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Etiology of Chronic Renal Failure in Hodeida, Yemen 也门荷台达慢性肾衰竭的病因学
IF 5.7 2区 医学 Q1 UROLOGY & NEPHROLOGY Pub Date : 2025-12-20 DOI: 10.1016/j.ekir.2025.103738
Nabeel Yahya Al-madwahi , Ahmed Hasan Batah , Muneer Gamil Alwesabi , Haitham Mohammed Jowah
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引用次数: 0
The Case for Combination Treatment of CKD in Type 2 Diabetes Mellitus 2型糖尿病慢性肾病联合治疗一例
IF 5.7 2区 医学 Q1 UROLOGY & NEPHROLOGY Pub Date : 2025-12-19 DOI: 10.1016/j.ekir.2025.103736
Viktor Rotbain Curovic
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引用次数: 0
Prospective Cohort Study of Obinutuzumab in Rituximab-Resistant Primary Membranous Nephropathy Obinutuzumab治疗耐利妥昔单抗原发性膜性肾病的前瞻性队列研究
IF 5.7 2区 医学 Q1 UROLOGY & NEPHROLOGY Pub Date : 2025-12-18 DOI: 10.1016/j.ekir.2025.103728
Matias Trillini , Valentina Portalupi , Alessia Gennarini , Livia Surdi , Annachiara Currado , Maddalena Marasà , Manuel Alfredo Podestà , Alessandro Villa , Tobia Peracchi , Diego Fidone , Nadia Rubis , Olimpia Diadei , Chiara Guarinoni , Federica Casiraghi , Marta Todeschini , Daniela Cugini , Fabiola Carrara , Davide Martinetti , Paola Rizzo , Giuseppe Remuzzi , Piero Ruggenenti

Introduction

Approximately 30% of patients with primary membranous nephropathy (MN) and persistent nephrotic syndrome (NS) fail rituximab therapy through mechanisms that could be overcome by obinutuzumab.

Methods

In this prospective, single-arm, single-center, open-label trial, 20 consenting adults with MN and rituximab-resistant NS received three 1000 mg obinutuzumab infusions at the Bergamo Nephrology Unit (Italy) between March 2022 and February 2024 and were monitored for ≤ 12 months. The primary outcome was a composite end point of normo-albuminemia and complete (proteinuria < 0.3 g/d) or partial (proteinuria < 3.5 g/d with ≥ 50% reduction from baseline) NS remission at 12-month follow-up. Twenty-four-hour proteinuria and glomerular filtration rate (GFR) were evaluated at baseline and at 3, 6, 9, 12, 18, and 24 months posttreatment.

Results

At 12 months, 16 patients met the combined end point, 4 with complete remission. No patient relapsed after remission. Median (interquartile range) 24-h proteinuria decreased from 5.7 (4.7–8.1) to 1.3 (0.5–2.7) g/24 h. Albumin and IgG fractional clearances also decreased, whereas serum albumin increased from 2.9 ± 0.6 g/dl to 3.9 ± 0.3 g/dl (P < 0.0001 for all changes). Dyslipidemia, hypocalcemia, and hypo-gammaglobulinemia improved significantly and GFR stabilized. At 12 months, total B cells and circulating anti–phospholipase A2 receptor (PLA2R) antibodies were depleted. Similar findings were observed in the cohort of 10 patients who completed 24 months of follow-up. However, total B cells reemerged in the circulation without an increase in anti-PLA2R antibodies and proteinuria, or relapses. Treatment was safe and well-tolerated.

Conclusion

Obinutuzumab treatment is extremely effective and safe in patients with MN and rituximab-resistant NS and can achieve persistent remission in this population.
大约30%的原发性膜性肾病(MN)和持续性肾病综合征(NS)患者通过可通过obinutuzumab克服的机制使利妥昔单抗治疗失败。在这项前瞻性、单组、单中心、开放标签的试验中,20名同意患有MN和利妥昔单抗耐药NS的成年人在2022年3月至2024年2月期间在意大利贝加莫肾病科接受了3次1000 mg的obinutuzumab输注,并进行了≤12个月的监测。在12个月的随访中,主要终点是正常白蛋白血症和完全(蛋白尿0.3 g/d)或部分(蛋白尿3.5 g/d,较基线减少≥50%)NS缓解的复合终点。在基线和治疗后3、6、9、12、18和24个月评估24小时蛋白尿和肾小球滤过率(GFR)。结果12个月后,16例患者达到联合终点,4例患者完全缓解。缓解后无患者复发。24小时蛋白尿中位数(四分位数范围)从5.7 (4.7-8.1)g/24小时下降到1.3 (0.5-2.7)g/24小时。白蛋白和IgG分数清除率也下降,而血清白蛋白从2.9±0.6 g/dl上升到3.9±0.3 g/dl (P < 0.0001)。血脂异常、低钙血症和低γ球蛋白血症显著改善,GFR稳定。12个月时,总B细胞和循环抗磷脂酶A2受体(PLA2R)抗体被耗尽。在完成24个月随访的10名患者队列中观察到类似的结果。然而,总B细胞在循环中重新出现,抗pla2r抗体和蛋白尿没有增加,也没有复发。治疗是安全且耐受性良好的。结论奥比妥珠单抗治疗在MN和利妥昔单抗耐药NS患者中是非常有效和安全的,并且可以在这一人群中实现持续缓解。
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引用次数: 0
Improving Lifelong Comprehensive Care Coordination in Nephropathic Cystinosis: Multidisciplinary Perspectives 改善肾病型胱氨酸病终身综合护理协调:多学科视角
IF 5.7 2区 医学 Q1 UROLOGY & NEPHROLOGY Pub Date : 2025-12-18 DOI: 10.1016/j.ekir.2025.103735
Ladan Golestaneh , Elizabeth G. Ames , Maya H. Doyle , Cybele Ghossein , Paul C. Grimm , Jennifer L. Hewlett , Alaena Lim , Lauren S. Marzinelli , Kimberly J. Schmidt , Bonnie Smeryage , Joshua J. Zaritsky , Frederick J. Kaskel
Nephropathic cystinosis is a rare lysosomal storage disorder characterized by cellular cystine accumulation and progressive multiorgan damage. Before advances in diagnostics, transplantation, and disease-modifying therapy, cystinosis was considered a fatal pediatric disease, with most patients reaching kidney failure by approximately 10 years of age. Life expectancy has now expanded into the 50s and beyond, and the disease has been transformed into a chronic condition with a predominantly extrarenal phenotype in adulthood. Traditionally, nephrologists have played a central role as cystinosis “care quarterbacks” in pediatric settings, and patients often expect this to continue after transitioning to adult care. As such, nephrologists are increasingly tasked with monitoring for and addressing complications in organs other than the kidneys, in addition to coordinating referrals to clinicians within the nephrology clinic (e.g., nurses, advanced practice providers, pharmacists, dietitians, social workers, care coordinators, and transplant specialists) and across multiple specialties (e.g., neurologists, gastroenterologists, endocrinologists, ophthalmologists, and orthopedists). Although recently published expert guidance offers recommendations for the multidisciplinary management of cystinosis, gaps in the literature exist around strategies to reduce care fragmentation, overcome collaboration challenges, and enhance patient/family and clinician experiences. This review aimed to explore various approaches for care delivery optimization across the lifespan of patients with cystinosis as well as improve the current understanding of disease-, patient-, provider-, and system-related factors that influence treatment adherence, engagement, and long-term outcomes.
肾病型胱氨酸病是一种罕见的溶酶体贮积性疾病,以细胞胱氨酸积累和进行性多器官损害为特征。在诊断、移植和疾病改善治疗取得进展之前,胱氨酸病被认为是一种致命的儿科疾病,大多数患者在大约10岁时达到肾衰竭。预期寿命现在已经扩展到50岁以上,并且这种疾病已经转变为一种慢性疾病,在成年期主要表现为外源性表型。传统上,肾病学家在儿科设置中扮演了胱氨酸病“护理四分卫”的核心角色,患者通常期望在过渡到成人护理后继续这样做。因此,除了协调肾内科诊所的临床医生(如护士、高级执业医师、药剂师、营养师、社会工作者、护理协调员和移植专家)和跨多个专业(如神经科医生、胃肠科医生、内分泌科医生、眼科医生和骨科医生)的转诊外,肾内科医生越来越多地承担监测和处理肾脏以外器官并发症的任务。尽管最近出版的专家指南为胱氨酸病的多学科管理提供了建议,但在减少护理碎片化、克服协作挑战以及增强患者/家庭和临床医生经验的策略方面,文献存在空白。本综述旨在探索胱氨酸病患者整个生命周期中优化护理交付的各种方法,并提高当前对影响治疗依从性、参与和长期结果的疾病、患者、提供者和系统相关因素的理解。
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引用次数: 0
Corrigendum to “Supportive Evidence of Surrogate End Points Based on Treatment Effect of Corticosteroid in IgA Nephropathy” [Kidney International Reports Volume 11, Issue 2, 2026, Article 103588] “基于皮质类固醇治疗IgA肾病疗效的替代终点的支持性证据”的勘误表[肾脏国际报告第11卷,第2期,2026,第103588条]
IF 5.7 2区 医学 Q1 UROLOGY & NEPHROLOGY Pub Date : 2025-12-18 DOI: 10.1016/j.ekir.2025.103716
Takaya Sasaki , Nobuo Tsuboi , Kentaro Koike , Hiroyuki Ueda , Masahiro Okabe , Shinya Yokote , Akihiro Shimizu , Keita Hirano , Tetsuya Kawamura , Takashi Yokoo , Yusuke Suzuki , J-IGACS Working Group
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引用次数: 0
Licorice-Induced Pseudohyperaldosteronism Highlights an Underestimated Etiology of Hypertension 甘草诱导的假性醛固酮增多症强调了一个被低估的高血压病因
IF 5.7 2区 医学 Q1 UROLOGY & NEPHROLOGY Pub Date : 2025-12-17 DOI: 10.1016/j.ekir.2025.103731
Jean-Joseph Bendjilali-Sabiani , Antoine Cardinale , Anaïs Lamy , Alix-Marie Pouget , Jean-Christophe Boyer , Véronique Taillard , Pascal Reboul
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引用次数: 0
CKD in Migrants: From Epidemiology to Challenges 移民慢性肾病:从流行病学到挑战
IF 5.7 2区 医学 Q1 UROLOGY & NEPHROLOGY Pub Date : 2025-12-16 DOI: 10.1016/j.ekir.2025.103726
Karen Courville , Vicente Sánchez-Polo , Roberta Arena , Greta Borelli , Lilio Hu , Miriam Di Nunzio , Gianluigi Zaza , Gaetano La Manna , Luca De Nicola , Michele Provenzano
Chronic kidney disease (CKD) is now considered a global health burden. In addition to the well-known clinical risk factors, social determinants of the disease such as poverty, low birthweight, and lack of access to health services play an important role, with the prevalence of disease modified by various socioeconomic factors, including migration. Herein, we explore the intersection of CKD and migration by examining how migrant populations can modify global prevalence and therapy of CKD global prevalence, including the challenges faced by migrant populations in accessing nephrology units. In addition, we provide suggestions for improving their care.
慢性肾脏疾病(CKD)现在被认为是一个全球性的健康负担。除了众所周知的临床风险因素外,该病的社会决定因素,如贫穷、低出生体重和缺乏获得保健服务的机会也起着重要作用,各种社会经济因素(包括移徙)改变了该病的流行程度。在此,我们通过研究移民人口如何改变CKD全球患病率和治疗方法,包括移民人口在进入肾脏病单位时面临的挑战,来探索CKD和移民的交集。此外,我们还提出了改善护理的建议。
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引用次数: 0
A Pathway to High Quality Clinical Trials in IgA Vasculitis Nephritis: Meeting Proceedings From a Multiprofessional International Collaborative Workshop IgA血管炎肾炎的高质量临床试验途径:多专业国际合作研讨会会议记录
IF 5.7 2区 医学 Q1 UROLOGY & NEPHROLOGY Pub Date : 2025-12-16 DOI: 10.1016/j.ekir.2025.103729
Louise Oni , Rona Smith , Seza Ozen , Chloe Williams , Elin Davies , Charlotte King , Paul Brogan , Mark Haas , Jonathan Barratt , Jeffrey Hafkin , Despina Eleftheriou , Karuna Keat , EMD Smith , Wen Ding , Chee Cheung , Caroline Platt , Evangéline Pillebout , Andrew Chetwynd , Areefa Alladin , Augusto Vaglio , Alan Salama
IgA vasculitis (IgAV) is an autoimmune disease that affects the small vessels of the skin, joints, gastrointestinal (GI) tract, and kidneys. In the long term, IgAV associated with nephritis (IgAV-N) can progress to kidney failure. Evidence-based clinical studies of IgAV-N are few, leading to huge variations in treatment approaches and suboptimal outcomes. The wealth of emerging efficacious treatments for IgA nephrology brings new opportunities to this disease. The aim of this report is to describe the proceedings of a multiprofessional collaborative workshop convened to identify the barriers to developing high quality evidence for patients with IgAV-N. A multiprofessional group consisting of 53 attendees from 13 countries met. The meeting was represented by a variety of professional backgrounds, including lay attendees, with different levels of expertise (32% professors and 19% midcareer doctors). Using predefined aims, key themes were extracted, and an action plan developed. Consensus was obtained that there is sufficient similarity between adults and children in terms of the organs involved, pathophysiology, histological features, and likely response to treatment. Important differences included the greater spontaneous improvement in children and worse kidney outcomes in some populations. It was agreed that patients at greatest risk of kidney failure should be the primary focus of initial clinical trials. Important considerations included the following: diagnostic classification for adult onset IgAV, observational data, evidence of scientific similarity to IgA nephropathy (IgAN), an age-inclusive approach to trial design, systemic disease secondary end points, and the inclusion of patient-reported outcomes. This manuscript communicates an expert-informed pathway to high-quality evidence for IgAV-N.
IgA血管炎(IgAV)是一种自身免疫性疾病,影响皮肤、关节、胃肠道和肾脏的小血管。长期来看,与肾炎相关的IgAV (IgAV- n)可发展为肾衰竭。基于证据的IgAV-N临床研究很少,导致治疗方法差异很大,结果不理想。新出现的有效治疗IgA肾病的方法为这种疾病带来了新的机会。本报告的目的是描述一个多专业合作研讨会的会议记录,该研讨会旨在确定为IgAV-N患者开发高质量证据的障碍。由来自13个国家的53名与会者组成的多专业小组举行了会议。与会人员来自各行各业,包括专业水平不同的非专业人士(32%为教授,19%为职业中期医生)。利用预先确定的目标,提取了关键主题,并制定了行动计划。人们一致认为,成人和儿童在受累器官、病理生理学、组织学特征和可能的治疗反应方面有足够的相似性。重要的差异包括儿童更大的自发改善和一些人群更差的肾脏预后。人们一致认为,肾功能衰竭风险最大的患者应该是初始临床试验的主要焦点。重要的考虑因素包括:成人发病IgAV的诊断分类、观察数据、与IgA肾病(IgAN)的科学相似性证据、试验设计的年龄包容性方法、全身性疾病次要终点,以及纳入患者报告的结局。这份手稿为IgAV-N提供了一条专家知情的高质量证据途径。
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引用次数: 0
Atypical Anti–Glomerular Basement Membrane Disease 非典型抗肾小球基底膜病
IF 5.7 2区 医学 Q1 UROLOGY & NEPHROLOGY Pub Date : 2025-12-16 DOI: 10.1016/j.ekir.2025.103732
Loreto Fernández-Lorente , Amir Shabaka , José María Mora-Gutiérrez , Fernando Caravaca-Fontán , María Maldonado-Martín , Virginia Cabello , Helena Marco , Alba Temprado , Carlos Rivera , Lucía Facal , Xoana Barros , Carmen Calderón González , Joaquín Manrique , Ángel Panizo Santos , Gema Fernández-Juárez
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引用次数: 0
Nephrology Survey on Reproductive Health and Kidney Disease 生殖健康与肾脏疾病肾病学调查
IF 5.7 2区 医学 Q1 UROLOGY & NEPHROLOGY Pub Date : 2025-12-16 DOI: 10.1016/j.ekir.2025.103730
Nedas Semaska , Rachael Nolan , Darren Lee , Silvi Shah

Introduction

Chronic kidney disease (CKD) affects ≤6% of females of childbearing age, with pregnancy posing considerable health risks. Despite this, contraceptive use among this population remains low, and nephrologists’ perspectives remain underexplored.

Methods

In this cross-sectional study, we used a 52-item electronic survey of US nephrologists to assess their practices and confidence related to reproductive health care for female patients with CKD. Descriptive and bivariate analyses were used to explore associations with provider characteristics.

Results

Among 104 respondents, most practiced in academic settings, and more than half had seen > 15 female patients of childbearing age with CKD in the past year. Reproductive health issues were infrequently addressed, with most “rarely/never” discussing sexual dysfunction (82%), menstrual disorders (59%), or contraception (31%). More than half were “not at all confident” in managing sexual dysfunction (54%), followed by menstrual disorders (42%). Common barriers included lack of standardized guidelines (39%), limited time (38%), and knowledge gaps (33%). More than half reported that the absence of guidelines decreased their likelihood of counseling. A patient receiving a kidney transplant was the factor that most positively influenced the provider’s decision to provide reproductive health counseling. Nephrologists who saw more female patients annually reported significantly higher confidence in managing contraception (30% vs. 11%, P = 0.043), breastfeeding (38% vs. 14%, P = 0.0107), antihypertensives (85% vs. 57%, P = 0.0029), and immunosuppression (63% vs. 36%, P = 0.0117).

Conclusion

US nephrologists reported limited confidence and inconsistent practices in reproductive counseling, underscoring the need for a standardized approach and best practices at a national level to guide care for female patients.
慢性肾脏疾病(CKD)影响≤6%的育龄女性,妊娠带来相当大的健康风险。尽管如此,这一人群的避孕药具使用率仍然很低,肾病学家的观点仍未得到充分探讨。方法在这项横断面研究中,我们对美国肾病学家进行了52项电子调查,以评估他们对女性慢性肾病患者生殖保健的做法和信心。使用描述性和双变量分析来探索与提供者特征的关联。结果104名受访者中,大多数在学术机构执业,超过一半的人在过去一年中见过15名育龄女性CKD患者。生殖健康问题很少被提及,大多数人“很少/从不”讨论性功能障碍(82%)、月经紊乱(59%)或避孕(31%)。超过一半的人对处理性功能障碍“完全没有信心”(54%),其次是月经紊乱(42%)。常见的障碍包括缺乏标准化指南(39%)、时间有限(38%)和知识差距(33%)。超过一半的人报告说,缺乏指导方针降低了他们接受咨询的可能性。接受肾脏移植的病人是对提供者决定提供生殖健康咨询影响最大的因素。每年看到更多女性患者的肾病学家报告说,她们对避孕(30%对11%,P = 0.043)、母乳喂养(38%对14%,P = 0.0107)、抗高血压(85%对57%,P = 0.0029)和免疫抑制(63%对36%,P = 0.0117)的管理有更高的信心。结论:美国肾病学家报告了生殖咨询的可信度有限和不一致的实践,强调了在国家层面上指导女性患者护理的标准化方法和最佳实践的必要性。
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引用次数: 0
期刊
Kidney International Reports
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