Introduction: Tolvaptan is the only FDA- and EMA approved disease-modifying treatment for autosomal dominant polycystic kidney disease (ADPKD), although its tolerability is associated by hydration status and aquaretic adverse effects. Environmental and climatic conditions may affect thirst perception and fluid loss; however, their influence on tolvaptan treatment outcomes has not been adequately investigated. Methods : This retrospective multicenter observational cohort study included 205 adults with ADPKD from 11 nephrology centers in Türkiye who received tolvaptan therapy for at least 12 months between 2020 and 2022. Demographic, clinical, laboratory, and imaging data were collected from medical records. Meteorological parameters including temperature, humidity, sunshine duration, latitude, and longitude were obtained from the Turkish National Meteorology Institute. Correlations between climatic variables and treatment-related parameters were evaluated using Spearman correlation analysis. Results: Treatment interruption occurred in 27.6
Primary Sjögren’s syndrome (pSS) is a systemic autoimmune disorder most commonly affects older women. It can involve the kidneys, most commonly causing distal renal tubular acidosis (dRTA). Overt nephrogenic diabetes insipidus (NDI) is rare, and the simultaneous occurrence of both tubular defects as the presenting feature of pSS, particularly in adolescents, is exceptional and presents a challenging diagnostic puzzle. We present a case of a 17-year-old female with a 5-year history of polydipsia and polyuria (daily intake/output 6–7 L), who was found to have severe hypokalemia, hyperchloremic normal anion-gap metabolic acidosis, and inappropriately alkaline urine, confirming distal RTA. Water deprivation test with desmopressin demonstrated urine osmolality that remained below 150 mOsm/kg despite rising serum osmolality and lacked significant increase after desmopressin, strongly supporting the diagnosis of NDI. Positive anti-SSA/SSB antibodies, objective sicca findings, and renal biopsy revealing active tubulointerstitial nephritis led to the diagnosis of pSS with dual tubular manifestations. Treatment with prednisone, mycophenolate mofetil, hydrochlorothiazide, and high-dose potassium supplements led to significant clinical and biochemical improvement. This case illustrates a systematic approach to polyuria and hypokalemia in an adolescent, unraveling a rare dual tubulopathy as the initial manifestation of pSS. We hypothesize that NDI in this setting may result from a “two-hit” mechanism: direct autoimmune injury to the collecting duct and profound hypokalemia-induced downregulation of aquaporin-2. Recognizing this association and initiating early immunosuppression can partially reverse tubular dysfunction and improve long-term outcomes.
Beta-D-glucan (BDG) levels are elevated in people receiving haemodialysis (HD), perhaps reflecting translocation of dietary BDG and gut-derived microbial products, and are a putative cause of HD-associated inflammation. BDG has been advocated as a marker of increased gut permeability. We evaluated an oral BDG challenge as a potential test for gut permeability in chronically inflamed HD patients. Twenty individuals receiving either high-flux HD (HF-HD; n = 5) or haemodiafiltration (HDF; n = 15) with chronic inflammation (defined by a baseline 3-monthly median serum high sensitivity CRP ≥ 5 mg/L in the absence of a clinically detectable inflammatory condition) received a meal enriched with BDG, immediately prior to a dialysis session. Twenty people with normal kidney function (healthy comparator group) received the same meal for comparison. Serum BDG levels, measured using the Fungitell® assay, were taken at baseline, 0.5, 1, 1.5, 2, 3, 4 and 6 hours. Analysable data was available in 19 dialysis patients and 20 in the healthy comparator group. The dialysis group was significantly older, more comorbid and had a higher BMI. Serum BDG levels were higher in the dialysis group than in the comparator group at all timepoints. There was no significant increment post-BDG challenge in the comparator group nor in the dialysis group as a whole. However, despite similar baseline subgroup characteristics, there were disparate results in the HDF and HF-HD subgroups, with serum BDG levels rising significantly between 4 and 6 hours post challenge in the HDF group and falling slightly in the HF-HD group over the same period. BDG levels were significantly higher the dialysis group than in the healthy comparator group but there were no significant changes in serum levels following dietary challenge in either group overall. This masked unexpected subgroup differences with serum BDG levels rising significantly post-challenge in the HDF group and falling in the HF-HD group. These subgroup findings, based on small numbers, are exploratory and underpowered, and should therefore be interpreted with caution and require confirmation in larger cohorts.
Unplanned hemodialysis initiation is associated with poor outcomes and may occur in patients with greater clinical severity and inadequate predialysis preparation. However, the clinical profile and cumulative mortality during the first year among patients undergoing unplanned initiation remain incompletely characterized, particularly in contemporary Asian cohorts. This study aimed to characterize the clinical profile of patients undergoing unplanned hemodialysis initiation and to compare cumulative mortality during the first year between patients undergoing planned and unplanned initiation. This retrospective cohort study included incident maintenance hemodialysis patients at Lampang Hospital, Thailand. Hemodialysis initiation was classified as planned or unplanned by medical record review using prespecified criteria. Unplanned initiation was defined as urgent or emergent initiation due to life-threatening complications or inadequate predialysis preparation. The primary outcome was 1-year all-cause mortality, with 90-day and 180-day mortality as secondary outcomes. An exploratory multivariable Cox model adjusted for age, diabetes mellitus, serum creatinine, and serum albumin. Among 233 incident hemodialysis patients, 135 underwent unplanned and 98 underwent planned initiation. Patients undergoing unplanned initiation had greater clinical and metabolic severity at baseline, including more frequent uremic symptoms, volume overload, respiratory failure, metabolic acidosis, and lower hemoglobin and serum albumin levels. During the first year, 26 deaths occurred. Cumulative mortality was higher in the unplanned group at 90 days (4.4
Patients with malignant hypertension (mHTN)-associated renal thrombotic microangiopathy (TMA) often present with progressive renal dysfunction, and some require dialysis at the time of diagnosis. Previous evidence has suggested that some patients may discontinue dialysis during follow-up after intensive antihypertensive therapy. However, the incidence of dialysis discontinuation and its associated factors remain unclear. This study aimed to investigate the incidence and associated factors of dialysis discontinuation in patients with mHTN-associated renal TMA who required dialysis at baseline. 132 patients with biopsy-proven mHTN-associated renal TMA who were dialysis-dependent at baseline were enrolled in this study. Patients were categorized into the dialysis discontinuation group (Group 1) and the dialysis-dependent group (Group 2). Cox regression analysis was performed to identify factors associated with dialysis discontinuation. Among the 132 patients, 50 (37.9
Sarcopenia is a prevalent complication in maintenance hemodialysis (MHD) patients and is associated with adverse outcomes. This study aimed to investigate the relationship between sarcopenia and bone metabolism markers and to develop a clinical screening model for sarcopenia in MHD patients. In this cross-sectional study, total of 292 MHD patients were classified into sarcopenia and non-sarcopenia groups according to the European Working Group on Sarcopenia in Older People 2 (EWGSOP2) critical value. Demographic characteristics, laboratory parameters, and anthropometric measurements, including body composition assessed by bioelectrical impedance analysis (BIA), were collected. A nomogram was constructed to screen for sarcopenia. The overall prevalence of sarcopenia was 26.4
Nafamostat mesylate (NM) is frequently used for continuous renal replacement therapy (CRRT) anticoagulation when clinicians wish to avoid sustained systemic anticoagulation, whereas low-molecular-weight heparin (LMWH) is commonly selected when systemic anticoagulation is considered acceptable. However, direct evidence comparing NM with LMWH in adult intensive care unit (ICU) patients undergoing CRRT remains limited. This study compared filter lifespan, circuit-related failure and safety outcomes between NM and LMWH in a real-world ICU CRRT cohort. This single-center retrospective cohort study included adult ICU patients receiving CRRT with NM or LMWH between January 2022 and January 2023. The primary outcome was filter lifespan. In the primary time-to-event analysis, circuit-related filter failure was defined as clotting-related failure or access/device-related failure, while planned and clinical-event-related terminations were censored. Kaplan–Meier analysis, Cox regression with patient-level cluster-robust standard errors, shared-frailty Cox regression, and propensity score–matched analyses were used to compare filter survival, account for repeated circuits within patients, adjust for CRRT prescription factors, and assess robustness. A total of 116 patients contributing 344 filters were included: 31 patients with 132 filters in the NM group and 85 patients with 212 filters in the LMWH group. NM-treated patients had greater illness severity and more pronounced coagulation abnormalities. Unadjusted Kaplan–Meier analysis showed shorter filter survival with NM than with LMWH (log-rank P = 0.007). However, anticoagulant strategy was not significantly associated with circuit-related filter failure in the primary Cox model (HR 1.41; 95
Kidney disease is a leading cause of morbidity and mortality globally. Glomerulonephritis is a leading cause of kidney disease that is best characterized by biopsy. There are limited data available on glomerulonephritis in the Kenyan population. Factors such as the prevalence of APOL1 high risk alleles, endemic HIV infection, high prevalence of other infectious diseases and increased susceptibility to autoimmune diseases may influence the prevalence of kidney disease in sub–Saharan Africa. This study aimed to describe selected clinical and histopathological findings in patients with medical kidney disease who had biopsies examined at the Aga Khan University Hospital, Nairobi. A retrospective chart review was done of the laboratory reports and clinical records of patients who had native medical kidney biopsies reported at Aga Khan University Hospital Nairobi from 2018 to 2024. Three hundred and forty-seven cases were reviewed. The commonest indication for renal biopsy was nephrotic syndrome (33.4
People undergoing hemodialysis (HD) experience falls with a high incidence and frequency, which has an important impact on morbidity, quality of life, and the use of healthcare resources. Therefore, the early identification of risk factors and adequate screening tools are key for developing efficient prevention strategies. The aim was to estimate the incidence of falls among people undergoing dialysis and to analyze the associated factors, as well as to assess the performance of the Downton scale on the identification of fall risk, and to compare it with the Index of Frailty-VIG (IF-VIG). A prospective observational cohort study with a 12-month follow-up was conducted on 102 hemodialysis patients. A baseline assessment was performed of the clinical, pharmacological, functional, and social factors. The fall events that occurred during the follow-up period were recorded. A descriptive and analytical analysis was performed, using the chi-square test for the bivariate analysis, a multivariate analysis to identify associated factors, and ROC curves to evaluate the discriminatory ability of the screening tools. A total of 102 patients undergoing hemodialysis were included (mean age 72.3 ± 11.6 years; 70.6
Cardiovascular disease is a leading cause of early mortality after kidney transplantation; however, the associations of pre-transplant cardiac risk markers and coronary management strategy with early post-transplant outcomes remain incompletely characterized. This study aimed to evaluate the association between pre-transplant cardiac risk phenotype, coronary management strategy, and 1-year mortality in living-donor kidney transplant recipients. In this retrospective single-center cohort, 178 adult living-donor kidney transplant recipients (June 2021–August 2024) with at least 12 months of follow-up were included. Patients were categorized according to pre-transplant coronary management: no revascularization (n = 155), percutaneous coronary intervention (PCI) (n = 15), or minimally invasive coronary artery bypass grafting (MIDCAB) (n = 8). The primary outcome was 1-year all-cause mortality. Survival was assessed using Kaplan–Meier analysis and log-rank testing. To minimize overfitting given the limited number of events (n = 13), the primary multivariable Cox model included three clinically pre-specified variables reflecting global vulnerability: age, left ventricular ejection fraction (EF < 55
Venous valve–related occlusion (VVRO) is an underdiagnosed cause of arteriovenous fistula (AVF) dysfunction in patients with end-stage renal disease (ESRD). Data regarding the long-term outcomes and prognostic factors associated with ultrasound-guided percutaneous transluminal angioplasty (PTA) for VVRO remain limited. This retrospective cohort study included ESRD patients with VVRO treated by ultrasound-guided PTA following thrombolysis between January 2017 and December 2023. VVRO was diagnosed based on post-thrombolysis duplex ultrasound findings and classified as type I (≤ 3 cm from the anastomosis) or type II (> 3 cm). Patency rates were analyzed using the Kaplan-Meier method. Univariable and multivariable Cox models were used to identify potential factors associated with primary patency. Among 94 patients, 50 had type I VVRO and 44 had type II. The mean age was 56 years, and the median dialysis duration was 35.3 months. Most patients were male (60.6
Hyperphosphatemia is a common complication of chronic kidney disease (CKD) and has been implicated in systemic inflammation, protein-energy wasting, anemia, and skeletal muscle wasting. Hypoalbuminemia is also frequent in CKD and is associated with poor clinical outcomes. Although CKD-associated hypoalbuminemia is generally interpreted in the context of malnutrition, inflammation, protein loss, or increased albumin catabolism, whether extracellular phosphate affects hepatocyte albumin-producing capacity remains unclear. HepaRG cells were treated with vehicle, inorganic phosphate (Pi), fibroblast growth factor 23 (FGF23), or Pi plus FGF23. Albumin, HNF4A, and IL6 expression levels were examined using RNA sequencing, and additional transcriptomic overview analyses, including TCC-based differential-expression visualization and TRRUST-based HNF4A target gene analysis, were performed. Albumin secretion into culture supernatants was measured by enzyme-linked immunosorbent assay. Cell viability was assessed by MTT assay. ERK phosphorylation after short-term Pi stimulation was evaluated by immunoblot analysis. Pi exposure reduced albumin expression in HepaRG cells, whereas FGF23 alone did not exert a comparable suppressive effect. Pi exposure was also associated with reduced HNF4A expression, a key transcriptional regulator of hepatocyte differentiation and liver-specific gene expression. Additional transcriptomic overview analyses, including TCC-based differential-expression visualization and TRRUST-based HNF4A target gene analysis, suggested attenuation of hepatocyte-function/HNF4A-associated gene programs in Pi-treated cells. Consistent with the transcriptomic findings, Pi, but not FGF23 alone, significantly reduced albumin secretion into the culture supernatant. This reduction was not attributable to decreased cell viability. Pi rapidly increased ERK phosphorylation. Notably, Pi-associated reductions in albumin and HNF4A were not accompanied by robust IL6 induction under the present experimental conditions. Under the present acute, supraphysiological exposure conditions, phosphate-salt treatment was associated with attenuation of the albumin-producing phenotype in HepaRG cells, accompanied by HNF4A downregulation and ERK activation. Because sodium/osmolality-matched controls and particle characterization were not included, these findings cannot be attributed specifically to dissolved phosphate. Further studies under physiologically relevant conditions are required to determine phosphate specificity and potential relevance to CKD-associated hypoalbuminemia.
The objectives of this study were to compare the sensitivity of two urine dipstick tests commercially available for detecting proteinuria and to project the potential impact of replacing one with the other for chronic kidney disease (CKD) screening in a large Brazilian health insurance company. Urine spot tests were prospectively collected from a convenience sample of 410 adults with ≥ 1 CKD risk factor from a specialized outpatient clinic. Proteinuria was assessed by three methods: (1) total protein strip (“S10”, the method currently used); (2) albumin-to-creatinine ratio strip (“S12”, the index test), and (3) quantitative measurement of albumin-to-creatinine ratio (UACR, the standard reference test). The UACR measurements were categorized into three severity levels, in accordance with current guidelines: mild (< 30 mg/g), moderate (30-300 mg/g), and severe (> 300 mg/g). To project the potential impact of replacing S10 with S12, the one-year amount of ambulatory routine urinalysis tests was assessed. Out of 410 study participants, 200 had quantitative UACR ≥ 30 mg/g. S12 showed greater sensitivity than S10 for detecting UACR ≥ 30 mg/g (95
Hypoxia-inducible factor-prolyl hydroxylase inhibitors (HIF-PHIs) are novel drugs used to treat renal anemia patients.However, their efficacy in elevating hemoglobin levels varies among patients. We aimed to identify characteristics that could influence hemoglobin level in renal anemia patients treated with HIF-PHIs. We conducted a retrospective cohort study in renal anemia patients treated with HIF-PHIs (roxadustat and enarodustat) with CKD stages 3–5 from 2023 to 2025, and both non-dialysis patients and dialysis patients were included. Patients with CKD stage 5 requiring dialysis were classified as having end-stage kidney disease (ESKD), these patients were also included. Patients were assigned into Hb responder or non-responder group based on hemoglobin changes (elevation, non-elevation) from 0 to 3 months. Logistic regression was applied to explore characteristics associated with the effect of HIF-PHI on achieving targeted hemoglobin levels in renal anemia patients. A total of 459 renal anemia patients were included, including 341 and 118 in the Hb responder and non-responder groups, respectively. There were significant differences in lymphocyte percentage, neutrophil percentage, phosphorus, chloride, blood urea nitrogen, and serum creatinine between the Hb responder and non-responder groups (P < 0.05). A higher probability of Hb response to HIF-PHI therapy was observed when hemoglobin levels were between 100 and 110 g/L. Treatment response was superior when HIF-PHIs were initiated prior to chronic kidney disease stage 4. Logistic regression analysis identified initial hemoglobin levels of 80–90 g/L as an independent risk factor affecting HIF-PHI efficacy, with satisfactory model performance. Renal function stage and electrolyte disturbances were associated with Hb response to HIF-PHIs in patients with renal anemia. Early and appropriate treatment timing may enhance the efficacy of HIF-PHIs to elevate hemoglobin levels in these patients.
Kidney transplantation is the optimal treatment for kidney failure, but whether primary care use among those on dialysis is associated with increased rates of kidney transplantation is unknown. We conducted a retrospective cohort study using United States Renal Data System (USRDS) and Medicare Parts A, B, and D claims to estimate the association between any primary care use (vs none) and both kidney transplantation and “active” kidney transplant waitlisting. Primary care use was defined as ≥ 1 outpatient visit to a family, general, geriatric, or internal medicine provider from January 1st, 2018 to December 31st, 2018. Patients were followed January 1st2019 until December 31st2020. We identified 74,028 (60
Patients undergoing haemodialysis require lifelong treatment and often suffer from symptoms such as fatigue and pruritus, which negatively affect their comfort, health outcomes, and quality of life. However, few studies have investigated the complex relationships among these symptoms. Therefore, in this study, we investigated relationships among pruritus, fatigue, and comfort in patients undergoing haemodialysis, and examined whether fatigue mediates the relationship between pruritus and comfort. This study used a cross-sectional design and convenience sampling. In total, 328 patients receiving haemodialysis were recruited from two hospitals. Instruments included participant background information, and the Vietnamese versions of the Post-Dialysis Fatigue Scale, the 5-D Itch Scale, and the Hemodialysis Comfort Scale version II. Data were analysed using independent t-tests, one-way ANOVAs, Pearson’s correlations, hierarchical multiple regressions, and bootstrapping for mediation analyses. Average scores of comfort, fatigue, and pruritus were 97.14 ± 13.32, 38.85 ± 8.97, and 10.15 ± 4.47, respectively. Both fatigue and pruritus were significantly and negatively associated with comfort levels. Fatigue explained 13.4
Chronic kidney disease (CKD) is characterized by progressive decline in glomerular filtration rate (GFR) and is associated with activation of neurohormonal pathways, including the arginine vasopressin system. Copeptin, the C-terminal fragment of pre-pro-vasopressin, has emerged as a stable surrogate marker of vasopressin activity. This cross-sectional study compared serum copeptin levels across clinically distinct study groups and evaluated their associations with renal and biochemical parameters. The study included 120 participants: 30 healthy controls and 90 patients with CKD, comprising stage 3 CKD, stage 4 CKD, and end-stage kidney disease (ESKD) receiving maintenance hemodialysis groups (n = 30 each). In patients receiving maintenance hemodialysis, fasting blood samples were obtained immediately before the scheduled dialysis session. Serum copeptin concentrations were measured using an enzyme-linked immunosorbent assay (ELISA). Renal function was assessed using 24-hour urinary creatinine clearance; in patients receiving maintenance hemodialysis, these values were interpreted as approximate estimates of residual kidney function. Intergroup comparisons were performed using one-way ANOVA, and correlations were analyzed using Pearson’s test. A two-tailed p value < 0.05 was considered statistically significant. Serum copeptin levels were significantly higher in the hemodialysis group than in the control, stage 3 CKD, and stage 4 CKD groups (overall p < 0.001). No significant differences were observed among the control, stage 3 CKD, and stage 4 CKD groups. Copeptin was negatively correlated with 24-hour urinary creatinine clearance (r = − 0.237, p < 0.01) and positively correlated with creatinine, parathyroid hormone, and glucose. No significant correlations were observed with C-reactive protein, calcium, phosphorus, or urea. In this cohort, copeptin levels were significantly elevated in patients receiving maintenance hemodialysis, whereas no significant differences were observed between controls and patients with stage 3 or stage 4 CKD. Because non-dialysis stage 5 CKD was not evaluated, these findings should be considered exploratory. Larger prospective studies incorporating appropriate multivariable analyses and longitudinal follow-up are required to clarify the significance of copeptin in CKD.
Louis XIV’s rapidly progressive terminal illness may be better explained by necrotizing soft-tissue infection than primary calciphylaxis. Severe distal pain, ascending gangrene, and fatal sepsis within three weeks suggest fulminant infection, plausibly arising from a diabetic foot lesion. Clinical chronology should remain central when evaluating competing retrospective clinicopathological hypotheses.
Home- and community-based services (HCBS) serve as a non-institutional care and support model, exhibiting application potential in chronic disease management. However, longitudinal evidence supporting the association between HCBS and the risk of incident chronic kidney disease (CKD) in patients with diabetes remains scarce. To investigate the potential association between HCBS and incident CKD risk in diabetic patients. Based on 2015–2020 data from the China Health and Retirement Longitudinal Study (CHARLS), this longitudinal cohort study included individuals with prevalent diabetes in 2015 and no CKD before 2018. Participants were grouped by HCBS utilization in 2018. Weighted logistic regression models were used to examine the association between HCBS and the risk of incident CKD among patients with diabetes. Subgroup analyses were performed stratified by age, sex, residence, education level, marital status, smoking, alcohol consumption, hypertension and dyslipidemia. Sensitivity analysis, E-value calculation and weighted Poisson regression with robust standard errors were conducted to verify the robustness of the results. A total of 1,269 diabetic patients were enrolled, among whom 283 (22.3
The spectrum of biopsy-proven kidney diseases varies across geographic regions and is influenced by biopsy practices and referral patterns. In India, the absence of a national kidney biopsy registry limits the availability of epidemiological data. We analysed kidney biopsies performed over a 12-year period at a large tertiary care referral centre in Northern India to describe the demographic, clinical, and histopathological spectrum of kidney diseases. This retrospective, observational study included all native kidney biopsies performed between January 2013 and October 2024. Demographic characteristics, clinical indications, comorbidities, and histopathological diagnoses were retrieved from hospital records. Biopsies were evaluated using light microscopy (LM) and immunofluorescence (IF) in all cases, with electron microscopy (EM) performed selectively. Diagnoses were categorised into primary glomerular disease, secondary glomerular disease, tubulointerstitial disease, vascular disease, and advanced sclerosis. Data was analysed descriptively. A total of 1,664 kidney biopsies were included (mean age 41 ± 16.9 years; 64.4
