NephrologyPub Date : 2024-09-01DOI: 10.1111/nep.14344
Renato C Monteiro
{"title":"Closing remarks from the IIGANN2023 Tokyo symposium-40 years of study progress in IgA nephropathy.","authors":"Renato C Monteiro","doi":"10.1111/nep.14344","DOIUrl":"https://doi.org/10.1111/nep.14344","url":null,"abstract":"","PeriodicalId":19264,"journal":{"name":"Nephrology","volume":"29 Suppl 2 ","pages":"80-83"},"PeriodicalIF":2.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142350958","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
NephrologyPub Date : 2024-09-01DOI: 10.1111/nep.14341
Bruce A Julian
{"title":"My lifetime in IgA nephropathy: An unexpected journey.","authors":"Bruce A Julian","doi":"10.1111/nep.14341","DOIUrl":"10.1111/nep.14341","url":null,"abstract":"","PeriodicalId":19264,"journal":{"name":"Nephrology","volume":"29 Suppl 2 ","pages":"55-59"},"PeriodicalIF":2.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11441621/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142350964","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
NephrologyPub Date : 2024-09-01DOI: 10.1111/nep.14349
Sean J Barbour
{"title":"The epidemiology of IgA nephropathy: East versus West.","authors":"Sean J Barbour","doi":"10.1111/nep.14349","DOIUrl":"https://doi.org/10.1111/nep.14349","url":null,"abstract":"","PeriodicalId":19264,"journal":{"name":"Nephrology","volume":"29 Suppl 2 ","pages":"65-67"},"PeriodicalIF":2.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142350968","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
NephrologyPub Date : 2024-09-01Epub Date: 2024-07-18DOI: 10.1111/nep.14369
Pei Chen, Jicheng Lv
{"title":"Low dose glucocorticoids, mycophenolate mofetil and hydroxychloroquine in IgA nephropathy, an update of current clinical trials.","authors":"Pei Chen, Jicheng Lv","doi":"10.1111/nep.14369","DOIUrl":"10.1111/nep.14369","url":null,"abstract":"<p><p>This mini-review explores glucocorticoids, mycophenolate mofetil (MMF), and hydroxychloroquine (HCQ) in IgA nephropathy (IgAN). It discusses conflicting findings from pivotal trials like TESTING and STOP-IgAN regarding glucocorticoid efficacy, emphasizing reduced-dose protocols as potentially safer options. MMF's effectiveness varies among populations, demonstrating promise in Chinese cohorts but yielding inconclusive results elsewhere. HCQ shows potential in reducing proteinuria, with ongoing trials investigating its long-term benefits.</p>","PeriodicalId":19264,"journal":{"name":"Nephrology","volume":" ","pages":"25-29"},"PeriodicalIF":2.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141634103","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
NephrologyPub Date : 2024-09-01DOI: 10.1111/nep.14365
Jonathan Barratt
{"title":"Mucosal targeting in IgA nephropathy targeting the gut associated lymphoid tissue.","authors":"Jonathan Barratt","doi":"10.1111/nep.14365","DOIUrl":"10.1111/nep.14365","url":null,"abstract":"<p><p>IgA nephropathy is a mucosally driven disease and new therapeutic approaches are specifically targeting the mucosal production of IgA in the hope that this will lead to a reduction in circulating IgA immune complexes and mesangial IgA deposition. In this lecture, I discuss the rationale for targeting the mucosal immune system of the gut and the existing data from clinical trials supporting such an approach as a disease modifying treatment for IgA nephropathy.</p>","PeriodicalId":19264,"journal":{"name":"Nephrology","volume":"29 Suppl 2 ","pages":"34-36"},"PeriodicalIF":2.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142350962","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
NephrologyPub Date : 2024-09-01Epub Date: 2024-07-16DOI: 10.1111/nep.14337
Anni Song, Ruiwei Yan, Yue Qiu, Xingjie Yin, Jing Xiong, Guangmin Yao, Chun Zhang
{"title":"Rhodojaponin VI ameliorates podocyte injury related with MDM2/Notch1 pathway in rat experimental membranous nephropathy.","authors":"Anni Song, Ruiwei Yan, Yue Qiu, Xingjie Yin, Jing Xiong, Guangmin Yao, Chun Zhang","doi":"10.1111/nep.14337","DOIUrl":"10.1111/nep.14337","url":null,"abstract":"<p><strong>Aim: </strong>Rhodojaponin VI (R-VI) is the key compound of Rhododendron molle G. Don (Ericaceae) (RM) with effective clinical application in rheumatoid arthritis and chronic glomerulonephritis. In our study, we tried to explore the effect of R-VI on the rat model of membranous nephropathy.</p><p><strong>Methods: </strong>The rat model of passive heymann nephritis (PHN) was established by injecting sheep anti-rat Fx1A serum at a single dose through the tail. The rats were orally administered R-VI (0.02 mg/kg) or FK506 (1 mg/kg) 1 day before PHN induction, which was kept for 4 weeks. Urine and blood samples as well as kidney tissue were collected for analysis. C5b-9-induced human podocyte cell (HPC) was employed for experiments in vitro.</p><p><strong>Results: </strong>R-VI could alleviate glomerulonephritis progression and podocyte injury in PHN rats, as indicated by the decreased proteinuria and the elevated level of albumin, accompanied with reduced immune deposits, reversed podocyte injury in the kidneys. Furthermore, R-VI suppressed murine double minute 2 (MDM2) expression without the alteration in the protein level of p53 and decreased Notch1 expression independent of Numb regulation. Pre-treatment with R-VI in C5b-9-induced HPC blocked MDM2/Notch1 signalling pathway.</p><p><strong>Conclusion: </strong>Thus, R-VI ameliorates podocyte injury in rats with PHN, which was probably related with MDM2/Notch1 signalling pathway.</p>","PeriodicalId":19264,"journal":{"name":"Nephrology","volume":" ","pages":"555-564"},"PeriodicalIF":2.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141620515","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
NephrologyPub Date : 2024-09-01Epub Date: 2024-06-04DOI: 10.1111/nep.14329
Liuyu Sun, Yali Ren, Baige Su, Suxia Wang, Xuhui Zhong, Yuwu Jiang, Fang Wang
{"title":"Glomerular basement membrane ultrastructural changes in a patient with COQ2 glomerulopathy: A case report.","authors":"Liuyu Sun, Yali Ren, Baige Su, Suxia Wang, Xuhui Zhong, Yuwu Jiang, Fang Wang","doi":"10.1111/nep.14329","DOIUrl":"10.1111/nep.14329","url":null,"abstract":"<p><p>Primary coenzyme Q10 deficiency-1, caused by COQ2 disease-causing variants, is an autosomal recessive disorder, and genetic testing is the gold standard for diagnosing this condition. A Chinese boy with steroid-resistant nephrotic syndrome, focal segmental glomerulosclerosis, and progressive kidney insufficiency was included in the study. Electron microscopy revealed the glomerular basement membrane with irregular thickness and lamellation with diffuse effacement of foot processes in the podocytes, and swollen mitochondria with abnormal cristae in the podocytes. Coenzyme Q10 supplementation started about 3 weeks after the onset of mild kidney dysfunction did not improve the proband's kidney outcome. Proband-only whole-exome sequencing and Sanger sequencing revealed two heteroallelic COQ2 variants: a maternally inherited novel variant c.1013G > A[p.(Gly338Glu)] in exon 6 and a variant of unknown origin c.1159C > T[p.(Arg387*)] in exon 7. Subsequent long-read sequencing demonstrated these two variants were located on different alleles. Our report extends the phenotypic and genotypic spectrum of COQ2 glomerulopathy.</p>","PeriodicalId":19264,"journal":{"name":"Nephrology","volume":" ","pages":"612-616"},"PeriodicalIF":2.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141262442","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
NephrologyPub Date : 2024-09-01DOI: 10.1111/nep.14343
Ian S D Roberts
{"title":"Pathology of IgA nephropathy: A global perspective.","authors":"Ian S D Roberts","doi":"10.1111/nep.14343","DOIUrl":"https://doi.org/10.1111/nep.14343","url":null,"abstract":"<p><p>Worldwide adoption of the Oxford Classification of IgA nephropathy (IgAN) has enabled comparison of pathology data from clinicopathological studies in different regions of the world. It is apparent that the frequency of Oxford Classification MEST-C scores shows geographic variations. These in part reflect differences in the stage of disease at diagnosis, criteria for performing biopsies and inclusion in clinical studies, and pathologist reporting practice. However, there appears to be a true geographic difference in the frequency of glomerular inflammation and crescents with a 2-3 fold greater proportion of patients showing these changes in East Asia when compared to Europe and North America. This indicates that the pathology of IgAN is influenced by genetic background. Geographic differences in the pathology of IgAN might underly the reported differences in clinical presentation and outcome in different regions of the world, and has important implications for clinical trials and patient management.</p>","PeriodicalId":19264,"journal":{"name":"Nephrology","volume":"29 Suppl 2 ","pages":"71-74"},"PeriodicalIF":2.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142350966","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
NephrologyPub Date : 2024-09-01DOI: 10.1111/nep.14364
Mitchell Hunter-Dickson, Muh Geot Wong
{"title":"The role of endothelin receptor antagonists in IgA nephropathy.","authors":"Mitchell Hunter-Dickson, Muh Geot Wong","doi":"10.1111/nep.14364","DOIUrl":"https://doi.org/10.1111/nep.14364","url":null,"abstract":"<p><p>There is growing evidence of endothelin receptor antagonists (ERAs) in renoprotection in proteinuric kidney disease including IgA nephropathy (IgAN). Here, we review current evidence, including the use of sparsentan, atrasentan and zibotentan in IgAN. Recent trails of combination therapy including SGLT2 inhibitors and ERAs suggest possible benefit in further reduction of proteinuria and reducing ERA fluid-retention side effects although more evidence is needed for clinical applications.</p>","PeriodicalId":19264,"journal":{"name":"Nephrology","volume":"29 Suppl 2 ","pages":"30-33"},"PeriodicalIF":2.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142350969","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}