Zi Chan, Ka Lok Chan, Chi Kwan Lam, Wai Ping Law, Wai Lun Will Pak, Yick Hei Wong, Sunny Sze Ho Wong
{"title":"Peritoneal Dialysis for End-Stage Kidney Disease due to Mitochondrial Encephalopathy, Lactic Acidosis, and Stroke-Like Episodes (MELAS): A Case Report.","authors":"Zi Chan, Ka Lok Chan, Chi Kwan Lam, Wai Ping Law, Wai Lun Will Pak, Yick Hei Wong, Sunny Sze Ho Wong","doi":"10.1111/nep.70283","DOIUrl":"10.1111/nep.70283","url":null,"abstract":"<p><p>We report a 23-year-old male with kidney failure secondary to childhood Focal Segmental Glomerulosclerosis who presented with sepsis, severe lactic acidosis, and encephalopathy. Following stabilization of the acute condition with Continuous Veno-Venous Hemofiltration, a diagnosis of Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-like episodes (MELAS) was confirmed by muscle biopsy and the identification of the m.3243A>G mutation. This case highlights the diagnostic difficulties of this rare disease and the challenges in selecting a long-term renal replacement therapy. Specifically, the use of lactate-buffered peritoneal dialysis fluids in these patients remains controversial. In our case, the patient demonstrated poor tolerance to intermittent haemodialysis due to MELAS-associated cardiomyopathy. However, despite theoretical concerns regarding lactate load, steady-state serum lactate remained stable on Continuous Ambulatory Peritoneal Dialysis (CAPD), and the patient achieved good functional recovery. We conclude that while acute decompensation requires aggressive management, CAPD is a feasible long-term dialysis modality for patients with kidney failure secondary to MELAS.</p>","PeriodicalId":520716,"journal":{"name":"Nephrology (Carlton, Vic.)","volume":"31 9","pages":"e70283"},"PeriodicalIF":1.9,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13574547/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148972953","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":"Physiologically-Guided Ultrafiltration: A Proposed Ultrasound Framework for Individualized Volume Removal During Hemodialysis.","authors":"Sharad Patel, Arwa Bootwala, Adam Green","doi":"10.1111/nep.70272","DOIUrl":"10.1111/nep.70272","url":null,"abstract":"<p><p>Ultrafiltration during hemodialysis is the most hemodynamically stressful intervention dialysis patients endure, yet its prescription relies on interdialytic weight gain and clinical gestalt. Rates exceeding 13 mL/kg/h carry substantially higher cardiovascular mortality, and recurrent intradialytic hypotension drives myocardial stunning, accelerated loss of residual kidney function, and progressive cardiac dysfunction. Existing volume management tools, including bioimpedance spectroscopy, relative blood volume monitoring, and even lung ultrasound, address fragments of this problem but none integrates the two questions that should govern every prescription: how much volume can be removed, and how fast? This review synthesizes principles from critical care hemodynamics and nephrology to propose an integrated ultrasound-guided framework. We apply the Frank-Starling relationship to volume removal, translating the principles of active fluid removal to maintenance hemodialysis. We approach the safety of fluid removal in terms of preload responsiveness: removal is best tolerated when the patient is preload-unresponsive, operating on the flat portion of the cardiac function curve. The framework uses a rapid screen (inferior vena cava diameter and lung ultrasound) to identify patients near dry weight versus those requiring further assessment. When congestion is present, a two-axis decision matrix combines venous congestion quantification (portal vein pulsatility) with preload dependence testing (passive leg raising with a stroke volume surrogate) to guide both volume target and removal rate. Preload responsiveness is defined by a rise of at least 10% to 12% in the left ventricular outflow tract velocity-time integral after passive leg raising, and significant venous congestion by an inferior vena cava diameter of 2 cm or greater with portal vein pulsatility; preload-unresponsive patients without congestion may tolerate rates toward the 13 mL/kg/h safety ceiling, whereas preload-responsive or severely congested patients warrant slower removal over extended or additional sessions. We propose a two-tiered protocol adaptable to diverse practice settings. This integrated framework has not been tested prospectively and should be regarded as hypothesis-generating; that said, the individual components are validated and the physiological rationale is sound, and the potential to reduce ultrafiltration-related morbidity, from intradialytic symptoms to myocardial stunning and residual function loss, is substantial.</p>","PeriodicalId":520716,"journal":{"name":"Nephrology (Carlton, Vic.)","volume":"31 9","pages":"e70272"},"PeriodicalIF":1.9,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13545514/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148893353","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":"Salt Intake and Kidney Outcomes in Older Adults With Chronic Kidney Disease: A Stage-Specific Cohort Study.","authors":"Hiroaki Miyauchi, Tatsuhiko Azegami, Takashin Nakayama, Chisato Mitsue, Hirotaka Shibata, Kaori Hayashi","doi":"10.1111/nep.70285","DOIUrl":"10.1111/nep.70285","url":null,"abstract":"<p><strong>Aim: </strong>In patients with chronic kidney disease (CKD), salt restriction has been shown to lower blood pressure and proteinuria and to provide kidney protective effects. However, the benefit of salt restriction in older adults with CKD remains unclear, particularly in advanced stages.</p><p><strong>Methods: </strong>We conducted a single-center retrospective cohort study of patients aged ≥ 75 years with CKD (estimated glomerular filtration rate [eGFR] < 60 mL/min/1.73 m<sup>2</sup>). Salt intake was estimated from spot urine using the Tanaka formula and categorized into quartiles. The primary outcome was a composite kidney endpoint of ≥ 30% eGFR decline or initiation of kidney replacement therapy. Cox proportional hazards models and competing risk analyses were performed, stratified by CKD stage.</p><p><strong>Results: </strong>A total of 462 patients (median age, 79 years; 68.6% male) were included. Of these, 305 had CKD Stage 3 and 157 had Stages 4-5. During a median follow-up of 2.79 years, 121 patients (26.2%) experienced the composite outcome. In Stage 3 CKD, higher salt intake was significantly associated with increased risk (adjusted HR 2.70; 95% CI 1.02-7.12; highest vs. lowest quartile), and findings were consistent across sensitivity analyses. In contrast, no significant association was observed in Stages 4-5.</p><p><strong>Conclusion: </strong>Among older adults with CKD, excessive salt intake was associated with adverse kidney outcomes in CKD Stage 3 but not in Stages 4-5. These results suggest that salt restriction may be most beneficial in the early stages of CKD in this population.</p>","PeriodicalId":520716,"journal":{"name":"Nephrology (Carlton, Vic.)","volume":"31 9","pages":"e70285"},"PeriodicalIF":1.9,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13585483/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148993641","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}
Xiao-Juan Chen, Min Kou, Mei Feng, Li-Hua Wang, Jing-Jing Wei
{"title":"Nephrogenic Syndrome of Inappropriate Antidiuresis: Case Report and Genetic Perspectives.","authors":"Xiao-Juan Chen, Min Kou, Mei Feng, Li-Hua Wang, Jing-Jing Wei","doi":"10.1111/nep.70250","DOIUrl":"10.1111/nep.70250","url":null,"abstract":"<p><strong>Aim: </strong>To report a case of nephrogenic syndrome of inappropriate antidiuresis (NSIAD) caused by a de novo hemizygous hotspot variant (c.409C>T p.R137C) in the AVPR2 gene in a young male child, and to discuss the diagnostic approach, therapeutic management, and follow-up of NSIAD.</p><p><strong>Methods: </strong>A 3-year and 2-month-old male child presenting with intermittent convulsions persisting for over one year and chronic hyponatremia was clinically evaluated. Genetic testing was performed to identify potential causative variants. Water restriction therapy was implemented, with the patient spontaneously limiting water intake to prevent infections and regulating fluid intake during illnesses. Follow-up evaluations were conducted at 3 and 6 months.</p><p><strong>Results: </strong>Genetic testing identified a de novo hemizygous hotspot variant, c.409C>T (p.R137C), in the AVPR2 gene. The patient adhered to spontaneous water restriction and regulated fluid intake during illnesses. Follow-up evaluations at 3 and 6 months demonstrated normal serum sodium levels, with no recurrence of convulsions or other symptoms.</p><p><strong>Conclusion: </strong>NSIAD should be included in the differential diagnosis of hyponatremia of unknown etiology. Genetic testing is crucial for achieving an early diagnosis, optimizing treatment regimens, and enhancing treatment compliance. Water restriction therapy, under careful monitoring, remains a safe and effective treatment modality, particularly during periods of illness.</p>","PeriodicalId":520716,"journal":{"name":"Nephrology (Carlton, Vic.)","volume":"31 9","pages":"e70250"},"PeriodicalIF":1.9,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13537857/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148883330","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}
Tennille L Vitagliano, Nicole L De La Mata, Peter S Hsu, Stephen I Alexander, Kate Wyburn, Melanie L R Wyld
{"title":"Sex and Intersectional Disparities in Access to Deceased Donor Kidney Transplantation: A 17-Year Population-Based Cohort Study.","authors":"Tennille L Vitagliano, Nicole L De La Mata, Peter S Hsu, Stephen I Alexander, Kate Wyburn, Melanie L R Wyld","doi":"10.1111/nep.70276","DOIUrl":"10.1111/nep.70276","url":null,"abstract":"<p><strong>Background: </strong>While sex disparities in access to transplant have been shown in a number of countries, the ways additional measures of disadvantage, such as intersectional and clinical disadvantage, influence sex-based disparities is less well understood. Access to transplant by sex or intersectional disadvantage has not been studied in Australia.</p><p><strong>Methods: </strong>We conducted a population-based cohort study of incident dialysis patients (2006-2023) using Australia & New Zealand Dialysis and Transplant Registry data. Cox proportional hazards models, interaction analyses and competing-risk approaches were used to evaluate associations between sex, waitlisting and deceased donor transplantation, adjusting for intersectional and clinical characteristics.</p><p><strong>Results: </strong>Among 47 884 incident dialysis patients contributing 147 510 person-years of follow-up, females were 19% less likely to be waitlisted than males (aHR 0.81, 95% CI 0.77-0.84). Sex-based disparities were greatest among females with intersectional disadvantage, including ethnic minority status (Aboriginal & Torres Strait Islander: aHR 0.57, 95% CI 0.49-0.66), diabetic kidney disease (aHR 0.61, 95% CI 0.56-0.67), having ≥ 3 comorbidities (aHR 0.66, 95% CI 0.56-0.79) and obesity (aHR 0.69, 95% CI 0.65-0.75), compared to male peers. Once waitlisted, females and males had similar likelihood of deceased donor transplantation (aHR 1.04, 95% CI 0.99-1.10) and comparable waitlist mortality (aHR 0.91, 95% CI 0.76-1.08).</p><p><strong>Conclusions: </strong>Females in Australia experience substantially reduced access to the kidney transplant waitlist, with the greatest inequities affecting those facing overlapping social and/or clinical disadvantage. The absence of sex-disparity after waitlisting indicates that inequities arise earlier in the referral and evaluation pathway. Interventions to improve equity must target these upstream stages and address intersecting drivers of disadvantage.</p>","PeriodicalId":520716,"journal":{"name":"Nephrology (Carlton, Vic.)","volume":"31 9","pages":"e70276"},"PeriodicalIF":1.9,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13542340/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148890117","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":"CKD-Associated Pruritus in Home Haemodialysis: A Signal for Routine Symptom Surveillance Rather Than a Severity Gradient.","authors":"Mengting Wu, Juan Jin, Qiang He","doi":"10.1111/nep.70249","DOIUrl":"10.1111/nep.70249","url":null,"abstract":"","PeriodicalId":520716,"journal":{"name":"Nephrology (Carlton, Vic.)","volume":"31 9","pages":"e70249"},"PeriodicalIF":1.9,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13558460/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148922549","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":"Association Between Tolvaptan Exposure and Venous Thromboembolism Risk in ADPKD: A Real-World Propensity-Matched EHR Study.","authors":"To-Pang Chen, Shang-Feng Tsai","doi":"10.1111/nep.70280","DOIUrl":"10.1111/nep.70280","url":null,"abstract":"<p><strong>Aim: </strong>Venous thromboembolism (VTE) has increasingly been reported in autosomal dominant polycystic kidney disease (ADPKD), although the mechanisms underlying this association remain incompletely understood. Whether tolvaptan exposure influences VTE risk in ADPKD has not been well studied.</p><p><strong>Methods: </strong>We performed a retrospective cohort study using the TriNetX Global Collaborative Network, including adults with ADPKD (ICD-10-CM Q61.2) from 2000 to 2025. Patients with inherited thrombophilia or prothrombin G20210A mutation were excluded. Propensity score matching (1:1) was conducted across 24 covariates to balance demographics, comorbidities, medications, and laboratory data. Exposure was analysed as a fixed treatment variable defined at cohort entry. Primary outcomes were VTE incidence, all-cause mortality, and use of antithrombotic medications.</p><p><strong>Results: </strong>Among 39 180 adults with ADPKD, 1338 received tolvaptan therapy, whereas 37 362 were without tolvaptan. After 1:1 propensity score matching, 1333 matched pairs were analysed. Tolvaptan exposure was associated with lower risks of VTE (2.1% vs. 3.9%; RR 0.538, 95% CI 0.342-0.847; p = 0.006) and all-cause mortality (1.28% vs. 3.9%; RR 0.327, 95% CI 0.190-0.562; p < 0.001). Lower utilisation of warfarin, aspirin, DOAC, heparin, or enoxaparin was observed (14.8% vs. 21.4%; RR 0.689, 95% CI 0.572-0.830; p < 0.001). Subgroup analyses demonstrated generally consistent directional associations across several demographic categories, although some subgroup analyses were limited by low event counts.</p><p><strong>Conclusion: </strong>In this large propensity-matched real-world cohort, tolvaptan exposure was associated with lower risks of VTE and mortality among patients with ADPKD. Given the retrospective observational design and the possibility of residual confounding, these findings should be interpreted as associative and hypothesis-generating rather than causal. Further prospective studies incorporating imaging-based measures of disease severity are warranted.</p>","PeriodicalId":520716,"journal":{"name":"Nephrology (Carlton, Vic.)","volume":"31 9","pages":"e70280"},"PeriodicalIF":1.9,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13574548/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148972672","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":"Light Chain Crystalline Proximal Tubulopathy and Podocytopathy With Staining for Both Light and Heavy Chain Immunoglobulin.","authors":"Seoyoung Choi, Kyung Chul Moon","doi":"10.1111/nep.70251","DOIUrl":"10.1111/nep.70251","url":null,"abstract":"<p><p>A 69-year-old male patient presented with weight loss and proteinuria. His medical history included papillary thyroid carcinoma. Laboratory findings showed proteinuria and high κ light chain levels in both urine and serum. Bone marrow aspiration revealed plasma cell myeloma of the IgA-κ type. Kidney biopsy revealed crystal inclusions in multiple locations, including podocytes and proximal tubular epithelial cells. Immunofluorescence and immunohistochemistry revealed positive staining for κ light chain and IgA. The patient was diagnosed with light chain crystalline podocytopathy and proximal tubulopathy. Our case presented some peculiarities: positive staining for heavy-chain IgA in addition to κ light chain and the absence of concurrent Fanconi syndrome or focal segmental glomerulosclerosis.</p>","PeriodicalId":520716,"journal":{"name":"Nephrology (Carlton, Vic.)","volume":"31 8","pages":"e70251"},"PeriodicalIF":1.9,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13413242/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148612430","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}
Jungwon Chun, Dohwi Yun, Juyeong Kim, Hyunjee Kim, Yesol Yim, Yoon Lee, Dongjin Yeo, Selin Woo, Dong Keon Yon
{"title":"Burden of Chronic Kidney Disease in Asia From 1990 to 2023: A Global Burden of Disease Study 2023.","authors":"Jungwon Chun, Dohwi Yun, Juyeong Kim, Hyunjee Kim, Yesol Yim, Yoon Lee, Dongjin Yeo, Selin Woo, Dong Keon Yon","doi":"10.1111/nep.70253","DOIUrl":"10.1111/nep.70253","url":null,"abstract":"<p><strong>Aim: </strong>Despite growing recognition of chronic kidney disease (CKD) as a major health threat in Asia, its subregional variations are often overlooked. This study aimed to assess the CKD burden across five Asian subregions.</p><p><strong>Methods: </strong>This study analysed data from the Global Burden of Disease Study 2023 to estimate the burden of CKD in Asia. CKD cases were classified using International Classification of Diseases codes. Age-standardized mortality rates (ASMRs) and age-standardized disability-adjusted life-year (DALY) rates (ASDRs) per 100 000 population were estimated from 1990 to 2023 across 34 Asian countries, which were grouped into five subregions: Central, East, South, Southeast Asia and high-income Asia Pacific. Estimates were stratified by age, sex and underlying causes and reported with 95% uncertainty intervals (UIs), along with an assessment of major risk factors.</p><p><strong>Results: </strong>In 2023, Southeast Asia experienced the heaviest CKD burden, with the highest ASMR (24.73 [95% UI, 19.98-29.83] per 100 000 population) and ASDR (816.37 [695.11-964.14]). Females under 35 years in South and Southeast Asia had a higher DALY burden than males. In Southeast Asia, type 1 diabetes contributed significantly to CKD deaths (ASMR, 3.18 [95% UI, 2.38-4.18] per 100 000 population), whereas CKD deaths in Central Asia were primarily due to other causes (7.48 [6.51-8.49]). High fasting plasma glucose levels were the leading risk factor across most regions, except in Central Asia.</p><p><strong>Conclusion: </strong>Although CKD is often considered uniform in Asia, our findings reveal distinct subregional drivers. Effective CKD control requires a shift from aggregated targets to precision- and region-specific strategies.</p>","PeriodicalId":520716,"journal":{"name":"Nephrology (Carlton, Vic.)","volume":"31 8","pages":"e70253"},"PeriodicalIF":1.9,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13446003/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148681660","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}