Nephrology Dialysis Transplantation最新文献

{"title":"Extrarenal manifestations in inherited kidney diseases.","authors":"Julia Hoefele, Julian Eble, Tobias Hermle, Matthias Wuttke, Ulla T Schultheiss","doi":"10.1093/ndt/gfae176","DOIUrl":"https://doi.org/10.1093/ndt/gfae176","url":null,"abstract":"<p><p>Monogenic kidney diseases result from an abundance of potential genes carrying pathogenic variants. These conditions are primarily recognized for manifesting as kidney disorders, defined as an impairment of the structure and/or function of the kidneys. However, the impact of these genetic disorders extends far beyond the kidneys, giving rise to a diverse spectrum of extrarenal manifestations. These manifestations can affect any organ system throughout the body, leading to a complex clinical presentation that demands a comprehensive understanding and interdisciplinary management of affected persons. The intricate interplay between genetic variants, molecular pathways, and systemic interactions underscores the importance of exploring the extrarenal aspects of inherited kidney diseases. This exploration not only deepens our comprehension of the diseases themselves but also opens avenues for more holistic diagnostics, treatment strategies, and improved interdisciplinary patient care. This article delves into the intricate realm of extrarenal manifestations in inherited kidney diseases, shedding light on the far-reaching impacts that these genetic conditions can exert beyond the confines of the kidney system.</p>","PeriodicalId":19078,"journal":{"name":"Nephrology Dialysis Transplantation","volume":null,"pages":null},"PeriodicalIF":4.8,"publicationDate":"2024-08-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141889775","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Association between urinary C4d levels and disease progression in IgA nephropathy.","authors":"Yaping Dong, Zi Wang, Weiyi Guo, Li Zhu, Xujie Zhou, Sufang Shi, Lijun Liu, Jicheng Lv, Hong Zhang","doi":"10.1093/ndt/gfae001","DOIUrl":"10.1093/ndt/gfae001","url":null,"abstract":"<p><strong>Background: </strong>C4d mesangial deposition, a hallmark of lectin pathway activation in immunoglobulin A nephropathy (IgAN), has been shown to be associated with risk of kidney failure. To date, the relationship between urinary C4d and renal outcome remain unelucidated.</p><p><strong>Methods: </strong>A total of 508 patients with biopsy-proven IgAN were enrolled in this study, whose baseline urine samples at the time of biopsy were collected and the levels of urinary C4d were quantified by enzyme-linked immunosorbent assay. The time-averaged C4d (TA-C4d) and the change in proteinuria were measured in sequential urine samples obtained from IgAN patients. The kidney progression event was defined as a 50% estimated glomerular filtration rate (eGFR) decline or end-stage kidney disease or death.</p><p><strong>Results: </strong>After a median follow-up of 36 months, 70 (13.8%) of the participants reached the kidney progression event. Higher levels of urinary C4d/Ucr were found to be associated with decreased eGFR, massive proteinuria, lower serum albumin levels, hypertension, and severe Oxford E and T scores. Upon adjusting for traditional risk factors (including demographics, eGFR, proteinuria, hypertension, Oxford pathologic score and immunosuppressive therapy), elevated levels of urinary C4d/Ucr were independently associated with an increased risk of chronic kidney disease progression [adjusted hazard ratio (HR) per standard deviation increment of log-transformed C4d/Ucr: 1.46; 95% CI 1.04-2.06; P = .030]. In reference to the low C4d group, the risk of poor renal outcome increased for the high C4d group (adjusted HR 1.93; 95% CI 1.05-3.54; P = .033). Additionally, a low baseline C4d level was independently associated with a favorable proteinuria response to immunosuppressive therapy at 3 months (adjusted relative risk 2.20; 95% CI 1.04-4.63, P = .038).</p><p><strong>Conclusion: </strong>The urinary C4d, serving as a non-invasive biomarker, is associated with the progression of IgAN and holds the potential to predict proteinuria response in this disease.</p>","PeriodicalId":19078,"journal":{"name":"Nephrology Dialysis Transplantation","volume":null,"pages":null},"PeriodicalIF":4.8,"publicationDate":"2024-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139098289","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Outcome of immunosuppression in children with IgA vasculitis-related nephritis.","authors":"Katharina Rohner, Matko Marlais, Yo Han Ahn, Alaa Ali, Abrar Alsharief, Anja Blejc Novak, Marta Brambilla, Evrim Kargin Cakici, Cengiz Candan, Nur Canpolat, Eugene Yu-Hin Chan, Stéphane Decramer, Madeleine Didsbury, Filipa Durao, Anne M Durkan, Ali Düzova, Thomas Forbes, Valentina Gracchi, Tulin Güngör, Tomoko Horinouchi, Belde Kasap Demir, Yasuko Kobayashi, Mikael Koskela, Eda Didem Kurt-Sukur, Claudio La Scola, Dean Langan, Xiaozhong Li, Gabriele Malgieri, Antonio Mastrangelo, Jeesu Min, Malgorzata Mizerska-Wasiak, Nabila Moussaoui, Aytul Noyan, Matti Nuutinen, Jennifer O'Gormon, Takayuki Okamoto, Louise Oni, Michiel Oosterveld, Malgorzata Pańczyk-Tomaszewska, Gonul Parmaksiz, Andrea Pasini, Pornpimol Rianthavorn, Joris Roelofs, Yunyan Shen, Rajiv Sinha, Rezan Topaloglu, Diletta Domenica Torres, Tomohiro Udagawa, Martin Wennerström, Yok Chin Yap, Kjell Tullus","doi":"10.1093/ndt/gfae009","DOIUrl":"10.1093/ndt/gfae009","url":null,"abstract":"<p><strong>Background: </strong>Immunoglobulin A vasculitis with nephritis (IgAVN) is the most common vasculitis in children. Due to a lack of evidence, treatment recommendations are based on expert opinion, resulting in variation. The aim of this study was to describe the clinical presentation, treatment and outcome of an extremely large cohort of children with biopsy-proven IgAVN in order to identify prognostic risk factors and signals of treatment efficacy.</p><p><strong>Methods: </strong>Retrospective data were collected on 1148 children with biopsy-proven IgAVN between 2005 and 2019 from 41 international paediatric nephrology centres across 25 countries and analysed using multivariate analysis. The primary outcome was estimated glomerular filtration rate (eGFR) and persistent proteinuria at last follow-up.</p><p><strong>Results: </strong>The median follow-up was 3.7 years (interquartile range 2-6.2). At last follow-up, 29% of patients had an eGFR <90 mL/min/1.73 m2, 36% had proteinuria and 3% had chronic kidney disease stage 4-5. Older age, lower eGFR at onset, hypertension and histological features of tubular atrophy and segmental sclerosis were predictors of poor outcome. There was no evidence to support any specific second-line immunosuppressive regimen being superior to others, even when further analysing subgroups of children with reduced kidney function, nephrotic syndrome or hypoalbuminemia at onset. Delayed start of immunosuppressive treatment was associated with a lower eGFR at last follow-up.</p><p><strong>Conclusion: </strong>In this large retrospective cohort, key features associated with disease outcome are highlighted. Importantly, there was no evidence to support that any specific immunosuppressive treatments were superior to others. Further discovery science and well-conducted clinical trials are needed to define accurate treatment and improve outcomes of IgAVN.</p>","PeriodicalId":19078,"journal":{"name":"Nephrology Dialysis Transplantation","volume":null,"pages":null},"PeriodicalIF":4.8,"publicationDate":"2024-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139425174","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Capillary rarefication as a possible cause of long-COVID syndrome.","authors":"Irina Osiaevi, Philipp Kümpers, Julia Weinmann-Menke, Hermann Pavenstädt, Alexandros Rovas","doi":"10.1093/ndt/gfae026","DOIUrl":"10.1093/ndt/gfae026","url":null,"abstract":"","PeriodicalId":19078,"journal":{"name":"Nephrology Dialysis Transplantation","volume":null,"pages":null},"PeriodicalIF":4.8,"publicationDate":"2024-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139672303","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Alkaline phosphatase treatment of acute kidney injury-an update.","authors":"Thei S Steenvoorden, Janneke A J Rood, Frederike J Bemelman, Roberto Armstrong, Henri G D Leuvenink, Joost W van der Heijden, Liffert Vogt","doi":"10.1093/ndt/gfae028","DOIUrl":"10.1093/ndt/gfae028","url":null,"abstract":"<p><p>Through improved insights into the increasing incidence and detrimental effects of acute kidney injury (AKI), its clinical relevance has become more and more apparent. Although treatment strategies for AKI have also somewhat improved, an adequate remedy still does not exist. Finding one is complicated by a multifactorial pathophysiology and by heterogeneity in the patient population. Alkaline phosphatase (ALP) has been suggested as a therapy for sepsis-associated AKI because of its protective effects against lipopolysaccharide (LPS)-induced inflammation and kidney injury in animals. However, its effectiveness as an AKI treatment has not been demonstrated definitively. Because the anti-inflammatory properties of ALP are likely not reliant on a direct effect on LPS itself, we postulate that other pathways are much more important in explaining the renoprotective properties ascribed to ALP. The re-evaluation of which properties of the ALP enzyme are responsible for the benefit seen in the lab is an important step in determining where the true potential of ALP as a treatment strategy for AKI in the clinic lies. In this review we will discuss how ALP can prevent activation of harmful pro-inflammatory receptors, redirect cell-cell signalling and protect barrier tissues, which together form the basis for current knowledge of the role of ALP in the kidney. With this knowledge in mind and by analysing currently available clinical evidence, we propose directions for new research that can determine whether ALP as a treatment strategy for AKI has a future in the clinical field.</p>","PeriodicalId":19078,"journal":{"name":"Nephrology Dialysis Transplantation","volume":null,"pages":null},"PeriodicalIF":4.8,"publicationDate":"2024-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11334066/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139940332","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}

{"title":"Effects of HIF-PHD inhibitors in kidney development.","authors":"Soichiro Haraguchi, Kenji Tsuji, Hiroyuki Nakanoh, Kazuhiko Fukushima, Shinji Kitamura, Jun Wada","doi":"10.1093/ndt/gfae078","DOIUrl":"10.1093/ndt/gfae078","url":null,"abstract":"","PeriodicalId":19078,"journal":{"name":"Nephrology Dialysis Transplantation","volume":null,"pages":null},"PeriodicalIF":4.8,"publicationDate":"2024-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140326908","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pros and cons of the use of fluoroquinolone antibiotics in patients with kidney disease.","authors":"Fatemeh Ahmadi, Y Joseph Hwang, Flory T Muanda","doi":"10.1093/ndt/gfae037","DOIUrl":"10.1093/ndt/gfae037","url":null,"abstract":"","PeriodicalId":19078,"journal":{"name":"Nephrology Dialysis Transplantation","volume":null,"pages":null},"PeriodicalIF":4.8,"publicationDate":"2024-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139707357","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Outcomes for clinical trials involving adults with chronic kidney disease: a multinational Delphi survey involving patients, caregivers and health professionals.","authors":"Andrea Matus Gonzalez, Nicole Evangelidis, Martin Howell, Allison Jaure, Benedicte Sautenet, Magdalena Madero, Gloria Ashuntantang, Samaya Anumudu, Amelie Bernier-Jean, Louese Dunn, Yeoungjee Cho, Laura Cortes Sanabria, Ian H de Boer, Samuel Fung, Daniel Gallego, Chandana Guha, Andrew S Levey, Adeera Levin, Eduardo Lorca, Ikechi G Okpechi, Patrick Rossignol, Nicole Scholes-Robertson, Laura Sola, Armando Teixeira-Pinto, Tim Usherwood, Andrea K Viecelli, David C Wheeler, Katherine Widders, Martin Wilkie, Jonathan C Craig","doi":"10.1093/ndt/gfae010","DOIUrl":"10.1093/ndt/gfae010","url":null,"abstract":"<p><strong>Background: </strong>Many outcomes of high priority to patients and clinicians are infrequently and inconsistently reported across trials in chronic kidney disease (CKD), which generates research waste and limits evidence-informed decision making. We aimed to generate consensus among patients/caregivers and health professionals on critically important outcomes for trials in CKD prior to kidney failure and the need for kidney replacement therapy, and to describe the reasons for their choices.</p><p><strong>Methods: </strong>This was an online two-round international Delphi survey. Adult patients with CKD (all stages and diagnoses), caregivers and health professionals who could read English, Spanish or French were eligible. Participants rated the importance of outcomes using a Likert scale (7-9 indicating critical importance) and a Best-Worst Scale. The scores for the two groups were assessed to determine absolute and relative importance. Comments were analysed thematically.</p><p><strong>Results: </strong>In total, 1399 participants from 73 countries completed Round 1 of the Delphi survey, including 628 (45%) patients/caregivers and 771 (55%) health professionals. In Round 2, 790 participants (56% response rate) from 63 countries completed the survey including 383 (48%) patients/caregivers and 407 (52%) health professionals. The overall top five outcomes were: kidney function, need for dialysis/transplant, life participation, cardiovascular disease and death. In the final round, patients/caregivers indicated higher scores for most outcomes (17/22 outcomes), and health professionals gave higher priority to mortality, hospitalization and cardiovascular disease (mean difference >0.3). Consensus was based upon the two groups yielding median scores of ≥7 and mean scores >7, and the proportions of both groups rating the outcome as 'critically important' being >50%. Four themes reflected the reasons for their priorities: imminent threat of a health catastrophe, signifying diminishing capacities, ability to self-manage and cope, and tangible and direct consequences.</p><p><strong>Conclusion: </strong>Across trials in CKD, the outcomes of highest priority to patients, caregivers and health professionals were kidney function, need for dialysis/transplant, life participation, cardiovascular disease and death.</p>","PeriodicalId":19078,"journal":{"name":"Nephrology Dialysis Transplantation","volume":null,"pages":null},"PeriodicalIF":4.8,"publicationDate":"2024-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139491316","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Long-term kidney function of Lowe syndrome: a nationwide study of paediatric and adult patients.","authors":"Taro Ando, Kenichiro Miura, Tomoo Yabuuchi, Yoko Shirai, Kiyonobu Ishizuka, Shoichiro Kanda, Yutaka Harita, Kyoko Hirasawa, Riku Hamada, Kenji Ishikura, Eisuke Inoue, Motoshi Hattori","doi":"10.1093/ndt/gfae080","DOIUrl":"10.1093/ndt/gfae080","url":null,"abstract":"","PeriodicalId":19078,"journal":{"name":"Nephrology Dialysis Transplantation","volume":null,"pages":null},"PeriodicalIF":4.8,"publicationDate":"2024-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140862039","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Treatment strategies of the thromboembolic risk in kidney failure patients with atrial fibrillation.","authors":"Simonetta Genovesi, A John Camm, Adrian Covic, Alexandru Burlacu, Björn Meijers, Casper Franssen, Valerie Luyckx, Vassilios Liakopoulos, Gaetano Alfano, Christian Combe, Carlo Basile","doi":"10.1093/ndt/gfae121","DOIUrl":"10.1093/ndt/gfae121","url":null,"abstract":"<p><p>The incidence and prevalence of atrial fibrillation (AF) in patients affected by kidney failure, i.e. glomerular filtration rate <15 ml/min/1.73 m2, is high and probably underestimated. Numerous uncertainties remain regarding how to prevent thromboembolic events in this population because both cardiology and nephrology guidelines do not provide clear recommendations. The efficacy and safety of oral anticoagulant therapy (OAC) in preventing thromboembolism in patients with kidney failure and AF has not been demonstrated for either vitamin K antagonists (VKAs) or direct anticoagulants (DOACs). Moreover, it remains unclear which is more effective and safer, because estimated creatinine clearance <25-30 ml/min was an exclusion criterion in the randomized controlled trials (RCTs). Three RCTs comparing DOACs and VKAs in kidney failure failed to reach the primary endpoint, as they were underpowered. The left atrial appendage is the main source of thromboembolism in the presence of AF. Left atrial appendage closure (LAAC) has recently been proposed as an alternative to OAC. RCTs comparing the efficacy and safety of LAAC versus OAC in kidney failure were terminated prematurely due to recruitment failure. A recent prospective study showed a reduction in thromboembolic events in haemodialysis patients with AF and undergoing LAAC compared with patients taking or not taking OAC. We review current treatment standards and discuss recent developments in managing the thromboembolic risk in kidney failure patients with AF. The importance of shared decision-making with the multidisciplinary team and the patient to consider individual risks and benefits of each treatment option is underlined.</p>","PeriodicalId":19078,"journal":{"name":"Nephrology Dialysis Transplantation","volume":null,"pages":null},"PeriodicalIF":4.8,"publicationDate":"2024-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11288792/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141180130","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}