Pub Date : 2026-02-01Epub Date: 2025-12-11DOI: 10.1016/j.xkme.2025.101199
Na’amah Razon , Yi Zhang , Bethney Bonilla-Herrera , Lorien S. Dalrymple , Amanda K. Stennett , Baback Roshanravan , Daniel Tancredi , Joshua J. Fenton
<div><h3>Rationale & Objective</h3><div>Transportation insecurity is a social risk factor of particular importance to individuals with end-stage kidney disease (ESKD), as most individuals need to travel multiple times a week to dialysis treatment. Advancing home modalities for individuals with ESKD experiencing transportation insecurity may be beneficial by reducing travel burden and improving access.</div></div><div><h3>Study Design</h3><div>Retrospective cohort study.</div></div><div><h3>Setting & Participants</h3><div>Individuals with ESKD treated with in-center hemodialysis (HD) at a large, national dialysis organization.</div></div><div><h3>Exposures</h3><div>The main transportation mode to HD is categorized into private transportation (individuals who drive themselves or have a family member/friend drive) or those who lack private transportation (Medicaid non-emergency medical transportation, paratransit, public transportation, private pay non-emergency medical transportation, and other).</div></div><div><h3>Outcomes</h3><div>Transition to home dialysis is defined as an individual who has completed at least 1 training treatment for home therapies or at least 1 dialysis treatment at home.</div></div><div><h3>Analytic Approach</h3><div>Log-binomial multivariate regression models to estimate adjusted incidence rate ratios of home dialysis transition by transportation mode.</div></div><div><h3>Results</h3><div>Individuals who lacked private transportation were significantly less likely to transition to home dialysis compared with those who drove themselves or had a family member/friend drive them to HD. Adjusted incidence rate ratios for home dialysis transition were 47%-58% lower in nonprivate transportation groups compared with those with private transportation, ranging from 0.42 in individuals relying on Medicaid transportation benefits (95% confidence interval, 0.35-0.50; <em>P</em> < 0.001) to 0.53 (95% confidence interval, 0.41-0.67; <em>P</em> < 0.001) among paratransit users.</div></div><div><h3>Limitations</h3><div>Single transportation assessment, exclusion of individuals already on home dialysis, and absence of caregiver data.</div></div><div><h3>Conclusions</h3><div>Individuals with ESKD receiving in-center HD who lack private transportation may have reduced access to home dialysis, even though this group may benefit from home modalities. Better identifying transportation barriers and targeting home modalities for those with transportation insecurity may reduce the adverse consequences of missed dialysis related to transportation barriers and be an additional opportunity to increase home dialysis uptake.</div></div><div><h3>Plain-Language Summary</h3><div>Transportation is a key barrier for many individuals receiving in-center dialysis care. Nonetheless, the majority of individuals in the United States receive their dialysis treatment at an in-center facility. In a study of patients with end-stage kidney disease treate
{"title":"Association Between Transportation and Home Dialysis Transition: Retrospective Cohort Study","authors":"Na’amah Razon , Yi Zhang , Bethney Bonilla-Herrera , Lorien S. Dalrymple , Amanda K. Stennett , Baback Roshanravan , Daniel Tancredi , Joshua J. Fenton","doi":"10.1016/j.xkme.2025.101199","DOIUrl":"10.1016/j.xkme.2025.101199","url":null,"abstract":"<div><h3>Rationale & Objective</h3><div>Transportation insecurity is a social risk factor of particular importance to individuals with end-stage kidney disease (ESKD), as most individuals need to travel multiple times a week to dialysis treatment. Advancing home modalities for individuals with ESKD experiencing transportation insecurity may be beneficial by reducing travel burden and improving access.</div></div><div><h3>Study Design</h3><div>Retrospective cohort study.</div></div><div><h3>Setting & Participants</h3><div>Individuals with ESKD treated with in-center hemodialysis (HD) at a large, national dialysis organization.</div></div><div><h3>Exposures</h3><div>The main transportation mode to HD is categorized into private transportation (individuals who drive themselves or have a family member/friend drive) or those who lack private transportation (Medicaid non-emergency medical transportation, paratransit, public transportation, private pay non-emergency medical transportation, and other).</div></div><div><h3>Outcomes</h3><div>Transition to home dialysis is defined as an individual who has completed at least 1 training treatment for home therapies or at least 1 dialysis treatment at home.</div></div><div><h3>Analytic Approach</h3><div>Log-binomial multivariate regression models to estimate adjusted incidence rate ratios of home dialysis transition by transportation mode.</div></div><div><h3>Results</h3><div>Individuals who lacked private transportation were significantly less likely to transition to home dialysis compared with those who drove themselves or had a family member/friend drive them to HD. Adjusted incidence rate ratios for home dialysis transition were 47%-58% lower in nonprivate transportation groups compared with those with private transportation, ranging from 0.42 in individuals relying on Medicaid transportation benefits (95% confidence interval, 0.35-0.50; <em>P</em> < 0.001) to 0.53 (95% confidence interval, 0.41-0.67; <em>P</em> < 0.001) among paratransit users.</div></div><div><h3>Limitations</h3><div>Single transportation assessment, exclusion of individuals already on home dialysis, and absence of caregiver data.</div></div><div><h3>Conclusions</h3><div>Individuals with ESKD receiving in-center HD who lack private transportation may have reduced access to home dialysis, even though this group may benefit from home modalities. Better identifying transportation barriers and targeting home modalities for those with transportation insecurity may reduce the adverse consequences of missed dialysis related to transportation barriers and be an additional opportunity to increase home dialysis uptake.</div></div><div><h3>Plain-Language Summary</h3><div>Transportation is a key barrier for many individuals receiving in-center dialysis care. Nonetheless, the majority of individuals in the United States receive their dialysis treatment at an in-center facility. In a study of patients with end-stage kidney disease treate","PeriodicalId":17885,"journal":{"name":"Kidney Medicine","volume":"8 2","pages":"Article 101199"},"PeriodicalIF":3.4,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146023299","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}
Pub Date : 2026-02-01Epub Date: 2025-12-12DOI: 10.1016/j.xkme.2025.101212
Jeong-Hoon Lim , Youn-Sik Oh , Man-Hoon Han , You Hyun Jeon , Hee-Yeon Jung , Ji-Young Choi , Jang-Hee Cho , Sun-Hee Park , Chan-Duck Kim , Yong-Lim Kim , Yong-Jin Kim
Proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID) is a rare form of monoclonal gammopathy of renal significance (MGRS). The natural history and optimal treatment are poorly defined, particularly when circulating monoclonal proteins or hematologic clones are undetectable. We report a 57-year-old woman with biopsy-confirmed PGNMID who underwent 3 sequential kidney biopsies. The initial biopsy showed a classic membranoproliferative pattern with monotypic IgG-kappa deposits, whereas bone marrow examination showed no clonal plasma cell or B-cell proliferation. Despite corticosteroid-based immunosuppression, proteinuria persisted, and histology worsened on the second biopsy. Rituximab was administered 10 months after diagnosis; a third biopsy 3 months later showed resolution of immune deposits and glomerular proliferation. At 8 months post-rituximab, the patient remained in clinical remission with stable kidney function. This case underscores the value of sequential kidney biopsies to monitor disease activity and treatment response in PGNMID. Rituximab can induce clinical and histologic remission, even when circulating monoclonal proteins are undetectable.
{"title":"Rituximab-Induced Remission in Proliferative Glomerulonephritis With Monoclonal Immunoglobulin G Deposits: A Case Report With Serial Kidney Biopsies","authors":"Jeong-Hoon Lim , Youn-Sik Oh , Man-Hoon Han , You Hyun Jeon , Hee-Yeon Jung , Ji-Young Choi , Jang-Hee Cho , Sun-Hee Park , Chan-Duck Kim , Yong-Lim Kim , Yong-Jin Kim","doi":"10.1016/j.xkme.2025.101212","DOIUrl":"10.1016/j.xkme.2025.101212","url":null,"abstract":"<div><div>Proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID) is a rare form of monoclonal gammopathy of renal significance (MGRS). The natural history and optimal treatment are poorly defined, particularly when circulating monoclonal proteins or hematologic clones are undetectable. We report a 57-year-old woman with biopsy-confirmed PGNMID who underwent 3 sequential kidney biopsies. The initial biopsy showed a classic membranoproliferative pattern with monotypic IgG-kappa deposits, whereas bone marrow examination showed no clonal plasma cell or B-cell proliferation. Despite corticosteroid-based immunosuppression, proteinuria persisted, and histology worsened on the second biopsy. Rituximab was administered 10 months after diagnosis; a third biopsy 3 months later showed resolution of immune deposits and glomerular proliferation. At 8 months post-rituximab, the patient remained in clinical remission with stable kidney function. This case underscores the value of sequential kidney biopsies to monitor disease activity and treatment response in PGNMID. Rituximab can induce clinical and histologic remission, even when circulating monoclonal proteins are undetectable.</div></div>","PeriodicalId":17885,"journal":{"name":"Kidney Medicine","volume":"8 2","pages":"Article 101212"},"PeriodicalIF":3.4,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145978510","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}
Pub Date : 2026-02-01Epub Date: 2025-12-15DOI: 10.1016/j.xkme.2025.101218
Swetha R. Kanduri , Pooja Amarapurkar , Mohamed Hassanein , Sabine Karam , Juan Carlos Q. Velez , Kenar D. Jhaveri
Hypercalcemia can affect multiple organ systems and, depending on its severity, presents with a wide range of clinical manifestations. Severe hypercalcemia may lead to serious complications such as confusion and encephalopathy. Malignancy-associated hypercalcemia (MAH) occurs in up to 10%-30% of patients with cancer, with a higher incidence in those with advanced disease, and is associated with a poor prognosis. Several mechanisms have been proposed to explain MAH, the most prominent being humoral hypercalcemia of malignancy and osteolytic hypercalcemia. Although less common, recent advances have identified additional mechanisms of MAH, including novel cancer medications such as immune checkpoint inhibitors and prolonged immobilization. While various treatment modalities are available for managing hypercalcemia, dosing of antihypercalcemic medications in patients with kidney dysfunction remains challenging. This comprehensive review illustrates the mechanisms of MAH, discusses relevant investigations, and focuses on management strategies tailored to the severity of hypercalcemia in patients with cancer. Additionally, we address the dose adjustments for antihypercalcemic agents in patients with reduced glomerular filtration rate and explore potential areas for developing targeted therapies to better address MAH.
{"title":"Malignancy-Associated Hypercalcemia and Kidney Disease: Facts, Controversies, and Management","authors":"Swetha R. Kanduri , Pooja Amarapurkar , Mohamed Hassanein , Sabine Karam , Juan Carlos Q. Velez , Kenar D. Jhaveri","doi":"10.1016/j.xkme.2025.101218","DOIUrl":"10.1016/j.xkme.2025.101218","url":null,"abstract":"<div><div>Hypercalcemia can affect multiple organ systems and, depending on its severity, presents with a wide range of clinical manifestations. Severe hypercalcemia may lead to serious complications such as confusion and encephalopathy. Malignancy-associated hypercalcemia (MAH) occurs in up to 10%-30% of patients with cancer, with a higher incidence in those with advanced disease, and is associated with a poor prognosis. Several mechanisms have been proposed to explain MAH, the most prominent being humoral hypercalcemia of malignancy and osteolytic hypercalcemia. Although less common, recent advances have identified additional mechanisms of MAH, including novel cancer medications such as immune checkpoint inhibitors and prolonged immobilization. While various treatment modalities are available for managing hypercalcemia, dosing of antihypercalcemic medications in patients with kidney dysfunction remains challenging. This comprehensive review illustrates the mechanisms of MAH, discusses relevant investigations, and focuses on management strategies tailored to the severity of hypercalcemia in patients with cancer. Additionally, we address the dose adjustments for antihypercalcemic agents in patients with reduced glomerular filtration rate and explore potential areas for developing targeted therapies to better address MAH.</div></div>","PeriodicalId":17885,"journal":{"name":"Kidney Medicine","volume":"8 2","pages":"Article 101218"},"PeriodicalIF":3.4,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146023167","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}
Pub Date : 2026-02-01Epub Date: 2025-12-13DOI: 10.1016/j.xkme.2025.101216
Rivaldo José Melo Tavares , Mariana Póvoa-Corrêa , Iandy de Souza Mateus Tarricone , Simone Collopy , Roberta Lins Gonçalves , Carlos Alberto da Silva Magliano , Gaudencio Espinosa Lopez
Rationale & Objective
Patients with chronic kidney disease (CKD) often reach a point where their options for hemodialysis access are exhausted, when transhepatic and translumbar access becomes an option. The aim of this study is to compare the prevalence of complications associated with both types of catheters through a systematic review and meta-analysis.
Study Design
Literature-based systematic review and meta-analysis were accomplished in 2021/2022. Studies were obtained from 11 registries, including Medline/PubMed, Embase, and Scopus.
Setting & Study Populations
Included studies involved patients with CKD in access exhaustion who underwent translumbar or transhepatic catheter placement.
Selection Criteria for Studies
Eligible designs included clinical trials, quasi-experimental studies, observational studies, and case series; case reports were excluded.
Data Extraction
Two independent researchers used a tailored sheet to extract data from the studies.
Analytical Approach
A fixed-effect model for proportions was used to assess complications across 18 observational studies involving 649 catheters.
Results
Compared to the translumbar group, the transhepatic group showed significantly higher proportions per 100 catheter-days of irreversible infections (0.085 [95% CI, 0.051-0.118] vs 0.015 [95% CI, 0.007-0.023]; P < 0.001) and irreversible dysfunction (0.259 [95% CI, 0.205-0.313] vs 0.071 [95% CI, 0.054-0.089]; P < 0.001). Total infections (P < 0.001), thrombosis (P < 0.001), and catheter displacement (P < 0.001) were also significantly more frequent in the transhepatic group.
Limitations
The main challenge was the variability in study designs and the lack of randomized clinical trials, which was expected given the nature of the intervention.
Conclusions
Translumbar access in CKD is associated with fewer complications; however, transhepatic access remains a viable option as a bridge to definitive access or transplantation.
基本原理和目的慢性肾脏疾病（CKD）患者经常达到他们的血液透析途径选择用尽的地步，当经肝和经腰椎途径成为一种选择。本研究的目的是通过系统回顾和荟萃分析来比较两种导管相关并发症的发生率。研究设计基于文献的系统评价和荟萃分析于2021/2022年完成。研究从11个注册中心获得，包括Medline/PubMed、Embase和Scopus。背景和研究人群纳入的研究涉及经腰椎或经肝置管的CKD通路衰竭患者。研究选择标准：符合条件的设计包括临床试验、准实验研究、观察性研究和病例系列；病例报告被排除在外。数据提取两名独立研究人员使用定制的表格从研究中提取数据。采用比例固定效应模型对涉及649根导管的18项观察性研究的并发症进行评估。结果与经腰椎组相比，经肝组每100个导管日发生不可逆感染（0.085 [95% CI, 0.051-0.118] vs 0.015 [95% CI, 0.007-0.023]; P < 0.001）和不可逆功能障碍（0.259 [95% CI, 0.205-0.313] vs 0.071 [95% CI, 0.054-0.089]; P < 0.001）的比例显著高于经肝组。总感染（P < 0.001）、血栓形成（P < 0.001）和导管移位（P < 0.001）在经肝组也明显更频繁。主要的挑战是研究设计的可变性和缺乏随机临床试验，考虑到干预的性质，这是意料之中的。结论CKD患者经腰椎通路并发症较少；然而，经肝进入仍然是一个可行的选择，作为最终进入或移植的桥梁。
{"title":"Translumbar and Transhepatic Catheters for Hemodialysis in Chronic Kidney Disease: A Systematic Review and Meta-Analysis","authors":"Rivaldo José Melo Tavares , Mariana Póvoa-Corrêa , Iandy de Souza Mateus Tarricone , Simone Collopy , Roberta Lins Gonçalves , Carlos Alberto da Silva Magliano , Gaudencio Espinosa Lopez","doi":"10.1016/j.xkme.2025.101216","DOIUrl":"10.1016/j.xkme.2025.101216","url":null,"abstract":"<div><h3>Rationale & Objective</h3><div>Patients with chronic kidney disease (CKD) often reach a point where their options for hemodialysis access are exhausted, when transhepatic and translumbar access becomes an option. The aim of this study is to compare the prevalence of complications associated with both types of catheters through a systematic review and meta-analysis.</div></div><div><h3>Study Design</h3><div>Literature-based systematic review and meta-analysis were accomplished in 2021/2022. Studies were obtained from 11 registries, including Medline/PubMed, Embase, and Scopus.</div></div><div><h3>Setting & Study Populations</h3><div>Included studies involved patients with CKD in access exhaustion who underwent translumbar or transhepatic catheter placement.</div></div><div><h3>Selection Criteria for Studies</h3><div>Eligible designs included clinical trials, quasi-experimental studies, observational studies, and case series; case reports were excluded.</div></div><div><h3>Data Extraction</h3><div>Two independent researchers used a tailored sheet to extract data from the studies.</div></div><div><h3>Analytical Approach</h3><div>A fixed-effect model for proportions was used to assess complications across 18 observational studies involving 649 catheters.</div></div><div><h3>Results</h3><div>Compared to the translumbar group, the transhepatic group showed significantly higher proportions per 100 catheter-days of irreversible infections (0.085 [95% CI, 0.051-0.118] vs 0.015 [95% CI, 0.007-0.023]; <em>P</em> < 0.001) and irreversible dysfunction (0.259 [95% CI, 0.205-0.313] vs 0.071 [95% CI, 0.054-0.089]; <em>P</em> < 0.001). Total infections (<em>P</em> < 0.001), thrombosis (<em>P</em> < 0.001), and catheter displacement (<em>P</em> < 0.001) were also significantly more frequent in the transhepatic group.</div></div><div><h3>Limitations</h3><div>The main challenge was the variability in study designs and the lack of randomized clinical trials, which was expected given the nature of the intervention.</div></div><div><h3>Conclusions</h3><div>Translumbar access in CKD is associated with fewer complications; however, transhepatic access remains a viable option as a bridge to definitive access or transplantation.</div></div>","PeriodicalId":17885,"journal":{"name":"Kidney Medicine","volume":"8 2","pages":"Article 101216"},"PeriodicalIF":3.4,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146023169","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}
Pub Date : 2026-02-01Epub Date: 2025-12-10DOI: 10.1016/j.xkme.2025.101202
William Morello , Greta Armaroli , Donatella Milani , Anita Sofia Bellotti , Paola Castelli , Elena Cicchetti , Alessandro Del Gobbo , Alessandra De Franco , Giovanni Montini
Proteinuria has been linked to several genetic disorders, providing valuable insights into its pathophysiology. ReNU syndrome, a recently described condition caused by heterozygous variants in the RNU4-2 gene, is characterized by intellectual disability, microcephaly, and multisystemic features. Kidney involvement has been reported exclusively as anatomical abnormalities.
Here, we presented a girl with isolated proteinuria and ReNU syndrome. Her prenatal history was notable for a small head circumference and reduced brain hemispheres. She was referred to our clinic at 3 months of age for isolated proteinuria. Physical examination revealed microsomia, strabismus, and dysmorphic features. Kidney ultrasound was unremarkable, and edema was never observed. A kidney biopsy showed minimal change disease with slight podocyte effacement. Treatment with prednisone was ineffective, and antiproteinuric agents were started. At her last follow-up, at age 16 years, nephrotic-range proteinuria persists with normal kidney function. Genetic testing before 2024 yielded no diagnosis, but whole-genome sequencing analysis later identified a de novo variant in the RNU4-2 gene (n.64_65insT), confirming ReNU syndrome.
This case is the first documented report of isolated, persistent proteinuria in ReNU syndrome. We recommend testing for RNU4-2 variants in patients with unexplained proteinuria and syndromic features and suggest regular monitoring for proteinuria in individuals with ReNU syndrome.
{"title":"ReNU Syndrome due to a de novo RNU4-2 Variant as a Novel Genetic Cause of Proteinuria","authors":"William Morello , Greta Armaroli , Donatella Milani , Anita Sofia Bellotti , Paola Castelli , Elena Cicchetti , Alessandro Del Gobbo , Alessandra De Franco , Giovanni Montini","doi":"10.1016/j.xkme.2025.101202","DOIUrl":"10.1016/j.xkme.2025.101202","url":null,"abstract":"<div><div>Proteinuria has been linked to several genetic disorders, providing valuable insights into its pathophysiology. ReNU syndrome, a recently described condition caused by heterozygous variants in the <em>RNU4-2</em> gene, is characterized by intellectual disability, microcephaly, and multisystemic features. Kidney involvement has been reported exclusively as anatomical abnormalities.</div><div>Here, we presented a girl with isolated proteinuria and ReNU syndrome. Her prenatal history was notable for a small head circumference and reduced brain hemispheres. She was referred to our clinic at 3 months of age for isolated proteinuria. Physical examination revealed microsomia, strabismus, and dysmorphic features. Kidney ultrasound was unremarkable, and edema was never observed. A kidney biopsy showed minimal change disease with slight podocyte effacement. Treatment with prednisone was ineffective, and antiproteinuric agents were started. At her last follow-up, at age 16 years, nephrotic-range proteinuria persists with normal kidney function. Genetic testing before 2024 yielded no diagnosis, but whole-genome sequencing analysis later identified a de novo variant in the <em>RNU4-2</em> gene (n.64_65insT), confirming ReNU syndrome.</div><div>This case is the first documented report of isolated, persistent proteinuria in ReNU syndrome. We recommend testing for <em>RNU4-2</em> variants in patients with unexplained proteinuria and syndromic features and suggest regular monitoring for proteinuria in individuals with ReNU syndrome.</div></div>","PeriodicalId":17885,"journal":{"name":"Kidney Medicine","volume":"8 2","pages":"Article 101202"},"PeriodicalIF":3.4,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146023172","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}
Pub Date : 2026-02-01Epub Date: 2025-12-16DOI: 10.1016/j.xkme.2025.101228
Yujia Wang , Kaiqi Huang , Caiming Chen , Keng Ye , Kunmei Lai , Xuan Tao , Hong Chen , Yanfang Xu
Drug-induced type II crescentic glomerulonephritis is an uncommon but severe renal syndrome, rarely linked to pharmaceutical agents, with no established association to Chinese herbal medicines to date. In this case report, we described 4 patients who developed biopsy-confirmed type II crescentic glomerulonephritis within 1-4 weeks following exposure to Chinese herbal products. Key histopathologic findings included crescent formation in ≥50% of glomeruli and immunofluorescence demonstrating granular mesangial deposits of immunoglobulin G and C3, accompanied by prominent acute tubulointerstitial injury. Clinically, 75% of cases presented with macroscopic hematuria, reflecting severe glomerular capillary damage. The therapeutic regimen comprised intravenous methylprednisolone, cyclophosphamide, and plasma exchange. Persistent hematuria and a longer duration of drug exposure were associated with slower and incomplete renal recovery during follow-up. Our case report suggested that Chinese herbal medicine may be a potential environmental trigger for crescentic glomerulonephritis and highlight the value of clinical inquiry into herbal product use in cases of rapidly progressive glomerulonephritis. Further toxicovigilance and compound-specific risk assessments are urgently needed.
{"title":"Chinese Herb-Induced Type II Crescentic Glomerulonephritis: A Case Report of Four Patients","authors":"Yujia Wang , Kaiqi Huang , Caiming Chen , Keng Ye , Kunmei Lai , Xuan Tao , Hong Chen , Yanfang Xu","doi":"10.1016/j.xkme.2025.101228","DOIUrl":"10.1016/j.xkme.2025.101228","url":null,"abstract":"<div><div>Drug-induced type II crescentic glomerulonephritis is an uncommon but severe renal syndrome, rarely linked to pharmaceutical agents, with no established association to Chinese herbal medicines to date. In this case report, we described 4 patients who developed biopsy-confirmed type II crescentic glomerulonephritis within 1-4 weeks following exposure to Chinese herbal products. Key histopathologic findings included crescent formation in ≥50% of glomeruli and immunofluorescence demonstrating granular mesangial deposits of immunoglobulin G and C3, accompanied by prominent acute tubulointerstitial injury. Clinically, 75% of cases presented with macroscopic hematuria, reflecting severe glomerular capillary damage. The therapeutic regimen comprised intravenous methylprednisolone, cyclophosphamide, and plasma exchange. Persistent hematuria and a longer duration of drug exposure were associated with slower and incomplete renal recovery during follow-up. Our case report suggested that Chinese herbal medicine may be a potential environmental trigger for crescentic glomerulonephritis and highlight the value of clinical inquiry into herbal product use in cases of rapidly progressive glomerulonephritis. Further toxicovigilance and compound-specific risk assessments are urgently needed.</div></div>","PeriodicalId":17885,"journal":{"name":"Kidney Medicine","volume":"8 2","pages":"Article 101228"},"PeriodicalIF":3.4,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146023174","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}
Fanconi syndrome is a generalized dysfunction of the renal proximal tubule, leading to growth failure and rickets during childhood. There are few reports of this syndrome in neonates, especially in extremely low-birth-weight infants. We present a case of an extremely low-birth-weight Asian girl with bone fragility because of Fanconi syndrome without underlying diseases. She was born at 29 weeks of gestation and weighed 418 g (–5.2SD). Based on blood and urine analyses, she was diagnosed with Fanconi syndrome. Metabolic acidosis was easily corrected with bicarbonate supplementation. However, the control of the rickets was very difficult, with multiple bone fractures observed despite supplementation of calcium, phosphorus, and vitamin D. Her renal tubular function finally improved at 6 months of age, and oral supplementation of bicarbonate, calcium, and phosphorus was discontinued before discharge. The genetic test for inherited causes of Fanconi syndrome showed no abnormalities. Hypoperfusion during the fetal period was assumed to be one of the causes of Fanconi syndrome in this case.
{"title":"An Extremely Low-Birth-Weight Infant With Bone Fragility Due to Fanconi Syndrome","authors":"Rei Yoshida, Miku Hosokawa, Toshiko Ukawa, Chisa Tsurisawa, Yoshiya Hisaeda, Shusuke Amagata, Tomohiro Takeda, Atsushi Nakao","doi":"10.1016/j.xkme.2025.101227","DOIUrl":"10.1016/j.xkme.2025.101227","url":null,"abstract":"<div><div>Fanconi syndrome is a generalized dysfunction of the renal proximal tubule, leading to growth failure and rickets during childhood. There are few reports of this syndrome in neonates, especially in extremely low-birth-weight infants. We present a case of an extremely low-birth-weight Asian girl with bone fragility because of Fanconi syndrome without underlying diseases. She was born at 29 weeks of gestation and weighed 418 g (–5.2SD). Based on blood and urine analyses, she was diagnosed with Fanconi syndrome. Metabolic acidosis was easily corrected with bicarbonate supplementation. However, the control of the rickets was very difficult, with multiple bone fractures observed despite supplementation of calcium, phosphorus, and vitamin D. Her renal tubular function finally improved at 6 months of age, and oral supplementation of bicarbonate, calcium, and phosphorus was discontinued before discharge. The genetic test for inherited causes of Fanconi syndrome showed no abnormalities. Hypoperfusion during the fetal period was assumed to be one of the causes of Fanconi syndrome in this case.</div></div>","PeriodicalId":17885,"journal":{"name":"Kidney Medicine","volume":"8 2","pages":"Article 101227"},"PeriodicalIF":3.4,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146023175","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}
Pub Date : 2026-02-01Epub Date: 2025-12-16DOI: 10.1016/j.xkme.2025.101223
Savino Sciascia , Massimo Radin , Irene Cecchi , Alice Barinotti , Francesco Trevisani , Roberta Fenoglio , Emanuele De Simone , Dario Roccatello , Paola Cassoni , Antonella Barreca
<div><h3>Rationale & Objective</h3><div>Thrombotic microangiopathy (TMA) as seen in antiphospholipid syndrome nephropathy (APSN) is associated with poor kidney outcomes. Caveolin-1 (Cav-1) has emerged as a potential marker of endothelial damage and microvascular injury.</div></div><div><h3>Study Design</h3><div>This retrospective study investigates the diagnostic potential value of Cav-1 immunohistochemistry in APSN-related TMA.</div></div><div><h3>Setting & Participants</h3><div>Patients were retrieved from the Piedmont and Aosta Valley Rare Disease Registry, from which a total of 771 APS cases had been recorded.</div></div><div><h3>Exposure</h3><div>Only case with biopsy-proven renal involvement were included.</div></div><div><h3>Outcomes</h3><div>Cases were classified into three histopathological clusters: TMA (APSN-TMA), hyperplastic vasculopathy, and subendothelial edema. Immunohistochemical staining for Cav-1 was performed and graded based on expression in peritubular and glomerular capillaries.</div></div><div><h3>Analytical Approach</h3><div>Clinical and laboratory data, including antiphospholipid antibody profiles, were correlated with Cav-1 expression.</div></div><div><h3>Results</h3><div>Out of 771 patients with APS, a total of 11 patients with primary APS and renal involvement were retrieved (1.43%). APSN-TMA was identified in 7 (63.3%) cases, hyperplastic vasculopathy in 2 (18.1%), and subendothelial edema in 2 (18.1%). Cav-1 expression was significantly higher in APSN-TMA cases in both glomerular (<em>P</em> < 0.001) and peritubular (<em>P</em> < 0.05) capillaries compared with other clusters, with 5 of 7 APSN-TMA cases exhibiting diffuse (grade III) staining. Triple antiphospholipid antibody positive testing was more prevalent in APSN-TMA cases, reinforcing their distinct pathophysiological profile.</div></div><div><h3>Limitations</h3><div>The small sample size warrants validation in larger cohorts, and Cav-1 expression, although associated with APSN, might not be disease-specific.</div></div><div><h3>Conclusions</h3><div>APSN-TMA is a rare manifestation of a rare disease. Cav-1 is strongly associated with APSN-TMA and may serve as a novel marker for its diagnosis and stratification. Given the poor renal prognosis of APSN-TMA, identifying affected patients is crucial for optimizing management strategies.</div></div><div><h3>Plain-Language Summary</h3><div>Antiphospholipid syndrome (APS) is an autoimmune condition that can cause blood clots in many organs, including the kidneys. In some patients, a type of severe small-vessel injury called thrombotic microangiopathy develops, leading to poor kidney outcomes. Diagnosing this form of kidney damage can be challenging. In our study, we examined kidney tissue from people with APS using a special stain for a protein called Caveolin-1, which is found in damaged blood vessel cells. We found that high Caveolin-1 levels were strongly linked to the most aggressive form of APS-rel
{"title":"Caveolin-1 as a Marker of Endothelial Damage in Primary Antiphospholipid Syndrome Nephropathy","authors":"Savino Sciascia , Massimo Radin , Irene Cecchi , Alice Barinotti , Francesco Trevisani , Roberta Fenoglio , Emanuele De Simone , Dario Roccatello , Paola Cassoni , Antonella Barreca","doi":"10.1016/j.xkme.2025.101223","DOIUrl":"10.1016/j.xkme.2025.101223","url":null,"abstract":"<div><h3>Rationale & Objective</h3><div>Thrombotic microangiopathy (TMA) as seen in antiphospholipid syndrome nephropathy (APSN) is associated with poor kidney outcomes. Caveolin-1 (Cav-1) has emerged as a potential marker of endothelial damage and microvascular injury.</div></div><div><h3>Study Design</h3><div>This retrospective study investigates the diagnostic potential value of Cav-1 immunohistochemistry in APSN-related TMA.</div></div><div><h3>Setting & Participants</h3><div>Patients were retrieved from the Piedmont and Aosta Valley Rare Disease Registry, from which a total of 771 APS cases had been recorded.</div></div><div><h3>Exposure</h3><div>Only case with biopsy-proven renal involvement were included.</div></div><div><h3>Outcomes</h3><div>Cases were classified into three histopathological clusters: TMA (APSN-TMA), hyperplastic vasculopathy, and subendothelial edema. Immunohistochemical staining for Cav-1 was performed and graded based on expression in peritubular and glomerular capillaries.</div></div><div><h3>Analytical Approach</h3><div>Clinical and laboratory data, including antiphospholipid antibody profiles, were correlated with Cav-1 expression.</div></div><div><h3>Results</h3><div>Out of 771 patients with APS, a total of 11 patients with primary APS and renal involvement were retrieved (1.43%). APSN-TMA was identified in 7 (63.3%) cases, hyperplastic vasculopathy in 2 (18.1%), and subendothelial edema in 2 (18.1%). Cav-1 expression was significantly higher in APSN-TMA cases in both glomerular (<em>P</em> < 0.001) and peritubular (<em>P</em> < 0.05) capillaries compared with other clusters, with 5 of 7 APSN-TMA cases exhibiting diffuse (grade III) staining. Triple antiphospholipid antibody positive testing was more prevalent in APSN-TMA cases, reinforcing their distinct pathophysiological profile.</div></div><div><h3>Limitations</h3><div>The small sample size warrants validation in larger cohorts, and Cav-1 expression, although associated with APSN, might not be disease-specific.</div></div><div><h3>Conclusions</h3><div>APSN-TMA is a rare manifestation of a rare disease. Cav-1 is strongly associated with APSN-TMA and may serve as a novel marker for its diagnosis and stratification. Given the poor renal prognosis of APSN-TMA, identifying affected patients is crucial for optimizing management strategies.</div></div><div><h3>Plain-Language Summary</h3><div>Antiphospholipid syndrome (APS) is an autoimmune condition that can cause blood clots in many organs, including the kidneys. In some patients, a type of severe small-vessel injury called thrombotic microangiopathy develops, leading to poor kidney outcomes. Diagnosing this form of kidney damage can be challenging. In our study, we examined kidney tissue from people with APS using a special stain for a protein called Caveolin-1, which is found in damaged blood vessel cells. We found that high Caveolin-1 levels were strongly linked to the most aggressive form of APS-rel","PeriodicalId":17885,"journal":{"name":"Kidney Medicine","volume":"8 2","pages":"Article 101223"},"PeriodicalIF":3.4,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146023239","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}
Pub Date : 2026-02-01Epub Date: 2025-12-09DOI: 10.1016/j.xkme.2025.101198
Ramy Magdy Hanna , Shruti Chaturvedi , Moh-Lim Ong , Arpita Nag , Rui Song , Lynn Huynh , Jordan A. Burdeau , Mei Sheng Duh , Yan Wang
<div><h3>Rationale & Objective</h3><div>Atypical hemolytic uremic syndrome (aHUS) is a rare form of thrombotic microangiopathy (TMA) caused by complement dysregulation. Ravulizumab, a complement C5 inhibitor (C5i), is approved for aHUS; however, published evidence in a real-world setting is limited.</div></div><div><h3>Study Design</h3><div>Retrospective, longitudinal, physician panel-based chart review.</div></div><div><h3>Setting & Population</h3><div>C5i-naive adults with aHUS in the United States treated with ravulizumab. Physicians randomly selected 1-5 patients who had ≥6 months of follow-up after ravulizumab initiation; patients who died within 6 months of initiation were eligible.</div></div><div><h3>Exposure(s) or Predictor(s)</h3><div>Ravulizumab.</div></div><div><h3>Outcomes</h3><div>The clinical outcomes evaluated included hematologic and renal outcomes, complete TMA response (a composite hematolgic/renal endpoint), and dialysis use.</div></div><div><h3>Analytical Approach</h3><div>Descriptive statistics, Kaplan-Meier estimators, and generalized linear models.</div></div><div><h3>Results</h3><div>Overall, 79 C5i-naive adults with aHUS (enrolled by 31 physicians) initiated ravulizumab and were included in the study. Statistically significant improvements from baseline occurred as early as day 4 (lactate dehydrogenase and percent change in serum creatinine; both <em>P</em> < 0.001) and day 8 (platelet count; <em>P</em> < 0.001). The proportions of patients with normalization of platelet counts and lactate dehydrogenase levels, and ≥25% improvement in serum creatinine levels, were 14 out of 67 (21%), 12 out of 58 (21%), and 10 out of 65 (15%) at day 4, and 40 out of 48 (83%), 35 out of 38 (92%), and 42 out of 48 (88%) at 12 months after ravulizumab initiation, respectively. Complete TMA response rates were 60% and 68% within 6 and 12 months after ravulizumab initiation, respectively, and the median (interquartile range) time to complete TMA response was 3.1 (1.0-14.0) months. Of the 20 patients who received any dialysis at baseline, 14 (70.0%) did not have dialysis during follow-up.</div></div><div><h3>Limitations</h3><div>The study design relies on available medical record data and has potential responder bias.</div></div><div><h3>Conclusions</h3><div>This study supports the immediate and sustained benefits of initiating ravulizumab in patients with aHUS as seen by the early response and continued improvement in clinical outcomes.</div></div><div><h3>Plain-Language Summary</h3><div>This study evaluates the use of a treatment called ravulizumab for atypical hemolytic uremic syndrome (aHUS), a rare disease that causes clots in small blood vessels and can lead to kidney failure. Researchers reviewed the medical records of 79 adults with aHUS in the United States who were treated with ravulizumab and had never used similar treatments before. Significant improvements in laboratory measures including lactate dehydrogenase and se
{"title":"Real-World Effectiveness of Ravulizumab Among C5 Inhibitor-Naive Patients With Atypical Hemolytic Uremic Syndrome: A Physician Panel-Based Chart Review (aHUS IMPACT Study)","authors":"Ramy Magdy Hanna , Shruti Chaturvedi , Moh-Lim Ong , Arpita Nag , Rui Song , Lynn Huynh , Jordan A. Burdeau , Mei Sheng Duh , Yan Wang","doi":"10.1016/j.xkme.2025.101198","DOIUrl":"10.1016/j.xkme.2025.101198","url":null,"abstract":"<div><h3>Rationale & Objective</h3><div>Atypical hemolytic uremic syndrome (aHUS) is a rare form of thrombotic microangiopathy (TMA) caused by complement dysregulation. Ravulizumab, a complement C5 inhibitor (C5i), is approved for aHUS; however, published evidence in a real-world setting is limited.</div></div><div><h3>Study Design</h3><div>Retrospective, longitudinal, physician panel-based chart review.</div></div><div><h3>Setting & Population</h3><div>C5i-naive adults with aHUS in the United States treated with ravulizumab. Physicians randomly selected 1-5 patients who had ≥6 months of follow-up after ravulizumab initiation; patients who died within 6 months of initiation were eligible.</div></div><div><h3>Exposure(s) or Predictor(s)</h3><div>Ravulizumab.</div></div><div><h3>Outcomes</h3><div>The clinical outcomes evaluated included hematologic and renal outcomes, complete TMA response (a composite hematolgic/renal endpoint), and dialysis use.</div></div><div><h3>Analytical Approach</h3><div>Descriptive statistics, Kaplan-Meier estimators, and generalized linear models.</div></div><div><h3>Results</h3><div>Overall, 79 C5i-naive adults with aHUS (enrolled by 31 physicians) initiated ravulizumab and were included in the study. Statistically significant improvements from baseline occurred as early as day 4 (lactate dehydrogenase and percent change in serum creatinine; both <em>P</em> < 0.001) and day 8 (platelet count; <em>P</em> < 0.001). The proportions of patients with normalization of platelet counts and lactate dehydrogenase levels, and ≥25% improvement in serum creatinine levels, were 14 out of 67 (21%), 12 out of 58 (21%), and 10 out of 65 (15%) at day 4, and 40 out of 48 (83%), 35 out of 38 (92%), and 42 out of 48 (88%) at 12 months after ravulizumab initiation, respectively. Complete TMA response rates were 60% and 68% within 6 and 12 months after ravulizumab initiation, respectively, and the median (interquartile range) time to complete TMA response was 3.1 (1.0-14.0) months. Of the 20 patients who received any dialysis at baseline, 14 (70.0%) did not have dialysis during follow-up.</div></div><div><h3>Limitations</h3><div>The study design relies on available medical record data and has potential responder bias.</div></div><div><h3>Conclusions</h3><div>This study supports the immediate and sustained benefits of initiating ravulizumab in patients with aHUS as seen by the early response and continued improvement in clinical outcomes.</div></div><div><h3>Plain-Language Summary</h3><div>This study evaluates the use of a treatment called ravulizumab for atypical hemolytic uremic syndrome (aHUS), a rare disease that causes clots in small blood vessels and can lead to kidney failure. Researchers reviewed the medical records of 79 adults with aHUS in the United States who were treated with ravulizumab and had never used similar treatments before. Significant improvements in laboratory measures including lactate dehydrogenase and se","PeriodicalId":17885,"journal":{"name":"Kidney Medicine","volume":"8 2","pages":"Article 101198"},"PeriodicalIF":3.4,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146023245","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}
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