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Acquired cystinuria in a kidney transplant recipient. 肾移植受者获得性胱氨酸尿症。
IF 4.6 2区 医学 Q1 UROLOGY & NEPHROLOGY Pub Date : 2025-11-06 eCollection Date: 2025-12-01 DOI: 10.1093/ckj/sfaf330
Cécile Martin, Stessy Kutchukian, Laure Ecotière, Héloïse Ducousso, Antoine Thierry, Bertrand Knebelmann, Frank Bridoux

Cystinuria is a rare autosomal recessive hereditary disorder characterized by increased urine cystine excretion and recurrent kidney stone formation. Acquired cystinuria after kidney transplantation is extremely rare, with, to our knowledge, a single case described in the literature, albeit without genetic explorations. We herein report a 59-year-old male kidney transplant recipient, without history of urolithiasis, who developed cystine stones in the allograft. Genetic studies revealed a homozygous pathogenic mutation in the SLC3A1 gene in the deceased donor. This case demonstrates the potential for transmission of cystinuria through the kidney allograft and underlines the value of genetic analysis in the donor.

胱氨酸尿症是一种罕见的常染色体隐性遗传疾病,其特征是尿中胱氨酸排泄增加和肾结石复发。肾移植后获得性胱氨酸尿是极其罕见的,据我们所知,文献中只有一例,尽管没有基因探索。我们在此报告一位59岁男性肾移植受者,无尿石症病史,在同种异体移植中出现胱氨酸结石。遗传学研究显示,在已故供者的SLC3A1基因中存在纯合致病性突变。该病例显示了胱氨酸尿通过移植肾传播的可能性,并强调了供体遗传分析的价值。
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引用次数: 0
Calciphylaxis diagnosis, management and future directions: a comprehensive update on behalf of the European Renal Association CKD-MBD Working Group. 钙化反应诊断、管理和未来方向:代表欧洲肾脏协会CKD-MBD工作组的全面更新。
IF 4.6 2区 医学 Q1 UROLOGY & NEPHROLOGY Pub Date : 2025-11-06 eCollection Date: 2025-12-01 DOI: 10.1093/ckj/sfaf338
Sharon Huish, Sacha Moore, Carlo Alfieri, Antonio Bellasi, Daniel Cejka, Martin H de Borst, Juan Miguel Diaz-Tocados, Pietro Manuel Ferraro, Maria Fusaro, Mathias Haarhaus, Ditte Hansen, Mehmet Kanbay, Markus Ketteler, Smeeta Sinha

Calciphylaxis, or calcific uraemic arteriolopathy, is a rare and life-threatening condition predominantly affecting people receiving dialysis. Characterized by painful necrotic skin lesions due to arteriolar calcification and thrombosis, calciphylaxis is associated with high morbidity and mortality. Diagnosis is frequently delayed due to misdiagnosis and an absence of specific diagnostic tests. Current treatment approaches are largely based on registry data and small uncontrolled studies. This update brings together the latest understanding of calciphylaxis pathogenesis, diagnostic approaches and management, highlighting recent advances and future directions. Pathophysiological mechanisms include vascular smooth muscle cell osteogenic transformation, loss of endogenous calcification inhibitors (fetuin-A, matrix Gla protein, pyrophosphate), systemic inflammation and thrombosis. The potential prognostic role of biomarkers, including the calciprotein particle crystallization test (T50) and plasma pyrophosphate, are also discussed. Management remains complex, with no proven treatments. A multifaceted, and multi-professional team approach is fundamental. Sodium thiosulfate remains widely used despite the lack of trial evidence. Recent investigational therapies, including SNF472 and INZ-701, target key calcification pathways and offer promise. The Better Evidence and Translation for Calciphylaxis (BEAT-Calci) adaptive platform trial represents a landmark step in evaluating multiple therapies systematically. National registries remain vital for informing prevalence estimates and improving real-world outcome data. Looking ahead, future research should prioritize the development and validation of diagnostic criteria, and prognostic tools integrating clinical risk factors with biomarkers. In addition, we propose the routine inclusion of patient-reported experience measures in calciphylaxis studies to better capture treatment impact in this vulnerable population.

钙化性尿毒性动脉病是一种罕见的危及生命的疾病,主要影响透析患者。以动脉钙化和血栓形成引起的皮肤疼痛坏死为特征,钙化反应具有高发病率和死亡率。由于误诊和缺乏特定的诊断测试,诊断经常被延误。目前的治疗方法主要基于注册数据和小型非对照研究。该更新汇集了对钙化反应发病机制、诊断方法和管理的最新了解,突出了最近的进展和未来的方向。病理生理机制包括血管平滑肌细胞成骨转化、内源性钙化抑制剂(胎蛋白a、基质Gla蛋白、焦磷酸盐)的丧失、全身性炎症和血栓形成。生物标志物的潜在预后作用,包括钙蛋白颗粒结晶试验(T50)和血浆焦磷酸盐,也进行了讨论。管理仍然很复杂,没有行之有效的治疗方法。一个多方面、多专业的团队方法是基本的。尽管缺乏试验证据,硫代硫酸钠仍被广泛使用。最近的研究疗法,包括SNF472和INZ-701,靶向关键的钙化途径,并提供了希望。更好的钙化治疗证据和翻译(BEAT-Calci)适应性平台试验是系统评估多种疗法的里程碑式的一步。国家登记对于通报流行率估计和改进实际结果数据仍然至关重要。展望未来,未来的研究应优先开发和验证诊断标准,以及将临床危险因素与生物标志物结合起来的预后工具。此外,我们建议在钙化反应研究中常规纳入患者报告的经验措施,以更好地捕捉治疗对这一弱势群体的影响。
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引用次数: 0
Implementing a model of integrated CKD management between primary and secondary care. 在初级和二级医疗之间实施CKD综合管理模式。
IF 4.6 2区 医学 Q1 UROLOGY & NEPHROLOGY Pub Date : 2025-11-04 eCollection Date: 2026-01-01 DOI: 10.1093/ckj/sfaf335
Philippa Jones, Hannah O'Keeffe, Rupert W Major, James Ritchie, Nil Sanganee, Smeeta Sinha, James O Burton

Chronic kidney disease (CKD) is a common condition and important cardiovascular risk factor. However, CKD remains underdiagnosed and evidence-based medicines underutilized. In most healthcare systems, most CKD is managed in primary care. Optimal management in this setting can only be achieved with integration of care including early identification, prioritization, and use of the tools and skill mix available. This narrative review focuses on the importance of screening and identification in primary care, looking at innovative solutions and methods from other long-term conditions, particularly cardio-renal-metabolic conditions. Integrated care virtual multidisciplinary reviews, have demonstrated clinical and economic benefits, improved medication optimization, and reduced unnecessary referrals. However, implementation remains inconsistent, and prescribing of both established and novel therapies remains sub-optimal. Optimizing CKD care requires a system-wide approach that reinforces primary-secondary care collaboration, prioritizes early detection, and facilitates timely, evidence-based interventions. The inclusion of urine albumin: creatinine ratio testing, integrated digital tools, and shared accountability frameworks must be urgently adopted to realize improved outcomes and reduce the burden of CKD on individuals and healthcare systems alike.

慢性肾脏疾病(CKD)是一种常见疾病,也是重要的心血管危险因素。然而,慢性肾病仍未得到充分诊断,循证药物也未得到充分利用。在大多数医疗保健系统中,大多数CKD是在初级保健中管理的。在这种情况下,只有通过整合护理,包括早期识别、优先排序和使用现有工具和技能组合,才能实现最佳管理。这篇叙述性综述的重点是筛查和识别在初级保健中的重要性,从其他长期疾病,特别是心肾代谢疾病中寻找创新的解决方案和方法。综合护理虚拟多学科审查,已经证明了临床和经济效益,改善药物优化,并减少不必要的转诊。然而,实施仍然不一致,既定和新疗法的处方仍然不是最佳的。优化CKD护理需要一种全系统的方法,加强初级和二级护理合作,优先考虑早期发现,并促进及时的循证干预。必须立即采用尿白蛋白:肌酐比值检测、集成的数字工具和共享的责任框架,以实现改善的结果,减轻CKD对个人和医疗系统的负担。
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引用次数: 0
Association between serum interleukin 8 levels and cardiovascular events in patients with chronic kidney disease. 慢性肾病患者血清白细胞介素8水平与心血管事件的关系
IF 4.6 2区 医学 Q1 UROLOGY & NEPHROLOGY Pub Date : 2025-11-04 eCollection Date: 2025-12-01 DOI: 10.1093/ckj/sfaf336
Maxime Pluquet, Ziad A Massy, Youssef Bennis, Said Kamel, Nicolas Mansencal, Christian Combe, Natalia Alencar de Pinho, Solène M Laville, Sophie Liabeuf

Introduction: Chronic kidney disease (CKD) is associated with systemic inflammation and elevated pro-inflammatory cytokines. While interleukin (IL)-8 has shown harmful cardiovascular effects in preclinical studies, its role in CKD remains underexplored. The study aimed to (i) determine serum IL-8 concentrations across CKD stages, (ii) identify factors associated with IL-8 concentrations, and (iii) evaluate its association with major adverse cardiovascular events (MACEs) and all-cause mortality.

Methods: The Chronic Kidney Disease-Renal Epidemiology and Information Network (CKD-REIN) prospective cohort includes CKD patients with an estimated glomerular filtration rate (eGFR) below 60 mL/min/1.73 m² not on kidney replacement therapy. Baseline serum IL-8 concentrations were centrally measured. MACE was defined as any cardiovascular death, myocardial infarction, stroke and hospital admission for heart failure. Multivariable linear regression was used to identify factors associated with IL-8 concentrations. Adjusted cause-specific Cox proportional hazard models were used to estimate hazard ratios [hazard ratio (HR) (95% confidence interval)] for the first MACE and for mortality.

Results: Among 2389 included patients (66% men; median age 68 years; mean eGFR 34.8 mL/min/1.73 m²), median serum IL-8 concentration was 12.2 pg/mL. Higher IL-8 levels correlated with more advanced CKD (P < .001), and were independently associated with lower eGFR, diabetes, prior cardiovascular disease, anemia, elevated C-reactive protein, more medications and lower serum albumin. Elevated baseline IL-8 was associated with a greater adjusted hazard of MACEs in women [HR for 1-unit change in log(IL-8): 1.75 (1.26; 2.43)] but not in men [HR 1.16 (0.93; 1.45)]. The adjusted HR for all-cause mortality was 1.70 (1.40; 2.06), with no difference between men and women.

Conclusion: In a large cohort of patients with moderate-to-advanced CKD, higher IL-8 levels were associated with a greater risk of MACEs in women (but not in men) and higher mortality in both sexes. Further research is needed to assess the potential of IL-8 as a cardiovascular risk biomarker, clarify the clinical significance of the sex difference observed here and determine whether targeting IL-8 could reduce cardiovascular risk in CKD.

慢性肾脏疾病(CKD)与全身性炎症和促炎细胞因子升高有关。虽然白细胞介素(IL)-8在临床前研究中显示出有害的心血管作用,但其在CKD中的作用仍未得到充分探讨。该研究旨在(i)确定CKD分期的血清IL-8浓度,(ii)确定与IL-8浓度相关的因素,以及(iii)评估其与主要不良心血管事件(mace)和全因死亡率的关系。方法:慢性肾脏疾病-肾脏流行病学和信息网络(CKD- rein)前瞻性队列包括肾小球滤过率(eGFR)估计低于60 mL/min/1.73 m²的未接受肾脏替代治疗的CKD患者。中心测量基线血清IL-8浓度。MACE定义为任何心血管死亡、心肌梗死、中风和因心力衰竭住院。采用多变量线性回归确定与IL-8浓度相关的因素。使用校正的病因特异性Cox比例风险模型来估计首次MACE和死亡率的风险比[风险比(HR)(95%置信区间)]。结果:在纳入的2389例患者中(66%为男性,中位年龄68岁,平均eGFR 34.8 mL/min/1.73 m²),血清中位IL-8浓度为12.2 pg/mL。结论:在大量中晚期CKD患者中,较高的IL-8水平与女性(而非男性)更高的mace风险和两性更高的死亡率相关。需要进一步的研究来评估IL-8作为心血管风险生物标志物的潜力,阐明本研究中观察到的性别差异的临床意义,并确定靶向IL-8是否可以降低CKD的心血管风险。
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引用次数: 0
Severe obesity associates with maladaptive glomerular haemodynamics clustered with insulin resistance and endothelial dysfunction. 严重肥胖与肾小球血流动力学失调相关,并伴有胰岛素抵抗和内皮功能障碍。
IF 4.6 2区 医学 Q1 UROLOGY & NEPHROLOGY Pub Date : 2025-11-03 eCollection Date: 2025-11-01 DOI: 10.1093/ckj/sfaf334
Diego Moriconi, Chiara Rovera, Francesco Raggi, Silvia Armenia, Rosa Maria Bruno, Anna Solini

Background: Obesity is a leading risk factor for chronic kidney disease, with glomerular hyperfiltration as one of its earliest manifestations. However, absolute glomerular filtration rate (GFR) does not distinguish well between a pressure-driven hyperfiltration and the physiological forms. Insulin resistance and endothelial dysfunction have been proposed as key correlates of maladaptive renal haemodynamics, but their interplay remains unclear.

Methods: Cross-sectional pilot study involving 27 adults with severe obesity (mean body mass index of 43.9 kg/m²). Measured GFR (mGFR) was assessed by iohexol plasma clearance and effective renal plasma flow (ERPF) by 123I-ortho-iodohippurate clearance. A hyperfiltration index (P-score) was derived as the standardized difference between filtration fraction and effective renal plasma flow with higher values reflecting a maladaptive phenotype. Endothelial function was assessed by brachial artery flow-mediated dilation and insulin sensitivity by oral glucose insulin sensitivity.

Results: Participants were stratified into tertiles of P-score. Across tertiles, mGFR did not differ, but ERPF declined (756 ± 113 vs 505 ± 130 mL/min, P = .001) and of filtration fraction increased (16 ± 3% vs 26 ± 5%, P = .001) from the lowest to the highest tertile. Insulin sensitivity significantly decreased with higher P-score (384 ± 50 vs 308 ± 33, P = .019). Endothelial function was reduced in the highest vs lowest tertile (3.44 ± 1.49 vs 6.21 ± 1.76%, P = .014), while responses to nitroglycerin did not differ. In univariate analyses, P-score was inversely associated with insulin sensitivity (r = -0.36, P = .036) and endothelial dysfunction (r = -0.40, P = .022), whereas measured mGFR showed no associations. In multivariable models, the link between P-score and insulin sensitivity remained significant after adjustment.

Conclusion: A maladaptive phenotype characterized by elevated filtration fraction relative to renal plasma flow clustered with insulin resistance and endothelial dysfunction, whereas mGFR alone failed to capture these relationships. Comprehensive haemodynamic profiling may help to refine risk stratification in obesity.

背景:肥胖是慢性肾脏疾病的主要危险因素,肾小球过滤是其最早的表现之一。然而,绝对肾小球滤过率(GFR)不能很好地区分压力驱动的超滤过和生理形式的超滤过。胰岛素抵抗和内皮功能障碍被认为是肾脏血流动力学失调的关键相关因素,但它们之间的相互作用尚不清楚。方法:横断面初步研究涉及27名严重肥胖(平均体重指数为43.9 kg/m²)的成年人。测量GFR (mGFR)通过碘己醇血浆清除率评估,有效肾血浆流量(ERPF)通过123i - orthoiodohippurate清除率评估。高滤过指数(P-score)是滤过分数和有效肾血浆流量之间的标准化差异,较高的值反映了不适应表型。通过肱动脉血流介导的扩张评估内皮功能,通过口服葡萄糖胰岛素敏感性评估胰岛素敏感性。结果:以P-score为分位数对参与者进行分层。不同类型的mGFR差异不大,但ERPF从低到高依次下降(756±113 vs 505±130 mL/min, P = .001),过滤分数增加(16±3% vs 26±5%,P = .001)。P值越高,胰岛素敏感性越低(384±50 vs 308±33,P = 0.019)。内皮功能在最高和最低的各组中降低(3.44±1.49 vs 6.21±1.76%,P = 0.014),而对硝酸甘油的反应没有差异。在单变量分析中,P评分与胰岛素敏感性(r = -0.36, P = 0.036)和内皮功能障碍(r = -0.40, P = 0.022)呈负相关,而测量的mGFR没有显示相关。在多变量模型中,p -评分与胰岛素敏感性之间的联系在调整后仍然显著。结论:一种以相对于肾血浆流量的滤过分数升高为特征的不适应表型与胰岛素抵抗和内皮功能障碍聚集在一起,而单独的mGFR无法捕获这些关系。全面的血流动力学分析可能有助于细化肥胖的风险分层。
{"title":"Severe obesity associates with maladaptive glomerular haemodynamics clustered with insulin resistance and endothelial dysfunction.","authors":"Diego Moriconi, Chiara Rovera, Francesco Raggi, Silvia Armenia, Rosa Maria Bruno, Anna Solini","doi":"10.1093/ckj/sfaf334","DOIUrl":"10.1093/ckj/sfaf334","url":null,"abstract":"<p><strong>Background: </strong>Obesity is a leading risk factor for chronic kidney disease, with glomerular hyperfiltration as one of its earliest manifestations. However, absolute glomerular filtration rate (GFR) does not distinguish well between a pressure-driven hyperfiltration and the physiological forms. Insulin resistance and endothelial dysfunction have been proposed as key correlates of maladaptive renal haemodynamics, but their interplay remains unclear.</p><p><strong>Methods: </strong>Cross-sectional pilot study involving 27 adults with severe obesity (mean body mass index of 43.9 kg/m²). Measured GFR (mGFR) was assessed by iohexol plasma clearance and effective renal plasma flow (ERPF) by <sup>123</sup>I-ortho-iodohippurate clearance. A hyperfiltration index (P-score) was derived as the standardized difference between filtration fraction and effective renal plasma flow with higher values reflecting a maladaptive phenotype. Endothelial function was assessed by brachial artery flow-mediated dilation and insulin sensitivity by oral glucose insulin sensitivity.</p><p><strong>Results: </strong>Participants were stratified into tertiles of P-score. Across tertiles, mGFR did not differ, but ERPF declined (756 ± 113 vs 505 ± 130 mL/min, <i>P</i> = .001) and of filtration fraction increased (16 ± 3% vs 26 ± 5%, <i>P</i> = .001) from the lowest to the highest tertile. Insulin sensitivity significantly decreased with higher P-score (384 ± 50 vs 308 ± 33, <i>P</i> = .019). Endothelial function was reduced in the highest vs lowest tertile (3.44 ± 1.49 vs 6.21 ± 1.76%, <i>P</i> = .014), while responses to nitroglycerin did not differ. In univariate analyses, P-score was inversely associated with insulin sensitivity (r = -0.36, <i>P</i> = .036) and endothelial dysfunction (r = -0.40, <i>P</i> = .022), whereas measured mGFR showed no associations. In multivariable models, the link between P-score and insulin sensitivity remained significant after adjustment.</p><p><strong>Conclusion: </strong>A maladaptive phenotype characterized by elevated filtration fraction relative to renal plasma flow clustered with insulin resistance and endothelial dysfunction, whereas mGFR alone failed to capture these relationships. Comprehensive haemodynamic profiling may help to refine risk stratification in obesity.</p>","PeriodicalId":10435,"journal":{"name":"Clinical Kidney Journal","volume":"18 11","pages":"sfaf334"},"PeriodicalIF":4.6,"publicationDate":"2025-11-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12635466/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145585886","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Afatinib-associated pseudo-acute kidney injury. 阿法替尼相关假性急性肾损伤。
IF 4.6 2区 医学 Q1 UROLOGY & NEPHROLOGY Pub Date : 2025-11-03 eCollection Date: 2025-12-01 DOI: 10.1093/ckj/sfaf333
Jacqueline Nguyen, Devika Das, Chintan V Shah
{"title":"Afatinib-associated pseudo-acute kidney injury.","authors":"Jacqueline Nguyen, Devika Das, Chintan V Shah","doi":"10.1093/ckj/sfaf333","DOIUrl":"10.1093/ckj/sfaf333","url":null,"abstract":"","PeriodicalId":10435,"journal":{"name":"Clinical Kidney Journal","volume":"18 12","pages":"sfaf333"},"PeriodicalIF":4.6,"publicationDate":"2025-11-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12665980/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145660288","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Genetic testing in autosomal dominant polycystic kidney disease: why it matters in 2025. 常染色体显性多囊肾病的基因检测:为什么它在2025年很重要。
IF 4.6 2区 医学 Q1 UROLOGY & NEPHROLOGY Pub Date : 2025-10-31 eCollection Date: 2025-12-01 DOI: 10.1093/ckj/sfaf331
Emilie Cornec-Le Gall, Albert C M Ong

Autosomal dominant polycystic kidney disease (ADPKD) is the most common monogenic cause of kidney failure globally, and a significant cause of morbidity and mortality. It is now recognized that it may result from both major and minor genes with associated differences in disease penetrance, symptom burden and clinical outcomes. Genetic testing is now readily available to discriminate between different genotypes and is being increasingly utilized for diagnostic and prognostic indications. In this short review, we summarize the reasons why testing should become part of standard care for ADPKD patients where available and highlight some current limitations and challenges to testing. Defining the genetic landscape in ADPKD for all ethnic groups will be key to the future development and deployment of individualized patient-centered management in this condition.

常染色体显性多囊肾病(ADPKD)是全球肾衰竭最常见的单基因原因,也是发病率和死亡率的重要原因。现在认识到,它可能是由主要和次要基因引起的,它们在疾病外显率、症状负担和临床结果方面存在相关差异。基因检测现在很容易用于区分不同的基因型,并越来越多地用于诊断和预后指征。在这篇简短的综述中,我们总结了为什么检测应该成为ADPKD患者标准治疗的一部分的原因,并强调了目前检测的一些局限性和挑战。确定所有种族的ADPKD的遗传景观将是未来发展和部署以患者为中心的个性化管理的关键。
{"title":"Genetic testing in autosomal dominant polycystic kidney disease: why it matters in 2025.","authors":"Emilie Cornec-Le Gall, Albert C M Ong","doi":"10.1093/ckj/sfaf331","DOIUrl":"10.1093/ckj/sfaf331","url":null,"abstract":"<p><p>Autosomal dominant polycystic kidney disease (ADPKD) is the most common monogenic cause of kidney failure globally, and a significant cause of morbidity and mortality. It is now recognized that it may result from both major and minor genes with associated differences in disease penetrance, symptom burden and clinical outcomes. Genetic testing is now readily available to discriminate between different genotypes and is being increasingly utilized for diagnostic and prognostic indications. In this short review, we summarize the reasons why testing should become part of standard care for ADPKD patients where available and highlight some current limitations and challenges to testing. Defining the genetic landscape in ADPKD for all ethnic groups will be key to the future development and deployment of individualized patient-centered management in this condition.</p>","PeriodicalId":10435,"journal":{"name":"Clinical Kidney Journal","volume":"18 Suppl 2","pages":"ii17-ii25"},"PeriodicalIF":4.6,"publicationDate":"2025-10-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12699653/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145755468","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Dietary salt and protein intake and urinary cystine excretion in patients with cystinuria. 胱氨酸尿患者膳食盐和蛋白质摄入与尿胱氨酸排泄的关系。
IF 4.6 2区 医学 Q1 UROLOGY & NEPHROLOGY Pub Date : 2025-10-29 eCollection Date: 2025-11-01 DOI: 10.1093/ckj/sfaf329
Francesca Bermond, Laura Fabbrini, Laura Rivoli, Andrea Spasiano, Marta Leporati, Michele Petrarulo, Andrea Ricotti, Lucia Borsotti, Martino Marangella, Domenico Cosseddu, Pietro Manuel Ferraro, Corrado Vitale

Background: Cystinuria is a rare autosomal recessive disorder characterized by impaired renal reabsorption of cystine and dibasic amino acids, leading to recurrent nephrolithiasis. While dietary salt and protein restriction are commonly recommended, evidence supporting their effectiveness in reducing urinary cystine excretion is limited. This study investigated whether intra-individual changes in dietary salt and protein intake are associated with changes in cystine excretion over time.

Methods: We conducted a retrospective cohort study of 41 adult patients with recurrent cystine stones treated at a tertiary kidney stone clinic between 2004 and 2023. All patients underwent five 24-hour urine collections at intervals of 6-12 months. Urinary sodium and urea excretions were used as surrogates for salt and protein intake, respectively. Mixed-effects linear regression models assessed within-person associations between dietary intake and urinary cystine excretion, adjusting for age, sex, and time.

Results: Cystine excretion showed considerable intra-individual variability (intraclass correlation coefficient: 0.457). An increase in urinary urea of 3.5 g/24 h (reflecting ∼10 g/day higher protein intake) was associated with a 164 µmol/24 h increase in cystine excretion (95% CI: 57 to 271, P = .003). In contrast, a 17 mmol/24 h increase in urinary sodium (∼1 g/day salt intake) was associated with a non-significant 46 µmol/24 h increase in cystine excretion (95% CI: -5 to 97, P = .081).

Conclusions: Protein intake is moderately associated with urinary cystine excretion, whereas salt intake has minimal effect. These findings suggest that, while protein moderation may contribute to cystine reduction, fluid intake and urine alkalinization remain the primary determinants of urinary cystine levels.

背景:胱氨酸尿症是一种罕见的常染色体隐性遗传病,其特征是肾脏对胱氨酸和二碱性氨基酸的重吸收受损,导致肾结石复发。虽然通常建议限制饮食中的盐和蛋白质,但支持其减少尿胱氨酸排泄的有效性的证据有限。本研究调查了个体内饮食盐和蛋白质摄入量的变化是否与胱氨酸排泄随时间的变化有关。方法:我们对2004年至2023年间在三级肾结石诊所治疗的41例复发性胱氨酸结石成年患者进行了回顾性队列研究。所有患者每隔6-12个月进行5次24小时尿液收集。尿钠和尿素排泄量分别作为盐和蛋白质摄入量的替代物。混合效应线性回归模型评估了饮食摄入和尿胱氨酸排泄之间的人际关系,调整了年龄、性别和时间。结果:胱氨酸排泄具有显著的个体差异性(类内相关系数为0.457)。尿尿素增加3.5 g/24 h(反映蛋白质摄入量增加~ 10 g/天)与胱氨酸排泄增加164µmol/24 h相关(95% CI: 57至271,P = 0.003)。相比之下,尿钠增加17 mmol/24 h(约1 g/天盐摄入量)与胱氨酸排泄增加46µmol/24 h无关(95% CI: -5至97,P = 0.081)。结论:蛋白质摄入与尿胱氨酸排泄适度相关,而盐摄入影响最小。这些研究结果表明,虽然蛋白质调节可能有助于胱氨酸减少,但液体摄入和尿碱化仍然是尿胱氨酸水平的主要决定因素。
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引用次数: 0
The BestShape artificial intelligence system for real-time prediction of intradialytic hypotension-clinical outcomes after four-year follow-up. 用于实时预测分析性低血压的BestShape人工智能系统——四年随访后的临床结果。
IF 4.6 2区 医学 Q1 UROLOGY & NEPHROLOGY Pub Date : 2025-10-28 eCollection Date: 2025-11-01 DOI: 10.1093/ckj/sfaf323
Cheng-Jui Lin, Hong-Mou Shih, Ying-Ying Chen, Shiow-Huey Chen, Hui-Fang Su, Homtin Chen, Chih-Jen Wu

Background: Intradialytic hypotension (IDH) is a serious complication of chronic hemodialysis (HD). BestShape is a novel artificial intelligence-driven system developed recently for real-time prediction of IDH. This study aimed to report the clinical results and health-economic benefits following the implementation of BestShape.

Methods: Medical records from two institutions in Taiwan that incorporated BestShape into all HD practices were retrospectively reviewed. Data from HD sessions from January 2020 to December 2023, following BestShape implementation, constituted the "BestShape group." Data from January 2016 through the end of 2019 served as the historical control group. The primary outcome was IDH frequency, and secondary outcomes were rates of cardiopulmonary resuscitation (CPR), falls, mortality, medical personnel satisfaction, and estimated cost reduction.

Results: In total, 213 071 HD sessions of 18 141 patients were included (BestShape 152 792 sessions; control 60 279 sessions). The mean monthly IDH rate significantly reduced from 27% in 2019 to 21% in 2023 (P < .001). The need for CPR during dialysis decreased from eight to three times per month, and post-dialysis falls decreased from 21 to seven times. The estimated annual cost savings by implementing BestShape were USD 115 658. The mortality rate during HD was not significantly different before and after BestShape implementation (8% vs 9.3%, P = .260). Medical personnel expressed satisfaction with BestShape, with a mean satisfaction score of 86.

Conclusion: Preliminary clinical data show BestShape is associated with reductions in IDH, CPR, and falls in patients undergoing HD, and a reduction in healthcare costs and high medical personnel satisfaction.

背景:分析性低血压(IDH)是慢性血液透析(HD)的严重并发症。BestShape是最近开发的用于IDH实时预测的新型人工智能驱动系统。本研究旨在报告实施BestShape后的临床结果和健康经济效益。方法:回顾性分析台湾两家机构将BestShape纳入所有HD实践的医疗记录。在BestShape实施之后,从2020年1月到2023年12月的高清会话数据构成了“BestShape组”。2016年1月至2019年底的数据作为历史对照组。主要结局是IDH频率,次要结局是心肺复苏(CPR)率、跌倒、死亡率、医务人员满意度和估计成本降低。结果:共纳入18141例患者的213071例HD (BestShape 152 792例,对照组60 279例)。平均每月IDH率从2019年的27%显著下降到2023年的21% (P P = 0.260)。医务人员对BestShape表示满意,平均满意度为86分。结论:初步临床数据显示,BestShape与HD患者IDH、心肺复苏术和跌倒的减少、医疗费用的降低和医务人员的高满意度有关。
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引用次数: 0
Age-adapted versus age-independent eGFR thresholds to diagnose CKD: integrating the debate and charting a balanced path forward. 年龄适应与年龄独立的eGFR阈值诊断CKD:整合争论并绘制平衡的前进路径。
IF 4.6 2区 医学 Q1 UROLOGY & NEPHROLOGY Pub Date : 2025-10-24 eCollection Date: 2025-11-01 DOI: 10.1093/ckj/sfaf328
Pierre Delanaye, Priya Vart, Jürgen Floege, Carmine Zoccali

The debate over whether to use age-adapted or age-independent estimated glomerular filtration rate (eGFR) cut-offs for diagnosing chronic kidney disease (CKD) reflects a fundamental tension between physiological understanding and clinical pragmatism. Age-adapted proponents highlight robust evidence that GFR declines naturally with age, with many healthy older adults falling below the traditional 60 ml/min/1.73 m2 threshold. They argue that fixed cut-offs risk overdiagnosing CKD in the elderly, potentially leading to unnecessary anxiety and treatment, while underrecognizing risk in younger individuals whose GFRs are abnormal for their age. Physiological and longitudinal studies support this view, suggesting age-specific percentiles may better reflect true pathology. Conversely, advocates for age-independent thresholds emphasize that age-related GFR decline is not benign. Large epidemiological studies show that older adults with an eGFR of 45-59 ml/min/1.73 m2 are at increased risk of kidney failure, cardiovascular events and mortality, even in the absence of albuminuria. They caution that redefining CKD based on age could exclude high-risk older adults from beneficial therapies and research and that 'healthy' reference populations may harbour undiagnosed disease. A balanced approach recognizes the physiological realities of aging while not ignoring the clinical risks associated with reduced kidney function in older adults. Individualized risk prediction tools and shared decision-making can help tailor diagnosis and management. Ultimately, moving beyond arbitrary thresholds toward a patient-centred, risk-based strategy may best serve individuals across their lifespans, ensuring that CKD diagnosis and care are both scientifically sound and clinically meaningful.

关于是否使用年龄适应或年龄无关的肾小球滤过率(eGFR)临界值来诊断慢性肾脏疾病(CKD)的争论反映了生理理解和临床实用主义之间的根本紧张关系。年龄适应的支持者强调了GFR随年龄自然下降的有力证据,许多健康老年人的GFR低于传统的60 ml/min/1.73 m2阈值。他们认为,在老年人中,固定的临界值可能会导致过度诊断CKD的风险,这可能会导致不必要的焦虑和治疗,而在年轻人中,他们的gfr在他们的年龄中是异常的。生理学和纵向研究支持这一观点,表明特定年龄的百分位数可能更好地反映真实的病理。相反,年龄无关阈值的倡导者强调,与年龄相关的GFR下降不是良性的。大型流行病学研究表明,即使没有蛋白尿,eGFR为45-59 ml/min/1.73 m2的老年人发生肾衰竭、心血管事件和死亡的风险也会增加。他们警告说,根据年龄重新定义CKD可能会将高风险的老年人排除在有益的治疗和研究之外,并且“健康”的参考人群可能存在未确诊的疾病。一个平衡的方法认识到衰老的生理现实,同时不忽视与老年人肾功能下降相关的临床风险。个性化的风险预测工具和共同决策可以帮助定制诊断和管理。最终,超越任意阈值,转向以患者为中心、以风险为基础的策略,可能最好地服务于整个生命周期的个体,确保CKD的诊断和护理既科学合理又有临床意义。
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Clinical Kidney Journal
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