Sumedh Jayanti, Jennifer Li, Jasveen Renthawa, Ming-Wei Lin, Vincent Lee
{"title":"Double Positive Anti-PR3 ANCA Vasculitis and Anti-GBM Vasculitis in a Pregnant Woman: Case Report.","authors":"Sumedh Jayanti, Jennifer Li, Jasveen Renthawa, Ming-Wei Lin, Vincent Lee","doi":"10.1111/nep.70124","DOIUrl":"10.1111/nep.70124","url":null,"abstract":"<p><p>Pulmonary-renal syndrome (PRS) caused by double-positive ANCA-associated vasculitis and anti-GBM disease is rare, and management is primarily guided by case series evidence. We present an even rarer case of a 36-year-old female who developed PRS in early pregnancy due to double-positive disease. She required intensive care admission for respiratory support and was treated with high-dose steroids and two doses of rituximab (1 g), achieving a good pulmonary response. However, her renal function subsequently deteriorated. Given the high maternal and foetal risks associated with her condition, she chose to terminate her pregnancy at 8 weeks. A subsequent kidney biopsy revealed crescentic glomerulonephritis secondary to anti-GBM disease. She was treated with plasma exchange and cyclophosphamide, leading to normalisation of her kidney function. She was weaned off prednisone and completed a course of intravenous pulsed cyclophosphamide (500 mg ×6 fortnightly). At 1 year post-diagnosis, she remains in biochemical and clinical remission on maintenance rituximab every 6 months. This case highlights the complexity of managing double-positive disease in pregnancy, where evidence is limited, and decisions require careful consideration of maternal and foetal risks. Furthermore, it underscores the importance of early anti-GBM-specific treatment-plasma exchange and cyclophosphamide-in achieving remission.</p>","PeriodicalId":520716,"journal":{"name":"Nephrology (Carlton, Vic.)","volume":"30 9","pages":"e70124"},"PeriodicalIF":1.9,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145071610","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Zibya Barday, Nicola Wearne, Erika S W Jones, Mignon McCulloch, Jacqueline Hoare, Kathryn Manning, Suzanne Pretorius, Bianca J Davidson
{"title":"Clinical Outcomes of a Dedicated Kidney Adolescent and Young Adult Clinic (KAYAC) in South Africa.","authors":"Zibya Barday, Nicola Wearne, Erika S W Jones, Mignon McCulloch, Jacqueline Hoare, Kathryn Manning, Suzanne Pretorius, Bianca J Davidson","doi":"10.1111/nep.70110","DOIUrl":"https://doi.org/10.1111/nep.70110","url":null,"abstract":"<p><strong>Aim: </strong>Adolescents and young adults (AYAs) are increasingly utilising kidney health care services. However, there is no data on the impact of kidney transition clinics, as well as the AYA spectrum of kidney diseases, in South Africa (SA). This study evaluates kidney outcomes and patient survival amongst AYA patients attending a dedicated kidney AYA clinic (KAYAC).</p><p><strong>Methods: </strong>This 5-year retrospective study included AYA (aged 13-25) with kidney disease, attending a tertiary nephrology service. A comparative analysis of outcomes between patients who attended the KAYAC and those attending the standard-of-care adult kidney clinics was performed. The primary composite outcome assessed included doubling of creatinine, reduction in eGFR > 40%, kidney failure, requirement for kidney replacement therapy, or death. Logistic regression evaluated the associations between relevant variables, death, and loss to follow-up (LTFU).</p><p><strong>Results: </strong>The AYA cohort consisted of 292 patients: 111 (38.0%) attended KAYAC and 181 (62.0%) attended adult clinics. Glomerular diseases (72.6%), congenital urinary tract anomalies (10.6%) and hereditary conditions (8.2%) were the most common causes of kidney disease. The KAYAC group had delayed progression to kidney failure with an improved composite outcome (p = 0.018), lower mortality (p = 0.046) and less LTFU (p = 0.001). Both groups demonstrated high rates of non-adherence, with a prevalence of 33.9% in the total cohort.</p><p><strong>Conclusion: </strong>AYA are a unique population who could benefit from KAYAC transition clinics. A dedicated KAYAC has been found to be associated with better kidney outcomes, lower mortality and less LTFU, underscoring its critical role in resource-limited settings.</p>","PeriodicalId":520716,"journal":{"name":"Nephrology (Carlton, Vic.)","volume":"30 9","pages":"e70110"},"PeriodicalIF":1.9,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12377949/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144986654","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}
Christopher Sparks, Adam G Steinberg, Nigel D Toussaint
{"title":"Identifying and Characterising a Chronic Kidney Disease Electronic-Phenotype Using Electronic Health Record-Derived Data: A Narrative Review of Strategies and Applications.","authors":"Christopher Sparks, Adam G Steinberg, Nigel D Toussaint","doi":"10.1111/nep.70118","DOIUrl":"10.1111/nep.70118","url":null,"abstract":"<p><p>Chronic kidney disease (CKD) represents a significant and growing healthcare burden. As CKD is defined and staged using laboratory values, it can be readily identified and characterised via data points derived from the electronic health record (EHR). This narrative literature review describes various strategies that have been employed to develop such a CKD 'e-phenotype,' evaluating accuracy, fidelity, and practicality. Methods discussed include the use of International Classification of Diseases (ICD) codes, estimated glomerular filtration rate (eGFR) and proteinuria criteria, free-text analysis and natural language processing (NLP), and machine learning techniques. Considerable variability in algorithm performance and complexity exists, with the use of eGFR and proteinuria criteria likely constituting the most practical and reliable basis for a CKD e-phenotype. In addition, promising current and future applications of the CKD e-phenotype have been outlined, such as characterising the burden of CKD complications and comorbid disease, and use as a tool to encourage optimisation of CKD management with quality, guideline-directed care. Future directions and challenges may involve integration of risk stratification and clinical decision support systems, alongside applications across public health resourcing and clinical trial recruitment.</p>","PeriodicalId":520716,"journal":{"name":"Nephrology (Carlton, Vic.)","volume":"30 9","pages":"e70118"},"PeriodicalIF":1.9,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12432484/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145056618","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}
{"title":"60th ANZSN Congress 30 August - 3 September 2025 Perth, Western Australia.","authors":"","doi":"10.1111/nep.70080","DOIUrl":"https://doi.org/10.1111/nep.70080","url":null,"abstract":"","PeriodicalId":520716,"journal":{"name":"Nephrology (Carlton, Vic.)","volume":"30 Suppl 3 ","pages":"3"},"PeriodicalIF":1.9,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145234864","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"60th ANZSN Congress 30 August - 3 September 2025 Perth, Western Australia.","authors":"","doi":"10.1111/nep.70085","DOIUrl":"https://doi.org/10.1111/nep.70085","url":null,"abstract":"","PeriodicalId":520716,"journal":{"name":"Nephrology (Carlton, Vic.)","volume":"30 Suppl 3 ","pages":"9"},"PeriodicalIF":1.9,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145234811","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Chondroitin Sulfate N-Acetylgalactosaminyltransferase 1 Promotes the Progression of Renal Fibrosis Mediated by Versican 1 in Mouse Remnant Kidney.","authors":"Yoshikatsu Kaneko, Yuya Suzuki, Kaho Sato, Kosei Takeuchi, Michihiro Igarashi, Ichiei Narita","doi":"10.1111/nep.70109","DOIUrl":"10.1111/nep.70109","url":null,"abstract":"<p><strong>Aim: </strong>Renal fibrosis is a final common pathway for progressive chronic kidney diseases. Immune cell infiltration and production of tumour growth factor-β (TGF-β) are essential factors for fibrosis development. We examined the role of chondroitin sulfate (CS) proteoglycan, which is one of the main extracellular matrix components induced by TGF-β signalling. We also examined CS N-acetylgalactosaminyltransferase 1 (T1), an enzyme that catalyses the first step of CS-specific synthesis.</p><p><strong>Methods: </strong>T1<sup>-/-</sup> mice, genetically lacking T1, and T1<sup>+/+</sup> mice underwent 5/6 nephrectomy (Nx) or sham operation. Kidney function, urine marker, mRNA expression, and TGF-β signalling were evaluated 1 month after Nx or sham operation. Renal fibrotic area was quantified 3 months later.</p><p><strong>Results: </strong>Both T1<sup>+/+</sup> and T1<sup>-/-</sup> mice with Nx showed equivalent loss of kidney function; however, a tubular damage marker, upregulation of TGF-β and collagen expression, and renal fibrosis were suppressed in T1<sup>-/-</sup> mice with Nx. Versican, one of the core proteins of CS proteoglycan, was exclusively upregulated in T1<sup>+/+</sup> mice with Nx. Among the versican splicing variants, versican 1 (V1) was expressed in the medullary interstitium of the remnant kidney in T1<sup>+/+</sup> mice. V1 was produced in the interstitial macrophages, fibroblasts/myofibroblasts, and endothelial cells, whereas TGF-β was expressed in fibroblasts/myofibroblasts. Phosphorylation of the TGF-β signalling molecules Smad2/3 was not induced in T1<sup>-/-</sup> mice with Nx. In vivo administration of TGF-β inhibitor into Nx mice reduced V1 and Tgfb expression.</p><p><strong>Conclusion: </strong>T1 was essential for effective TGF-β signalling, V1 upregulation, and subsequent renal fibrosis.</p>","PeriodicalId":520716,"journal":{"name":"Nephrology (Carlton, Vic.)","volume":"30 8","pages":"e70109"},"PeriodicalIF":1.9,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144877928","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"60th ANZSN Congress 30 August - 3 September 2025 Perth, Western Australia.","authors":"","doi":"10.1111/nep.70088","DOIUrl":"https://doi.org/10.1111/nep.70088","url":null,"abstract":"","PeriodicalId":520716,"journal":{"name":"Nephrology (Carlton, Vic.)","volume":"30 Suppl 3 ","pages":"66-113"},"PeriodicalIF":1.9,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145234828","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"60th ANZSN Congress 30 August - 3 September 2025 Perth, Western Australia.","authors":"","doi":"10.1111/nep.70087","DOIUrl":"https://doi.org/10.1111/nep.70087","url":null,"abstract":"","PeriodicalId":520716,"journal":{"name":"Nephrology (Carlton, Vic.)","volume":"30 Suppl 3 ","pages":"10-23"},"PeriodicalIF":1.9,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145234806","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"60th ANZSN Congress 30 August - 3 September 2025 Perth, Western Australia.","authors":"","doi":"10.1111/nep.70086","DOIUrl":"https://doi.org/10.1111/nep.70086","url":null,"abstract":"","PeriodicalId":520716,"journal":{"name":"Nephrology (Carlton, Vic.)","volume":"30 Suppl 3 ","pages":"8"},"PeriodicalIF":1.9,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145234813","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}