glomerular diseases

肾小球疾病
  • 文章类型: Journal Article
    这项回顾性分析调查了2010年至2021年在ARNASG.Brotzu进行的682例肾脏活检的结果和并发症。我们的研究结果表明,轻微并发症发生率为9.1%,严重并发症极为罕见,为0.3%。年龄不会增加风险,强调该程序在各年龄组的安全性。从2019年4月至2023年12月,临床催眠被纳入部分患者(n=45)的活检方案。超过90%的患者报告对手术没有感知,60%没有疼痛。根据STAY考试成绩,这种方法显著降低了术后焦虑(p=0.001);该组未观察到主要或次要并发症.虽然我们的研究重申了肾脏活检中严重并发症的风险非常低,它还强调了辅助临床催眠在增强患者舒适和合作过程中的潜在益处。这项探索为进一步研究开辟了一条有希望的途径,以改善肾脏活检的患者体验和手术结果。
    This retrospective analysis investigates the outcomes and complications of 682 kidney biopsies performed at ARNAS G. Brotzu from 2010 to 2021. Our findings indicate a minor complication rate of 9.1%, with severe complications being exceedingly rare at 0.3%. Age did not contribute to an increased risk, underscoring the procedure\'s safety across age groups. Clinical hypnosis was incorporated into the biopsy protocol in a subset of patients (n = 45) from April 2019 to December 2023. Over 90% of these patients reported no perception of the procedure, and 60% experienced no pain. According to STAY-Y test scores, this approach significantly reduced anxiety post-procedure (p = 0.001); no major or minor complications were observed in this group. While our study reaffirms the very low risk of severe complications in kidney biopsies, it also highlights the potential benefits of adjunct clinical hypnosis in enhancing patient comfort and cooperation during the procedure. This exploration opens a promising avenue for further investigation to improve patient experiences and procedural outcomes in kidney biopsies.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

  • 文章类型: Journal Article
    引言:药物不良反应是现代社会的重大问题,由于处方药的增加,非处方药,和整体的多重用药。肾小球疾病是经常报道的与药物使用相关的肾脏疾病之一。VigiBase是评估与药物使用相关的事件的重要工具,and,根据作者的知识,尚未有研究评估该数据库以确定与肾小球疾病相关的主要药物.材料和方法:我们从VigiBase收集了54年的数据,并根据全球频率对数据进行了评估,不相称性(IC025值),肾毒性潜力,和病理生理学机制。结果:在评估期间,33.932.051对VigiBase中报告的药物不良反应的自发通知进行了评估,从中提取了106.775项药物相关肾小球疾病的通知。分离的药物被归类为“潜在的肾毒素”(47.0%),40%的药物缺乏科学参考来报告与肾小球疾病发展的任何关联。在评估的药物中,Inotersen(IC025of8.3),青霉胺(IC0256.8),贝伐单抗(IC0255.9)和伦伐替尼(IC0255.4)被鉴定为与这些肾小球疾病的相关性最强。对于分类为“非肾毒性”的药物,观察到高的不成比例指数,提示可能被认为是新的潜在肾毒素的药物。结论:药物诱导的肾小球疾病与没有确定的肾毒性作用但在VigiBase中显示出高比例指数的药物显着相关。这些新所谓的肾毒性药物需要在专门的研究中进一步评估,以评估其真正的肾毒性潜力。
    Introduction: Adverse drug reactions are a significant problem in modern society, stemming from the increase in prescribed medications, over-the-counter drugs, and overall polypharmacy. Glomerular disorders are one of the frequently reported renal conditions associated with medication use. VigiBase is a significant tool for evaluating events associated with drug use, and, to the authors\' knowledge, no study has yet assessed this database to identify the primary medications associated with glomerular disorders. Materials and Methods: We collected data from VigiBase for 54 years and evaluated data based on global frequencies, disproportionality (IC025 values), nephrotoxic potential, and physiopathological mechanisms. Results: Over the evaluation period, 33.932.051 spontaneous notifications of adverse drug reactions reported in VigiBase were assessed, from which 106.775 notifications of drug-associated glomerular disorders were extracted. The isolated medications were classified as \'potential nephrotoxins\' (47.0%), with 40% of the medications lacking scientific references to report any association with the development of glomerular disorders. Among the evaluated medications, Inotersen (IC025 of 8.3), Penicillamine (IC025 6.8), Bevacizumab (IC025 5.9) and Lenvatinib (IC025 5.4) were identified as having the strongest association with these glomerular disorders. For medications classified as \'non-nephrotoxic\', a high disproportionality index was observed, suggesting drugs that might be considered as new potential nephrotoxins. Conclusions: Drug-induced glomerular disorders were significantly associated with medications that had no established nephrotoxic role but demonstrated a high disproportionality index in VigiBase. These newly alleged nephrotoxic drugs warrant further evaluation in dedicated studies to assess their true nephrotoxic potential.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Journal Article
    A vérvizelés hátterében a betegségek széles spektruma állhat gyermekkorban, az egyszerű és gyorsan gyógyuló húgyúti fertőzésektől a gyorsan progrediáló súlyos glomerulonephritisig, mely a vesefunkció gyors romlásához vezet. A vérvizelés kivizsgálása során ezért elsődleges célunk az orvosi szempontból jelentős esetek elkülönítése. A vérvizeléshez társuló fehérjevizelés, a magas vérnyomás vagy a beszűkült vesefunkció progresszív vesebetegségre hívja fel a figyelmet. Amennyiben a vérvizelés makroszkópos, vagy az egyéb klinikai tünetek jellegzetesek, a differenciáldiagnózis gyakran könnyű és egyértelmű. Komplikáltabb esetekben azonban további noninvazív vizsgálómódszerek lehetnek szükségesek, mint az ultrahangvizsgálat, a vörösvértestek morfológiai vizsgálata, kalciumürítés, egyéb vizeletkémiai paraméterek, illetve a vesefunkció és a fehérjevizelés mértékének meghatározása. A családi anamnézis részletes felvétele a vérvizelés örökletes okainak elkülönítésében segít. Tünetmentes vérvizelés esetén központokként változhat a vesebiopszia elvégzésének indikációja. A glomerularis betegségek felismerésének arányát jelentősen befolyásolja, hogy mely kritériumok teljesülése esetén végzünk vesebiopsziát. Összefoglaló közleményünkben a vérvizelés diagnosztikájának kihívásait mutatjuk be. Részletezzük a vérvizelés definícióját, a glomerularis és postglomerularis vérvizelés elkülönítésének lépéseit, áttekintjük a hátterükben álló leggyakoribb betegségeket, melyek vagy gyakoriságuk, vagy gyors lefolyásuk és rossz prognózisuk miatt kihívást jelentenek mindennapi gyermeknefrológiai orvosi gyakorlatunk során. Orv Hetil. 2024; 165(28): 1067–1078.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

  • 文章类型: Journal Article
    肾小球疾病(GD),终末期肾病的重要原因,通过基于肾脏活检(KB)的流行病学研究更好地理解,提供了对其患病率和特征的重要见解。本研究旨在分析2008年至2017年在罗马尼亚最大的参考中心诊断的GD的临床病理特征。在这项长达十年的研究中,纳入1254名诊断为GD的成年患者。使用四个组织病理学病变计算每个KB的局部先前验证的肾组织病理学预后评分:整体肾小球硬化,肾小管萎缩,间质纤维化和纤维细胞/纤维新月。病人的平均年龄是50岁,男性占主导地位(57%)。主要转诊原因为肾病综合征(46%),肾病综合征(37%),慢性肾脏病(12%),无症状的泌尿异常(4%),和急性肾损伤(1%)。免疫球蛋白A肾病(IgAN)是最常见的诊断GD(20%),与欧洲登记册报告的频率保持一致。糖尿病性肾小球肾病是最常见的继发性GD(10%)。它还呈现最高的中位肾组织病理学预后评分(2),表明预后较差。较低的eGFR和较高的蛋白尿与较高的评分独立相关。这项长达十年的研究强调IgAN是KB诊断最常见的GD。糖尿病性肾小球肾病被确定为最常见的继发性GD。肾组织病理学预后评分,在糖尿病肾小球肾病患者中尤其高,与较低的eGFR和较高的蛋白尿相关,强调其临床相关性。
    Glomerular diseases (GDs), significant causes of end-stage kidney disease, are better understood through epidemiological studies based on kidney biopsies (KBs), which provide important insights into their prevalence and characteristics. This study aims to analyze the clinicopathological features of GDs diagnosed from 2008 to 2017 at Romania\'s largest reference center. In this decade-long study, 1254 adult patients diagnosed with GDs were included. The local previously validated renal histopathological prognostic score was calculated for each KB using four histopathologic lesions: global glomerulosclerosis, tubular atrophy, interstitial fibrosis and fibrocellular/fibrous crescents. The mean patient age was 50 years, with a male predominance (57%). The primary referral reasons were nephrotic syndrome (46%), nephritic syndrome (37%), chronic kidney disease (12%), asymptomatic urinary abnormalities (4%), and acute kidney injury (1%). Immunoglobulin A nephropathy (IgAN) was the most frequently diagnosed GD (20%), aligning with frequencies reported in European registries. Diabetic glomerular nephropathy was the most common secondary GD (10%). It also presented the highest median renal histopathological prognostic score (2), indicating a poorer prognosis. Lower eGFR and higher proteinuria were independently associated with higher scores. This decade-long study highlights IgAN as the most frequent GD diagnosed by KB. Diabetic glomerular nephropathy was identified as the most common secondary GD. The renal histopathological prognostic score, notably high in diabetic glomerular nephropathy patients, was correlated with lower eGFR and higher proteinuria, underlining its clinical relevance.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Journal Article
    乙型肝炎病毒(HBV)和丙型肝炎病毒(HCV)感染了大量的全球个体和他们的肝外表现,包括肾小球疾病,建立得很好。此外,肝病相关的IgA肾病是继发性IgA肾病的主要原因,其病程从无症状性泌尿系统异常到进行性肾损伤不等.在这里,我们提供了一份关于流行病学的最新综述,发病机制,临床表现,和治疗HBV和HCV相关的肾小球肾炎以及肝病患者的IgA肾病。最常见的HBV相关性肾小球肾炎是膜性肾病,尽管已经描述了膜增生性肾小球肾炎和足细胞病变。描述最好的HCV相关性肾小球肾炎是大约30%的混合性冷球蛋白性血管炎患者发生的冷球蛋白性肾小球肾炎。HBV-GN和HCV-GN的主要治疗方法是抗病毒治疗,自直接作用抗病毒药物出现以来,结果显着改善。然而,具有严重病理和/或更具侵袭性的疾病轨迹的病例可以提供一个疗程的免疫抑制,常用抗CD20治疗,特别是在冷球蛋白血症性肾小球肾炎的情况下。
    Hepatitis B virus (HBV) and hepatitis C virus (HCV) infect a significant number of individuals globally and their extra-hepatic manifestations, including glomerular disease, are well established. Additionally, liver disease-associated IgA nephropathy is the leading cause of secondary IgA nephropathy with disease course varying from asymptomatic urinary abnormalities to progressive kidney injury. Herein we provide an updated review on the epidemiology, pathogenesis, clinical manifestations, and treatment of HBV- and HCV-related glomerulonephritis as well as IgA nephropathy in patients with liver disease. The most common HBV-related glomerulonephritis is membranous nephropathy, although membranoproliferative glomerulonephritis and podocytopathies have been described. The best described HCV-related glomerulonephritis is cryoglobulinemic glomerulonephritis occurring in about 30% of patients with mixed cryoglobulinemic vasculitis. The mainstay of treatment for HBV-GN and HCV-GN is antiviral therapy, with significant improvement in outcomes since the emergence of the direct-acting antivirals. However, cases with severe pathology and/or a more aggressive disease trajectory can be offered a course of immunosuppression, commonly anti-CD20 therapy, particularly in the case of cryoglobulinemic glomerulonephritis.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

  • 文章类型: Journal Article
    第一个空间分辨转录组学平台,GeoMx(Nanostring)和Visium(10xGenomics)于2019年推出,并在2020年被《自然方法》认可为年度方法。随后对这些技术和其他技术进行改进和扩展,以增加复杂性,用福尔马林固定石蜡包埋的组织工作,而分析蛋白质除了基因表达外,仅增加了它们对生物医学的意义和影响。从这个角度来看,我们专注于空间转录组学的两个平台,GeoMx和Vvium,以及这些平台如何被用来提供对肾脏疾病的新见解。平台的选择将在很大程度上取决于实验问题和设计。将这些技术应用于临床来源的活检提供了识别特定组织生物标志物的机会,这些生物标志物有助于确定疾病病因,并在未来更精确地针对治疗性干预措施。
    在这篇评论中,我们提供了对现有和新兴技术的描述,这些技术可用于从组织中捕获空间分辨的基因和蛋白质表达数据。这些技术为疾病的空间异质性提供了新的见解,对疾病的反应是如何在组织中分布的,哪些细胞受到影响,和预测疾病和治疗反应的分子途径。
    未来几年将看到大量使用空间转录组学技术来更好地定义肾脏疾病的病理生理学,并开发新的诊断测试来指导患者的个性化治疗。
    UNASSIGNED: The first spatially resolved transcriptomics platforms, GeoMx (Nanostring) and Visium (10x Genomics) were launched in 2019 and were recognized as the method of the year by Nature Methods in 2020. The subsequent refinement and expansion of these and other technologies to increase -plex, work with formalin-fixed paraffin-embedded tissue, and analyze protein in addition to gene expression have only added to their significance and impact on the biomedical sciences. In this perspective, we focus on two platforms for spatial transcriptomics, GeoMx and Visium, and how these platforms have been used to provide novel insight into kidney disease. The choice of platform will depend largely on experimental questions and design. The application of these technologies to clinically sourced biopsies presents the opportunity to identify specific tissue biomarkers that help define disease etiology and more precisely target therapeutic interventions in the future.
    UNASSIGNED: In this review, we provide a description of the existing and emerging technologies that can be used to capture spatially resolved gene and protein expression data from tissue. These technologies have provided new insight into the spatial heterogeneity of diseases, how reactions to disease are distributed within a tissue, which cells are affected, and molecular pathways that predict disease and response to therapy.
    UNASSIGNED: The upcoming years will see intense use of spatial transcriptomics technologies to better define the pathophysiology of kidney diseases and develop novel diagnostic tests to guide personalized treatments for patients.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Journal Article
    肾小球肾病的有效管理,慢性肾脏病(CKD)的主要类别之一,需要准确的诊断,进展的预后,评估治疗效果,and,理想情况下,药物反应的预测。文献中已经报道了用于评估肾小球疾病特定方面的多种生物标志物和算法。不过,其中绝大多数尚未在临床实践中实施,或者由于访问权限有限而无法在全球范围内使用,缺少医疗基础设施,或经济和政治原因。这项审查的目的是汇编目前所有可用的诊断信息,预后,和目前可用于肾小球疾病管理的预测性生物标志物,并为这些生物标志物的应用提供指导。由于收集了可用的不同生物标志物的证据,我们提出了一个非侵入性的决策树,生物标志物指导的诊断路径。目前可用的数据表明,对于大多数肾小球疾病患者,有效的生物标志物是可用的。然而,尽管肾活检有明显的缺点,具有侵入性,不适用于监测,特别是在罕见CKD病因的背景下,肾活检仍不能被非侵入性策略所取代.
    Effective management of glomerular kidney disease, one of the main categories of chronic kidney disease (CKD), requires accurate diagnosis, prognosis of progression, assessment of therapeutic efficacy, and, ideally, prediction of drug response. Multiple biomarkers and algorithms for the assessment of specific aspects of glomerular diseases have been reported in the literature. Though, the vast majority of these have not been implemented in clinical practice or are not available on a global scale due to limited access, missing medical infrastructure, or economical as well as political reasons. The aim of this review is to compile all currently available information on the diagnostic, prognostic, and predictive biomarkers currently available for the management of glomerular diseases, and provide guidance on the application of these biomarkers. As a result of the compiled evidence for the different biomarkers available, we present a decision tree for a non-invasive, biomarker-guided diagnostic path. The data currently available demonstrate that for the large majority of patients with glomerular diseases, valid biomarkers are available. However, despite the obvious disadvantages of kidney biopsy, being invasive and not applicable for monitoring, especially in the context of rare CKD etiologies, kidney biopsy still cannot be replaced by non-invasive strategies.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Journal Article
    肾脏病学领域最近已将相当多的注意力集中在干扰素基因刺激因子(STING)分子上,因为它似乎是慢性肾脏疾病(CKD)的有效驱动因素。STING及其活化剂,环GMP-AMP合酶(cGAS),连同细胞内RIG样受体(RLRs)和toll样受体(TLRs),是I型干扰素(IFN-I)表达的有效诱导物。这些细胞因子长期以来一直被认为是先天免疫系统用来对抗病毒感染的机制的一部分;然而,他们是否参与无菌性炎症尚不清楚.越来越多的证据表明IFN-I途径参与无菌性肾脏炎症,提供了对先天免疫系统和肾单位最敏感段损伤之间复杂相互作用的潜在见解。肾小球。STING途径通常被认为是不归因于病毒感染的肾脏疾病的一个原因。相反,该途径可以识别宿主来源的核酸并发出信号,也被RLRs和TLRs认可。目前还不清楚,然而,肾脏疾病的发展是否取决于随后的IFN-I诱导或涉及的其他过程。本文旨在探讨IFN-I在肾小球细胞中的主要内源性诱导剂,讨论自分泌和旁分泌信号对IFN-I诱导有什么影响,并确定与肾小球损伤发展有关的途径。
    The field of nephrology has recently directed a considerable amount of attention towards the stimulator of interferon genes (STING) molecule since it appears to be a potent driver of chronic kidney disease (CKD). STING and its activator, the cyclic GMP-AMP synthase (cGAS), along with intracellular RIG-like receptors (RLRs) and toll-like receptors (TLRs), are potent inducers of type I interferon (IFN-I) expression. These cytokines have been long recognized as part of the mechanism used by the innate immune system to battle viral infections; however, their involvement in sterile inflammation remains unclear. Mounting evidence pointing to the involvement of the IFN-I pathway in sterile kidney inflammation provides potential insights into the complex interplay between the innate immune system and damage to the most sensitive segment of the nephron, the glomerulus. The STING pathway is often cited as one cause of renal disease not attributed to viral infections. Instead, this pathway can recognize and signal in response to host-derived nucleic acids, which are also recognized by RLRs and TLRs. It is still unclear, however, whether the development of renal diseases depends on subsequent IFN-I induction or other processes involved. This review aims to explore the main endogenous inducers of IFN-I in glomerular cells, to discuss what effects autocrine and paracrine signaling have on IFN-I induction, and to identify the pathways that are implicated in the development of glomerular damage.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Journal Article
    免疫球蛋白A肾病(IgAN)仍然是全球原发性肾小球疾病的主要原因。进展性慢性肾病和肾衰竭的发生率很高,结果很差,这有助于全球医疗保健成本。尽管已经描述了这种疾病实体,直到最近还没有针对疾病的治疗方法,目前的护理标准侧重于最佳支持措施,包括改变生活方式和优化肾素-血管紧张素-醛固酮阻断。然而,在过去的十年中,人们对IgAN发病机制的认识取得了重大进展,以及接受加速药物批准的替代结果,已经测试了许多针对这种疾病的新研究药物。随着这些代理变得可用,我们设想了一种多管齐下的治疗策略,同时针对持续的肾单位损失的后果,停止任何肾小球炎症,抑制肾小球和肾小管间质中的促纤维化信号,并抑制致病性IgA分子的产生。这篇评论是对2021年发表的先前评论的更新,我们旨在总结IgAN治疗策略的发展和更新,并强调可能增加我们的医疗设备的有希望的发现。
    Immunoglobulin A nephropathy (IgAN) remains the leading cause of primary glomerular disease worldwide. Outcomes are poor with high rates of progressive chronic kidney disease and kidney failure, which contributes to global healthcare costs. Although this disease entity has been described, there were no disease-specific treatments until recently, with the current standard of care focusing on optimal supportive measures including lifestyle modifications and optimization of the renin-angiotensin-aldosterone blockade. However, with significant advances in the understanding of the pathogenesis of IgAN in the past decade, and the acceptance of surrogate outcomes for accelerated drug approval, there have been many new investigational agents tested to target this disease. As these agents become available, we envision a multi-pronged treatment strategy that simultaneously targets the consequences of ongoing nephron loss, stopping any glomerular inflammation, inhibiting pro-fibrotic signals in the glomerulus and tubulo-interstitium, and inhibiting the production of pathogenic IgA molecules. This review is an update on a previous review published in 2021, and we aim to summarize the developments and updates in therapeutic strategies in IgAN and highlight the promising discoveries that are likely to add to our armamentarium.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Journal Article
    肾病综合征具有重要的临床重要性,其特征是尿液中大量蛋白质丢失。肾小球基底膜或足细胞的损伤通常是肾蛋白丢失的基础。人们越来越相信补语系统的参与,先天免疫系统的一部分,在这些条件下。了解补体系统和肾小球结构之间的相互作用不断发展,挑战传统的血尿屏障作为被动过滤器的观点。临床研究表明,在各个点精确抑制补体系统可能很快变得可行。然而,彻底了解当前的知识对于规划肾病肾小球疾病如膜性肾小球病的未来治疗至关重要,膜增生性肾小球肾炎,狼疮性肾炎,局灶性节段肾小球硬化,和微小变化的疾病。这篇综述概述了互补系统,它与肾小球结构的相互作用,以及对表现出肾病病程的特定肾小球疾病的见解。此外,我们探索新的诊断工具和未来的治疗方法。
    The nephrotic syndrome holds significant clinical importance and is characterized by a substantial protein loss in the urine. Damage to the glomerular basement membrane or podocytes frequently underlies renal protein loss. There is an increasing belief in the involvement of the complement system, a part of the innate immune system, in these conditions. Understanding the interactions between the complement system and glomerular structures continually evolves, challenging the traditional view of the blood-urine barrier as a passive filter. Clinical studies suggest that a precise inhibition of the complement system at various points may soon become feasible. However, a thorough understanding of current knowledge is imperative for planning future therapies in nephrotic glomerular diseases such as membranous glomerulopathy, membranoproliferative glomerulonephritis, lupus nephritis, focal segmental glomerulosclerosis, and minimal change disease. This review provides an overview of the complement system, its interactions with glomerular structures, and insights into specific glomerular diseases exhibiting a nephrotic course. Additionally, we explore new diagnostic tools and future therapeutic approaches.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

公众号