Nephron[JOURNAL]
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200 papers
Lysinuric Protein Intolerance: Not Only a Disorder for Pediatric Nephrologists - Case Report.
Rigoldi M, Mele C, Breno M … +6 more , Noris M, Imeraj A, Gamba S, Schieppati A, Daina E, Italian Undiagnosed Rare Diseases Network (IURDN)
Nephron · 2025 · PMID 39293417 · Publisher ↗
INTRODUCTION: Lysinuric protein intolerance (LPI) is a multisystemic inborn error of metabolism with a variable clinical expressivity that usually begins in childhood with growth failure and gastroenterological/neurologi...
INTRODUCTION: Lysinuric protein intolerance (LPI) is a multisystemic inborn error of metabolism with a variable clinical expressivity that usually begins in childhood with growth failure and gastroenterological/neurological problems related to the altered urea cycle and, later, with complications involving the renal, pulmonary, and immunohematological systems. CASE REPORT: We present the case of a 40-year-old woman suffering from chronic kidney disease in the context of a LPI, whose diagnosis was challenging because the signs of the disease were always blurred and the patient never manifested critical episodes typical of this multisystemic disease. In addition to renal disease, splenomegaly, thrombocytopenia, elevated lactate dehydrogenase (LDH), hyperferritinemia, and hypertriglyceridemia were also present. A thorough investigation of the patient's food preferences revealed her spontaneous aversion to protein-containing foods and excessive drowsiness during the occurrence of infectious episodes or on the rare occasions of excessive protein intake, although without ever coming to medical attention. These nuanced signs led us to suspect an impairment of the urea cycle and ultimately allowed us to narrow down the diagnosis to LPI through biochemical and genetic investigations. CONCLUSION: Nephrologists should consider LPI in the differential diagnosis, whenever a patient presents with mixed proteinuria, tubular dysfunction, and/or chronic kidney disease of unknown origin. In these circumstances, we suggest looking for other signs such as growth failure, signs and symptoms ascribed to urea-cycle impairment, pulmonary involvement, hepatosplenomegaly, and laboratory alterations such as pancytopenia, hyperferritinemia, lipid abnormalities, and elevated LDH.
The Impact of Age and Body Composition on the Agreement between Estimated and Measured GFR in Heart Transplant Recipients.
Hornum M, Jørgensen MB, Sidenius Nelson LM … +5 more , Feldt-Rasmussen B, Rossing K, Porrini E, Oturai P, Gustafsson F
Nephron · 2025 · PMID 39278210 · Publisher ↗
BACKGROUND: Estimated GFR (eGFR) has shown poor agreement with measured GFR (mGFR) in several populations. We investigated the impact of age and body composition on the accuracy and precision of eGFR in heart transplant...
BACKGROUND: Estimated GFR (eGFR) has shown poor agreement with measured GFR (mGFR) in several populations. We investigated the impact of age and body composition on the accuracy and precision of eGFR in heart transplant (HTx) recipients. METHODS: In a longitudinal, observational, retrospective study design, patients receiving first-time HTx with at least one registered mGFR value within 15 months after HTx and a corresponding plasma creatinine were included. GFR was measured by 51Cr-EDTA and eGFR calculated by creatinine-based CKD-EPI formula. RESULTS: A total of 150 patients with a total of 723 mGFR measurements were included. During the first year after HTx, mean weight increased by 4.2 kg (CI: 3.2 to 5.1) followed by an annual decrease of 0.35 kg/year (Cl: -0.05 to 0.74). mGFR increased by 7.5 mL/min (Cl: 3.2 to 11.8) the first year but was stable hereafter (0.0 mL/min/year; CI: -1.0 to 1.0). The initial weigh gain and increase in mGFR were most pronounced in patients <45 years. Neither eGFR adjusted nor unadjusted for BSA detected the initial increase in mGFR. At 1 year after HTx, limits of agreement on the Bland-Altman plot were -37.2 to 33.1 mL/min with a bias of -2.1 mL/min (Cl: -5.0 to 0.9). In patients <45 years, eGFR significantly overestimated mGFR by 7.1 mL/min (Cl: 1.0 to 13.2) and showed a significant lower precision than patients >45 years. There was no effect of BMI class, weight, BSA, or change in BMI class on the difference between eGFR and mGFR. CONCLUSION: eGFR is, on average, accurate but imprecise in HTx patients. The agreement is affected by age but not body composition.
Preferences for Advance Care Planning in Patients with End-Stage Kidney Disease: A Cross-Sectional Survey among Nephrology Healthcare Providers in a Tertiary Care Center in India.
Naik B, Ghoshal A, Damani A … +5 more , Singhai P, Prabhu Attur R, Salins N, Nayak AM, Nagaraju SP
Nephron · 2025 · PMID 39250897 · Full text
INTRODUCTION: Advance care planning (ACP) is essential in managing patients with end-stage kidney disease (ESKD), yet its integration into clinical practice remains limited, particularly in low- and middle-income countri...
INTRODUCTION: Advance care planning (ACP) is essential in managing patients with end-stage kidney disease (ESKD), yet its integration into clinical practice remains limited, particularly in low- and middle-income countries. This study explores the preferences, attitudes and perceived barriers of nephrology healthcare providers toward ACP for patients with ESKD in a tertiary care center in India. METHODS: A cross-sectional survey was conducted among nephrology healthcare providers at a tertiary care center in India. The survey, developed from literature reviews and pretested, covered demographics, ACP knowledge and attitudes, current practices, and perceived barriers and facilitators. Data collection occurred from September 2022 to March 2023. Quantitative data were analyzed descriptively, and qualitative data through thematic analysis. RESULTS: A total of 50 healthcare providers participated. While 36% acknowledged the importance of ACP, only 8% routinely engaged in ACP discussions. Major barriers included inadequate training (22%), lack of awareness about the importance of discussing ACP among stakeholders (20%), cultural barriers (18%), lack of time (14%), and the absence of institutional protocols for discussion on ACP (14%). Additional barriers included instances where families withhold health information from patients due to fear of losing hope (16%) and patient/family discomfort in discussing ACP (12%). Providers expressed a need for structured ACP protocols and educational programs. CONCLUSION: Despite recognizing its importance, ACP is underutilized in the care of patients with ESKD in India. Addressing the identified barriers through targeted interventions may enhance ACP practices and improve patient outcomes.
Acute Renal Infarction: A 12-Year Retrospective Analysis.
Greenberg S, Jana K, Janga K … +2 more , Lee MH, Lockwood M
Nephron · 2025 · PMID 39250895 · Publisher ↗
INTRODUCTION: Acute renal infarction (ARI) is a relatively rare and underdiagnosed condition. Presenting symptoms are nonspecific, and imaging is the mainstay for diagnosis. This study attempts to characterize the profil...
INTRODUCTION: Acute renal infarction (ARI) is a relatively rare and underdiagnosed condition. Presenting symptoms are nonspecific, and imaging is the mainstay for diagnosis. This study attempts to characterize the profile of patients with ARI and identify possible risk factors. METHODS: All inpatients admitted with diagnosis of ARI between 2010 and 2022 were included in this single-center retrospective observational study. Patients with chronic renal infarction, iatrogenic causes, and without radiographic evidence of ARI were excluded. Clinical, laboratory, and radiological findings of patients were collected. Patients were grouped into three groups based on probable etiology: cardiovascular, hypercoagulable disorders, and idiopathic, and analyzed. RESULTS: Eighty-five patients were included. Mean age of patients was 61.6 ± 17.54 years. Cardiovascular group had the highest number of patients (49.4%) of which atrial fibrillation was the most common etiology (59.5%). Malignancy was the most common etiology in the hypercoagulable disorder group (69.3%). Patients in the idiopathic group were significantly younger and had higher mean body mass index than the other 2 groups at presentation. Smokers had 9 times higher risk of renal infarction in cardiovascular group and 1.7 times higher risk in hypercoagulable when compared to the idiopathic group. 48.2% of patients developed renal infarction though they were on antiplatelets/anticoagulants. CONCLUSION: ARI is a rare and often underdiagnosed condition that can have residual renal dysfunction. It is important to consider ARI as a differential especially in young patients with risk factors even if they are on anticoagulation medication.
Efficacy and Safety of Telitacicept in IgA Nephropathy: A Retrospective, Multicenter Study.
Liu L, Liu Y, Li J … +32 more , Tang C, Wang H, Chen C, Long H, Chen X, Xing G, Cheng J, Liang J, Peng X, Wang L, Shao S, Lin Y, Chen T, Tang Y, Shen S, Sun L, Wu H, Yu Y, Du X, Liu H, He L, Liu H, Ye M, Chen W, Wen Q, Zhang H, Cao H, Yuan J, Chen H, Wang M, Lv J, Zhang H
Nephron · 2025 · PMID 39250892 · Publisher ↗
INTRODUCTION: The efficacy of telitacicept treatment in reducing proteinuria in patients with IgA nephropathy (IgAN) was indicated in a phase II clinical trial with small sample size. In this study, we conducted a large...
INTRODUCTION: The efficacy of telitacicept treatment in reducing proteinuria in patients with IgA nephropathy (IgAN) was indicated in a phase II clinical trial with small sample size. In this study, we conducted a large multicenter retrospective study to explore the efficacy and safety of telitacicept in patients with IgAN. METHODS: This study recruited patients with IgAN from 19 sites from China who were treated with telitacicept and had been followed up at least once or with side effect reported, since April 1, 2021, to April 1, 2023. The primary outcomes of the study were the changing in proteinuria and eGFR over time. RESULTS: A cohort of 97 patients with IgAN who were treated with telitacicept were recruited, with a median follow-up duration of 3 months. The median baseline proteinuria was 2.3 [1.3, 3.9] g/day and eGFR was 45.0 [26.8, 73.7] mL/min/1.73 m2. There was a significant reduction of proteinuria at 2, 4, 6 months when compared with baseline (2.3 [1.5, 4.1] vs. 1.5 [0.8, 2.3] g/day; 2.3 [1.1, 3.7] vs. 1.1 [0.6, 1.9] g/day; 2.1 [1.0, 2.7] vs. 0.9 [0.5, 1.7] g/day, all p values <0.01). The level of eGFR were comparable between at the baseline and 2, 4, 6 months of follow-up time (41.5 [29.7, 72.0] vs. 42.5 [28.8, 73.3] mL/min/1.73 m2; 41.0 [26.8, 67.7] vs. 44.7 [31.0, 67.8] mL/min/1.73 m2; 33.7 [24.0, 58.5] vs. 32.6 [27.8, 57.5] mL/min/1.73 m2, all p values >0.26). Telitacicept was well tolerated in the patients. CONCLUSIONS: This study indicates that telitacicept alone or on top of steroids therapy can significantly and safely reduce proteinuria in patients with IgAN. The long-term kidney protection still needs to be confirmed in large phase III trial.
The Glomerular Sieving Coefficient of Albumin Is Really Very Low.
Tanner GA
Nephron · 2024 · PMID 39186923 · Publisher ↗
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Lipophagy and Mitophagy in Renal Pathophysiology.
Dupont N, Terzi F
Nephron · 2025 · PMID 39182483 · Publisher ↗
BACKGROUND: The lysosomal autophagic pathway plays a fundamental role in cellular and tissue homeostasis, and its deregulation is linked to human pathologies including kidney diseases. Autophagy can randomly degrade cyto...
BACKGROUND: The lysosomal autophagic pathway plays a fundamental role in cellular and tissue homeostasis, and its deregulation is linked to human pathologies including kidney diseases. Autophagy can randomly degrade cytoplasmic components in a nonselective manner commonly referred to as bulk autophagy. In contrast, selective forms of autophagy specifically target cytoplasmic structures such as organelles and protein aggregates, thereby being important for cellular quality control and organelle homeostasis. SUMMARY: Research during the past decades has begun to elucidate the role of selective autophagy in kidney physiology and kidney diseases. KEY MESSAGES: In this review, we will summarize the knowledge on lipophagy and mitophagy, two forms of selective autophagy important in renal epithelium homeostasis, and discuss how their deregulations contribute to renal disease progression.
Severe Leptospirosis with Acute Kidney Injury: A Case Description and Literature Review.
Daschner C, Schübler AS, Jung M … +6 more , Ayasse N, Yücel G, Husain-Syed F, Leipe J, Krämer BK, Yazdani B
Nephron · 2024 · PMID 39102808 · Publisher ↗
INTRODUCTION: Leptospirosis is a globally transmitted zoonotic disease caused by Leptospira spp., a highly mobile, obligate aerobic, spiral-shaped bacteria. Described first by Adolf Weil in 1886, leptospirosis in Germany...
INTRODUCTION: Leptospirosis is a globally transmitted zoonotic disease caused by Leptospira spp., a highly mobile, obligate aerobic, spiral-shaped bacteria. Described first by Adolf Weil in 1886, leptospirosis in Germany is rare, leading to a delayed diagnosis due to diverse symptoms. Most cases are mild, but severe forms, like Weil's disease, cause life-threatening complications such as fever, jaundice, hemoptysis, and acute kidney injury (AKI). The aim of this work was to provide a literature review of leptospirosis with renal manifestation based on a case report. CASE PRESENTATION: We report the case of an 81-year-old male patient with initially unclear oliguric AKI, bilateral pulmonary infiltrates, and jaundice. After excluding common AKI causes, the expanded patient history suggested possible rat contact in his chicken coop. Finally, we serologically identified an infection with Leptospira spp. by positive IgM, proving that the illness was compatible with classical Weil's disease. The patient underwent temporary hemodialysis and antibiotic treatment with intravenous penicillin G for 2 weeks. Under therapy, the AKI, hyperbilirubinemia, and clinical condition of the patient improved. The patient was discharged after 2 weeks. In the following controls, slightly impaired kidney function was observed, indicating a progress of his chronic kidney disease (CKD). CONCLUSION: Although leptospirosis is rare, there are some cases with a fulminant course. Impairment of renal function often correlates with severity of the disease requiring antibiotic treatment. In some cases, AKI progresses to CKD demonstrating the need to raise awareness for leptospirosis.
Systems Biology and Novel Biomarkers for the Early Detection of Diabetic Kidney Disease.
Villalvazo P, Villavicencio C, Gonzalez de Rivera M … +2 more , Fernandez-Fernandez B, Ortiz A
Nephron · 2025 · PMID 39074450 · Publisher ↗
Diabetic kidney disease is the most common driver of chronic kidney disease (CKD)-associated mortality and kidney replacement therapy. Despite recent therapeutic advances (sodium glucose co-transporter 2 [SGLT2] inhibito...
Diabetic kidney disease is the most common driver of chronic kidney disease (CKD)-associated mortality and kidney replacement therapy. Despite recent therapeutic advances (sodium glucose co-transporter 2 [SGLT2] inhibitors, finerenone), the residual kidney and mortality risk remains high for patients already diagnosed of having CKD (i.e., estimated glomerular filtration rate <60 mL/min/1.73 m2 or urinary albumin:creatinine ratio >30 mg/g). The challenge for the near future is to identify patients at higher risk of developing CKD to initiate therapy before CKD develops (primary prevention of CKD) and to identify patients with CKD and high risk of progression or death, in order to intensify therapy. We now discuss recent advances in biomarkers that may contribute to the identification of such high-risk individuals for clinical trials of novel primary prevention or treatment approaches for CKD. The most advanced biomarker from a clinical development point of view is the urinary peptidomics classifier CKD273, that integrates prognostic information from 273 urinary peptides and identifies high-risk individuals before CKD develops.
Predicting Outcomes after Transplantation of Deceased Donor Kidneys of Marginal Quality within the Eurotransplant Service Area.
Scurt FG, Fischer-Fröhlich CL, Ernst A … +3 more , Mertens PR, Becker JU, Chatzikyrkou C
Nephron · 2024 · PMID 39047699 · Full text
INTRODUCTION: Kidneys of marginal quality are increasingly being used to overcome the shortage of donor organs. However, accurate prediction of outcome is needed to optimize the use of these kidneys. We aimed to test the...
INTRODUCTION: Kidneys of marginal quality are increasingly being used to overcome the shortage of donor organs. However, accurate prediction of outcome is needed to optimize the use of these kidneys. We aimed to test the performance of a recently proposed score consisting of delayed graft function (DGF), renal function recovery (RFR), and glomerular filtration rate (GFR) <30 mL/min per 1.73 m2 90 days after transplantation for risk assessment of patient and graft survival. MATERIAL AND METHODS: A total of 221 adult brain death donors with marginal kidneys transplanted into 223 recipients within Eurotransplant were included in the analysis. Multivariable Cox proportional hazards models were constructed to assess death-censored and all-cause censored graft failure and recipient mortality at 1 and 3 years. RESULTS: Recipients with DGF had a higher risk of death-censored graft loss (HR, 95% CIs: 3.058 [1.195-7.825]). Recipients with a GFR <30 mL/min/1.73 m2 at 90 days after transplantation had a higher risk of death censored and all-cause graft failure (HR, 95% CIs: 2.122 [1.129-3.990] and 2.122 [1.129-3.990]). None of the three components of the proposed score was associated with a higher risk of mortality. CONCLUSION: DGF and estimated GFR <30 mL/min/1.73 m2 but not RFR at 90 days predicted graft failure after transplantation of marginal kidneys. However, no combination of these factors was able to predict short-term patient and graft survival.
The Relationship between Serum Sodium Concentration and Albuminuria: A Retrospective Cohort Study.
Cole NI, Swift PA, Suckling RJ … +5 more , He FJ, Gallagher H, van Vlymen J, Byford R, de Lusignan S
Nephron · 2024 · PMID 39038444 · Publisher ↗
BACKGROUND: Lowering dietary salt intake reduces albuminuria, an early marker of renal damage and a sensitive predictor of adverse cardiovascular outcomes. The mechanisms underlying this effect are uncertain but small ch...
BACKGROUND: Lowering dietary salt intake reduces albuminuria, an early marker of renal damage and a sensitive predictor of adverse cardiovascular outcomes. The mechanisms underlying this effect are uncertain but small changes in serum sodium concentration may be important: this retrospective cohort study investigated the hypothesis that higher serum sodium concentration is a risk factor for albuminuria (defined as a urine albumin:creatinine ratio [UACR], ≥3 mg/mmol). METHODS: Primary care data from the Royal College of General Practitioners Research and Surveillance Centre were used to identify 47,294 individuals with a UACR result available between April 2010 and March 2015, and no known albuminuria prior to this. Exclusion criteria were missing or abnormal serum sodium concentration at baseline (<135 or >146 mmol/L); age <18 years; diabetes mellitus; decompensated liver disease; heart failure; and stage 5 chronic kidney disease. RESULTS: After adjustment for known risk factors, there was a significant "U-shaped" relationship between serum sodium concentration and albuminuria. The lowest risk was associated with a serum sodium of 138-140 mmol/L. In comparison, the risk of albuminuria was 18% higher with a serum sodium of 135-137 mmol/L and 19% higher with a serum sodium of 144-146 mmol/L. There was no association between serum sodium concentration and blood pressure. CONCLUSION: The finding of a positive association between higher serum sodium concentration and albuminuria is in support of the hypothesis, but the inverse relationship between serum sodium concentration and albuminuria at lower concentrations warrants further explanation.
Impact of Renin-Angiotensin System Blockade on Mortality and Allograft Loss among Renal Transplant Recipients: A Systematic Review and Meta-Analysis.
Kung CW, Lin YC, Tseng CS … +1 more , Chou YH
Nephron · 2024 · PMID 39008959 · Publisher ↗
INTRODUCTION: The blockade of the renin-angiotensin system (RAS) has a beneficial effect on reducing the levels of proteinuria and blood pressure in patients with chronic kidney disease (CKD) and reduces the risk of deve...
INTRODUCTION: The blockade of the renin-angiotensin system (RAS) has a beneficial effect on reducing the levels of proteinuria and blood pressure in patients with chronic kidney disease (CKD) and reduces the risk of developing end-stage kidney disease in CKD patients. Nonetheless, a debate persists regarding the impact of RAS inhibitors on outcomes such as mortality and graft survival in renal transplant patients. To assess the effect of RAS inhibitors on graft recipients in the past decade, we conducted a systematic review and meta-analysis. METHODS: We searched Embase, PubMed, and the Cochrane Central Register of Clinical Trials from January 1, 2012, to August 1, 2022. We included 14 articles, comprising 5 randomized controlled trials (RCTs) and 9 cohort studies, including 45,377 patients. These studies compared patient or graft survival between an RAS inhibitor treatment arm and a control arm. RESULTS: The meta-analysis revealed that RAS blockade was significantly associated with lower mortality in cohort studies (risk ratio [RR] = 0.66, 95% confidence interval [CI]: 0.55-0.79), reduced allograft loss in cohort studies (RR = 0.62, 95% CI: 0.54-0.71), and significant changes in systolic blood pressure in RCTs. Subgroup analysis of the groups of interest (interventions involving RAS blockade, follow-up period of ≥5 years) showed consistently reduced mortality (RR = 0.67, 95% CI: 0.56-0.81) and reduced allograft loss (RR = 0.61, 95% CI: 0.54-0.70). CONCLUSIONS: Our results demonstrated that the application of RAS blockade among renal transplant recipients was associated with lower mortality and allograft loss in cohort studies but not in RCTs. More powered clinical trials are needed to evaluate the effects of RAS blockade in renal transplant recipients.
Burden of Chronic Kidney Disease of Undetermined Aetiology (CKDu) in a Tertiary Care Public Hospital in North India.
Maity I, Sati H, Singh G … +3 more , Bhowmik D, Agarwal SK, Bagchi S
Nephron · 2024 · PMID 38964302 · Publisher ↗
INTRODUCTION: Chronic kidney disease of undetermined aetiology (CKDu) is an important public health problem. Indian data are mostly based on studies from rural regions in south and east India. We examined the burden and...
INTRODUCTION: Chronic kidney disease of undetermined aetiology (CKDu) is an important public health problem. Indian data are mostly based on studies from rural regions in south and east India. We examined the burden and profile of CKDu in patients attending a tertiary care hospital in north India. METHODS: We assessed records of consecutive new CKD patients registered in a nephrology clinic from January 2015 to June 2022. Patients were classified as having CKDu based on predefined inclusion and exclusion criteria. Clinical and laboratory parameters at presentation and kidney biopsy when done were noted. RESULTS: Records of 32,369 patients with CKD were screened, and 29,663 were included (2,706 excluded due to inadequate data). A total of 370 (1.2%) patients were categorized as CKDu. Mean age was 41 ± 14.7 years, 58.1% being male. Of them, 158 (42.7%) patients were in CKD stage 3, 89 (24.1%) in stage 4, 84 (22.7%) in stage 5, and 39 (10.5%) were dialysis dependent at presentation; 232 (62.7%) patients had proteinuria <0.5 gm/day and 138 (37.3%) between 0.5 and 1 gm/day. Renal histology was available for 65 CKDu patients: 62 had chronic tubulointerstitial nephritis (CTIN) and 3 had non-specific changes. CONCLUSION: When defined using strict criteria with intensive diagnostic workup, burden of CKDu is low in our hospital-based cohort of CKD patients. CTIN is the predominant histopathological finding in kidney biopsy.
Unsupervised Clustering of Membranoproliferative Glomerulonephritis and C3 Glomerulopathy Patients Discovers Distinct Patient Groups unlike the Current Classification.
Kovala M, Seppälä M, Wojnicki M … +4 more , Honkanen E, Meri S, Kaartinen K, Räisänen-Sokolowski A
Nephron · 2024 · PMID 38964287 · Publisher ↗
INTRODUCTION: Membranoproliferative glomerulonephritis is currently divided into immunoglobulin-mediated glomerulonephritis (IC-MPGN) and C3 glomerulopathy (C3G); however, the patients often overlap with histology, compl...
INTRODUCTION: Membranoproliferative glomerulonephritis is currently divided into immunoglobulin-mediated glomerulonephritis (IC-MPGN) and C3 glomerulopathy (C3G); however, the patients often overlap with histology, complement, clinical and prognostic factors. Our aim was to investigate if an unsupervised clustering method finds different patient groups in 44 IC-MPGN/C3G patients using only histological and clinical data available in everyday clinical work. METHODS: Primary IC-MPGN/C3G adult patients were included whose diagnostic (baseline) native biopsy was obtained in 2006-2017. The biopsies were reassessed and the clinical data at baseline and during follow-up were obtained from the medical records. There were 39 baseline histological and clinical variables included in the unsupervised clustering. Follow-up information was combined with the clustering results. RESULTS: The clustering resulted in two clusters (n = 24 and n = 20 patients for clusters 1-2, respectively), where cluster 1 had a significantly higher baseline plasma creatinine (mean 213 vs. 104, respectively, p value <0.001) and a lower baseline eGFR than cluster 2 (mean 37 vs. 70, respectively, p value <0.001). Regarding histology, chronic changes such as lobulated glomeruli, mesangial matrix expansion, and glomeruli double contours were more prevalent in cluster 1 (p value <0.001). Biopsy morphology was more often crescentic and membranoproliferative in cluster 1 (p value <0.001). Although the differences were insignificant, cluster 1 patients were in dialysis in the last follow-up or had a progressive disease more often than cluster 2 patients (21% vs. 5%, 38% vs. 10%). CONCLUSIONS: Our results indicate that these patients share greater similarity than the current classification IC-MPGN versus C3G indicates.
8th Update on Fabry Disease: Biomarkers, Progression and Treatment Opportunities in 2024.
Nephron · 2024 · PMID 38934177 · Publisher ↗
Fabry Update.
Fabry Update.
Reporting Practices for Animal Studies on Peritoneal Dialysis Conducted in 2021-2023 after the Introduction of the ARRIVE 2.0 Guidelines.
Witowski J, Sikorska D, Catar R
Nephron · 2024 · PMID 38934165 · Full text
INTRODUCTION: The first version of Animal Research: Reporting of in vivo Experiments (ARRIVE 1.0) guidelines was introduced to improve reporting of animal research but did not lead to major improvements in this respect....
INTRODUCTION: The first version of Animal Research: Reporting of in vivo Experiments (ARRIVE 1.0) guidelines was introduced to improve reporting of animal research but did not lead to major improvements in this respect. This applied also to animal studies on peritoneal dialysis (PD). Here, we examined the performance of the revised version of these guidelines (ARRIVE 2.0). METHODS: Eighty-nine relevant articles published in 2018-2020 (ARRIVE 1.0 period) and 97 published in 2021-2023 (ARRIVE 2.0 period) were identified in PubMed® and analyzed for completeness and transparency of reporting. RESULTS: In both periods, most studies were carried out in Asia, on rodents, and concerned the peritoneal pathophysiology. During ARRIVE 2.0, more studies were published in higher impact factor journals with the focus on pharmacology and immunology. Compared to ARRIVE 1.0, general aspects of study design and reporting improved during ARRIVE 2.0 period in studies generated in Europe and USA but did not change significantly in Asia. Detailed analysis showed no global improvement in completeness of reporting key information included in the ARRIVE 2.0 Essential 10 checklist. Articles from both periods were deficient in sample size calculations, use of blinding, recording adverse events and drop-outs, and specification of appropriate statistical methods. The level of reporting during ARRIVE 2.0 did not correspond to the journal impact factor and the presence of recommendations for the use of ARRIVE 2.0 in their instructions to authors. CONCLUSION: So far, ARRIVE 2.0 has not produced significant improvements in the reporting of animal studies in PD.
Magnesium Derangement among Critically Ill Patients with Acute Kidney Injury: An Association with Acute Kidney Disease.
Suppadungsuk S, Thongprayoon C, Nikravangolsefid N … +4 more , Singh W, Cheungpasitporn W, Dong Y, Kashani KB
Nephron · 2024 · PMID 38861941 · Publisher ↗
INTRODUCTION: The association between magnesium level and progression to acute kidney disease (AKD) in acute kidney injury (AKI) patients was not well studied. With AKI transition to AKD, the burden of the disease on mor...
INTRODUCTION: The association between magnesium level and progression to acute kidney disease (AKD) in acute kidney injury (AKI) patients was not well studied. With AKI transition to AKD, the burden of the disease on mortality, morbidity, and healthcare costs increases. Serum magnesium disturbances are linked with a decline in renal function and increased risk of death in CKD and hemodialysis patients. This study aims to assess the significance of magnesium derangements as a risk factor for the progression of AKI to AKD in critically ill patients. METHODS: This study was conducted among patients with AKI admitted to the intensive care units at Mayo Clinic from 2007 to 2017. Serum magnesium at AKI onset was categorized into five groups of <1.7, 1.7-1.9, 1.9-2.1, 2.1-2.3, and ≥2.3 mg/dL, with 1.9-2.1 mg/dL as the reference group. AKD was defined as AKI that persisted >7 days following the AKI onset. Logistic regression was used to evaluate the association between magnesium and AKD. RESULTS: Among 20,198 critically ill patients with AKI, the mean age was 66 ± 16 years, and 57% were male. The mean serum magnesium at AKI onset was 1.9 ± 0.4 mg/dL. The overall incidence of AKD was 31.4%. The association between serum magnesium and AKD followed a U-shaped pattern. In multivariable analysis, serum magnesium levels were associated with increased risk of AKD with the odds ratio of 1.17 (95% CI: 1.07-1.29), 1.13 (95% CI: 1.01-1.26), and 1.65 (95% CI: 1.48-1.84) when magnesium levels were <1.7, 2.1-2.3, and ≥2.3 mg/dL, respectively. CONCLUSION: Among patients with AKI, magnesium level derangement was an independent risk for AKD in critically ill AKI patients. Monitoring serum magnesium and proper correction in critically ill patients with AKI should be considered an AKD preventive intervention in future trials.
Comorbidity and Medication Trends in Chronic Kidney Disease and Incident Atrial Fibrillation: A Nationwide Cohort Study.
Jyrkilä H, Kaartinen K, Martola L … +10 more , Halminen O, Haukka J, Linna M, Mustonen P, Putaala J, Teppo K, Kinnunen J, Hartikainen J, Airaksinen KEJ, Lehto M
Nephron · 2024 · PMID 38861935 · Publisher ↗
INTRODUCTION: Chronic kidney disease (CKD) is associated with an increased incidence of atrial fibrillation (AF). Also, patients with AF are prone to adverse kidney outcomes. We examined comorbidities and medication use...
INTRODUCTION: Chronic kidney disease (CKD) is associated with an increased incidence of atrial fibrillation (AF). Also, patients with AF are prone to adverse kidney outcomes. We examined comorbidities and medication use in patients with CKD and incident AF. METHODS: The Finnish AntiCoagulation in Atrial Fibrillation (FinACAF) is a nationwide retrospective register-linkage study including data from 168,233 patients with incident AF from 2007 to 2018, with laboratory data from 2010 onwards. Estimated glomerular filtration rate (eGFR) was available for 124,936 patients. The cohort was divided into 5 CKD stages with separate groups for dialysis and kidney transplantation. RESULTS: At AF diagnosis eGFR <60 mL/min/1.73 m2 was found in 27%, while 318 (0.3%) patients were on dialysis, and 188 (0.2%) had a functioning kidney transplant. Lowering eGFR yielded more comorbidities and medications. During 2010-2018 in patients with eGFR <60 mL/min/1.73 m2 prevalence of hypertension, dyslipidaemia, and diabetes increased from 82 to 88%, from 50 to 66% and from 25 to 33%, respectively (<0.001). Throughout the observation period, lipid-lowering medication was underused. CONCLUSION: More than one-fourth of patients with incident AF also had CKD stage 3-5 (eGFR <60 mL/min/1.73 m2). Both comorbidities and medication use increased with worsening kidney function. Prevalence of major cardiovascular (CV) risk factors increased during 2010-2018, but the use of survival-affecting medications, such as lipid-lowering medication, was suboptimal at all stages of CKD. More attention should be given to the optimal treatment of risk factors in this high CV risk population.
Risk Factors and Clinical Outcomes of Renal Thrombotic Microangiopathy in Children with Lupus Nephritis in Terms of Pathological and Clinical Features.
Zhang P, Yang X, Fu MZ … +3 more , Gao CL, Fang X, Xia ZK
Nephron · 2024 · PMID 38852577 · Publisher ↗
BACKGROUND: Thrombotic microangiopathy (TMA) is an important risk factor for the prognosis of lupus nephritis (LN). Patients with LN complicated with TMA tend to be critically ill with high mortality and poor prognosis....
BACKGROUND: Thrombotic microangiopathy (TMA) is an important risk factor for the prognosis of lupus nephritis (LN). Patients with LN complicated with TMA tend to be critically ill with high mortality and poor prognosis. In the present study, we retrospectively analyzed the clinical manifestations, laboratory results, renal pathological manifestations, and prognosis of children with LN-TMA and analyzed the risk factors for end-stage renal disease (ESRD) in children with LN-TMA. METHODS: Seventy-four patients with LN and renal TMA (rTMA) were selected and compared to 128 LN controls without TMA (1:2 ratio) matched according to demographics, pathological type and treatments. RESULTS: The mean values of systolic blood pressure, diastolic blood pressure (DBP), lactate dehydrogenase (LDH), blood urea nitrogen (BUN), urinary protein quantitation (PRO), urine red blood cells, N-acetyl-β-D-glucosidase (NAG), retinol-binding protein, systemic lupus erythematosus disease activity score (SLEDAI), and activity index (AI) scores in the TMA group were all higher than those in the non-TMA group (p < 0.05 and p < 0.01). The mean values of complement C3, hemoglobin, platelets, estimated glomerular filtration rate, and chronic index (CI) score in the TMA group were all lower than those in the non-TMA group (p < 0.05 and p < 0.01). The number of cases of glomerular crescent, fibrous crescent, endocapillary proliferation, tubular atrophy, interstitial fibrosis, C3 and C1q deposition in the TMA group was higher than that in the non-TMA group (p < 0.05 and p < 0.01). The 3-year and 5-year renal survival rates in the TMA group (88.93% vs. 97.00%, p < 0.05) and TMA group (61.41% vs. 82.31%, p < 0.05) were significantly lower than those in the non-TMA group. Multivariate Cox regression analysis showed that serum creatinine before treatment (≥110 μmol/L), TMA and interstitial fibrosis were independent risk factors for the development of ESRD in LN children. CONCLUSION: The general condition of children with TMA is critical, and the prognosis is poor. Early detection, early treatment and the development of new treatments are key to improving LN-TMA outcomes in children.
The SLC6A18 Transporter Is Most Likely a Na-Dependent Glycine/Urea Antiporter Responsible for Urea Secretion in the Proximal Straight Tubule: Influence of This Urea Secretion on Glomerular Filtration Rate.
Bankir L, Crambert G, Vargas-Poussou R
Nephron · 2024 · PMID 38824912 · Full text
BACKGROUND: Urea is the major end-product of protein metabolism in mammals. In carnivores and omnivores, a large load of urea is excreted daily in urine, with a concentration that is 30-100 times above that in plasma. Th...
BACKGROUND: Urea is the major end-product of protein metabolism in mammals. In carnivores and omnivores, a large load of urea is excreted daily in urine, with a concentration that is 30-100 times above that in plasma. This is important for the sake of water economy. Too little attention has been given to the existence of energy-dependent urea transport that plays an important role in this concentrating activity. SUMMARY: This review first presents functional evidence for an energy-dependent urea secretion that occurs exclusively in the straight part of the proximal tubule (PST). Second, it proposes a candidate transmembrane transporter responsible for this urea secretion in the PST. SLC6A18 is expressed exclusively in the PST and has been identified as a glycine transporter, based on findings in SLC6A18 knockout mice. We propose that it is actually a glycine/urea antiport, secreting urea into the lumen in exchange for glycine and Na. Glycine is most likely recycled back into the cell via a transporter located in the brush border. Urea secretion in the PST modifies the composition of the tubular fluid in the thick ascending limb and, thus, contributes, indirectly, to influence the "signal" at the macula densa that plays a crucial role in the regulation of the glomerular filtration rate (GFR) by the tubulo-glomerular feedback. KEY MESSAGES: Taking into account this secondary active secretion of urea in the mammalian kidney provides a new understanding of the influence of protein intake on GFR, of the regulation of urea excretion, and of the urine-concentrating mechanism.
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