Kidney international最新文献

{"title":"A kidney organoid-based readout to assess disease activity in primary and recurrent focal segmental glomerulosclerosis.","authors":"Ashwani Kumar Gupta, Ekta Minocha, Kyle M Koss, Bilal A Naved, Luisa Safar-Boueri, Jason A Wertheim, Lorenzo Gallon","doi":"10.1016/j.kint.2025.01.018","DOIUrl":"https://doi.org/10.1016/j.kint.2025.01.018","url":null,"abstract":"<p><p>Primary Focal Segmental Glomerulosclerosis (pFSGS) is an acquired kidney disorder that frequently leads to kidney failure and confers an elevated risk of recurrence after kidney transplantation, termed recurrent pFSGS. Unfortunately, there is no diagnostic method to foresee recurrence of pFSGS after kidney transplantation. Progress in developing assays to test disease activity is hampered by few preclinical models to replicate disease and inability of in vitro cultured primary podocytes to remain terminally differentiated. In recent years, advancements in kidney organoid biology have led to the development of kidney tissues with glomeruli and major nephron segments including podocytes. To develop a pFSGS model, we studied the effect of plasma from patients diagnosed with pFSGS on kidney organoids differentiated from human pluripotent stem cells. The pFSGS plasma treatment induced podocytopathy, extracellular matrix protein deposition, fibrosis and apoptosis within organoids, whereas non-recurrent plasma did not affect organoid structure. pFSGS plasma also led to loss of normal expression patterns of podocyte specific proteins, nephrin and podocin within podocytes. Further, cytokine array profiling revealed that pFSGS plasma induced secretion of cytokines associated with inflammation and angiogenesis. Additionally, kidney organoids treated with plasma obtained after therapeutic plasma exchange for recurrent pFSGS led to lower cell death in organoids after sequential exchanges with the final exchange showing the least apoptotic cells without morphological abnormality. Overall, our results demonstrate the potential of kidney organoids in advancing kidney disease modeling. These insights could be applied in clinical settings to assist in gauging FSGS recurrence risk prior to kidney transplantation.</p>","PeriodicalId":17801,"journal":{"name":"Kidney international","volume":" ","pages":""},"PeriodicalIF":14.8,"publicationDate":"2025-02-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143365218","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Prenatal and preimplantation genetic testing for monogenic kidney disorders","authors":"Nine V.A.M. Knoers","doi":"10.1016/j.kint.2024.06.031","DOIUrl":"10.1016/j.kint.2024.06.031","url":null,"abstract":"<div><div>In recent years, advances in genetic sequencing techniques and in the analysis of sequencing data have significantly improved our ability to diagnose genetic kidney diseases. Identification of the disease-causing genetic variant(s) is crucial not only for prognostication and personalized management, but also for providing genetic counseling and guiding family planning decisions. It is particularly important that patients desiring children receive advice on their reproductive choices early, ideally before conception. This concise review focuses on the options available for prenatal and preimplantation genetic testing in the context of monogenic kidney diseases, including the latest progress and the legal and ethical issues associated with these reproductive technologies. Although these tests could be performed for all monogenic disorders where the disease-causing variant(s) has (have) been identified in the index patient, invasive prenatal testing is currently primarily performed for severe childhood-onset monogenic kidney disorders. Noninvasive prenatal diagnosis for monogenic disorders is a rapidly developing field that promises to provide an accurate and acceptable alternative to invasive procedures once several technical challenges have been addressed. Preimplantation genetic testing allows for the selection and implantation of embryos free from the disease-causing genetic variants, significantly lowering the risk of affected pregnancies. This option is becoming more popular among individuals with monogenic kidney diseases, particularly those with disorders that manifest later in life, such as autosomal dominant polycystic kidney disease. This review covers the procedure, its outcomes, and the technical, ethical and legal challenges of preimplantation genetic testing for monogenic kidney diseases.</div></div>","PeriodicalId":17801,"journal":{"name":"Kidney international","volume":"107 2","pages":"Pages 255-261"},"PeriodicalIF":14.8,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142546141","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"When two signals cross paths: cGAS-STING and ER stress in kidney disease progression","authors":"Ryo Yamada ,&nbsp;Motoko Yanagita","doi":"10.1016/j.kint.2024.11.023","DOIUrl":"10.1016/j.kint.2024.11.023","url":null,"abstract":"<div><div>Previous reports have suggested that both the endoplasmic reticulum (ER) stress and cyclic guanosine monophosphate–adenosine monophosphate synthase–stimulator of interferon genes pathways contribute to the progression of chronic kidney disease; however, the relationship between these 2 pathways in kidney injury has not been fully elucidated. Andrade-Silva <em>et al.</em> revealed that the cyclic guanosine monophosphate–adenosine monophosphate synthase–stimulator of interferon genes pathway can enhance ER stress through the protein kinase R-like ER kinase (PERK)–mediated signaling cascade in kidney tubular epithelial cells and sequentially augment fibrosis during kidney injury. Further studies are needed to elucidate the precise mechanisms by which the cyclic guanosine monophosphate–adenosine monophosphate synthase–stimulator of interferon genes pathway activates PERK-dependent ER stress in kidney tubular epithelial cells post injury.</div></div>","PeriodicalId":17801,"journal":{"name":"Kidney international","volume":"107 2","pages":"Pages 227-229"},"PeriodicalIF":14.8,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143029074","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"KDIGO 2025 clinical practice guideline for the evaluation, management, and treatment of autosomal dominant polycystic kidney disease (ADPKD): executive summary","authors":"Vicente E. Torres ,&nbsp;Curie Ahn ,&nbsp;Thijs R.M. Barten ,&nbsp;Godela Brosnahan ,&nbsp;Melissa A. Cadnapaphornchai ,&nbsp;Arlene B. Chapman ,&nbsp;Emilie Cornec-Le Gall ,&nbsp;Joost P.H. Drenth ,&nbsp;Ron T. Gansevoort ,&nbsp;Peter C. Harris ,&nbsp;Tess Harris ,&nbsp;Shigeo Horie ,&nbsp;Max C. Liebau ,&nbsp;Michele Liew ,&nbsp;Andrew J. Mallett ,&nbsp;Changlin Mei ,&nbsp;Djalila Mekahli ,&nbsp;Dwight Odland ,&nbsp;Albert C.M. Ong ,&nbsp;Luiz F. Onuchic ,&nbsp;Olivier Devuyst","doi":"10.1016/j.kint.2024.07.010","DOIUrl":"10.1016/j.kint.2024.07.010","url":null,"abstract":"<div><div>The <em>Kidney Disease: Improving Global Outcomes (KDIGO) 2025 Clinical Practice Guideline for the Evaluation, Management, and Treatment of Autosomal Dominant Polycystic Kidney Disease</em> (ADPKD) represents the first KDIGO guideline on this subject. Its scope includes nomenclature, diagnosis, prognosis, and prevalence; kidney manifestations; chronic kidney disease (CKD) management and progression, kidney failure, and kidney replacement therapy; therapies to delay progression of kidney disease; polycystic liver disease; intracranial aneurysms and other extrarenal manifestations; lifestyle and psychosocial aspects; pregnancy and reproductive issues; pediatric issues; and approaches to the management of people with ADPKD. The guideline has been developed with patient partners, clinicians, and researchers around the world, with the goal to generate a useful resource for healthcare providers and patients by providing actionable recommendations. The development of this guideline followed an explicit process of evidence review and appraisal, based on a rigorous, formal systematic literature review. The strength of recommendations follows the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) approach. The guideline also provides practice points serving to direct clinical care or activities relating to areas for which a systematic review was not conducted. Limitations of the evidence are discussed. Research recommendations to address gaps in knowledge, and implications for policy and payment, are provided. The guideline targets a broad audience of healthcare providers, people living with ADPKD, and stakeholders involved in the various aspects of ADPKD care.</div></div>","PeriodicalId":17801,"journal":{"name":"Kidney international","volume":"107 2","pages":"Pages 234-254"},"PeriodicalIF":14.8,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143029067","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"KDIGO 2025 Clinical Practice Guideline for the Evaluation, Management, and Treatment of Autosomal Dominant Polycystic Kidney Disease (ADPKD)","authors":"","doi":"10.1016/j.kint.2024.07.009","DOIUrl":"10.1016/j.kint.2024.07.009","url":null,"abstract":"","PeriodicalId":17801,"journal":{"name":"Kidney international","volume":"107 2","pages":"Pages S1-S239"},"PeriodicalIF":14.8,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143029076","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"PI3Kα in the pathogenesis and treatment of lupus nephritis","authors":"Vivek Kasinath ,&nbsp;George C. Tsokos","doi":"10.1016/j.kint.2024.11.004","DOIUrl":"10.1016/j.kint.2024.11.004","url":null,"abstract":"","PeriodicalId":17801,"journal":{"name":"Kidney international","volume":"107 2","pages":"Pages 215-217"},"PeriodicalIF":14.8,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142668445","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Challenges in maturation and integration of kidney organoids for stem cell–based renal replacement therapy","authors":"Cathelijne W. van den Berg ,&nbsp;Sébastien J. Dumas ,&nbsp;Melissa H. Little ,&nbsp;Ton J. Rabelink","doi":"10.1016/j.kint.2024.10.028","DOIUrl":"10.1016/j.kint.2024.10.028","url":null,"abstract":"<div><div>Human pluripotent stem cell–derived kidney organoids hold promise for future applications in regenerative medicine. However, significant biological hurdles need to be overcome to enable their use as a transplantable stem cell–derived therapeutic graft. Current kidney organoid protocols do not recapitulate a complete integrated developing kidney, but embryonic kidney transplantations have provided clues for advancing maturation and functionality of kidney organoids. Transplantation, subsequent vascularization, and blood perfusion of kidney organoids improve nephron patterning and maturation, suggesting a role for angiocrine factors as well as metabolic wiring in these processes. Transplanted organoids exhibit filtration, but the resulting filtrate has no apparent exit path for excretion. Improved <em>in vitro</em> patterning of kidney organoids may be required such that a more structurally correct tissue is formed before transplant. Here we review current progress with transplantation of kidney organoids, as well as their engraftment and integration, and identify the key obstacles to therapeutic success and how these might be achieved.</div></div>","PeriodicalId":17801,"journal":{"name":"Kidney international","volume":"107 2","pages":"Pages 262-270"},"PeriodicalIF":14.8,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142687312","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The authors reply","authors":"Yoshio Funahashi ,&nbsp;Seung Hun Park ,&nbsp;Jessica F. Hebert ,&nbsp;Mahaba B. Eiwaz ,&nbsp;Adam C. Munhall ,&nbsp;Tahnee Groat ,&nbsp;Lingxue Zeng ,&nbsp;Jonghan Kim ,&nbsp;Hak Soo Choi ,&nbsp;Michael P. Hutchens","doi":"10.1016/j.kint.2024.11.010","DOIUrl":"10.1016/j.kint.2024.11.010","url":null,"abstract":"","PeriodicalId":17801,"journal":{"name":"Kidney international","volume":"107 2","pages":"Page 361"},"PeriodicalIF":14.8,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143029070","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The autoimmune architecture of childhood idiopathic nephrotic syndrome","authors":"Tho-Alfakar Al-Aubodah ,&nbsp;Ciriaco A. Piccirillo ,&nbsp;Howard Trachtman ,&nbsp;Tomoko Takano","doi":"10.1016/j.kint.2024.10.027","DOIUrl":"10.1016/j.kint.2024.10.027","url":null,"abstract":"<div><div>Idiopathic nephrotic syndrome, the most common glomerular disorder in children, has long been considered an immune-mediated disease based on the efficacy of glucocorticoids at inducing remission. Nevertheless, the immune processes leading to podocytopathy have largely remained elusive. The success of B-cell depletion with rituximab, descriptions of B-cell dysregulation during active disease, and the most recent discovery of autoantibodies targeting the major podocyte antigen nephrin point to an autoimmune humoral etiology for idiopathic nephrotic syndrome. Investigations of the immune factors involved in idiopathic nephrotic syndrome pathogenesis have uncovered common features with other autoimmune disorders that will aid in prognostication and in guiding the expansion of our glucocorticoid-sparing therapeutic arsenal. In this review, we discuss the emerging autoimmune architecture of idiopathic nephrotic syndrome, with a specific focus on pediatric steroid-sensitive disease, including the podocyte-reactive B-cell response that causes anti-podocyte antibodies, the predisposing genetic factors that shape the podocyte-reactive immune landscape, and the immune triggers driving active disease.</div></div>","PeriodicalId":17801,"journal":{"name":"Kidney international","volume":"107 2","pages":"Pages 271-279"},"PeriodicalIF":14.8,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142687346","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"journal club","authors":"","doi":"10.1016/j.kint.2024.11.024","DOIUrl":"10.1016/j.kint.2024.11.024","url":null,"abstract":"","PeriodicalId":17801,"journal":{"name":"Kidney international","volume":"107 2","pages":"Pages 212-214"},"PeriodicalIF":14.8,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143151879","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}