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"They Know You Better Than the Transplant Team": An Interpretive Description Study Exploring the Perspectives of Living Kidney Donors About Care Received From Family Physicians. “他们比移植团队更了解你”:一项探讨活体肾供者从家庭医生那里接受护理的观点的解释性描述研究。
IF 1.6 Q3 UROLOGY & NEPHROLOGY Pub Date : 2025-03-14 eCollection Date: 2025-01-01 DOI: 10.1177/20543581251324548
Katya Loban, Charo Rodriguez, Antoine Przybylak-Brouillard, Elie Fadel, Heather Badenoch, Peter Nugus, Ann Bugeja, Justin Gill, Marie-Chantal Fortin, Emilie Trinh, Scott McKay, Shaifali Sandal

Background: Given the significant benefits of living donor kidney transplantation, the nephrology and transplant communities are augmenting efforts to increase living kidney donation. However, prior living kidney donors (LKDs) report suboptimal experiences and unmet care needs. The LKDs are healthy, and the vast majority have good outcomes post-donation. Thus, in clinical practice, their care is primarily assumed by practitioners, such as family physicians (FPs).

Objective: This study aimed to better understand the integration of primary care in LKDs' donation trajectory from the point of view of the latter. Our specific research questions were: (1) How do LKDs perceive the role of FPs currently integrated into the donation trajectory? (2) What are their needs and expectations from their FPs?

Design: An interpretive description methodology.

Setting and participants: Canadian LKDs who donated a kidney prior to 2020.

Methods: Qualitative interviews and inductive thematic analysis.

Results: In our sample of 49 LKDs who donated between 2007 and 2020, 61.2% were women and 87.8% were white. Also, 87.8% and 83.7% were attached to an FP pre- and post-donation (1 by a nurse practitioner) with 16.3% reporting no regular FP post-donation. Although participants provided varying accounts, an overwhelming majority described challenges with timely access to needed care; lack of cohesive continuity of care; variability in the services offered by FPs; and challenges with coordination of care between providers. Many reported poor coordination and communication between FPs and donor teams. Most articulated the desire to see an expanded role for FPs. This included improvements in knowledge regarding living donor care, information and care brokerage, continuous integrative care, and mental and emotional support.

Limitations: Limited transferability of our findings to other countries with variable payment structures.

Conclusions: Our work suggests that improving LKD care requires developing care pathways that facilitate donor transition and care coordination between donor teams and primary care practitioners. Given the challenges being faced by primary care in Canada, we believe that pragmatic strategies to better support primary care practitioners and a stronger integration of primary care with the living kidney donation process are essential. In addition, strategies to better support the mental health of LKDs are also needed. The LKDs provide a valuable gift to our health systems and to patients with kidney failure. It is our responsibility to optimize their experiences and improve their care.

背景:鉴于活体肾移植的显著好处,肾脏学和移植界正在加大努力,以增加活体肾捐赠。然而,先前的活体肾脏供者(LKDs)报告不理想的经历和未满足的护理需求。LKDs是健康的,绝大多数捐献后的结果都很好。因此,在临床实践中,他们的护理主要由从业者承担,如家庭医生(FPs)。目的:本研究旨在从低龄儿童捐献轨迹的角度,更好地了解初级保健在低龄儿童捐献轨迹中的整合。我们的具体研究问题是:(1)LKDs如何看待目前整合到捐赠轨迹中的FPs的作用?(2)他们对FPs的需求和期望是什么?设计:解释性描述方法。背景和参与者:在2020年之前捐赠肾脏的加拿大LKDs。方法:定性访谈和归纳专题分析。结果:在2007年至2020年期间捐赠的49名LKDs样本中,61.2%是女性,87.8%是白人。此外,87.8%和83.7%的人在捐献前和捐献后都有计划生育(1名护士执业),16.3%的人在捐献后没有定期计划生育。尽管参与者提供了不同的说法,但绝大多数人描述了及时获得所需护理的挑战;缺乏连贯的连续性;FPs所提供服务的可变性;以及提供者之间协调护理的挑战。许多国家报告说,FPs和捐助小组之间的协调和沟通很差。大多数人都希望看到FPs扮演更大的角色。这包括对活体捐赠者护理、信息和护理代理、持续综合护理以及精神和情感支持方面知识的改进。局限性:我们的研究结果在其他支付结构多变的国家的可转移性有限。结论:我们的工作表明,改善LKD护理需要发展护理途径,促进供体过渡和供体团队和初级保健从业人员之间的护理协调。考虑到加拿大初级保健面临的挑战,我们认为,更好地支持初级保健从业人员和加强初级保健与活体肾脏捐赠过程的整合的务实战略是必不可少的。此外,还需要制定战略,更好地支持弱势群体的心理健康。LKDs为我们的卫生系统和肾衰竭患者提供了一份宝贵的礼物。我们有责任优化他们的体验,改善他们的护理。
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引用次数: 0
Using Unsupervised Clustering to Characterize Phenotypes Among Older Kidney Transplant Recipients: A Cohort Study. 使用无监督聚类来描述老年肾移植受者的表型:一项队列研究。
IF 1.6 Q3 UROLOGY & NEPHROLOGY Pub Date : 2025-03-14 eCollection Date: 2025-01-01 DOI: 10.1177/20543581251322576
Sareen Singh, Syed Sibte Raza Abidi, Syed Asil Ali Naqvi, Amanda J Vinson, Thomas A A Skinner, George Worthen, Samina Abidi, Kenneth A West, Karthik K Tennankore

Background: Older kidney transplant recipients have inferior outcomes compared to younger recipients, and this risk may be compounded by donor characteristics.

Objective: We applied an unsupervised machine learning clustering approach to group older recipients into similar phenotypes. We evaluated the association between each cluster and graft failure, and the impact of donor quality on outcomes.

Design: This is a nationally representative retrospective cohort study.

Setting and patients: Kidney transplant recipients aged ≥65 years identified from the Scientific Registry of Transplant Recipients (2000-2017).

Measurements and methods: We used unsupervised clustering to generate phenotypes using 16 recipient factors. Donor quality was evaluated using 2 approaches, including the Kidney Donor Risk Index (KDRI). All-cause graft failure was analyzed using multivariable Cox regression.

Results: Overall, 16 364 patients (mean age 69 years; 38% female) were separated into 3 clusters. Cluster 1 recipients were exclusively female; cluster 2 recipients were exclusively males without diabetes; and cluster 3 recipients were males with a higher burden of comorbidities. Compared to cluster 2, the risk of graft failure was higher for cluster 3 recipients (adjusted hazard ratio [aHR] = 1.25, 95% confidence interval [CI] = 1.19-1.32). Cluster 3 recipients of a lower quality (KDRI ≥1.45) kidney had the highest risk of graft failure (aHR = 1.74, 95% CI = 1.61-1.87) relative to cluster 2 recipients of a higher quality kidney.

Limitations: This study did not include an external validation cohort. The findings should be interpreted as exploratory and should not be used to inform individual risk prediction nor be applied to recipients <65 years of age.

Conclusions: In a national cohort of older kidney transplant recipients, unsupervised clustering generated 3 clinically distinct recipient phenotypes. These phenotypes may aid in complementing allocation decisions, providing prognostic information, and optimizing post-transplant care for older recipients.

背景:年龄较大的肾移植受者与年轻受者相比预后较差,并且这种风险可能因供者特征而加剧。目的:我们应用无监督机器学习聚类方法将老年受体分组为相似表型。我们评估了每个集群与移植物失败之间的关系,以及供体质量对结果的影响。设计:这是一项具有全国代表性的回顾性队列研究。环境和患者:年龄≥65岁的肾移植受者,从移植受者科学登记(2000-2017)中确定。测量和方法:我们使用无监督聚类产生表型使用16受体因子。采用肾供者风险指数(KDRI)等2种方法评价供者质量。采用多变量Cox回归分析全因移植物衰竭。结果:共16 364例患者(平均年龄69岁;女性占38%),分为3组。第1组受助人全是女性;第2组接受者均为无糖尿病的男性;第3组受体为男性,其合并症负担较高。与第2类相比,第3类受者移植失败的风险更高(校正风险比[aHR] = 1.25, 95%可信区间[CI] = 1.19-1.32)。第三组低质量(KDRI≥1.45)肾移植失败的风险最高(aHR = 1.74, 95% CI = 1.61-1.87),高于第二组高质量肾移植。局限性:本研究未包括外部验证队列。结论:在全国老年肾移植受者队列中,无监督聚类产生了3种临床不同的受者表型。这些表型可能有助于补充分配决策,提供预后信息,并优化老年受者的移植后护理。
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引用次数: 0
A Province-Wide Home Dialysis Program Review: Challenges, Strengths, and Future Strategies. 全省家庭透析项目回顾:挑战、优势和未来战略。
IF 1.6 Q3 UROLOGY & NEPHROLOGY Pub Date : 2025-03-14 eCollection Date: 2025-01-01 DOI: 10.1177/20543581251324560
Megan Borkum, Micheli Bevilacqua, Alexandra Romann, Krishna Poinen, Sushila Saunders, Linda Turnbull, R Suneet Singh, Adeera Levin, Michael A Copland

Purpose of review: British Columbia (BC) has a robust provincial kidney care program emphasizing patient-centered and goal-oriented dialysis care. Despite maintaining a home dialysis prevalence of approximately 30%, consistently above the national average, a review was conducted to examine intake and attrition rates and optimize these outcomes within a learning health system context.

Sources of information: This review draws on published articles, program reports, and insights from the provincial kidney care program framework. Key components include funding models, multidisciplinary committees, administrative support, and comprehensive training resources for staff and patients.

Methods: A structured analysis was conducted to evaluate factors influencing home dialysis rates. The approach focused on health care system dynamics, professional practices, and patient characteristics, emphasizing identifying barriers and opportunities for program optimization.

Key findings: Challenges identified include ongoing biases among health care professionals and logistical barriers in remote areas. Future initiatives aim to standardize patient screening, promote home dialysis champions, adopt environmentally friendly practices, and expand peer support networks.

Limitations: The review is constrained by potential regional variability within BC and limited generalizability to other provinces or countries. In addition, patient preferences and broader societal influences require further exploration.

Implications: A systematic approach to assessing and optimizing home dialysis programs is essential. The findings highlight the need to address health care system barriers, improve professional education, and promote patient engagement to increase home dialysis uptake and sustainability.

综述目的:不列颠哥伦比亚省(BC)有一个强大的省级肾脏护理计划,强调以患者为中心和以目标为导向的透析护理。尽管家庭透析的流行率保持在约30%,始终高于全国平均水平,但仍进行了一项审查,以检查摄入量和损耗率,并在学习卫生系统背景下优化这些结果。信息来源:本综述借鉴了已发表的文章、项目报告和来自省级肾脏护理项目框架的见解。关键组成部分包括资助模式、多学科委员会、行政支持以及对工作人员和患者的综合培训资源。方法:对影响家庭透析率的因素进行结构化分析。该方法侧重于卫生保健系统动态、专业实践和患者特征,强调识别程序优化的障碍和机会。主要发现:确定的挑战包括卫生保健专业人员中持续存在的偏见和偏远地区的后勤障碍。未来的举措旨在使患者筛查标准化,推广家庭透析冠军,采用环保做法,并扩大同伴支持网络。局限性:该综述受到不列颠哥伦比亚省内部潜在的区域差异和对其他省份或国家的有限推广的限制。此外,患者偏好和更广泛的社会影响需要进一步探索。意义:一个系统的方法来评估和优化家庭透析方案是必不可少的。研究结果强调需要解决卫生保健系统的障碍,改善专业教育,促进患者参与,以增加家庭透析的吸收和可持续性。
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引用次数: 0
Approximating the Proportion of Individuals With Kidney Failure Who Die Without Kidney Replacement Therapy in Ontario, Canada. 加拿大安大略省肾衰竭患者未接受肾脏替代治疗而死亡的近似比例。
IF 1.6 Q3 UROLOGY & NEPHROLOGY Pub Date : 2025-03-14 eCollection Date: 2025-01-01 DOI: 10.1177/20543581251323961
Andrea C Cowan, Nivethika Jeyakumar, Amit X Garg, Stephanie Dixon, Bin Luo, Peter G Blake

Background: Quantifying the number and proportion of people with kidney failure (KF) who receive conservative kidney management is vital for health care system benchmarking and planning. It is not easy to ascertain this value precisely at the population level, but we can approximate it using information from different data sources to estimate the proportion of patients with advanced kidney disease who die without receiving dialysis or a transplant and should receive conservative kidney management.

Objective: To approximate the proportion of people with KF in Ontario, Canada, who die without receiving kidney replacement therapy.

Design: A review of unpublished provincial renal agency reports of 3 retrospective population-based cohorts combined with clinical interpretation.

Patients: The 3 cohorts of people were: 1. those who died between January 1, 2013 and December 31, 2017, with an estimated glomerular filtration rate (eGFR) <10 mL/min/1.73 m2 and no evidence of receiving kidney replacement therapy; 2. those who initiated outpatient maintenance dialysis or received a preemptive transplant in the same period; and 3. those with a sustained low eGFR ≤ 10 mL/min/1.73 m2 between April 1, 2015 and March 31, 2018, and were followed for 1 year to determine if they started dialysis. In this last cohort, patients whose kidney function improved (evidence of an eGFR > 10 mL/min/1.73 m2) or who received a transplant during follow-up were excluded from the analysis.

Measurements and methods: The 3 cohorts were derived at ICES and used linked health care databases for the province of Ontario, Canada. In 2016, Ontario had a population of about 14 million people. Two nephrologists reviewed the data to provide the clinical approximation.

Results: There were 1891 individuals with KF who died without kidney replacement (the no KRT cohort). The median (25th, 75th percentile) eGFR prior to death was 7 (5, 8) mL/min/1.73 m2. During the same period, 13 511 individuals started dialysis or received a preemptive kidney transplant (the KRT cohort). There were 7259 individuals in the low eGFR cohort; over the following year, 66% started dialysis, 20% died without dialysis, and 14% were alive without starting dialysis. The clinical approximation is that between 13 and 16% of people with KF die without receiving kidney replacement therapy.

Limitations: The data reports lacked certain information to inform the clinical approximation. There was no information on the conversations health professionals had with people about kidney replacement therapy, any decisions made about receiving conservative care, or the circumstances that preceded death without kidney replacement therapy.

Conclusions: After reviewing data from the 3 cohorts, we clinically approximate

背景:量化接受保守肾管理的肾衰竭(KF)患者的数量和比例对于卫生保健系统的基准和规划至关重要。在人群水平上精确地确定这个值是不容易的,但我们可以使用来自不同数据源的信息来估计晚期肾脏疾病患者不接受透析或移植而死亡并应接受保守肾脏管理的比例。目的:估计加拿大安大略省未接受肾脏替代治疗而死亡的KF患者的比例。设计:回顾未发表的省级肾脏机构的3个回顾性人群队列报告,并结合临床解释。患者:三组患者分别为:1.;2013年1月1日至2017年12月31日期间死亡,估计肾小球滤过率(eGFR) 2且没有接受肾脏替代治疗的证据;2. 在同一时期进行过门诊维持性透析或接受过先期移植的患者;和3。2015年4月1日至2018年3月31日期间持续低eGFR≤10 mL/min/1.73 m2的患者,随访1年以确定是否开始透析。在最后一个队列中,在随访期间肾功能改善(eGFR bb0 10 mL/min/1.73 m2)或接受移植的患者被排除在分析之外。测量和方法:这3个队列来自ICES,并使用了加拿大安大略省的相关卫生保健数据库。2016年,安大略省的人口约为1400万。两位肾病学家回顾了这些数据,以提供临床近似值。结果:1891例KF患者死于无肾置换(无KRT队列)。死亡前eGFR中位数(25、75百分位)为7 (5,8)mL/min/1.73 m2。在同一时期,13 511人开始透析或接受了先发制人的肾移植(KRT队列)。低eGFR队列中有7259人;在接下来的一年里,66%的人开始透析,20%的人在没有透析的情况下死亡,14%的人在没有开始透析的情况下存活。临床估计是13 - 16%的KF患者在未接受肾脏替代治疗的情况下死亡。局限性:数据报告缺乏一定的信息来告知临床近似。没有关于卫生专业人员与患者就肾脏替代疗法进行的对话的信息,没有关于接受保守治疗的任何决定,也没有关于未接受肾脏替代疗法的死亡前情况的信息。结论:在回顾了三个队列的数据后,我们在临床上估计,加拿大安大略省6名KF患者中有1人在没有接受透析的情况下死亡,应该接受保守的肾脏管理。
{"title":"Approximating the Proportion of Individuals With Kidney Failure Who Die Without Kidney Replacement Therapy in Ontario, Canada.","authors":"Andrea C Cowan, Nivethika Jeyakumar, Amit X Garg, Stephanie Dixon, Bin Luo, Peter G Blake","doi":"10.1177/20543581251323961","DOIUrl":"https://doi.org/10.1177/20543581251323961","url":null,"abstract":"<p><strong>Background: </strong>Quantifying the number and proportion of people with kidney failure (KF) who receive conservative kidney management is vital for health care system benchmarking and planning. It is not easy to ascertain this value precisely at the population level, but we can approximate it using information from different data sources to estimate the proportion of patients with advanced kidney disease who die without receiving dialysis or a transplant and should receive conservative kidney management.</p><p><strong>Objective: </strong>To approximate the proportion of people with KF in Ontario, Canada, who die without receiving kidney replacement therapy.</p><p><strong>Design: </strong>A review of unpublished provincial renal agency reports of 3 retrospective population-based cohorts combined with clinical interpretation.</p><p><strong>Patients: </strong>The 3 cohorts of people were: 1. those who died between January 1, 2013 and December 31, 2017, with an estimated glomerular filtration rate (eGFR) <10 mL/min/1.73 m<sup>2</sup> and no evidence of receiving kidney replacement therapy; 2. those who initiated outpatient maintenance dialysis or received a preemptive transplant in the same period; and 3. those with a sustained low eGFR ≤ 10 mL/min/1.73 m<sup>2</sup> between April 1, 2015 and March 31, 2018, and were followed for 1 year to determine if they started dialysis. In this last cohort, patients whose kidney function improved (evidence of an eGFR > 10 mL/min/1.73 m<sup>2</sup>) or who received a transplant during follow-up were excluded from the analysis.</p><p><strong>Measurements and methods: </strong>The 3 cohorts were derived at ICES and used linked health care databases for the province of Ontario, Canada. In 2016, Ontario had a population of about 14 million people. Two nephrologists reviewed the data to provide the clinical approximation.</p><p><strong>Results: </strong>There were 1891 individuals with KF who died without kidney replacement (the no KRT cohort). The median (25th, 75th percentile) eGFR prior to death was 7 (5, 8) mL/min/1.73 m<sup>2</sup>. During the same period, 13 511 individuals started dialysis or received a preemptive kidney transplant (the KRT cohort). There were 7259 individuals in the low eGFR cohort; over the following year, 66% started dialysis, 20% died without dialysis, and 14% were alive without starting dialysis. The clinical approximation is that between 13 and 16% of people with KF die without receiving kidney replacement therapy.</p><p><strong>Limitations: </strong>The data reports lacked certain information to inform the clinical approximation. There was no information on the conversations health professionals had with people about kidney replacement therapy, any decisions made about receiving conservative care, or the circumstances that preceded death without kidney replacement therapy.</p><p><strong>Conclusions: </strong>After reviewing data from the 3 cohorts, we clinically approximate ","PeriodicalId":9426,"journal":{"name":"Canadian Journal of Kidney Health and Disease","volume":"12 ","pages":"20543581251323961"},"PeriodicalIF":1.6,"publicationDate":"2025-03-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11909665/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143647356","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Is Childhood IgA Nephropathy Different From Adult IgA Nephropathy? A Narrative Review. 儿童IgA肾病与成人IgA肾病不同吗?叙述性评论。
IF 1.6 Q3 UROLOGY & NEPHROLOGY Pub Date : 2025-03-12 eCollection Date: 2025-01-01 DOI: 10.1177/20543581251322571
Areefa Alladin-Karan, Susan M Samuel, Andrew W Wade, Pietro Ravani, Silviu Grisaru, Ngan N Lam, Kathryn A Bernie, Robert R Quinn
<p><strong>Purpose of the review: </strong>Immunoglobulin A (IgA) nephropathy (IgAN) is the most common primary glomerular kidney disease. Children and adults are presumed to have the same disease and are treated similarly. However, there are differences between childhood IgAN and adult IgAN that may require unique treatment considerations, even after transition to adult nephrology services. A narrative review was conducted to compare childhood and adult IgAN and to describe the distinct characteristics of childhood IgAN. Reframing childhood IgAN can inform guideline recommendations unique to childhood IgAN, the development of targeted therapies, and clinical trial design.</p><p><strong>Sources of information: </strong>Medline and Embase were searched for reports on children and adults with IgAN published between January 2013 and December 2023 (updated May 2024). The search was not restricted by age group, outcomes reported, language, or study design. Randomized controlled trials (RCTs), observational studies, review articles, and nephrology conference abstracts were included. A total of 3104 reports were retrieved. Forty-seven reports (37 primary studies and 10 reviews) were included in the review. Two RCTs and 35 observational studies included a total of 45 085 participants (9223 children and 35 862 adults).</p><p><strong>Method: </strong>Data were extracted for primary IgAN and not for IgA vasculitis-associated nephritis. Findings were described with no statistical comparisons due to variations in interventions and outcome definitions.</p><p><strong>Key findings: </strong>Gross hematuria was the obvious clinical difference between childhood IgAN and adult (60-88% vs 15-20%). Nephrotic syndrome was more common in children, approaching up to 44%, while <18% of adults had nephrotic syndrome. Children were biopsied sooner (6 vs 15 months) and had more inflammatory kidney lesions (mesangial hypercellularity: 41-82% vs 38-64%; endocapillary hypercellularity: 39-58% vs 17-34%). Chronic kidney lesions were more prevalent in adults (segmental sclerosis: 62-77% vs 8-51%; interstitial fibrosis/tubular atrophy: 34-37% vs 1-18%). The use of immunosuppressive therapy was higher in children (46-84% vs 35-56%). Children were started on immunosuppressive therapy sooner than adults. Adults were more likely to be optimized with renin-angiotensin system inhibitors (87-94% vs 49-75%). Children had better kidney function than adults at diagnosis (estimated glomerular filtration rate of 90-128 vs 50-88 ml/min/1.73 m<sup>2</sup>), and children also had better kidney survival, with kidney failure of 3.1% vs 13.4% at 5 years. Children had more risk alleles for IgAN and higher levels of mannose-binding lectin than adults.</p><p><strong>Limitations: </strong>Most studies were retrospective and observational, with limited data on children and disease mechanisms. Data were not pooled for analysis because of important differences in definitions and measurements of baseline
免疫球蛋白A (IgA)肾病(IgAN)是最常见的原发性肾小球肾病。儿童和成人被认为患有同样的疾病,并得到同样的治疗。然而,儿童IgAN和成人IgAN之间存在差异,可能需要独特的治疗考虑,即使在过渡到成人肾病服务之后。对儿童期和成年期IgAN进行了回顾性比较,并描述了儿童期IgAN的不同特征。重新构建儿童IgAN可以为儿童IgAN的独特指南建议、靶向治疗的发展和临床试验设计提供信息。信息来源:在Medline和Embase检索2013年1月至2023年12月(更新于2024年5月)发表的关于儿童和成人IgAN的报告。搜索不受年龄组、结果报告、语言或研究设计的限制。随机对照试验(RCTs)、观察性研究、综述文章和肾脏病会议摘要被纳入。总共检索了3104份报告。47份报告(37项初步研究和10项综述)被纳入本综述。两项随机对照试验和35项观察性研究共纳入45 085名参与者(9223名儿童和35 862名成人)。方法:提取原发性IgAN的数据,而不提取IgA血管炎相关性肾炎的数据。由于干预措施和结果定义的差异,研究结果没有进行统计比较。主要发现:肉眼血尿是儿童IgAN与成人的明显临床差异(60-88% vs 15-20%)。肾病综合征在儿童中更常见,接近44%,而2),儿童也有更好的肾脏存活率,5年肾衰竭为3.1%比13.4%。与成人相比,儿童有更多的IgAN风险等位基因和更高水平的甘露糖结合凝集素。局限性:大多数研究是回顾性和观察性的,关于儿童和疾病机制的数据有限。由于基线特征和结果的定义和测量存在重要差异,因此没有将数据汇总进行分析。已建立尿液筛查项目的国家与未建立尿液筛查项目的国家的数据有所不同。一些观察到的差异可能是由于实践差异和成人的延迟诊断(前置时间偏差)。设计良好的前瞻性研究和标准化的肾功能评估和结果测量方法可以减少异质性并改善综述的结果。结论:儿童IgAN与成人IgAN的内在差异可能是由于不同的疾病机制。将儿童IgAN作为一种单独的疾病来对待,可能会导致发现有针对性的治疗方法,并改善儿童时期和过渡到成人护理后的管理。
{"title":"Is Childhood IgA Nephropathy Different From Adult IgA Nephropathy? A Narrative Review.","authors":"Areefa Alladin-Karan, Susan M Samuel, Andrew W Wade, Pietro Ravani, Silviu Grisaru, Ngan N Lam, Kathryn A Bernie, Robert R Quinn","doi":"10.1177/20543581251322571","DOIUrl":"10.1177/20543581251322571","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Purpose of the review: &lt;/strong&gt;Immunoglobulin A (IgA) nephropathy (IgAN) is the most common primary glomerular kidney disease. Children and adults are presumed to have the same disease and are treated similarly. However, there are differences between childhood IgAN and adult IgAN that may require unique treatment considerations, even after transition to adult nephrology services. A narrative review was conducted to compare childhood and adult IgAN and to describe the distinct characteristics of childhood IgAN. Reframing childhood IgAN can inform guideline recommendations unique to childhood IgAN, the development of targeted therapies, and clinical trial design.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Sources of information: &lt;/strong&gt;Medline and Embase were searched for reports on children and adults with IgAN published between January 2013 and December 2023 (updated May 2024). The search was not restricted by age group, outcomes reported, language, or study design. Randomized controlled trials (RCTs), observational studies, review articles, and nephrology conference abstracts were included. A total of 3104 reports were retrieved. Forty-seven reports (37 primary studies and 10 reviews) were included in the review. Two RCTs and 35 observational studies included a total of 45 085 participants (9223 children and 35 862 adults).&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Method: &lt;/strong&gt;Data were extracted for primary IgAN and not for IgA vasculitis-associated nephritis. Findings were described with no statistical comparisons due to variations in interventions and outcome definitions.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Key findings: &lt;/strong&gt;Gross hematuria was the obvious clinical difference between childhood IgAN and adult (60-88% vs 15-20%). Nephrotic syndrome was more common in children, approaching up to 44%, while &lt;18% of adults had nephrotic syndrome. Children were biopsied sooner (6 vs 15 months) and had more inflammatory kidney lesions (mesangial hypercellularity: 41-82% vs 38-64%; endocapillary hypercellularity: 39-58% vs 17-34%). Chronic kidney lesions were more prevalent in adults (segmental sclerosis: 62-77% vs 8-51%; interstitial fibrosis/tubular atrophy: 34-37% vs 1-18%). The use of immunosuppressive therapy was higher in children (46-84% vs 35-56%). Children were started on immunosuppressive therapy sooner than adults. Adults were more likely to be optimized with renin-angiotensin system inhibitors (87-94% vs 49-75%). Children had better kidney function than adults at diagnosis (estimated glomerular filtration rate of 90-128 vs 50-88 ml/min/1.73 m&lt;sup&gt;2&lt;/sup&gt;), and children also had better kidney survival, with kidney failure of 3.1% vs 13.4% at 5 years. Children had more risk alleles for IgAN and higher levels of mannose-binding lectin than adults.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Limitations: &lt;/strong&gt;Most studies were retrospective and observational, with limited data on children and disease mechanisms. Data were not pooled for analysis because of important differences in definitions and measurements of baseline ","PeriodicalId":9426,"journal":{"name":"Canadian Journal of Kidney Health and Disease","volume":"12 ","pages":"20543581251322571"},"PeriodicalIF":1.6,"publicationDate":"2025-03-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11898040/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143613500","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Impact of Baseline Kidney Function on the Rate of Progressive Kidney Disease After Pregnancy: A Population-Based Cohort Study Research Protocol. 基线肾功能对妊娠后肾脏疾病进展率的影响:基于人群的队列研究方案
IF 1.6 Q3 UROLOGY & NEPHROLOGY Pub Date : 2025-02-28 eCollection Date: 2025-01-01 DOI: 10.1177/20543581251318836
Lavanya Bathini, Nivethika Jeyakumar, Jessica Sontrop, Eric McArthur, Yuguang Kang, Bin Luo, Aminu Bello, David Collister, Sofia Ahmed, Padma Kaul, Erik Youngson, Branko Braam, Nir Melamed, Michelle Hladunewich, Amit X Garg

Background: Better data are necessary to determine whether baseline level of kidney function affects the rate of progressive kidney disease following pregnancy.

Objective: The objective was to determine whether the baseline (pre-pregnancy) estimated glomerular filtration rate (eGFR) modifies the association between becoming pregnant and the subsequent rate of progressive kidney disease.

Design: Population-based cohort study using provincial administrative health care databases in Ontario and Alberta, Canada.

Setting: The sample will be accrued from April 1, 2007, to March 31, 2023, in Ontario and from April 1, 2012, to March 31, 2023, in Alberta. Follow-up for study outcomes will occur until March 31, 2024.

Participants: The pregnant group will include adult female residents of Ontario or Alberta with a record of a pregnancy of 20 to 46 weeks' gestation during the accrual period, and the non-pregnant group will include adult female residents with no prior record of pregnancy. The cohort entry dates in those in the pregnant group will be the estimated date of conception; the entry dates for those in the non-pregnant group will be randomly assigned following the distribution of dates in the pregnant group. To be eligible, individuals must be between 18 and 45 years old at cohort entry. They require at least 1 serum creatinine measurement within 2 years before entry and should not have received maintenance dialysis or a prior kidney transplant. Both groups will be categorized into one of 3 levels of baseline eGFR (≥60, 45-59, and <45 mL/min per 1.73 m2). Inverse probability of treatment weighting on a propensity score will be used to balance the pregnant and non-pregnant groups on baseline characteristics (including age, proteinuria, hypertension, and diabetes) within the 3 categories of baseline eGFR.

Measurements: The primary outcome, progressive kidney disease, will be defined as a composite of a persistent ≥40% drop in eGFR from the baseline value, a new persistent eGFR <15 mL/min per 1.73 m2, receipt of maintenance dialysis, or receipt of a kidney transplant. The secondary outcomes will be the components of the primary composite outcome examined separately and the annualized change in eGFR in mL/min per 1.73 m2 from baseline.

Methods: We will test for statistical interaction to determine whether the baseline category of eGFR modifies the rate of long-term progressive kidney disease after pregnancy. We hypothesize that a statistical interaction will be present. We will present weighted cause-specific hazard ratios (HRs) and cumulative incidence function (CIF) curves for up to 10 years of follow-up for the pregnant and non-pregnant groups stratified by each eGFR category. We will perform additional pre-specified analyses to confirm whether the findings are ro

背景:需要更好的数据来确定基线肾功能水平是否影响妊娠后肾脏疾病的进展率。目的:目的是确定基线(孕前)估计肾小球滤过率(eGFR)是否改变怀孕与随后进展性肾病发生率之间的关系。设计:基于人群的队列研究,使用加拿大安大略省和阿尔伯塔省的省级行政卫生保健数据库。设置:安大略省的采样时间为2007年4月1日至2023年3月31日,阿尔伯塔省的采样时间为2012年4月1日至2023年3月31日。研究结果的随访将持续到2024年3月31日。参与者:怀孕组将包括安大略省或阿尔伯塔省的成年女性居民,在应计期间怀孕20至46周,非怀孕组将包括没有怀孕记录的成年女性居民。怀孕组的队列入组日期为估计受孕日期;未怀孕组的入组日期将按照怀孕组的日期分布随机分配。要符合资格,个人必须在18至45岁之间在队列进入。他们需要在入院前2年内至少进行一次血清肌酐测量,并且不应该接受维持性透析或先前的肾移植。两组将被分为基线eGFR 3个水平(≥60、45-59和2)中的一个。倾向评分上的治疗加权逆概率将用于平衡妊娠组和非妊娠组在基线eGFR 3个类别中的基线特征(包括年龄、蛋白尿、高血压和糖尿病)。测量:主要终点,进展性肾脏疾病,将被定义为eGFR从基线值持续下降≥40%,新的持续eGFR 2,接受维护性透析或接受肾移植的复合。次要结果将是单独检查的主要复合结果的组成部分,以及eGFR从基线到mL/min / 1.73 m2的年化变化。方法:我们将检验统计相互作用,以确定eGFR的基线类别是否会改变妊娠后长期进展性肾脏疾病的发生率。我们假设将存在统计上的相互作用。我们将对妊娠组和非妊娠组进行为期10年的加权原因特异性风险比(hr)和累积发生率函数(CIF)曲线,并对每个eGFR类别进行分层。我们将进行额外的预先指定的分析,以确认这些发现是否可靠,并检查基线蛋白尿的关联。结果:基于安大略省ICES数据的可行性分析,我们预计该队列将包括超过40万怀孕女性和120万非怀孕女性。这包括至少395,000名基线eGFR≥60 mL/min/1.73 m2的孕妇,300名eGFR为45至59 mL/min/1.73 m2, 110名eGFR为2。中位随访预计为5年(范围= 1-17年),随访损失最小。局限性:肾功能测量将作为常规护理的一部分(不根据研究计划)。常规护理数据中经常缺少基线蛋白尿的测量,即使在高达15%的eGFR 2患者中也是如此。结论:本研究将探讨基线eGFR水平是否会改变妊娠后进展性肾病的发生率,并通过3种基线eGFR估计妊娠和非妊娠女性进展性肾病的累积发生率。
{"title":"Impact of Baseline Kidney Function on the Rate of Progressive Kidney Disease After Pregnancy: A Population-Based Cohort Study Research Protocol.","authors":"Lavanya Bathini, Nivethika Jeyakumar, Jessica Sontrop, Eric McArthur, Yuguang Kang, Bin Luo, Aminu Bello, David Collister, Sofia Ahmed, Padma Kaul, Erik Youngson, Branko Braam, Nir Melamed, Michelle Hladunewich, Amit X Garg","doi":"10.1177/20543581251318836","DOIUrl":"https://doi.org/10.1177/20543581251318836","url":null,"abstract":"<p><strong>Background: </strong>Better data are necessary to determine whether baseline level of kidney function affects the rate of progressive kidney disease following pregnancy.</p><p><strong>Objective: </strong>The objective was to determine whether the baseline (pre-pregnancy) estimated glomerular filtration rate (eGFR) modifies the association between becoming pregnant and the subsequent rate of progressive kidney disease.</p><p><strong>Design: </strong>Population-based cohort study using provincial administrative health care databases in Ontario and Alberta, Canada.</p><p><strong>Setting: </strong>The sample will be accrued from April 1, 2007, to March 31, 2023, in Ontario and from April 1, 2012, to March 31, 2023, in Alberta. Follow-up for study outcomes will occur until March 31, 2024.</p><p><strong>Participants: </strong>The pregnant group will include adult female residents of Ontario or Alberta with a record of a pregnancy of 20 to 46 weeks' gestation during the accrual period, and the non-pregnant group will include adult female residents with no prior record of pregnancy. The cohort entry dates in those in the pregnant group will be the estimated date of conception; the entry dates for those in the non-pregnant group will be randomly assigned following the distribution of dates in the pregnant group. To be eligible, individuals must be between 18 and 45 years old at cohort entry. They require at least 1 serum creatinine measurement within 2 years before entry and should not have received maintenance dialysis or a prior kidney transplant. Both groups will be categorized into one of 3 levels of baseline eGFR (≥60, 45-59, and <45 mL/min per 1.73 m<sup>2</sup>). Inverse probability of treatment weighting on a propensity score will be used to balance the pregnant and non-pregnant groups on baseline characteristics (including age, proteinuria, hypertension, and diabetes) within the 3 categories of baseline eGFR.</p><p><strong>Measurements: </strong>The primary outcome, progressive kidney disease, will be defined as a composite of a persistent ≥40% drop in eGFR from the baseline value, a new persistent eGFR <15 mL/min per 1.73 m<sup>2</sup>, receipt of maintenance dialysis, or receipt of a kidney transplant. The secondary outcomes will be the components of the primary composite outcome examined separately and the annualized change in eGFR in mL/min per 1.73 m<sup>2</sup> from baseline.</p><p><strong>Methods: </strong>We will test for statistical interaction to determine whether the baseline category of eGFR modifies the rate of long-term progressive kidney disease after pregnancy. We hypothesize that a statistical interaction will be present. We will present weighted cause-specific hazard ratios (HRs) and cumulative incidence function (CIF) curves for up to 10 years of follow-up for the pregnant and non-pregnant groups stratified by each eGFR category. We will perform additional pre-specified analyses to confirm whether the findings are ro","PeriodicalId":9426,"journal":{"name":"Canadian Journal of Kidney Health and Disease","volume":"12 ","pages":"20543581251318836"},"PeriodicalIF":1.6,"publicationDate":"2025-02-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11869263/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143540377","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Shared Decision-Making Aid for Stroke-Prevention Strategies in Patients With Atrial Fibrillation Receiving Maintenance Hemodialysis (SIMPLIFY-HD): A Mixed-Methods Study. 房颤维持性血液透析患者卒中预防策略的共享决策辅助(simplified - hd):一项混合方法研究
IF 1.6 Q3 UROLOGY & NEPHROLOGY Pub Date : 2025-02-21 eCollection Date: 2025-01-01 DOI: 10.1177/20543581241311077
Olivier Massé, Noémie Maurice, Yu Hong, Claudia Mercurio, Catherine Tremblay, Lysane Senécal, Amélie Bernier-Jean, Nicolas Dugré, Gabriel Dallaire
<p><strong>Background: </strong>Recent atrial fibrillation guidelines recommend shared decision-making between clinicians and patients when choosing stroke-prevention therapies. Although decision aids improve patients' knowledge and decisional conflicts, there is no decision aid for stroke-prevention strategies in people with atrial fibrillation receiving hemodialysis.</p><p><strong>Objective: </strong>The objective was to develop and field test the first decision aid for Atrial Fibrillation in HemoDialysis (AFHD-DA) for stroke prevention in atrial fibrillation and hemodialysis.</p><p><strong>Design: </strong>This is a sequential 3-phase mixed-methods study following the International Patient Decision Aid Standards and the Ottawa Decision Support Framework.</p><p><strong>Setting: </strong>This study was conducted in 2 ambulatory hemodialysis centers in Montreal and Laval (Canada).</p><p><strong>Participants: </strong>Adults with atrial fibrillation receiving hemodialysis and clinicians (physicians, pharmacists, or nurse practitioners) involved in their care.</p><p><strong>Methods: </strong>In phase 1, we conducted systematic and 2 rapid reviews and formed the steering committee to pilot the first version of AFHD-DA. In phase 2, we refined the AFHD-DA through 4 rounds of focus groups and interviews, using a qualitative analysis of transcripts and a descriptive analysis of acceptability and usability scores. In phase 3, we field-tested the decision aid during 16 simulated clinical consultations. We assessed decisional conflict and patient knowledge using before-and-after paired <i>t</i>-tests and compared the proportion of patients with high decisional conflict using McNemar's test. We used the Ottawa Hospital preparation for decision-making scale and participants' feedback to evaluate how AFHD-DA facilitated shared decision-making.</p><p><strong>Results: </strong>We enrolled 8 patients and 10 clinicians in phase 2. The predefined usability and acceptability thresholds (68 and 66, respectively) were reached. Theme saturation was achieved in the fourth round of focus groups and interviews. Four major themes emerged: acceptability, usability, decision-making process, and scientific value of the decision aid. Sixteen patients and 10 clinicians field-tested the decision aid in phase 3. In clinical settings, AFHD-DA significantly decreased the mean decisional conflict score from 41.0 to 13.6 (<i>P</i> < .001) and the proportion of patients with decisional conflicts from 81.3 to 18.8% (<i>P</i> = .002). It improved the patients' mean knowledge score from 62.7 to 76.6 (<i>P</i> = .001), and 81% of patients and 90% of clinicians felt highly prepared for decision-making. Clinical consultations lasted, on average, 21 minutes (standard deviation = 8).</p><p><strong>Limitations: </strong>The main limitations were the low quality of existing literature, the small number of participants, and the absence of a control group.</p><p><strong>Conclusions: </strong>The
背景:最近的房颤指南建议临床医生和患者在选择卒中预防治疗时共同决策。虽然决策辅助提高了患者的知识和决策冲突,但对接受血液透析的房颤患者的卒中预防策略没有决策辅助。目的:目的是开发和现场测试房颤血液透析第一决策辅助(AFHD-DA)预防房颤和血液透析卒中。设计:这是一项连续的3期混合方法研究,遵循国际患者决策辅助标准和渥太华决策支持框架。环境:本研究在加拿大蒙特利尔和拉瓦尔的2个流动血液透析中心进行。参与者:接受血液透析的房颤成人和临床医生(医生、药剂师或执业护士)参与他们的护理。方法:在第一阶段,我们进行了系统和两次快速审查,并成立了指导委员会,对第一版AFHD-DA进行试点。在第二阶段,我们通过4轮焦点小组和访谈完善了AFHD-DA,使用了对成绩单的定性分析和对可接受性和可用性评分的描述性分析。在第三阶段,我们在16次模拟临床咨询中对决策辅助进行了现场测试。我们使用前后配对t检验评估决策冲突和患者知识,并使用McNemar检验比较具有高决策冲突的患者比例。我们使用渥太华医院准备的决策量表和参与者的反馈来评估AFHD-DA如何促进共同决策。结果:我们在第二阶段招募了8名患者和10名临床医生。达到了预定义的可用性和可接受性阈值(分别为68和66)。在第四轮焦点小组和访谈中达到主题饱和。出现了四个主要主题:可接受性、可用性、决策过程和决策辅助的科学价值。在第三阶段,16名患者和10名临床医生对决策辅助进行了现场测试。在临床环境中,AFHD-DA显著降低了决策冲突的平均得分,从41.0分降至13.6分(P < 0.001),决策冲突的患者比例从81.3%降至18.8% (P = 0.002)。患者的平均知识得分从62.7分提高到76.6分(P = 0.001), 81%的患者和90%的临床医生对决策做好了充分的准备。临床咨询平均持续时间为21分钟(标准差= 8)。局限性:主要局限性是现有文献质量低,参与者人数少,缺乏对照组。结论:决策辅助促进了临床医生和患者之间的分时共享决策,提高了患者的知识,并减少了房颤接受血液透析患者在选择卒中预防策略方面的决策冲突。
{"title":"Shared Decision-Making Aid for Stroke-Prevention Strategies in Patients With Atrial Fibrillation Receiving Maintenance Hemodialysis (SIMPLIFY-HD): A Mixed-Methods Study.","authors":"Olivier Massé, Noémie Maurice, Yu Hong, Claudia Mercurio, Catherine Tremblay, Lysane Senécal, Amélie Bernier-Jean, Nicolas Dugré, Gabriel Dallaire","doi":"10.1177/20543581241311077","DOIUrl":"10.1177/20543581241311077","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Background: &lt;/strong&gt;Recent atrial fibrillation guidelines recommend shared decision-making between clinicians and patients when choosing stroke-prevention therapies. Although decision aids improve patients' knowledge and decisional conflicts, there is no decision aid for stroke-prevention strategies in people with atrial fibrillation receiving hemodialysis.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Objective: &lt;/strong&gt;The objective was to develop and field test the first decision aid for Atrial Fibrillation in HemoDialysis (AFHD-DA) for stroke prevention in atrial fibrillation and hemodialysis.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Design: &lt;/strong&gt;This is a sequential 3-phase mixed-methods study following the International Patient Decision Aid Standards and the Ottawa Decision Support Framework.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Setting: &lt;/strong&gt;This study was conducted in 2 ambulatory hemodialysis centers in Montreal and Laval (Canada).&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Participants: &lt;/strong&gt;Adults with atrial fibrillation receiving hemodialysis and clinicians (physicians, pharmacists, or nurse practitioners) involved in their care.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Methods: &lt;/strong&gt;In phase 1, we conducted systematic and 2 rapid reviews and formed the steering committee to pilot the first version of AFHD-DA. In phase 2, we refined the AFHD-DA through 4 rounds of focus groups and interviews, using a qualitative analysis of transcripts and a descriptive analysis of acceptability and usability scores. In phase 3, we field-tested the decision aid during 16 simulated clinical consultations. We assessed decisional conflict and patient knowledge using before-and-after paired &lt;i&gt;t&lt;/i&gt;-tests and compared the proportion of patients with high decisional conflict using McNemar's test. We used the Ottawa Hospital preparation for decision-making scale and participants' feedback to evaluate how AFHD-DA facilitated shared decision-making.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results: &lt;/strong&gt;We enrolled 8 patients and 10 clinicians in phase 2. The predefined usability and acceptability thresholds (68 and 66, respectively) were reached. Theme saturation was achieved in the fourth round of focus groups and interviews. Four major themes emerged: acceptability, usability, decision-making process, and scientific value of the decision aid. Sixteen patients and 10 clinicians field-tested the decision aid in phase 3. In clinical settings, AFHD-DA significantly decreased the mean decisional conflict score from 41.0 to 13.6 (&lt;i&gt;P&lt;/i&gt; &lt; .001) and the proportion of patients with decisional conflicts from 81.3 to 18.8% (&lt;i&gt;P&lt;/i&gt; = .002). It improved the patients' mean knowledge score from 62.7 to 76.6 (&lt;i&gt;P&lt;/i&gt; = .001), and 81% of patients and 90% of clinicians felt highly prepared for decision-making. Clinical consultations lasted, on average, 21 minutes (standard deviation = 8).&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Limitations: &lt;/strong&gt;The main limitations were the low quality of existing literature, the small number of participants, and the absence of a control group.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusions: &lt;/strong&gt;The","PeriodicalId":9426,"journal":{"name":"Canadian Journal of Kidney Health and Disease","volume":"12 ","pages":"20543581241311077"},"PeriodicalIF":1.6,"publicationDate":"2025-02-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11843691/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143482100","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Clinical and Pathological Characteristics of Non-AL Amyloidosis MGRS: A Single-Center Experience Over 10 Years. 非al淀粉样变的临床和病理特征:10年的单中心经验。
IF 1.6 Q3 UROLOGY & NEPHROLOGY Pub Date : 2025-02-21 eCollection Date: 2025-01-01 DOI: 10.1177/20543581251318830
Chunpeng Nie, Holly Lee, Kim Cheema, Peter Duggan, Sylvia McCulloch, Jason Tay, Paola Neri, Nizar J Bahlis, Victor H Jimenez-Zepeda

Objective: Monoclonal gammopathy of renal significance (MGRS) is a heterogeneous and relatively recently defined disorder that encompasses many kidney and hematologic pathologies. MGRS remains a rare disease and there is a need for more literature regarding its treatment and outcomes. In this study, we share our center's experience with MGRS including incidence of different kidney pathologies, clone type, kidney and hematologic response, and progression-free survival.

Methods: Data from 35 patients diagnosed with MGRS excluding light-chain amyloidosis between 2013 and 2022 at a single Canadian tertiary care center were retrospectively analyzed. All cases required kidney biopsy. Initial treatment included regimens containing bortezomib, rituximab, or cyclosporine, or steroids only. Parameters studied included incidence of different kidney pathologies, clone type, depth of hematologic response, kidney survival (KS), overall survival (OS), and progression-free survival (PFS).

Results: Out of 35 patients, there were 10 cases of monoclonal immunoglobulin deposition disease, 8 of proliferative glomerulonephritis with immune deposits, 5 of microtubular immune deposits including immunotactoid and types 1 and 2 cryoglobulinemic nephropathy, 3 of C3 glomerulonephritis, and 9 of other diagnoses. There were 21 cases with a plasma cell clone identified in bone marrow, 2 each of B cell and low-grade lymphoma, 1 atypical T cell clone, and 9 cases without an expanded clone on bone marrow biopsy. A total of 6 patients required kidney replacement therapy and 4 patients died; the median PFS was 59.3 months. Very good partial hematologic response or better was significantly associated with decreased proteinuria but not preserved eGFR. There was a non-significant trend toward better PFS with hematologic response.

Conclusion: Our experience confirms that MGRS is a heterogeneous disease and adds to the literature concerning the diagnosis and treatment of MGRS. Successful treatment of the underlying hematologic disorder with targeted therapy is more likely to lead to an improvement in kidney function.

目的:单克隆肾性伽玛病(MGRS)是一种异质性和相对较新定义的疾病,包括许多肾脏和血液病理。MGRS仍然是一种罕见的疾病,需要更多关于其治疗和结果的文献。在这项研究中,我们分享了我们中心在MGRS方面的经验,包括不同肾脏病理的发生率,克隆类型,肾脏和血液反应,以及无进展生存。方法:回顾性分析2013年至2022年在加拿大一家三级医疗中心诊断为MGRS(不包括轻链淀粉样变)的35例患者的数据。所有病例均行肾活检。初始治疗方案包括硼替佐米、利妥昔单抗或环孢素,或仅使用类固醇。研究参数包括不同肾脏病理的发生率、克隆类型、血液学反应深度、肾脏生存(KS)、总生存(OS)和无进展生存(PFS)。结果:本组35例患者中,单克隆性免疫球蛋白沉积病10例,增生性肾小球肾炎伴免疫沉积8例,微管性免疫沉积包括免疫因子样及1、2型冷球蛋白血症肾病5例,C3型肾小球肾炎3例,其他9例。骨髓活检发现浆细胞克隆21例,B细胞和低级别淋巴瘤各2例,非典型T细胞克隆1例,无扩增克隆9例。6例患者需要肾脏替代治疗,4例患者死亡;中位PFS为59.3个月。非常好的部分血液学反应或更好与蛋白尿减少显著相关,但与eGFR保存无关。随着血液学反应的改善,PFS有不显著的趋势。结论:我们的经验证实了MGRS是一种异质性疾病,并增加了有关MGRS诊断和治疗的文献。通过靶向治疗成功地治疗潜在的血液系统疾病更有可能导致肾功能的改善。
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引用次数: 0
Cultivating Innovative, Pragmatic, Randomized Controlled Registry Trials Embedded in Hemodialysis Care: Conference Proceeding From Gardener's Grove 2023. 在血液透析护理中培养创新、实用、随机对照注册试验:2023年加德纳格罗夫会议纪要。
IF 1.6 Q3 UROLOGY & NEPHROLOGY Pub Date : 2025-02-14 eCollection Date: 2025-01-01 DOI: 10.1177/20543581251318442
Bryn Tannar, Patricia Olar, Shane Kilburn, Kathleen Brown-Blake, Ahmed A Al-Jaishi, Peter G Blake, Kristin K Clemens, Charles Cook, Laura M Dember, Stephanie N Dixon, Cory E Goldstein, Areef Ishani, Catherine Joyes, Conor Judge, James C Kaufman, Susan Q Mackenzie, Taylor McLinden, Amber O Molnar, Alicia Murdoch, Gihad Nesrallah, Sanjay Pandeya, Claudio Rigatto, Pavel S Roshanov, Melissa Schorr, Samuel A Silver, Rona M Smith, Leanne Stalker, Navdeep Tangri, Monica Taljaard, Karthik K Tennankore, Hans Vorster, Charles Weijer, Myles Wolf, Merrick Zwarenstein, Amit X Garg

Purpose of the conference: Hemodialysis is a life-sustaining treatment for patients with end-stage kidney disease. However, patients on dialysis continue to face poor quality of life and short life expectancies. Despite this, the nephrology community conducts the fewest randomized controlled trials of any medical discipline, relying instead on expert opinion to guide many aspects of hemodialysis care. There is a need to conduct high-quality pragmatic randomized controlled trials in hemodialysis to drive evidence-based practice. To this end, the Innovative Clinical Trials in Hemodialysis Centers initiative, with the support of the Canadian Institutes of Health Research and its Strategy for Patient-Oriented Research, funded the development of 6 pragmatic trial protocols. Gardener's Grove 2023 created a space to support the development of these trials and increase awareness and knowledge of past, ongoing, and future innovative, pragmatic, randomized controlled registry trials embedded in routine hemodialysis care. This report summarizes the proceedings of this conference.

Sources of information: The conference included 6 panel presentations, each featuring an overview of a new pragmatic trial followed by expert panel feedback from patient partners, nephrologists, researchers, and health care providers. The conference also included 10 educational sessions led by clinicians and researchers with experience in the fields of kidney medicine and clinical trials.

Methods: Gardener's Grove 2023 was a 4-day virtual conference held in March of 2023. Recordings of all the conference presentations were later published on the Gardener's Grove website and are summarized in the Supplemental Appendix of this report.

Key findings: The conference brought together 118 Canadian and international researchers, patients, and health care providers to collaborate on 6 pragmatic trials intended to test interventions to improve hemodialysis care. The proposed trials included (1) PREventing FracturEs in REnal Disease (PREFERRED), (2) DIALysis with EXpanded solute removal (DIALEX), (3) Sodium fOr diaLysis oUTcome rEduction (SOLUTE), (4) Finding the right blood pressure target for patients on dialysis, (5) DIuretic Use in patients with Residual renal function on hemodialysis (DIURESED), and (6) Lower vs higher dialysate bicarbonate concentration in patients receiving hemodialysis (Dial-Bicarb). All of these interventions were widely supported and received valuable feedback from panelists and conference attendees. The education sessions focused on various design and execution elements of pragmatic, randomized controlled registry trials embedded in routine care.

Limitations: The conference could have been improved by streamlining session topics and pacing, allowing additional time for discussions, strengthening online network opportunities, and improving survey response

会议目的:血液透析是终末期肾病患者的一种维持生命的治疗方法。然而,接受透析治疗的患者仍然面临着生活质量差和预期寿命短的问题。尽管如此,肾脏病学界进行的随机对照试验是所有医学学科中最少的,而是依靠专家意见来指导血液透析护理的许多方面。有必要在血液透析中进行高质量的实用随机对照试验,以推动循证实践。为此,在加拿大卫生研究院及其面向患者的研究战略的支持下,血液透析中心创新临床试验倡议资助制定了6项实用试验方案。Gardener's Grove 2023为支持这些试验的发展创造了空间,并提高了对常规血液透析护理中过去、正在进行和未来创新、实用、随机对照注册试验的认识和了解。这份报告概述了这次会议的会议记录。信息来源:会议包括6个小组报告,每个小组报告都概述了一项新的实用试验,然后是来自患者伴侣、肾病学家、研究人员和卫生保健提供者的专家小组反馈。会议还包括由具有肾脏医学和临床试验领域经验的临床医生和研究人员领导的10个教育会议。方法:Gardener's Grove 2023是于2023年3月召开的为期4天的虚拟会议。所有会议演讲的录音后来都发布在园丁园网站上,并在本报告的补充附录中进行了总结。主要发现:会议汇集了118名加拿大和国际研究人员、患者和卫生保健提供者,就6项旨在测试干预措施以改善血液透析护理的实用试验进行合作。拟议的试验包括(1)预防肾脏疾病骨折(PREFERRED),(2)扩展溶质去除透析(DIALEX),(3)降低透析结局的钠(solute),(4)为透析患者找到合适的血压目标,(5)利尿剂在血液透析中残余肾功能患者中的使用(DIURESED),以及(6)血液透析患者中较低和较高的透析液碳酸氢盐浓度(Dial-Bicarb)。所有这些干预措施都得到了小组成员和与会者的广泛支持和宝贵反馈。教育课程侧重于日常护理中实用的随机对照注册试验的各种设计和执行要素。限制:会议可以通过简化会议主题和节奏、允许额外的讨论时间、加强在线网络机会和提高调查回复率来改进。计划在几年内举行一次后续会议,协调员将致力于执行这些变化。启示:Gardener's Grove 2023成功地为患者、研究人员和卫生保健提供者创造了一个空间,以支持在血液透析护理中开展6项新的实用试验。自园丁格罗夫2023年以来,其中一些试验获得了CIHR资助,获得了伦理批准,并正在积极准备启动他们的干预措施。
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引用次数: 0
Chronic Benign Tubular Albuminuria From Compound Heterozygous Variants in CUBN: A Case Report. 慢性良性小管性蛋白尿由复合杂合变异体引起:1例报告。
IF 1.6 Q3 UROLOGY & NEPHROLOGY Pub Date : 2025-02-05 eCollection Date: 2025-01-01 DOI: 10.1177/20543581251317016
Adam Pietrobon, Mark D Elliott

Rationale: Albuminuria is a commonly used parameter for predicting decline in kidney filtration function. Cubilin, encoded by CUBN, is a critical protein involved in protein reabsorption in the proximal tubule. Mutations in CUBN lead to Imerslund-Gräsbeck syndrome (IGS), a disorder characterized by vitamin B12 deficiency (and consequences related to that) with or without albuminuria. Recent evidence suggests that C-terminal variants in CUBN may lead to albuminuria without other features of IGS.

Presenting concerns of the patient: Here, we report a case of a 52-year-old male with chronic, albumin-predominant, subnephrotic range proteinuria since his teenage years, but preserved estimated glomerular filtration rate (eGFR).

Interventions: Neither angiotensin-converting enzyme (ACE) inhibition nor angiotensin Type II (AT-II) receptor blockade reduced his degree of albuminuria.

Diagnosis: Genetic testing identified 3 distinct pathogenic variants in CUBN that were confirmed by segregation analysis to be a compound heterozygous mode of inheritance. All variants were downstream of the intrinsic factor-vitamin B12 binding domain of cubilin. The patient had normal vitamin B12 levels and did not exhibit any features of IGS.

Outcomes: Kidney biopsy was not pursued for this patient as diagnostic clarification was achieved by non-invasive genetic testing alone.

Novel findings: This case highlights several important lessons. First, not all albuminuria is made equal, and forms of tubular albuminuria can exist without compromising kidney filtration function. Second, identifying genetic forms of tubular albuminuria is key to avoiding ineffective interventions (eg, ACE inhibition, AT-II receptor blockade, sodium-glucose cotransporter-2 [SGLT2] inhibition) and unnecessary invasive procedures (eg, kidney biopsy). Third, the location of CUBN variants dictates phenotypic consequences, with C-terminal variants leading to albuminuria without vitamin B12 deficiency.

理由:蛋白尿是预测肾滤过功能下降的常用参数。Cubilin由CUBN编码,是参与近端小管蛋白重吸收的关键蛋白。CUBN的突变导致Imerslund-Gräsbeck综合征(IGS),这是一种以维生素B12缺乏(及其相关后果)为特征的疾病,伴有或不伴有蛋白尿。最近的证据表明,CUBN的c端变异可能导致蛋白尿,而不具有IGS的其他特征。患者的关注:在这里,我们报告一例52岁男性,从青少年时期开始患有慢性,白蛋白为主,亚肾病范围蛋白尿,但保留了估计的肾小球滤过率(eGFR)。干预措施:血管紧张素转换酶(ACE)抑制和血管紧张素II型(AT-II)受体阻断都不能降低他的蛋白尿程度。诊断:基因检测在CUBN中发现3种不同的致病变异,通过分离分析证实为复合杂合遗传模式。所有变异都位于cubilin的内在因子-维生素B12结合域的下游。患者维生素B12水平正常,未表现出IGS的任何特征。结果:该患者未进行肾活检,因为仅通过非侵入性基因检测即可获得诊断澄清。新发现:这个案例突出了几个重要的教训。首先,不是所有的蛋白尿都是一样的,管状蛋白尿的形式可以存在而不损害肾脏过滤功能。其次,确定小管性蛋白尿的遗传形式是避免无效干预(如ACE抑制、AT-II受体阻断、钠-葡萄糖共转运体-2 [SGLT2]抑制)和不必要的侵入性手术(如肾活检)的关键。第三,CUBN变异的位置决定了表型后果,c端变异导致无维生素B12缺乏症的蛋白尿。
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引用次数: 0
期刊
Canadian Journal of Kidney Health and Disease
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