Kidney International Reports最新文献

英文中文

Acute Kidney Injury in Immune-Mediated Thrombotic Thrombocytopenic Purpura – Solving the Puzzle? 免疫介导的血栓性血小板减少性紫癜急性肾损伤-解决这个难题？

IF 5.7 2区医学 Q1 UROLOGY & NEPHROLOGY

Kidney International Reports

Pub Date : 2025-11-17 DOI: 10.1016/j.ekir.2025.11.018

Juliane Schneider , Camille Schlesser , Michael Fischereder , Anke von Bergwelt-Baildon , Ulf Schönermarck

引用次数: 0

Alport Syndrome is a Partial Tubulointerstitial Disease of the Kidney Alport综合征是一种部分肾小管间质性疾病

IF 5.7 2区医学 Q1 UROLOGY & NEPHROLOGY

Kidney International Reports

Pub Date : 2025-11-17 DOI: 10.1016/j.ekir.2025.11.019

Lisa Loderbauer , Karl X. Knaup , Daniel Reisenbüchler , Nicolas Kaiser , Stephanie Naas , Karen Schneider , Florian J. Wopperer , Antje Wiesener , Francesca Pasutto , Mario Schiffer , Christoph Daniel , Katharina A.E. Broeker , Dorit Merhof , Maike Buettner-Herold , Michael S. Wiesener

Introduction

Recent genetic studies have shown that Alport syndrome (AS) is much more prevalent than clinically recognized, suggesting that atypical cases may phenocopy other kidney diseases. To date, pathomechanistic studies of AS have focused exclusively on the glomerular membrane, yet equally strong expression of collagen α(IV) chains is found along the distal renal tubule. We hypothesized that genetically determined abnormality of the tubular collagen IV (α345) molecule contributes to kidney failure and may drive atypical phenotypes.

Methods

Histology and primary tubular cells (PTCs) of 8 patients with AS were investigated alongside controls.

Results

Collagen α5 (IV) was detected within the tubular basement membrane (BM) (TBM) of the distal segments of renal tubules by immunohistochemistry. In situ hybridization on human tissues and protein detection of collagen α5 (IV) in PTC cultures clearly showed that the distal tubular apparatus predominantly produces collagen IV for the TBM. Electron microscopy of biopsies from patients with AS demonstrated irregularities of the TBM, somewhat similar as described for the glomerular BM (GBM). Finally, computer-assisted analyses showed that in biopsies of patients with AS, interstitial fibrosis preferentially occurs in spatial vicinity of the affected distal tubules.

Conclusion

Our study demonstrates that the collagen IV (α345) molecule within the TBM is largely produced by the distal tubule itself. In AS, the TBM shows ultrastructural changes, which may induce fibrotic molecular signatures, as tubulointerstitial fibrosis appears to start in the vicinity of the distal tubule. Therefore, we postulate that the progression of kidney disease in AS may in part stem from the (distal) tubular apparatus.

最近的遗传学研究表明，阿尔波特综合征（AS）比临床认识到的更为普遍，这表明非典型病例可能是其他肾脏疾病的表型。迄今为止，AS的病理机制研究仅集中在肾小球膜上，然而沿远端肾小管也发现了同样强烈的胶原α(IV)链表达。我们假设，遗传决定的管状胶原IV （α345）分子异常有助于肾衰竭，并可能导致非典型表型。方法对8例AS患者的组织学和原代小管细胞（ptc）进行观察。结果免疫组化法在肾小管远段基底膜（TBM）内检测到α5 （IV）胶原蛋白。人体组织的原位杂交和PTC培养中α5 （IV）胶原蛋白的检测清楚地表明，远端管状器主要为TBM产生IV胶原。来自AS患者的活检电镜显示TBM不规则，与肾小球BM （GBM）的描述有些相似。最后，计算机辅助分析显示，在AS患者的活检中，间质纤维化优先发生在受影响的远端小管附近。结论TBM内的胶原IV （α345）分子主要由远端小管自身产生。在AS中，TBM显示超微结构变化，这可能诱发纤维化分子特征，因为小管间质纤维化似乎开始于远端小管附近。因此，我们假设AS肾脏疾病的进展可能部分源于（远端）肾小管。

{"title":"Alport Syndrome is a Partial Tubulointerstitial Disease of the Kidney","authors":"Lisa Loderbauer , Karl X. Knaup , Daniel Reisenbüchler , Nicolas Kaiser , Stephanie Naas , Karen Schneider , Florian J. Wopperer , Antje Wiesener , Francesca Pasutto , Mario Schiffer , Christoph Daniel , Katharina A.E. Broeker , Dorit Merhof , Maike Buettner-Herold , Michael S. Wiesener","doi":"10.1016/j.ekir.2025.11.019","DOIUrl":"10.1016/j.ekir.2025.11.019","url":null,"abstract":"<div><h3>Introduction</h3><div>Recent genetic studies have shown that Alport syndrome (AS) is much more prevalent than clinically recognized, suggesting that atypical cases may phenocopy other kidney diseases. To date, pathomechanistic studies of AS have focused exclusively on the glomerular membrane, yet equally strong expression of collagen α(IV) chains is found along the distal renal tubule. We hypothesized that genetically determined abnormality of the tubular collagen IV (α345) molecule contributes to kidney failure and may drive atypical phenotypes.</div></div><div><h3>Methods</h3><div>Histology and primary tubular cells (PTCs) of 8 patients with AS were investigated alongside controls.</div></div><div><h3>Results</h3><div>Collagen α5 (IV) was detected within the tubular basement membrane (BM) (TBM) of the distal segments of renal tubules by immunohistochemistry. <em>In situ</em> hybridization on human tissues and protein detection of collagen α5 (IV) in PTC cultures clearly showed that the distal tubular apparatus predominantly produces collagen IV for the TBM. Electron microscopy of biopsies from patients with AS demonstrated irregularities of the TBM, somewhat similar as described for the glomerular BM (GBM). Finally, computer-assisted analyses showed that in biopsies of patients with AS, interstitial fibrosis preferentially occurs in spatial vicinity of the affected distal tubules.</div></div><div><h3>Conclusion</h3><div>Our study demonstrates that the collagen IV (α345) molecule within the TBM is largely produced by the distal tubule itself. In AS, the TBM shows ultrastructural changes, which may induce fibrotic molecular signatures, as tubulointerstitial fibrosis appears to start in the vicinity of the distal tubule. Therefore, we postulate that the progression of kidney disease in AS may in part stem from the (distal) tubular apparatus.</div></div>","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"11 2","pages":"Article 103694"},"PeriodicalIF":5.7,"publicationDate":"2025-11-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145789063","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Obinutuzumab as a Rescue Therapy in Anti-Rituximab Antibody-Positive Primary Membranous Nephropathy Obinutuzumab作为抗利妥昔单抗抗体阳性原发性膜性肾病的拯救疗法

IF 5.7 2区医学 Q1 UROLOGY & NEPHROLOGY

Kidney International Reports

Pub Date : 2025-11-17 DOI: 10.1016/j.ekir.2025.11.015

Yang Liu , Di Wu , Rui Xue , Yuna Chen , Qijun Wan

引用次数: 0

Global Perspectives on Genetic Testing Among Nephrologists 肾病学家基因检测的全球视角

IF 5.7 2区医学 Q1 UROLOGY & NEPHROLOGY

Kidney International Reports

Pub Date : 2025-11-17 DOI: 10.1016/j.ekir.2025.11.012

Jaison George , Tushar Vachharajani , Xiangling Wang

引用次数: 0

A Systematic Review Evaluating Estimated GFR Performance in South Asian Populations 评估南亚人群GFR估计表现的系统综述

IF 5.7 2区医学 Q1 UROLOGY & NEPHROLOGY

Kidney International Reports

Pub Date : 2025-11-12 DOI: 10.1016/j.ekir.2025.11.004

Rouvick Mariano Gama , Ayush Sinha , Alexander Sarnowski , Kathryn Dalrymple , Pierre Delanaye , Kate Bramham

Introduction

Estimated glomerular filtration rate (eGFR) equations are widely used to measure kidney function; however, their performance in South Asian populations is variable. We performed a systematic review and meta-analysis to assess the performance of creatinine-based (eGFR_Cr), cystatin-C–based (eGFR_CysC) and combined (eGFR_Cr-CysC) eGFR equations compared with measured GFR (mGFR) in South Asians.

Methods

Pubmed/Medline, Embase (Ovid), Scopus, Web of Science, and Cochrane Library for Systematic Reviews and Clinical Trials were searched for relevant studies between January 1, 1976 and November 30, 2024. Eligible studies compared eGFR to mGFR in South Asian adult populations. Meta-analyses were performed to assess bias (standardized mean difference [SMD]) and 30% accuracy (meta proportion/percentage of eGFR results within 30% of the corresponding mGFR result [P30]) for eGFR equations compared with mGFR with multivariable meta-regression for key moderators.

Results

Thirty-five cohorts (n = 4725) were included. eGFR_Cr equations overestimated mGFR with the Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI-2009_Cr) equation showing the highest standardised mean difference (0.66; 95% confidence interval [CI]: 0.35–0.97). eGFR_CysC had the least standardized mean difference (−0.03 to 0.15). However, accuracy for eGFR_Cr and eGFR_CysC equations were < 75%. eGFR_Cr-CysC had the best P30, ranging from 79% to 82%. Meta-regression identified age, sex, ethnicity, and cohort type as having significant impacts on bias effect size.

Conclusion

eGFR equation accuracy in South Asians is suboptimal, consistently below the accepted clinical P30 threshold of 75%. eGFR_CysC equations had reduced bias compared with eGFR_Cr or eGFR_Cr-CysC; however, accuracy was better with eGFR_Cr-CysC. Emerging equations, such as the CKD-EPI-Pakistan variant and European Kidney Function Consortium (EKFC) equations warrant further investigation in diverse South Asian cohorts with population-specific calibration.

估计肾小球滤过率（eGFR）方程被广泛用于测量肾功能；然而，他们在南亚人群中的表现是不同的。我们进行了一项系统回顾和荟萃分析，以评估南亚地区基于肌酐（eGFRCr）、基于胱抑素c （eGFRCysC）和联合（eGFRCr- cysc） eGFR方程与测量GFR （mGFR）的性能。方法检索spubmed /Medline、Embase （Ovid）、Scopus、Web of Science、Cochrane Library for Systematic Reviews and Clinical Trials，检索1976年1月1日至2024年11月30日的相关研究。符合条件的研究比较了南亚成年人的eGFR和mGFR。进行meta分析以评估eGFR方程的偏差（标准化平均差[SMD]）和30%的准确性（eGFR结果在相应mGFR结果的30%内的meta比例/百分比[P30]），并将关键调节因子与具有多变量meta回归的mGFR进行比较。结果共纳入35个队列（n = 4725）。eGFRCr方程高估了mGFR，而慢性肾脏疾病流行病学合作（CKD-EPI-2009Cr）方程显示出最高的标准化平均差异（0.66；95%可信区间[CI]: 0.35-0.97）。eGFRCysC的标准化平均差异最小（- 0.03 ~ 0.15）。然而，eGFRCr和eGFRCysC方程的准确率为75%。eGFRCr-CysC的P30最佳，范围为79% ~ 82%。meta回归发现年龄、性别、种族和队列类型对偏倚效应大小有显著影响。结论南亚人的egfr方程准确性不理想，始终低于临床公认的P30阈值75%。与eGFRCr或eGFRCr- cysc相比，eGFRCysC方程的偏倚降低；然而，eGFRCr-CysC的准确性更好。新兴的方程，如ckd - epi -巴基斯坦变体和欧洲肾功能联盟（EKFC）方程，值得在南亚不同的队列中进行进一步的研究，并进行特定人群的校准。

{"title":"A Systematic Review Evaluating Estimated GFR Performance in South Asian Populations","authors":"Rouvick Mariano Gama , Ayush Sinha , Alexander Sarnowski , Kathryn Dalrymple , Pierre Delanaye , Kate Bramham","doi":"10.1016/j.ekir.2025.11.004","DOIUrl":"10.1016/j.ekir.2025.11.004","url":null,"abstract":"<div><h3>Introduction</h3><div>Estimated glomerular filtration rate (eGFR) equations are widely used to measure kidney function; however, their performance in South Asian populations is variable. We performed a systematic review and meta-analysis to assess the performance of creatinine-based (eGFR<sub>Cr</sub>), cystatin-C–based (eGFR<sub>CysC</sub>) and combined (eGFR<sub>Cr-CysC</sub>) eGFR equations compared with measured GFR (mGFR) in South Asians.</div></div><div><h3>Methods</h3><div>Pubmed/Medline, Embase (Ovid), Scopus, Web of Science, and Cochrane Library for Systematic Reviews and Clinical Trials were searched for relevant studies between January 1, 1976 and November 30, 2024. Eligible studies compared eGFR to mGFR in South Asian adult populations. Meta-analyses were performed to assess bias (standardized mean difference [SMD]) and 30% accuracy (meta proportion/percentage of eGFR results within 30% of the corresponding mGFR result [P30]) for eGFR equations compared with mGFR with multivariable meta-regression for key moderators.</div></div><div><h3>Results</h3><div>Thirty-five cohorts (<em>n</em> = 4725) were included. eGFR<sub>Cr</sub> equations overestimated mGFR with the Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI-2009<sub>Cr</sub>) equation showing the highest standardised mean difference (0.66; 95% confidence interval [CI]: 0.35–0.97). eGFR<sub>CysC</sub> had the least standardized mean difference (−0.03 to 0.15). However, accuracy for eGFR<sub>Cr</sub> and eGFR<sub>CysC</sub> equations were < 75%. eGFR<sub>Cr-CysC</sub> had the best P30, ranging from 79% to 82%. Meta-regression identified age, sex, ethnicity, and cohort type as having significant impacts on bias effect size.</div></div><div><h3>Conclusion</h3><div>eGFR equation accuracy in South Asians is suboptimal, consistently below the accepted clinical P30 threshold of 75%. eGFR<sub>CysC</sub> equations had reduced bias compared with eGFR<sub>Cr</sub> or eGFR<sub>Cr-CysC</sub>; however, accuracy was better with eGFR<sub>Cr-CysC</sub>. Emerging equations, such as the CKD-EPI-Pakistan variant and European Kidney Function Consortium (EKFC) equations warrant further investigation in diverse South Asian cohorts with population-specific calibration.</div></div>","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"11 2","pages":"Article 103677"},"PeriodicalIF":5.7,"publicationDate":"2025-11-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145926818","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Recurrence of C3 Glomerulopathy and Immune Complex–Mediated Membranoproliferative Glomerulonephritis After Kidney Transplantation: Challenges and Opportunities 肾移植后C3肾小球病变和免疫复合物介导的膜增生性肾小球肾炎的复发：挑战和机遇

IF 5.7 2区医学 Q1 UROLOGY & NEPHROLOGY

Kidney International Reports

Pub Date : 2025-11-12 DOI: 10.1016/j.ekir.2025.11.008

José Enrique Ruiz-Cabello , Hernando Trujillo , Teresa Cavero , Amado Andrés , Manuel Praga , Fernando Caravaca-Fontán

Recurrent C3 glomerulopathy (C3G) and primary immune complex–mediated membranoproliferative glomerulonephritis (IC-MPGN) remain major causes of kidney allograft dysfunction and loss. Their pathogenesis involves genetic susceptibility, acquired complement dysregulation, and transplant-related triggers, yet no clear validated predictors of recurrence exist. Recurrence rates after kidney transplantation frequently exceed 60% and are associated with progression to graft failure. Early detection, often achievable through protocol biopsies, offers an opportunity for timely intervention, although histologic recognition of early recurrence can be challenging. Comprehensive pretransplant evaluation, including genetic and molecular complement testing and screening for monoclonal gammopathies, may refine risk stratification. Conventional treatments, including intensified immunosuppression, rituximab, and plasma exchange, have shown limited and inconsistent efficacy. Conversely, complement-targeted therapies have yielded promising outcomes. Although eculizumab remains the most extensively studied agent, emerging proximal complement inhibitors such as iptacopan and pegcetacoplan have shown encouraging efficacy in both native and recurrent disease. However, optimal timing, prophylactic use, and long-term safety in transplant recipients remain uncertain. In this review, we outline the clinical spectrum, key diagnostic and therapeutic challenges, and emerging treatment strategies for recurrent C3G and IC-MPGN, highlighting advances that may ultimately improve graft survival and patient outcomes.

复发性C3肾小球病变（C3G）和原发性免疫复合物介导的膜增生性肾小球肾炎（IC-MPGN）仍然是导致同种异体肾功能障碍和丧失的主要原因。其发病机制涉及遗传易感性、获得性补体失调和移植相关的触发因素，但目前尚无明确的复发预测因素。肾移植后的复发率经常超过60%，并伴有移植物衰竭的进展。尽管早期复发的组织学识别可能具有挑战性，但通常通过方案活检可以实现早期检测，为及时干预提供了机会。全面的移植前评估，包括遗传和分子补体检测以及单克隆伽玛病筛查，可能会完善风险分层。常规治疗，包括强化免疫抑制、利妥昔单抗和血浆置换，显示出有限和不一致的疗效。相反，补体靶向治疗已经产生了有希望的结果。尽管eculizumab仍然是研究最广泛的药物，新兴的近端补体抑制剂，如伊普他科泮和pegcetacoplan，在原生和复发性疾病中都显示出令人鼓舞的疗效。然而，移植受者的最佳时机、预防使用和长期安全性仍不确定。在这篇综述中，我们概述了复发性C3G和IC-MPGN的临床谱、关键诊断和治疗挑战，以及新兴的治疗策略，强调了可能最终改善移植物存活和患者预后的进展。

{"title":"Recurrence of C3 Glomerulopathy and Immune Complex–Mediated Membranoproliferative Glomerulonephritis After Kidney Transplantation: Challenges and Opportunities","authors":"José Enrique Ruiz-Cabello , Hernando Trujillo , Teresa Cavero , Amado Andrés , Manuel Praga , Fernando Caravaca-Fontán","doi":"10.1016/j.ekir.2025.11.008","DOIUrl":"10.1016/j.ekir.2025.11.008","url":null,"abstract":"<div><div>Recurrent C3 glomerulopathy (C3G) and primary immune complex–mediated membranoproliferative glomerulonephritis (IC-MPGN) remain major causes of kidney allograft dysfunction and loss. Their pathogenesis involves genetic susceptibility, acquired complement dysregulation, and transplant-related triggers, yet no clear validated predictors of recurrence exist. Recurrence rates after kidney transplantation frequently exceed 60% and are associated with progression to graft failure. Early detection, often achievable through protocol biopsies, offers an opportunity for timely intervention, although histologic recognition of early recurrence can be challenging. Comprehensive pretransplant evaluation, including genetic and molecular complement testing and screening for monoclonal gammopathies, may refine risk stratification. Conventional treatments, including intensified immunosuppression, rituximab, and plasma exchange, have shown limited and inconsistent efficacy. Conversely, complement-targeted therapies have yielded promising outcomes. Although eculizumab remains the most extensively studied agent, emerging proximal complement inhibitors such as iptacopan and pegcetacoplan have shown encouraging efficacy in both native and recurrent disease. However, optimal timing, prophylactic use, and long-term safety in transplant recipients remain uncertain. In this review, we outline the clinical spectrum, key diagnostic and therapeutic challenges, and emerging treatment strategies for recurrent C3G and IC-MPGN, highlighting advances that may ultimately improve graft survival and patient outcomes.</div></div>","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"11 2","pages":"Article 103682"},"PeriodicalIF":5.7,"publicationDate":"2025-11-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145789061","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Kidney Tertiary Lymphoid Tissues and Poor Immunosuppressive Response in IgA Nephropathy IgA肾病的肾三级淋巴组织和不良免疫抑制反应

IF 5.7 2区医学 Q1 UROLOGY & NEPHROLOGY

Kidney International Reports

Pub Date : 2025-11-12 DOI: 10.1016/j.ekir.2025.11.003

Hao Zhao , Rui Ma , Zhuoni Zhong , Di Xie , Han Ouyang , Zhanmei Zhou , Cailing Su , Nan Jia , Xin Xu , Fan Fan Hou

Introduction

Ectopic lymphoid structures termed as tertiary lymphoid tissues (TLTs) are observed in certain kidney diseases, including IgA nephropathy (IgAN); however, clinical relevance of TLTs remains unclear.

Methods

This prospective cohort study included 845 Chinese patients with IgAN with proteinuria ≥ 1 g/d despite 3 months of optimized supportive care with renin-angiotensin system inhibitors. Patients received immunosuppression for a median of 22 months. Kidney interstitial TLTs, defined as lymphocyte aggregates, were counted using immunochemistry and staged by the absence (stage I) or presence of follicular dendritic cells (FDCs) (stage II) or germinal centers (GCs) (stage III). The outcome was response to immunosuppression, defined as no remission with up to 12 months of immunosuppression.

Results

Kidney interstitial TLTs were present in 429 of 845 patients with IgAN (50.8%), of whom 72 of 429 (16.8%) had TLTs at advanced stages (stage II or III). Compared with patients without TLTs, both the presence and stage of TLTs were associated with significantly increased risk of poor response to immunosuppression, with an adjusted odds ratio (95% confidence interval [CI]) of 3.07 (2.16–4.37), 2.97 (2.05–4.28), and 6.25 (3.39–11.53) in those with TLTs at any stage, stage I, and advanced stage, respectively. This grade association remained consistent across all prespecified subgroups. The stage and number of TLTs predicted poor response to immunosuppression with an area under receiver operating characteristic curve (AUC) of 0.81 (95% CI: 0.75–0.87). The AUC increased to 0.90 (95% CI: 0.87–0.94) when we combined TLTs with the clinical and glomerular macrophage data at biopsy.

Conclusion

The presence of interstitial TLTs at diagnosis was associated with poor response to immunosuppression in IgAN.

异位淋巴样结构被称为三级淋巴样组织（tlt），在某些肾脏疾病中观察到，包括IgA肾病（IgAN）；然而，tlt的临床意义尚不清楚。方法：本前瞻性队列研究纳入845例中国IgAN患者，患者在接受肾素-血管紧张素系统抑制剂优化支持治疗3个月后，蛋白尿≥1 g/d。患者接受免疫抑制的中位时间为22个月。肾间质tlt被定义为淋巴细胞聚集体，使用免疫化学方法进行计数，并根据滤泡树突状细胞（fdc）（II期）或生发中心（GCs）（III期）的缺失（I期）或存在进行分期。结果是对免疫抑制的反应，定义为免疫抑制长达12个月无缓解。结果845例IgAN患者中有429例（50.8%）出现肾间质tlt，其中72例（16.8%）在晚期（II期或III期）出现tlt。与没有tlt的患者相比，tlt的存在和分期与免疫抑制不良反应的风险显著增加相关，在任何阶段、一期和晚期tlt患者中，调整后的优势比（95%置信区间[CI]）分别为3.07（2.16-4.37）、2.97（2.05-4.28）和6.25（3.39-11.53）。这种分级关联在所有预先指定的亚组中保持一致。tlt的分期和数量预测免疫抑制不良反应，接受者工作特征曲线下面积（AUC）为0.81 （95% CI: 0.75-0.87）。当我们将TLTs与临床和肾小球巨噬细胞活检数据相结合时，AUC增加到0.90 （95% CI: 0.87-0.94）。结论诊断时间质性tlt的存在与IgAN免疫抑制反应较差有关。

{"title":"Kidney Tertiary Lymphoid Tissues and Poor Immunosuppressive Response in IgA Nephropathy","authors":"Hao Zhao , Rui Ma , Zhuoni Zhong , Di Xie , Han Ouyang , Zhanmei Zhou , Cailing Su , Nan Jia , Xin Xu , Fan Fan Hou","doi":"10.1016/j.ekir.2025.11.003","DOIUrl":"10.1016/j.ekir.2025.11.003","url":null,"abstract":"<div><h3>Introduction</h3><div>Ectopic lymphoid structures termed as tertiary lymphoid tissues (TLTs) are observed in certain kidney diseases, including IgA nephropathy (IgAN); however, clinical relevance of TLTs remains unclear.</div></div><div><h3>Methods</h3><div>This prospective cohort study included 845 Chinese patients with IgAN with proteinuria ≥ 1 g/d despite 3 months of optimized supportive care with renin-angiotensin system inhibitors. Patients received immunosuppression for a median of 22 months. Kidney interstitial TLTs, defined as lymphocyte aggregates, were counted using immunochemistry and staged by the absence (stage I) or presence of follicular dendritic cells (FDCs) (stage II) or germinal centers (GCs) (stage III). The outcome was response to immunosuppression, defined as no remission with up to 12 months of immunosuppression.</div></div><div><h3>Results</h3><div>Kidney interstitial TLTs were present in 429 of 845 patients with IgAN (50.8%), of whom 72 of 429 (16.8%) had TLTs at advanced stages (stage II or III). Compared with patients without TLTs, both the presence and stage of TLTs were associated with significantly increased risk of poor response to immunosuppression, with an adjusted odds ratio (95% confidence interval [CI]) of 3.07 (2.16–4.37), 2.97 (2.05–4.28), and 6.25 (3.39–11.53) in those with TLTs at any stage, stage I, and advanced stage, respectively. This grade association remained consistent across all prespecified subgroups. The stage and number of TLTs predicted poor response to immunosuppression with an area under receiver operating characteristic curve (AUC) of 0.81 (95% CI: 0.75–0.87). The AUC increased to 0.90 (95% CI: 0.87–0.94) when we combined TLTs with the clinical and glomerular macrophage data at biopsy.</div></div><div><h3>Conclusion</h3><div>The presence of interstitial TLTs at diagnosis was associated with poor response to immunosuppression in IgAN.</div></div>","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"11 2","pages":"Article 103676"},"PeriodicalIF":5.7,"publicationDate":"2025-11-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145789123","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

A Decade of C3 Glomerulopathy—A Nationwide Cohort Study C3肾小球病变的十年——一项全国队列研究

IF 5.7 2区医学 Q1 UROLOGY & NEPHROLOGY

Kidney International Reports

Pub Date : 2025-11-12 DOI: 10.1016/j.ekir.2025.11.007

Rick H. Overwijk , Fiona R. Kolbinger , Mark Eijgelsheim , Olaf M. Dekkers , Andreas Kronbichler , Ingeborg M. Bajema

Introduction

C3 glomerulopathy (C3G) is a rare but devastating disease affecting children and adults. It frequently leads to end-stage kidney failure, and currently no specific treatment exists. C3G is used as a collective term for dense deposit disease (DDD) and C3 glomerulonephritis (C3GN) and is thought to sometimes occur in postinfectious settings (C3-PIGN). Currently, little is known about the incidence and distribution of subtypes in the population. We analyzed a large cohort of patients with C3G in the Netherlands regarding incidence and disease subtype distribution in relation to geographical factors and histopathological findings.

Methods

A search in the Dutch Nationwide Pathology Databank (Palga) was performed to identify patients diagnosed with C3G from January 2014 until December 2023, subsequently ascertained by 2 independent observers. We assessed the correlation of disease subtypes with glomerular patterns, and the geographical distribution was charted.

Results

The selection resulted in a cohort of 280 patients consisting of C3GN (n = 101), DDD (n = 39), unspecified C3G (n = 106), C3-PIGN (n = 29), and others (n = 5). The median age at biopsy diagnosis was 19 (range: 4–75) years for DDD and 54 (range: 2–86) years for C3GN, showing age distribution depends on C3G subtype (P < 0.001). DDD and C3G were associated with membranoproliferative pattern and C3-PIGN with endocapillary or exudative pattern.

Conclusion

Our results show consistent assessment of kidney biopsies across the country and absence of geographical factors influencing disease development.

c3肾小球病（C3G）是一种罕见但严重的疾病，影响儿童和成人。它经常导致终末期肾衰竭，目前没有专门的治疗方法。C3G被用作致密沉积病（DDD）和C3肾小球肾炎（C3GN）的统称，被认为有时发生在感染后环境（C3- pign）。目前，对人群中亚型的发病率和分布知之甚少。我们分析了荷兰C3G患者的一大队列，分析了与地理因素和组织病理学结果相关的发病率和疾病亚型分布。方法在荷兰全国病理数据库（Palga）中检索2014年1月至2023年12月诊断为C3G的患者，随后由2名独立观察员确定。我们评估了疾病亚型与肾小球模式的相关性，并绘制了地理分布图。结果选择280例患者，包括C3GN (n = 101), DDD (n = 39)，未指定的C3G (n = 106), C3-PIGN （n = 29）和其他（n = 5）。活检诊断时DDD的中位年龄为19岁（范围：4-75岁），C3GN的中位年龄为54岁（范围：2-86岁），年龄分布取决于C3G亚型（P < 0.001）。DDD和C3G与膜增生性有关，C3-PIGN与毛细血管内或渗出性有关。结论我们的研究结果显示了全国肾活检评估的一致性，并且没有影响疾病发展的地理因素。

{"title":"A Decade of C3 Glomerulopathy—A Nationwide Cohort Study","authors":"Rick H. Overwijk , Fiona R. Kolbinger , Mark Eijgelsheim , Olaf M. Dekkers , Andreas Kronbichler , Ingeborg M. Bajema","doi":"10.1016/j.ekir.2025.11.007","DOIUrl":"10.1016/j.ekir.2025.11.007","url":null,"abstract":"<div><h3>Introduction</h3><div>C3 glomerulopathy (C3G) is a rare but devastating disease affecting children and adults. It frequently leads to end-stage kidney failure, and currently no specific treatment exists. C3G is used as a collective term for dense deposit disease (DDD) and C3 glomerulonephritis (C3GN) and is thought to sometimes occur in postinfectious settings (C3-PIGN). Currently, little is known about the incidence and distribution of subtypes in the population. We analyzed a large cohort of patients with C3G in the Netherlands regarding incidence and disease subtype distribution in relation to geographical factors and histopathological findings.</div></div><div><h3>Methods</h3><div>A search in the Dutch Nationwide Pathology Databank (Palga) was performed to identify patients diagnosed with C3G from January 2014 until December 2023, subsequently ascertained by 2 independent observers. We assessed the correlation of disease subtypes with glomerular patterns, and the geographical distribution was charted.</div></div><div><h3>Results</h3><div>The selection resulted in a cohort of 280 patients consisting of C3GN (<em>n</em> = 101), DDD (<em>n</em> = 39), unspecified C3G (<em>n</em> = 106), C3-PIGN (<em>n</em> = 29), and others (<em>n</em> = 5). The median age at biopsy diagnosis was 19 (range: 4–75) years for DDD and 54 (range: 2–86) years for C3GN, showing age distribution depends on C3G subtype (<em>P</em> < 0.001). DDD and C3G were associated with membranoproliferative pattern and C3-PIGN with endocapillary or exudative pattern.</div></div><div><h3>Conclusion</h3><div>Our results show consistent assessment of kidney biopsies across the country and absence of geographical factors influencing disease development.</div></div>","PeriodicalId":17761,"journal":{"name":"Kidney International Reports","volume":"11 2","pages":"Article 103681"},"PeriodicalIF":5.7,"publicationDate":"2025-11-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145789058","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Histopathological Spectrum of Acute Kidney Injury After Wasp Sting Envenomation 黄蜂蜇伤后急性肾损伤的组织病理学谱

IF 5.7 2区医学 Q1 UROLOGY & NEPHROLOGY

Kidney International Reports

Pub Date : 2025-11-11 DOI: 10.1016/j.ekir.2025.11.006

K.S. Jansi Prema , C.V. Malathi , Anila Abraham Kurien

引用次数: 0

Clinical Variability of ADPKD in Monozygotic Twins 同卵双胞胎ADPKD的临床变异

IF 5.7 2区医学 Q1 UROLOGY & NEPHROLOGY

Kidney International Reports

Pub Date : 2025-11-11 DOI: 10.1016/j.ekir.2025.11.002

Othmane Mohib , Stefan Van Cauwelaert , Laura Pölsler , Patricia Van Der Niepen , Carola Brussaard , Bruno Van Vlem , Rachel Schauer , Peter C. Harris , Peter Janssens

引用次数: 0

首页上一页

下一页尾页

类型

全部化学•材料生命科学医学物理工程技术环境•农林材料科学地球科学法学管理学化学环境科学与生态学计算机科学教育学经济学农林科学人文科学生物学数学物理与天体物理心理学综合性期刊其他工业工程理学历史学农学文学信息工程

数据库

全部 ACS Publications Elsevier ieeexplore Springer The Royal Society of Chemistry Wiley

期刊

Kidney International Reports

全部 Acc. Chem. Res. ACS Applied Bio Materials ACS Appl. Electron. Mater. ACS Appl. Energy Mater. ACS Appl. Mater. Interfaces ACS Appl. Nano Mater. ACS Appl. Polym. Mater. ACS BIOMATER-SCI ENG ACS Catal. ACS Cent. Sci. ACS Chem. Biol. ACS Chemical Health & Safety ACS Chem. Neurosci. ACS Comb. Sci. ACS Earth Space Chem. ACS Energy Lett. ACS Infect. Dis. ACS Macro Lett. ACS Mater. Lett. ACS Med. Chem. Lett. ACS Nano ACS Omega ACS Photonics ACS Sens. ACS Sustainable Chem. Eng. ACS Synth. Biol. Anal. Chem. BIOCHEMISTRY-US Bioconjugate Chem. BIOMACROMOLECULES Chem. Res. Toxicol. Chem. Rev. Chem. Mater. CRYST GROWTH DES ENERG FUEL Environ. Sci. Technol. Environ. Sci. Technol. Lett. Eur. J. Inorg. Chem. IND ENG CHEM RES Inorg. Chem. J. Agric. Food. Chem. J. Chem. Eng. Data J. Chem. Educ. J. Chem. Inf. Model. J. Chem. Theory Comput. J. Med. Chem. J. Nat. Prod. J PROTEOME RES J. Am. Chem. Soc. LANGMUIR MACROMOLECULES Mol. Pharmaceutics Nano Lett. Org. Lett. ORG PROCESS RES DEV ORGANOMETALLICS J. Org. Chem. J. Phys. Chem. J. Phys. Chem. A J. Phys. Chem. B J. Phys. Chem. C J. Phys. Chem. Lett. Analyst Anal. Methods Biomater. Sci. Catal. Sci. Technol. Chem. Commun. Chem. Soc. Rev. CHEM EDUC RES PRACT CRYSTENGCOMM Dalton Trans. Energy Environ. Sci. ENVIRON SCI-NANO ENVIRON SCI-PROC IMP ENVIRON SCI-WAT RES Faraday Discuss. Food Funct. Green Chem. Inorg. Chem. Front. Integr. Biol. J. Anal. At. Spectrom. J. Mater. Chem. A J. Mater. Chem. B J. Mater. Chem. C Lab Chip Mater. Chem. Front. Mater. Horiz. MEDCHEMCOMM Metallomics Mol. Biosyst. Mol. Syst. Des. Eng. Nanoscale Nanoscale Horiz. Nat. Prod. Rep. New J. Chem. Org. Biomol. Chem. Org. Chem. Front. PHOTOCH PHOTOBIO SCI PCCP Polym. Chem.

﹀