Pub Date : 2026-01-01Epub Date: 2025-08-13DOI: 10.1159/000547628
Hidenori Amaike, Masatoshi Kanda, Hirotsugu Yamazaki, Koki Nakamura, Li Ma, Ken Nagahata, Hiroyuki Nakamura, Arata Osanami, Naoya Yama, Masamitsu Hatakenaka, Masato Furuhashi, Hiroki Takahashi
Objectives: The aim of the study was to compare the efficacy of magnetic resonance imaging (MRI) with that of plain or contrast-enhanced computed tomography (CT) in the detection of renal parenchymal and pelvic lesions of immunoglobulin G4-related kidney disease (IgG4-RKD).
Methods: Patients with IgG4-RKD and controls, who performed plain, contrast-enhanced CT and MRI around the kidney region in our hospital, were enrolled. The diagnosis of IgG4-RKD was made by definite cases of IgG4-RKD diagnostic criteria in 2020. Five blinded observers independently assessed image datasets by confidence scores to assess diagnostic accuracy, sensitivity, specificity, areas under the receiver operating characteristic curve (AUROC), and Cronbach's alpha coefficient.
Results: A total of 31 patients were included in the study. Fourteen (45.2%) had IgG4-RKD. Five patients with IgG4-RKD had parenchymal lesions, 5 had renal pelvic lesions, and 4 had both. In the parenchymal lesions, there was no significant difference in diagnostic performance between contrast-enhanced CT and diffusion-weighted imaging (DWI)-b800. The AUROC and sensitivity were higher in DWI-b800 than in plain CT (p < 0.05). Cronbach's alpha coefficient was 0.44 for plain CT and over 0.80 for contrast-enhanced CT and DWI-b800. In the pelvic lesions, there were fewer differences in the performance among each sequence. Cronbach's alpha coefficient was over 0.80 for plain CT, contrast-enhanced CT, and DWI-b800.
Conclusion: Plain MRI, especially in DWI-b800, can effectively detect renal parenchymal lesions in IgG4-RKD. In cases where the use of a contrast agent of CT is difficult, DWI-b800 can be an alternative for the screening of IgG4-RKD.
{"title":"The Effectiveness of Lesion Detection for Immunoglobulin G4-Related Kidney Diseases by Diffusion-Weighted Imaging.","authors":"Hidenori Amaike, Masatoshi Kanda, Hirotsugu Yamazaki, Koki Nakamura, Li Ma, Ken Nagahata, Hiroyuki Nakamura, Arata Osanami, Naoya Yama, Masamitsu Hatakenaka, Masato Furuhashi, Hiroki Takahashi","doi":"10.1159/000547628","DOIUrl":"10.1159/000547628","url":null,"abstract":"<p><strong>Objectives: </strong>The aim of the study was to compare the efficacy of magnetic resonance imaging (MRI) with that of plain or contrast-enhanced computed tomography (CT) in the detection of renal parenchymal and pelvic lesions of immunoglobulin G4-related kidney disease (IgG4-RKD).</p><p><strong>Methods: </strong>Patients with IgG4-RKD and controls, who performed plain, contrast-enhanced CT and MRI around the kidney region in our hospital, were enrolled. The diagnosis of IgG4-RKD was made by definite cases of IgG4-RKD diagnostic criteria in 2020. Five blinded observers independently assessed image datasets by confidence scores to assess diagnostic accuracy, sensitivity, specificity, areas under the receiver operating characteristic curve (AUROC), and Cronbach's alpha coefficient.</p><p><strong>Results: </strong>A total of 31 patients were included in the study. Fourteen (45.2%) had IgG4-RKD. Five patients with IgG4-RKD had parenchymal lesions, 5 had renal pelvic lesions, and 4 had both. In the parenchymal lesions, there was no significant difference in diagnostic performance between contrast-enhanced CT and diffusion-weighted imaging (DWI)-b800. The AUROC and sensitivity were higher in DWI-b800 than in plain CT (p < 0.05). Cronbach's alpha coefficient was 0.44 for plain CT and over 0.80 for contrast-enhanced CT and DWI-b800. In the pelvic lesions, there were fewer differences in the performance among each sequence. Cronbach's alpha coefficient was over 0.80 for plain CT, contrast-enhanced CT, and DWI-b800.</p><p><strong>Conclusion: </strong>Plain MRI, especially in DWI-b800, can effectively detect renal parenchymal lesions in IgG4-RKD. In cases where the use of a contrast agent of CT is difficult, DWI-b800 can be an alternative for the screening of IgG4-RKD.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"1-11"},"PeriodicalIF":1.8,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144855851","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}
Pub Date : 2026-01-01Epub Date: 2025-11-13DOI: 10.1159/000548254
Mijie Guan, Haofei Hu, Haiying Song, Qijun Wan
Introduction: Oxalate nephropathy, characterized by calcium oxalate crystal deposition in renal tissue, represents an underrecognized etiology of acute and chronic kidney injury. Secondary hyperoxaluria can emerge from diverse pathogenetic mechanisms, including excessive oxalate precursor intake, augmented intestinal absorption, or iatrogenic interventions. The therapeutic potential of glucocorticoids in managing this condition remains incompletely elucidated.
Case presentation: A 66-year-old man with no significant prior medical history developed acute kidney injury (AKI), manifesting as a profound serum creatinine elevation from 81 µmol/L to 1,140.4 µmol/L. The patient had consumed a "corn germ powder" supplement containing lactitol for five consecutive months, concurrently experiencing persistent diarrhea. Initial laboratory investigations revealed severe renal dysfunction without notable proteinuria or hematuria. Renal ultrasonography demonstrated normal kidney morphology and dimensions. Definitive kidney biopsy revealed extensive calcium oxalate crystal deposition within renal tubular structures, conclusively diagnosing oxalate nephropathy. Therapeutic intervention comprised prednisone (60 mg daily) and comprehensive supportive management. Following a 3-month treatment protocol with gradual corticosteroid dose reduction, the patient's renal function demonstrated substantial improvement, with serum creatinine declining to 118.2 µmol/L.
Conclusion: This case underscores lactitol-induced secondary oxalate nephropathy as a rare yet clinically significant contributor to AKI. Prompt diagnostic recognition and targeted therapeutic intervention, potentially incorporating glucocorticoid therapy, may substantially facilitate renal functional recovery. Clinicians should maintain heightened awareness of nephrotoxic risks associated with over-the-counter laxative supplements and consider oxalate nephropathy in cryptogenic renal dysfunction scenarios.
{"title":"Lactitol-Induced Acute Kidney Injury with Oxalate Nephropathy: A Case Report.","authors":"Mijie Guan, Haofei Hu, Haiying Song, Qijun Wan","doi":"10.1159/000548254","DOIUrl":"10.1159/000548254","url":null,"abstract":"<p><strong>Introduction: </strong>Oxalate nephropathy, characterized by calcium oxalate crystal deposition in renal tissue, represents an underrecognized etiology of acute and chronic kidney injury. Secondary hyperoxaluria can emerge from diverse pathogenetic mechanisms, including excessive oxalate precursor intake, augmented intestinal absorption, or iatrogenic interventions. The therapeutic potential of glucocorticoids in managing this condition remains incompletely elucidated.</p><p><strong>Case presentation: </strong>A 66-year-old man with no significant prior medical history developed acute kidney injury (AKI), manifesting as a profound serum creatinine elevation from 81 µmol/L to 1,140.4 µmol/L. The patient had consumed a \"corn germ powder\" supplement containing lactitol for five consecutive months, concurrently experiencing persistent diarrhea. Initial laboratory investigations revealed severe renal dysfunction without notable proteinuria or hematuria. Renal ultrasonography demonstrated normal kidney morphology and dimensions. Definitive kidney biopsy revealed extensive calcium oxalate crystal deposition within renal tubular structures, conclusively diagnosing oxalate nephropathy. Therapeutic intervention comprised prednisone (60 mg daily) and comprehensive supportive management. Following a 3-month treatment protocol with gradual corticosteroid dose reduction, the patient's renal function demonstrated substantial improvement, with serum creatinine declining to 118.2 µmol/L.</p><p><strong>Conclusion: </strong>This case underscores lactitol-induced secondary oxalate nephropathy as a rare yet clinically significant contributor to AKI. Prompt diagnostic recognition and targeted therapeutic intervention, potentially incorporating glucocorticoid therapy, may substantially facilitate renal functional recovery. Clinicians should maintain heightened awareness of nephrotoxic risks associated with over-the-counter laxative supplements and consider oxalate nephropathy in cryptogenic renal dysfunction scenarios.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"99-106"},"PeriodicalIF":1.8,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145513623","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}
Pub Date : 2026-01-01Epub Date: 2025-08-02DOI: 10.1159/000547796
Gabriel Sartori Pacini, Renato George Eick, Renata Asnis Schuchmann, Mário Sergio Fernandes, Lucas Gobetti da Luz, Illan George Balestrin, Karla Lais Pêgas, Milton Kalil, Maurício Lutzky
Background: Thrombotic microangiopathy (TMA) encompasses a group of rare, life-threatening disorders characterized by microangiopathic hemolytic anemia, thrombocytopenia, and organ damage, most commonly affecting the kidneys. Complement-mediated TMA (CM-TMA), a subtype of TMA, is often associated with dysregulation of the complement system due to genetic mutations. Dengue virus has been recognized as a potential trigger of secondary TMA and may precipitate CM-TMA in genetically predisposed individuals.
Case presentation: We report the case of a 47-year-old woman with a history of thrombotic thrombocytopenic purpura (TTP) who presented with fever, gastrointestinal symptoms, anemia, thrombocytopenia, and acute kidney injury. Dengue infection was confirmed by a positive NS1 antigen. Laboratory and peripheral smear findings indicated TMA. Therapeutic plasma exchange was started due to previous history of TTP, with partial clinical response. ADAMTS13 activity was preserved at 60.7%. Kidney biopsy demonstrated features of TMA. Genetic testing identified a heterozygous pathogenic variant in the CD46 gene, supporting the diagnosis of CM-TMA. Notably, the patient showed sustained clinical improvement without the use of eculizumab.
Conclusion: This case illustrates the diagnostic challenges of TMA in patients with overlapping clinical features and potential infectious triggers. In dengue-endemic regions, the virus should be recognized as a possible precipitating factor for TMA, particularly in individuals harboring complement gene mutations. A multidisciplinary approach - integrating clinical, laboratory, histopathological, and genetic data - is essential for accurate diagnosis and personalized management of TMA syndromes.
{"title":"Differential Diagnosis of Thrombotic Microangiopathy: Overlapping Features of Thrombotic Thrombocytopenic Purpura and Complement-Mediated Thrombotic Microangiopathy in a Dengue-Infected Patient.","authors":"Gabriel Sartori Pacini, Renato George Eick, Renata Asnis Schuchmann, Mário Sergio Fernandes, Lucas Gobetti da Luz, Illan George Balestrin, Karla Lais Pêgas, Milton Kalil, Maurício Lutzky","doi":"10.1159/000547796","DOIUrl":"10.1159/000547796","url":null,"abstract":"<p><strong>Background: </strong>Thrombotic microangiopathy (TMA) encompasses a group of rare, life-threatening disorders characterized by microangiopathic hemolytic anemia, thrombocytopenia, and organ damage, most commonly affecting the kidneys. Complement-mediated TMA (CM-TMA), a subtype of TMA, is often associated with dysregulation of the complement system due to genetic mutations. Dengue virus has been recognized as a potential trigger of secondary TMA and may precipitate CM-TMA in genetically predisposed individuals.</p><p><strong>Case presentation: </strong>We report the case of a 47-year-old woman with a history of thrombotic thrombocytopenic purpura (TTP) who presented with fever, gastrointestinal symptoms, anemia, thrombocytopenia, and acute kidney injury. Dengue infection was confirmed by a positive NS1 antigen. Laboratory and peripheral smear findings indicated TMA. Therapeutic plasma exchange was started due to previous history of TTP, with partial clinical response. ADAMTS13 activity was preserved at 60.7%. Kidney biopsy demonstrated features of TMA. Genetic testing identified a heterozygous pathogenic variant in the CD46 gene, supporting the diagnosis of CM-TMA. Notably, the patient showed sustained clinical improvement without the use of eculizumab.</p><p><strong>Conclusion: </strong>This case illustrates the diagnostic challenges of TMA in patients with overlapping clinical features and potential infectious triggers. In dengue-endemic regions, the virus should be recognized as a possible precipitating factor for TMA, particularly in individuals harboring complement gene mutations. A multidisciplinary approach - integrating clinical, laboratory, histopathological, and genetic data - is essential for accurate diagnosis and personalized management of TMA syndromes.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"41-46"},"PeriodicalIF":1.8,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144775858","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}
Pub Date : 2026-01-01Epub Date: 2025-08-23DOI: 10.1159/000547798
Liyin Guo, Tian Wang
Introduction: This study examined the relationship between the dietary inflammatory index (DII) and anemia in patients with diabetic kidney disease (DKD).
Methods: All the data were obtained from the National Health and Nutrition Examination Survey (NHANES) from 2007 to 2018. The final analysis included 1918 DKD patients, with 500 experiencing anemia. Weighted multivariate logistic regression models were used to assess the association between DII and anemia, with results expressed as odds ratios.
Results: Elevated DII scores correlated with an increased incidence of anemia (OR = 1.13, 95% CI: 1.02-1.25). When analyzed as categorical variables, DII scores of 1.57-2.64 (OR = 1.77, 95% CI: 1.13-2.77) and >2.64 (OR = 1.78, 95% CI: 1.12-2.85) were linked to higher anemia risk. Subgroup analyses revealed consistent associations in individuals aged 65 and older (OR = 1.92, 95% CI: 1.16-3.17), those with serum iron levels <73 μg/dL (OR = 2.01, 95% CI: 1.11-3.62), and males (OR = 2.23, 95% CI: 1.21-4.11). Higher DII scores correlated with greater odds of moderate-severe anemia (OR = 1.22, 95% CI: 1.07-1.38).
Conclusion: The results indicate that elevated DII scores are associated with an increased occurrence of anemia in DKD patients, emphasizing the importance of lowering pro-inflammatory food consumption as a potential strategy to prevent anemia in this group.
{"title":"The Relationship of Dietary Inflammatory Index with Anemia in Diabetic Kidney Disease: A Cross-Sectional Study of the NHANES.","authors":"Liyin Guo, Tian Wang","doi":"10.1159/000547798","DOIUrl":"10.1159/000547798","url":null,"abstract":"<p><strong>Introduction: </strong>This study examined the relationship between the dietary inflammatory index (DII) and anemia in patients with diabetic kidney disease (DKD).</p><p><strong>Methods: </strong>All the data were obtained from the National Health and Nutrition Examination Survey (NHANES) from 2007 to 2018. The final analysis included 1918 DKD patients, with 500 experiencing anemia. Weighted multivariate logistic regression models were used to assess the association between DII and anemia, with results expressed as odds ratios.</p><p><strong>Results: </strong>Elevated DII scores correlated with an increased incidence of anemia (OR = 1.13, 95% CI: 1.02-1.25). When analyzed as categorical variables, DII scores of 1.57-2.64 (OR = 1.77, 95% CI: 1.13-2.77) and >2.64 (OR = 1.78, 95% CI: 1.12-2.85) were linked to higher anemia risk. Subgroup analyses revealed consistent associations in individuals aged 65 and older (OR = 1.92, 95% CI: 1.16-3.17), those with serum iron levels <73 μg/dL (OR = 2.01, 95% CI: 1.11-3.62), and males (OR = 2.23, 95% CI: 1.21-4.11). Higher DII scores correlated with greater odds of moderate-severe anemia (OR = 1.22, 95% CI: 1.07-1.38).</p><p><strong>Conclusion: </strong>The results indicate that elevated DII scores are associated with an increased occurrence of anemia in DKD patients, emphasizing the importance of lowering pro-inflammatory food consumption as a potential strategy to prevent anemia in this group.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"52-62"},"PeriodicalIF":1.8,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144962187","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}
Pub Date : 2026-01-01Epub Date: 2025-10-18DOI: 10.1159/000549074
Yamini Mallisetty, Ahmed Kabeil, Cameron Jackson Bergeron, Zhongji Han, Chi-Yang Chiu, Csaba P Kovesdy, Keiichi Sumida
Introduction: Elevated monocyte to lymphocyte ratio (MLR) represents a pro-inflammatory immune microenvironment and has been associated with poor clinical outcomes such as cardiovascular disease. However, little is known about its association with outcomes among patients on hemodialysis.
Methods: In a nationwide prospective cohort of 952 patients receiving maintenance hemodialysis from 2011-2013, we examined the association of baseline MLR with subsequent risk of cardiovascular events (cardiovascular-related hospitalization and cardiovascular death) and all-cause mortality, using multivariable Cox proportional-hazards models with adjustment for potential confounders. We also examined the mediation effect of inflammatory markers on the association between MLR and cardiovascular events and all-cause mortality, respectively.
Results: Overall, patients were 60.1 ± 13.4 years old; 53.3% were male; 39.7% were African American; and 56.9% were diabetic. The median dialysis vintage was 3.1 years. During a median follow-up of 2.1 years, 184 and 207 cases experienced cardiovascular events and all-cause death, respectively. A higher MLR was incrementally associated with higher risk of cardiovascular events and all-cause mortality. The multivariable-adjusted hazard ratios (95% confidence interval) of cardiovascular events and all-cause mortality for 0.1-unit increase in MLR were 1.22 (1.09-1.37) and 1.18 (1.07-1.30), respectively. There was a modest but significant mediation effect of plasma interleukin-6 (IL-6) on the association between MLR and cardiovascular events (28.2%) and all-cause mortality (24.1%).
Conclusion: A higher MLR was independently associated with higher risk of cardiovascular events and all-cause mortality in patients on hemodialysis. Our findings suggest the potential of MLR as a simple, cost-effective prognostic biomarker and support IL-6-targeted therapeutic interventions to improve clinical outcomes in patients with ESKD.
{"title":"Association of Monocyte-Lymphocyte Ratio with Cardiovascular Events and All-Cause Mortality in Patients Undergoing Hemodialysis: A Prospective Cohort Study.","authors":"Yamini Mallisetty, Ahmed Kabeil, Cameron Jackson Bergeron, Zhongji Han, Chi-Yang Chiu, Csaba P Kovesdy, Keiichi Sumida","doi":"10.1159/000549074","DOIUrl":"10.1159/000549074","url":null,"abstract":"<p><strong>Introduction: </strong>Elevated monocyte to lymphocyte ratio (MLR) represents a pro-inflammatory immune microenvironment and has been associated with poor clinical outcomes such as cardiovascular disease. However, little is known about its association with outcomes among patients on hemodialysis.</p><p><strong>Methods: </strong>In a nationwide prospective cohort of 952 patients receiving maintenance hemodialysis from 2011-2013, we examined the association of baseline MLR with subsequent risk of cardiovascular events (cardiovascular-related hospitalization and cardiovascular death) and all-cause mortality, using multivariable Cox proportional-hazards models with adjustment for potential confounders. We also examined the mediation effect of inflammatory markers on the association between MLR and cardiovascular events and all-cause mortality, respectively.</p><p><strong>Results: </strong>Overall, patients were 60.1 ± 13.4 years old; 53.3% were male; 39.7% were African American; and 56.9% were diabetic. The median dialysis vintage was 3.1 years. During a median follow-up of 2.1 years, 184 and 207 cases experienced cardiovascular events and all-cause death, respectively. A higher MLR was incrementally associated with higher risk of cardiovascular events and all-cause mortality. The multivariable-adjusted hazard ratios (95% confidence interval) of cardiovascular events and all-cause mortality for 0.1-unit increase in MLR were 1.22 (1.09-1.37) and 1.18 (1.07-1.30), respectively. There was a modest but significant mediation effect of plasma interleukin-6 (IL-6) on the association between MLR and cardiovascular events (28.2%) and all-cause mortality (24.1%).</p><p><strong>Conclusion: </strong>A higher MLR was independently associated with higher risk of cardiovascular events and all-cause mortality in patients on hemodialysis. Our findings suggest the potential of MLR as a simple, cost-effective prognostic biomarker and support IL-6-targeted therapeutic interventions to improve clinical outcomes in patients with ESKD.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"76-86"},"PeriodicalIF":1.8,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145318518","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}
Pub Date : 2026-01-01Epub Date: 2025-09-19DOI: 10.1159/000548245
Fernando Caravaca-Fontán, Fadi Fakhouri, Matthew C Pickering, Vikte Lionikaite, Alison Baird, Regina Horneff, Luis López-Lázaro, Lucia Quintana-Gallardo, Carly Rich
Introduction: Knowledge gaps and controversies remain regarding the natural history and variability of C3 glomerulopathy (C3G) and primary immune complex membranoproliferative glomerulonephritis (IC-MPGN). The objectives were to provide an overview of these diseases for the following outcomes of interest: clinical presentation, treatment patterns, and disease burden, including the association between proteinuria and kidney outcomes.
Methods: This systematic literature review (SLR) included studies of adults and children with C3G or primary IC-MPGN investigating outcomes of interest. Embase and MEDLINE were searched from January 2012 to February 22, 2024, combining terms for C3G or IC-MPGN and outcomes of interest. Supplementary congress searches and reference list checking of relevant articles were conducted. Study details, outcomes of interest, and key findings were extracted, and data were narratively summarized.
Results: In total, 148 articles were included. No clear trend for differences between C3G and primary IC-MPGN were observed for clinical presentation. Treatments included immunosuppressive therapies and off-label anti-complement agents. Kidney failure occurred in up to 50% and 37% of patients with C3G and primary IC-MPGN, respectively, and kidney transplantation was required in up to 32% and 24% of patients, respectively. Mortality was reported in up to 21% of patients. No clear trend of complete remission across treatments was observed. In longitudinal studies, proteinuria was associated with increasing risk of kidney failure. No articles reported on patient quality of life or caregiver burden. Several articles reported an economic burden according to length of hospital stay. Possible limitations include that terms used for electronic searches limited which articles were identified, many studies were retrospective and small (<10 participants), and risk of bias was not performed.
Conclusions: This SLR provides insights into C3G and primary IC-MPGN, emphasizing the need for new targeted and effective treatments. Proteinuria was identified to be an acceptable marker in assessing the efficacy of treatments on long-term kidney outcomes.
{"title":"Clinical Presentation, Treatment Patterns, Burden of Disease, and the Association of Proteinuria with Clinical Outcomes in C3 Glomerulopathy and Primary Immune Complex Membranoproliferative Glomerulonephritis: A Systematic Review.","authors":"Fernando Caravaca-Fontán, Fadi Fakhouri, Matthew C Pickering, Vikte Lionikaite, Alison Baird, Regina Horneff, Luis López-Lázaro, Lucia Quintana-Gallardo, Carly Rich","doi":"10.1159/000548245","DOIUrl":"10.1159/000548245","url":null,"abstract":"<p><strong>Introduction: </strong>Knowledge gaps and controversies remain regarding the natural history and variability of C3 glomerulopathy (C3G) and primary immune complex membranoproliferative glomerulonephritis (IC-MPGN). The objectives were to provide an overview of these diseases for the following outcomes of interest: clinical presentation, treatment patterns, and disease burden, including the association between proteinuria and kidney outcomes.</p><p><strong>Methods: </strong>This systematic literature review (SLR) included studies of adults and children with C3G or primary IC-MPGN investigating outcomes of interest. Embase and MEDLINE were searched from January 2012 to February 22, 2024, combining terms for C3G or IC-MPGN and outcomes of interest. Supplementary congress searches and reference list checking of relevant articles were conducted. Study details, outcomes of interest, and key findings were extracted, and data were narratively summarized.</p><p><strong>Results: </strong>In total, 148 articles were included. No clear trend for differences between C3G and primary IC-MPGN were observed for clinical presentation. Treatments included immunosuppressive therapies and off-label anti-complement agents. Kidney failure occurred in up to 50% and 37% of patients with C3G and primary IC-MPGN, respectively, and kidney transplantation was required in up to 32% and 24% of patients, respectively. Mortality was reported in up to 21% of patients. No clear trend of complete remission across treatments was observed. In longitudinal studies, proteinuria was associated with increasing risk of kidney failure. No articles reported on patient quality of life or caregiver burden. Several articles reported an economic burden according to length of hospital stay. Possible limitations include that terms used for electronic searches limited which articles were identified, many studies were retrospective and small (<10 participants), and risk of bias was not performed.</p><p><strong>Conclusions: </strong>This SLR provides insights into C3G and primary IC-MPGN, emphasizing the need for new targeted and effective treatments. Proteinuria was identified to be an acceptable marker in assessing the efficacy of treatments on long-term kidney outcomes.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"25-40"},"PeriodicalIF":1.8,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12795539/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145092201","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}
Pub Date : 2026-01-01Epub Date: 2025-11-26DOI: 10.1159/000549041
In the article by Jeon et al. entitled "The Impact of C-Reactive Protein-To-Albumin Ratio on Mortality in Patients with Acute Kidney Injury Requiring Continuous Renal Replacement Therapy: A Multicenter Retrospective Study" [Nephron. 2024;148:379-389; https://doi.org/10.1159/000534970], the license type has been changed to CC-BY-NC.The original article has been updated.
{"title":"Erratum.","authors":"","doi":"10.1159/000549041","DOIUrl":"10.1159/000549041","url":null,"abstract":"<p><p>In the article by Jeon et al. entitled \"The Impact of C-Reactive Protein-To-Albumin Ratio on Mortality in Patients with Acute Kidney Injury Requiring Continuous Renal Replacement Therapy: A Multicenter Retrospective Study\" [Nephron. 2024;148:379-389; https://doi.org/10.1159/000534970], the license type has been changed to CC-BY-NC.The original article has been updated.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"63-64"},"PeriodicalIF":1.8,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145636100","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}
Pub Date : 2026-01-01Epub Date: 2025-10-13DOI: 10.1159/000548903
Jeanette Finderup, Hanne Agerskov, Malene Deele, Christina Egmose Frandsen, Ingrid Villadsen Kristensen, Ann Bonner, Hanne Konradsen
Introduction: To co-design and test the effectiveness of a tailored COmplex symptom Management intervention FOR adulTs with advanced kidney disease and their informal caregivers along with healthcare professionals who deliver care and support to both patients and informal caregivers. Methods: Informed by Symptom Management Theory, COMFORT is a program of research involving multiple studies and structured around the United Kingdom Medical Research Council's framework for developing complex interventions. The program involves building capacity of nurse researchers across three studies: first development of an intervention using mixed methods, followed by co-design workshops and prototype testing; thereafter testing of the symptom intervention through a feasibility trial. Lastly, evaluation of the intervention's effectiveness and implementation using a type two hybrid randomised control trial design, focussing on both clinical outcomes and implementation fidelity. Conclusion: This research program is expected to demonstrate that the COMFORT intervention is superior to standard care in improving symptom burden among adults with advanced kidney disease and reduce caregiver burden among informal caregivers. The COMFORT Nursing Program addresses a critical gap in clinical practice by focusing on non-pharmacological interventions tailored to individual patient needs and symptom clusters. By integrating shared decision-making and self-management support, the program aims to empower patients and caregivers, enhances clinical practice, and contributes substantial evidence to the domain of symptom management in advanced kidney disease. The involvement of stakeholders throughout the research process ensures the relevance and applicability of the findings to real-world clinical settings.
{"title":"Tailored Complex Symptom Management Intervention for Adults with Advanced Kidney Disease and Their Informal Caregivers: Protocol for the COMFORT Research Program.","authors":"Jeanette Finderup, Hanne Agerskov, Malene Deele, Christina Egmose Frandsen, Ingrid Villadsen Kristensen, Ann Bonner, Hanne Konradsen","doi":"10.1159/000548903","DOIUrl":"10.1159/000548903","url":null,"abstract":"<p><p><p>Introduction: To co-design and test the effectiveness of a tailored COmplex symptom Management intervention FOR adulTs with advanced kidney disease and their informal caregivers along with healthcare professionals who deliver care and support to both patients and informal caregivers. Methods: Informed by Symptom Management Theory, COMFORT is a program of research involving multiple studies and structured around the United Kingdom Medical Research Council's framework for developing complex interventions. The program involves building capacity of nurse researchers across three studies: first development of an intervention using mixed methods, followed by co-design workshops and prototype testing; thereafter testing of the symptom intervention through a feasibility trial. Lastly, evaluation of the intervention's effectiveness and implementation using a type two hybrid randomised control trial design, focussing on both clinical outcomes and implementation fidelity. Conclusion: This research program is expected to demonstrate that the COMFORT intervention is superior to standard care in improving symptom burden among adults with advanced kidney disease and reduce caregiver burden among informal caregivers. The COMFORT Nursing Program addresses a critical gap in clinical practice by focusing on non-pharmacological interventions tailored to individual patient needs and symptom clusters. By integrating shared decision-making and self-management support, the program aims to empower patients and caregivers, enhances clinical practice, and contributes substantial evidence to the domain of symptom management in advanced kidney disease. The involvement of stakeholders throughout the research process ensures the relevance and applicability of the findings to real-world clinical settings. </p>.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"65-75"},"PeriodicalIF":1.8,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12707882/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145280845","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}
Pub Date : 2026-01-01Epub Date: 2025-10-30DOI: 10.1159/000549077
Rengin Çetin Güvenç, Tolga Sinan Güvenç, Ezgi Sude Karakaya, Hasan Açık, Aysu Korkmaz, Abdul Fattah Salem, Osman Şahin, Ferhat Ferhatoğlu, Alp Gürkan
Introduction: Pulmonary hypertension and right ventricular (RV) dysfunction are associated with an increase in mortality and worse prognosis in patients with end-stage kidney disease (ESKD), but pathophysiologic mechanisms underlying the progression of RV dysfunction remain incompletely understood. The present study aimed to understand right ventricular to pulmonary artery (RV-PA) coupling, which is an early indicator of transition to RV dysfunction, to better characterize adaptive RV response to increased afterload in ESKD patients and changes in RV-PA coupling following renal transplantation.
Methods: One hundred eleven patients with ESKD, including 49 patients scheduled for renal transplantation, underwent a detailed echocardiographic examination and measurement of tricuspid annular plane excursion to pulmonary artery systolic pressure (TAPSE/PASP) ratio, and a repeat examination was performed 6 months after the baseline examination.
Results: Patients with ESKD had significantly lower TAPSE/PASP ratio at baseline (1.02 [0.71-1.63] vs. 1.29 [1.09-1.96], p < 0.001). In 40 patients that underwent transplantation, TAPSE/PASP ratio increased significantly from (0.97 [0.72-1.42] to 1.30 [1.01-1.82], p = 0.03), while in 27 patients remained on dialysis, there was a nonsignificant reduction in TAPSE/PASP ratio (1.21 [0.71-1.62] vs. 0.84 [0.61-1.38], p = 0.44). The change in TAPSE/PASP ratio correlated significantly with the change in pulmonary vascular resistance (OR: 0.61, 95% CI: 0.51-0.72, p < 0.001) and left ventricular mass index (OR: 0.97, 95% CI: 0.96-0.99, p = 0.001) after adjustment.
Conclusions: Patients with ESKD had abnormal RV-PA coupling, as indicated by a reduced TAPSE/PASP ratio, which normalizes 6 months after renal transplantation.
{"title":"Abnormal Right Ventricular to Pulmonary Artery Coupling in Patients with End-Stage Kidney Disease and Normalization after Renal Transplantation: An Observational Study.","authors":"Rengin Çetin Güvenç, Tolga Sinan Güvenç, Ezgi Sude Karakaya, Hasan Açık, Aysu Korkmaz, Abdul Fattah Salem, Osman Şahin, Ferhat Ferhatoğlu, Alp Gürkan","doi":"10.1159/000549077","DOIUrl":"10.1159/000549077","url":null,"abstract":"<p><strong>Introduction: </strong>Pulmonary hypertension and right ventricular (RV) dysfunction are associated with an increase in mortality and worse prognosis in patients with end-stage kidney disease (ESKD), but pathophysiologic mechanisms underlying the progression of RV dysfunction remain incompletely understood. The present study aimed to understand right ventricular to pulmonary artery (RV-PA) coupling, which is an early indicator of transition to RV dysfunction, to better characterize adaptive RV response to increased afterload in ESKD patients and changes in RV-PA coupling following renal transplantation.</p><p><strong>Methods: </strong>One hundred eleven patients with ESKD, including 49 patients scheduled for renal transplantation, underwent a detailed echocardiographic examination and measurement of tricuspid annular plane excursion to pulmonary artery systolic pressure (TAPSE/PASP) ratio, and a repeat examination was performed 6 months after the baseline examination.</p><p><strong>Results: </strong>Patients with ESKD had significantly lower TAPSE/PASP ratio at baseline (1.02 [0.71-1.63] vs. 1.29 [1.09-1.96], p < 0.001). In 40 patients that underwent transplantation, TAPSE/PASP ratio increased significantly from (0.97 [0.72-1.42] to 1.30 [1.01-1.82], p = 0.03), while in 27 patients remained on dialysis, there was a nonsignificant reduction in TAPSE/PASP ratio (1.21 [0.71-1.62] vs. 0.84 [0.61-1.38], p = 0.44). The change in TAPSE/PASP ratio correlated significantly with the change in pulmonary vascular resistance (OR: 0.61, 95% CI: 0.51-0.72, p < 0.001) and left ventricular mass index (OR: 0.97, 95% CI: 0.96-0.99, p = 0.001) after adjustment.</p><p><strong>Conclusions: </strong>Patients with ESKD had abnormal RV-PA coupling, as indicated by a reduced TAPSE/PASP ratio, which normalizes 6 months after renal transplantation.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"87-98"},"PeriodicalIF":1.8,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145409537","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}
Pub Date : 2026-01-01Epub Date: 2025-09-27DOI: 10.1159/000548275
Saúl Neftalí Rolon-Barbosa, Alfonso Martín Cueto-Manzano, Nancy Gabriela Romero-Ornelas, Hugo Enrique Chavez-Chavez, Laura Margarita Díaz-Canchola, Mariana Avalos-Lopez, José Ignacio Cerrillos-Gutiérrez, Javier Ramón Zambrano-Melín, Enrique Rojas-Campos, Fabiola Martín-Del-Campo-Lopez
Introduction: Treatment conditions and progression of chronic kidney disease (CKD) are factors for work incapacity, related with physical limitations and social and emotional distress, affecting quality of life (QOL).
Methods: In a cross-sectional study, 343 patients with CKD were included: chronic kidney disease non-dialysis (CKD G3-G5ND) (n = 98), hemodialysis (HD) (n = 95), peritoneal dialysis (PD) (n = 96), and kidney transplant (KT) (n = 54). A clinical and nutritional assessment was carried out using the Subjective Global Assessment instrument. QOL was assessed using the Kidney Disease Quality of Life Short Form instrument.
Results: Employed patients (44%) were younger (40 ± 14 vs. 51 ± 16 years, p < 0.0001), had >9 years of schooling (49 vs. 29%, p < 0.0001), lower diabetes frequency (21 vs. 47%, p < 0.0001), hypertension (71 vs. 87%, p < 0.0001), cardiovascular disease (5 vs. 16%, p = 0.002), and better nutritional status score (6 ± 1 vs. 5 ± 1, p < 0.0001) than unemployed. Employed patients with KT had better QOL than employed patients on CKD G3-G5ND, HD, and PD (76 ± 6, 68 ± 13, 68 ± 12, and 67 ± 7, respectively, p < 0.05). In multivariate analysis, employment predicted QOL in all kidney replacement therapies (KRTs): HD (B = 10.1, 95% CI: 5.5-14.6), PD (B = 4.5, 95% CI: 0.08-8.9) and KT (B = 13.3, 95% CI: 6.1-20.5). Nutritional status predicted QOL in all groups: CKD G3-G5ND (B = 3.6, 95% CI: 1.24-5.97), HD (B = 2.44, 95% CI: 0.4-4.4), PD (B = 3.73, 95% CI: 2.1-5.3), and KT (B = 4.4, 95% CI: 0.05-8.8).
Conclusion: Only 44% of patients had employment. Employed patients had better QOL, were younger, more educated, had fewer comorbidities and better nutritional status than unemployed patients. Employment predicted QOL in all three KRTs but not in CKD G3-G5ND patients, and nutritional status was a predictor QOL in all groups.
{"title":"Association of Employment Status with Quality of Life in Patients across Chronic Kidney Disease G3-G5 Non-Dialysis and Kidney Replacement Therapies.","authors":"Saúl Neftalí Rolon-Barbosa, Alfonso Martín Cueto-Manzano, Nancy Gabriela Romero-Ornelas, Hugo Enrique Chavez-Chavez, Laura Margarita Díaz-Canchola, Mariana Avalos-Lopez, José Ignacio Cerrillos-Gutiérrez, Javier Ramón Zambrano-Melín, Enrique Rojas-Campos, Fabiola Martín-Del-Campo-Lopez","doi":"10.1159/000548275","DOIUrl":"10.1159/000548275","url":null,"abstract":"<p><strong>Introduction: </strong>Treatment conditions and progression of chronic kidney disease (CKD) are factors for work incapacity, related with physical limitations and social and emotional distress, affecting quality of life (QOL).</p><p><strong>Methods: </strong>In a cross-sectional study, 343 patients with CKD were included: chronic kidney disease non-dialysis (CKD G3-G5ND) (n = 98), hemodialysis (HD) (n = 95), peritoneal dialysis (PD) (n = 96), and kidney transplant (KT) (n = 54). A clinical and nutritional assessment was carried out using the Subjective Global Assessment instrument. QOL was assessed using the Kidney Disease Quality of Life Short Form instrument.</p><p><strong>Results: </strong>Employed patients (44%) were younger (40 ± 14 vs. 51 ± 16 years, p < 0.0001), had >9 years of schooling (49 vs. 29%, p < 0.0001), lower diabetes frequency (21 vs. 47%, p < 0.0001), hypertension (71 vs. 87%, p < 0.0001), cardiovascular disease (5 vs. 16%, p = 0.002), and better nutritional status score (6 ± 1 vs. 5 ± 1, p < 0.0001) than unemployed. Employed patients with KT had better QOL than employed patients on CKD G3-G5ND, HD, and PD (76 ± 6, 68 ± 13, 68 ± 12, and 67 ± 7, respectively, p < 0.05). In multivariate analysis, employment predicted QOL in all kidney replacement therapies (KRTs): HD (B = 10.1, 95% CI: 5.5-14.6), PD (B = 4.5, 95% CI: 0.08-8.9) and KT (B = 13.3, 95% CI: 6.1-20.5). Nutritional status predicted QOL in all groups: CKD G3-G5ND (B = 3.6, 95% CI: 1.24-5.97), HD (B = 2.44, 95% CI: 0.4-4.4), PD (B = 3.73, 95% CI: 2.1-5.3), and KT (B = 4.4, 95% CI: 0.05-8.8).</p><p><strong>Conclusion: </strong>Only 44% of patients had employment. Employed patients had better QOL, were younger, more educated, had fewer comorbidities and better nutritional status than unemployed patients. Employment predicted QOL in all three KRTs but not in CKD G3-G5ND patients, and nutritional status was a predictor QOL in all groups.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"119-130"},"PeriodicalIF":1.8,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145182193","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}