity (MM) composite and 30-day readmission (READMIT); the MM sub-components were secondary endpoints. Multivariable logistic regression models evaluated AF/AFL impact upon these endpoints while holding other factors constant. Results: Of 36,783 adults initially undergoing aortic valve replacement, 334 subsequently underwent r-AVR. Within this r-AVR group, 42.4% of repeat surgical (r-SAVR) patients had AF/AFL; 50.4% of repeat transcatheter (viv-TAVR) patients had AF/AFL. R-SAVR AF/AFL patients were older and had more comorbidities than those without AF/AFL. Viv-TAVR AF/AFL patients were similar to those without AF/AFL except for lower rates of chronic obstructive pulmonary disease. Comparing risk-adjusted r-AVR outcomes, AF/AFL did not impact MM [odds ratio (OR), 95% confidence interval (CI): 1.23, 0.66-2.28, P = 0.512] or READMIT (OR, 95% CI: 1.15, 0.60-2.19, P = 0.681). Black race (OR, 95% CI: 2.89, 1.01-8.32, P = 0.049) and Elixhauser mortality score (OR, 95% CI: 1.07, 1.04-1.10, P < 0.0001) predicted MM risk. Cerebrovascular disease (OR, 95% CI: 2.54, 1.23-5.25, P = 0.012) predicted READMIT risk, while viv-TAVR was protective compared to r-SAVR (OR, 95% CI: 0.44, 0.21-0.91, P = 0.027). Conclusion: AF/AFL was not associated with risk-adjusted short-term r-AVR outcomes. Black race, Elixhauser mortality score, and cerebrovascular disease predicted adverse outcomes.
目的:术前心房颤动或扑动(AF/AFL)对重复主动脉瓣置换术(r-AVR)患者经风险调整的短期预后的影响尚不清楚。方法:比较2005-2018年纽约州AF/AFL与非AF/AFL成人经风险调整的r-AVR结局。主要终点包括胸外科学会30天手术死亡率或主要发病率(MM)和30天再入院率(READMIT);MM子成分为次要终点。多变量逻辑回归模型在保持其他因素不变的情况下评估AF/AFL对这些终点的影响。结果:36783名成年人最初接受主动脉瓣置换术,334人随后接受了r-AVR。在r-AVR组中,42.4%的重复手术(r-SAVR)患者患有AF/AFL;50.4%的重复经导管(viv-TAVR)患者有AF/AFL。R-SAVR AF/AFL患者比无AF/AFL患者年龄更大,合并症更多。Viv-TAVR AF/AFL患者与无AF/AFL患者相似,但慢性阻塞性肺疾病的发生率较低。比较风险调整后的r-AVR结果,AF/AFL不影响MM[比值比(OR), 95%可信区间(CI): 1.23, 0.66-2.28, P = 0.512]或READMIT (OR, 95% CI: 1.15, 0.60-2.19, P = 0.681)。黑人(OR, 95% CI: 2.89, 1.01-8.32, P = 0.049)和Elixhauser死亡率评分(OR, 95% CI: 1.07, 1.04-1.10, P < 0.0001)预测MM风险。脑血管疾病(OR, 95% CI: 2.54, 1.23-5.25, P = 0.012)预测READMIT风险,而与r-SAVR相比,viv-TAVR具有保护作用(OR, 95% CI: 0.44, 0.21-0.91, P = 0.027)。结论:AF/AFL与经风险调整的短期r-AVR结果无关。黑人种族、Elixhauser死亡率评分和脑血管疾病预测不良结局。
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引用次数: 1
Unique contribution of one patient advocacy organization in advancing cerebral cavernous malformation awareness and research 一个患者倡导组织在推进脑海绵状血管瘤的认识和研究方面的独特贡献
Pub Date : 2022-01-01 DOI: 10.20517/2574-1209.2021.31
Cornelia Lee
Advocacy organizations have long played a role in advancing care and research for patients affected by rare disease. Angioma Alliance has served traditional functions of organizing scientific meetings and creating shared resources like a tissue bank and a patient registry. Uniquely, the organization has employed creative patient engagement methods like subsidized genetic testing as well as targeting special populations to expand research participation and understanding of the illness. Special populations include those with CCM3 mutations, the CCM1 Common Hispanic Mutation, and Black patients.
倡导组织长期以来一直在推进罕见疾病患者的护理和研究方面发挥作用。血管瘤联盟的传统职能是组织科学会议和创建组织库和患者登记等共享资源。独特的是,该组织采用了创造性的患者参与方法,如补贴基因检测,以及针对特殊人群扩大研究参与和对疾病的了解。特殊人群包括CCM3突变、CCM1常见西班牙裔突变和黑人患者。
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引用次数: 0
Survival and clinical outcomes of diabetic peripheral artery disease patients following a pain-free home-based walking program 糖尿病外周动脉疾病患者在无痛家庭步行计划后的生存和临床结果
Pub Date : 2022-01-01 DOI: 10.20517/2574-1209.2021.79
N. Lamberti, E. Tsolaki, F. Guerzoni, N. Napoli, L. Traina, Giovanni Piva, V. Gasbarro, P. Zamboni, S. Straudi, R. Manfredini, F. Manfredini
Aim: We retrospectively examined the impact on the rate of survival of pain-free home-based exercise in diabetic peripheral artery disease patients compared to patients receiving usual care. Methods: In total, 202 patients at Fontaine’s Stage II with diabetes were studied. Half were enrolled in a structured home-based exercise program (E), whereas the other half received walking advice as the active control group (C). Long-term clinical outcomes at five years were gathered from the Emilia-Romagna Health Service Registry, with survival probability selected as the primary outcome. Results: At baseline, the two groups did not differ for any demographic or clinical characteristics. High adherence to the program was recorded in Group E (88% of home-walking sessions executed, with an average distance walked during the program of 174 km). After five years, a survival rate of 90% for Group E and 60% for Group C was observed, with a significantly (P < 0.001) higher mortality risk for Group C [Hazard ratio (HR) = 3.92]. Additionally, among secondary outcomes, Group E showed a significantly (P = 0.048) lower rate of peripheral revascularizations than Group C (15% vs. 24%, respectively; HR = 1.91), all-cause hospitalizations (P = 0.007; 61% vs. 80%, HR = 1.58), and amputations (P = 0.049; 6% vs. 13%, HR = 2.47). In a Cox multivariate-proportional regression model of the entire population, the predictors of survival probability were age (HR = 1.05), Charlson index (HR = 1.24), lower ankle-brachial index (HR = 6.66), and control group (HR = 4.99). Conclusion: A simple sustainable program aimed at improving mobility of diabetic patients with claudication at high cardiovascular risk was associated with better survival and long-term clinical outcomes.
目的:我们回顾性研究了与接受常规护理的糖尿病外周动脉疾病患者相比,无痛家庭运动对生存率的影响。方法:对202例方丹氏II期糖尿病患者进行研究。其中一半参加了有组织的家庭锻炼计划(E),而另一半接受步行建议作为积极对照组(C)。5年的长期临床结果从艾米利亚-罗马涅健康服务登记处收集,生存率作为主要结果。结果:在基线时,两组在任何人口统计学或临床特征上没有差异。E组对该计划的坚持程度很高(88%的家庭步行会议执行,在计划期间平均步行距离为174公里)。5年后,E组和C组的生存率分别为90%和60%,其中C组的死亡率显著高于对照组(P < 0.001)[危险比(HR) = 3.92]。此外,在次要结局中,E组外周血运重建率显著(P = 0.048)低于C组(分别为15%对24%;HR = 1.91),全因住院率(P = 0.007;61%对80%,HR = 1.58),截肢(P = 0.049;6%对13%,HR = 2.47)。在全人群Cox多变量比例回归模型中,预测生存率的因素为年龄(HR = 1.05)、Charlson指数(HR = 1.24)、下踝肱指数(HR = 6.66)和对照组(HR = 4.99)。结论:一个简单的可持续的项目,旨在改善糖尿病合并跛行患者的高心血管风险的活动能力,与更好的生存和长期临床结果。
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引用次数: 2
Pharmacologic therapies for the low cardiac output syndrome in children after cardiac surgery: evidence of their efficacy and trends in their use 儿童心脏手术后低心输出量综合征的药物治疗:疗效和使用趋势的证据
Pub Date : 2022-01-01 DOI: 10.20517/2574-1209.2021.94
Raj Sahulee, Jaclyn McKinstry
The low cardiac output syndrome describes the phenomenon of the reduction of cardiac output that can occur following cardiac surgery requiring cardiopulmonary bypass. If unrecognized or untreated, this condition can result in significant morbidity and mortality. Along with non-pharmacologic therapies, pharmacologic agents used to help manage the low cardiac output syndrome include catecholamine inotropes, inodilators, systemic vasodilators, pulmonary vasodilators, and other classes of medications. We summarize the rationale and key evidence supporting the use of these therapies in children. In addition, utilizing provider surveys and registry reviews, we describe the current trends in the use of these medications and the variation demonstrated between providers and centers. Given the heterogeneous etiology of low cardiac output syndrome, successful management requires that pharmacologic therapies be tailored to the physiologic derangements of each patient.
低心输出量综合征描述了在需要体外循环的心脏手术后可能发生的心输出量减少的现象。如果不加以认识或治疗,这种情况可导致严重的发病率和死亡率。与非药物治疗一起,用于帮助控制低心排血量综合征的药物包括儿茶酚胺性肌力药物、消张剂、全身血管扩张剂、肺血管扩张剂和其他类型的药物。我们总结了支持在儿童中使用这些疗法的基本原理和关键证据。此外,利用提供者调查和注册审查,我们描述了这些药物使用的当前趋势以及提供者和中心之间的差异。考虑到低心排血量综合征的异质性病因,成功的治疗需要针对每个患者的生理紊乱量身定制药物治疗。
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引用次数: 2
Features of mitochondrial dynamics in monocytes in inflammatory and metabolic disorders 炎症和代谢紊乱中单核细胞线粒体动力学特征
Pub Date : 2022-01-01 DOI: 10.20517/2574-1209.2022.22
T. Tolstik, A. Bogatyreva, A. Grechko, Y. Oishi, A. Markin
Mitochondria do not exist as separate formations in the cell; they form a homogeneous network in which the processes of division and fusion continuously occur. A shift in this balance, as well as mitochondrial dysfunction, leads to the development of chronic and metabolic disorders. Metabolic changes in mitochondria control the formation and differentiation of monocytes. Pro-inflammatory activation of monocytes/macrophages leads to a decrease in oxidative phosphorylation and an increase in mitochondrial fusion. To date, the molecular mechanisms that regulate mitochondrial dynamics to control life and death in monocytes are not well understood. In addition, there is ample evidence that abnormal mitochondrial metabolism is involved in the pathogenesis of many diseases. Mitochondrial stress and damage contribute to cell death, metabolic disorders, and inflammation. In this review, we consider in detail the involvement of mitochondrial processes in the development of pathologies and discuss how mitochondria can be therapeutically affected. Attention is also drawn to possible diagnostic studies that target mitochondrial dynamics of disorders in monocytes.
线粒体在细胞中不是单独存在的;它们形成了一个同质的网络,在这个网络中,分裂和融合的过程不断发生。这种平衡的改变,以及线粒体功能障碍,导致慢性和代谢疾病的发展。线粒体的代谢变化控制单核细胞的形成和分化。单核/巨噬细胞的促炎激活导致氧化磷酸化的减少和线粒体融合的增加。迄今为止,调节线粒体动力学以控制单核细胞生死的分子机制尚不清楚。此外,有充分证据表明线粒体代谢异常参与了许多疾病的发病机制。线粒体应激和损伤导致细胞死亡、代谢紊乱和炎症。在这篇综述中,我们详细考虑了线粒体过程在病理发展中的参与,并讨论了线粒体如何在治疗上受到影响。注意也提请到可能的诊断研究,目标线粒体动力学的疾病在单核细胞。
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引用次数: 0
Diagnosis of cardiac murmurs in children 儿童心脏杂音的诊断
Pub Date : 2022-01-01 DOI: 10.20517/2574-1209.2021.105
P. Rao
Heart murmurs are frequently heard, and the murmurs are the usual cause for uncovering heart defects in pediatric patients. The murmurs are grouped into systolic murmurs, diastolic murmurs, and continuous murmurs. Cautious assessment of the murmur and associated abnormalities in physical examination are likely to produce correct diagnosis of the cause of the murmur. Sometimes it may be necessary to utilize noninvasive and invasive (rarely) investigations to achieve an accurate diagnosis. Nonetheless, such diagnostic studies may frequently be required for quantification, and before intervention either by transcatheter methods or by surgery.
心脏杂音是经常听到的,并且杂音通常是发现儿科患者心脏缺陷的原因。杂音分为收缩期杂音、舒张期杂音和持续性杂音。仔细评估杂音和体格检查中相关的异常可能会对杂音的病因做出正确的诊断。有时可能需要使用非侵入性和侵入性(很少)检查来获得准确的诊断。尽管如此,在经导管方法或手术干预之前,这种诊断研究可能经常需要量化。
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引用次数: 1
Cutaneous manifestations of anti-neutrophil cytoplasmic antibody associated vasculitis 抗中性粒细胞细胞质抗体相关性血管炎的皮肤表现
Pub Date : 2022-01-01 DOI: 10.20517/2574-1209.2021.40
M. Abdel-Halim, A. Mahmoud, Gaafar Ragab
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a distinct group of systemic vasculitis with severe multi-organ involvement. It includes three types: granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), and microscopic polyangiitis (MPA). Cutaneous manifestations are present at the time of presentation in around 35% of cases and are more frequently encountered in EGPA than in GPA or MPA. They can also occur during the course of the disease or mark a disease relapse. They can be classified into specific or non-specific according to the presence or absence of vasculitis, whether or not associated with granuloma, in skin biopsies. The most common presentation of vasculitic skin lesions in AAV is palpable purpura. Other manifestations include hemorrhagic blisters, tender subcutaneous nodules, livedo reticularis/racemosa, painful ulcers that may mimic pyoderma gangrenosum (PG), and digital gangrenes. A wide range of non-specific cutaneous manifestations can occur in association with AAV including urticarial rash, prurigo, sterile pustules, and oral lesions. The presence of cutaneous lesions is usually associated with severe organ involvement and systemic manifestations. Since skin lesions in AAV can occur in other types of vasculitis and in other disorders, attributing them to AAV requires meticulous clinical, laboratory, and serological correlation. Awareness of cutaneous lesions of AAV is important for all physicians working in the field of systemic vasculitis as a skin biopsy may provide an easy clue to diagnose AAV in such cases.
抗中性粒细胞细胞质抗体(ANCA)相关性血管炎(AAV)是一种严重累及多器官的系统性血管炎。它包括三种类型:肉芽肿病合并多血管炎(GPA)、嗜酸性肉芽肿病合并多血管炎(EGPA)和显微多血管炎(MPA)。约35%的病例在发病时出现皮肤表现,EGPA比GPA或MPA更常见。它们也可能发生在疾病过程中或标志着疾病复发。根据皮肤活检中是否存在血管炎,无论是否与肉芽肿相关,它们可分为特异性或非特异性。血管血管性皮肤病变在AAV中最常见的表现是可触及的紫癜。其他表现包括出血性水疱、柔软的皮下结节、网状/总状活动性溃疡、类似坏疽性脓皮病(PG)的疼痛性溃疡和指坏疽。与AAV相关的广泛的非特异性皮肤表现包括荨麻疹、痒疹、无菌脓疱和口腔病变。皮肤病变的出现通常伴有严重的器官受累和全身表现。由于AAV引起的皮肤损害可发生在其他类型的血管炎和其他疾病中,因此将其归因于AAV需要细致的临床、实验室和血清学相关性。对于所有从事全身性血管炎领域工作的医生来说,意识到AAV的皮肤病变是很重要的,因为皮肤活检可能为这种情况下的AAV诊断提供了一个简单的线索。
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引用次数: 4
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