Search

The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic led to rapid vaccine development and large global vaccination schemes. However, patients with immune-mediated kidney disease, chronic kidney diseases and kidney transplant recipients show high non-response rates to vaccination despite more than three vaccinations and, consequently, reduced viral clearance capacity when infected while receiving certain immunosuppressants, carrying an elevated risk for coronavirus disease 2019 (COVID-19)-related morbidity and mortality. SARS-CoV-2 evolution has been characterized by the emergence of novel variants and spike mutations contributing to waning efficacy of neutralizing antibodies. To this end, the therapeutic field expands from vaccination towards a combined approach of immunization, pre-exposure prophylaxis and early post-exposure treatment using direct-acting antivirals and neutralizing monoclonal antibodies to treat early in the disease course and avoid hospitalization. This expert opinion paper from the Immunonephrology Working Group of the European Renal Association (ERA-IWG) summarizes available prophylactic and/or early treatment options (i.e. neutralizing monoclonal antibodies and direct-acting antivirals) of SARS-CoV-2-infected patients with immune-mediated kidney disease, chronic kidney disease and kidney transplant recipients.

In the last decades several important advances have taken place in the understanding of the pathogenesis underlying membranous nephropathy, which have sparked renewed interest in its management. Four landmark trials in membranous nephropathy, and a fifth clinical trial-which was a pilot study-, have been published in the last years. The results from some of these trials have had a significant impact in the recommendations included in the 2021 Kidney Disease: Improving Global Outcomes (KDIGO) Guideline for the Management of Glomerular Diseases, which represent a significant step forward compared to the previous guideline in several aspects such as diagnosis, disease monitoring and treatment strategies. However, considering the rapidly evolving advances in the knowledge of membranous nephropathy and the recent publication of the STARMEN and RI-CYCLO trials, several recommendations contained in the guideline warrant updates. This manuscript provides a perspective of the Immunonephrology Working Group of the European Renal Association (ERA) regarding the management of membranous nephropathy in native kidneys of adult patients.

Purpose Because of the increase in globalization, clinicians all over the world are confronted the questions about safety of Ramadan fasting. However, there is no clear information about whether there is an obstacle for fasting patients with chronic disease. Hence, in the present study, we aimed to investigate the effects of Ramadan fasting on kidney and the factors affecting this relationship in patients with chronic kidney disease. Methods This study involving 117 patients [36 females, 81 males; mean age, 60 (46.0-70.0) years] with stage 2-3 chronic kidney and fasting. Patients were evaluated every 10 days for acute kidney injury (AKI) in Ramadan month. And, patients with acute kidney injury and patients without AKI were grouped. Results When the patients were evaluated for AKI, we observed that in 27 patients had acute kidney injury. While patients without AKI fasted for more days (p < 0.001), urea levels and frequency of hypertension were higher in the group with AKI (p = 0.019;p = 0.025 respectively). We also performed univariate and multiple binary logistic regression analysis to identify the risk factors of AKI. Hypertension and number of fasting day were found to be predictive of AKI (p = 0.02;p < 0.001 respectively). Conclusions We found a significant relationship between hypertension, the number of fasting days and acute kidney injury. Patients with chronic kidney damage and hypertension should be evaluated more carefully, informed about the importance of hydration after fasting and should be followed frequently for AKI.

© 2022 The Author(s). Published by Informa UK Limited, trading as Taylor & Francis Group.Background: We aimed to evaluate the features of primary membranous nephropathy (MNP) in Turkish people. Methods: This is a retrospective analysis of patients with biopsy-proven primary MNP. We obtained the data collected between 2009 and 2019 in the primary glomerulonephritis registry of the Turkish Society of Nephrology Glomerular Diseases Study Group (TSN-GOLD). Patients with a secondary cause for MNP were excluded. Clinical, demographic, laboratory, and histopathological findings were analyzed. Results: A total of 995 patients with primary MNP were included in the analyses. Males constituted the majority (58.8%). The mean age was 48.4 ± 13.9 years. The most common presentation was the presence of nephrotic syndrome (81.7%) and sub nephrotic proteinuria (10.3%). Microscopic hematuria was detected in one-third of patients. The median estimated glomerular filtration rate (eGFR) was 100.6 mL/min/1.73 m2 (IQR, 75.4–116.3), and median proteinuria was 6000 mg/d (IQR, 3656–9457). Serum C3 and C4 complement levels were decreased in 3.7 and 1.7% of patients, respectively. Twenty-four (2.4%) patients had glomerular crescents in their kidney biopsy samples. Basal membrane thickening was detected in 93.8% of cases under light microscopy. Mesangial proliferation and interstitial inflammation were evident in 32.8 and 55.9% of the patients, respectively. The most commonly detected depositions were IgG (93%), C3 complement (68.8%), and kappa and lambda immunoglobulin light chains (70%). Although renal functions were normal at presentation, vascular, interstitial, and glomerular findings were more prominent on biopsy in hypertensive patients. No significant effect of BMI on biopsy findings was observed. Conclusions: Despite some atypical findings, the main features of primary MNP in Turkey were similar to the published literature. This is the largest MNP study to date conducted in Turkish people.

Coronavirus disease 2019 (COVID-19) vaccine platforms are becoming available and are the most promising strategy to curb the spread of severe acute respiratory syndrome coronavirus 2 infections. However, numerous uncertainties exist regarding the pros and cons of vaccination, especially in patients with (immune-mediated) kidney diseases on immunosuppressive drugs. Here, members of the Immunonephrology Working Group of the European Renal Association–European Dialysis and Transplant Association discuss 13 frequently asked questions regarding the safety and efficacy of the most promising vaccine candidates. Post-marketing surveillance should be performed to estimate the rate of vaccine response (humoral and cellular) of different vaccine platforms and disease activity following the administration of COVID-19 vaccines. Some of the candidates induce signalling pathways, which also promote autoimmune kidney diseases, e.g. type I interferons in systemic lupus erythematosus. Efficacy estimates would thus far favour the use of selected COVID-19 vaccines, such as BNT162b2, mRNA-1273 or Gam-COVID-Vac. Humoral immune response after vaccination should be monitored using appropriate assays. Even in the absence of neutralizing antibodies, patients might be protected by a sufficient cellular immune response capable of reducing the severity of COVID-19. A reduced vaccine response after the use of CD20-depleting agents is anticipated and it is particularly important to discuss strategies to improve vaccine response with these patients. Distancing and shielding measures remain important, as not all vaccines fully protect from coronavirus infection. In-depth information about the most pressing vaccine questions is essential to reduce vaccine hesitancy of patients.

Recent studies claim that FGF23 is also associated with anemia and inflammation. In this study, the relationship between FGF23 and anemia in hemodialysis (HD) and renal transplantation patients (RTx patients) patients was investigated. This was a cross-sectional study involving 40 RTx patients (13 females, 27 males; mean age, 45.93 ± 12.49 years) who had transplantation at least 6 months before, 25 HD patients (12 females, 13 males; mean age, 54.72 ± 15.5 years), and 20 healthy control subjects (13 females, 7 males; mean age, 36.7 ± 9.38 years). FGF23 was studied using Elisa method. Parameters such as iron, ferritin, total iron binding capacity, and transferrin saturation were assessed. FGF23 level was significantly higher in HD patients when compared with the RTx patients and control groups. In the bivariate correlation analysis, hemoglobin was positively correlated with albumin (r = 0.681, p = 0.000), ferritin (r = 0.446, p = 0.043), and negatively correlated with CRP (r = − 0.476, p = 0.016) and FGF23 (r = 0.493, p = 0.043). FGF23 was found to be an independent predictor of decreased hemoglobin in HD patients. In addition, this association was observed to disappear after transplantation. While FGF23 is closely related to hemoglobin levels in HD patients, we have shown that this relationship disappears after transplantation.

Background. Patients on kidney replacement therapy comprise a vulnerable population and may be at increased risk of death from coronavirus disease 2019 (COVID-19). Currently, only limited data are available on outcomes in this patient population. Methods. We set up the ERACODA (European Renal Association COVID-19 Database) database, which is specifically designed to prospectively collect detailed data on kidney transplant and dialysis patients with COVID-19. For this analysis, patients were included who presented between 1 February and 1 May 2020 and had complete information available on the primary outcome parameter, 28-day mortality. Results. Of the 1073 patients enrolled, 305 (28%) were kidney transplant and 768 (72%) dialysis patients with a mean age of 60 ± 13 and 67 ± 14 years, respectively. The 28-day probability of death was 21.3% [95% confidence interval (95% CI) 14.3–30.2%] in kidney transplant and 25.0% (95% CI 20.2–30.0%) in dialysis patients. Mortality was primarily associated with advanced age in kidney transplant patients, and with age and frailty in dialysis patients. After adjusting for sex, age and frailty, in-hospital mortality did not significantly differ between transplant and dialysis patients [hazard ratio (HR) 0.81, 95% CI 0.59–1.10, P = 0.18]. In the subset of dialysis patients who were a candidate for transplantation (n = 148), 8 patients died within 28 days, as compared with 7 deaths in 23 patients who underwent a kidney transplantation Conclusions. The 28-day case-fatality rate is high in patients on kidney replacement therapy with COVID-19 and is primarily driven by the risk factors age and frailty. Furthermore, in the first year after kidney transplantation, patients may be at increased risk of COVID-19-related mortality as compared with dialysis patients on the waiting list for transplantation. This information is important in guiding clinical decision-making, and for informing the public and healthcare authorities on the COVID-19-related mortality risk in kidney transplant and dialysis patients.

Background and Aims Several glomerular disease registries in Europe, and they shared their data; however, there was a lack of current data on trends of primary glomerulopathy in Turkey. Glomerular disease patterns can change geographical areas and populations decade by decade. Our aim is to present Turkey's primary glomerular disease pattern from 2009 to 2017. Method 3878 native kidney biopsy records were assessed in the Turkish Society of Nephrology Glomerulopathy (TSN-GOLD) Working Group Registry. Secondary disease (lupus, etc.) and transplant biopsies do not enroll in the registry. These records divided into four periods, before 2009, 2009 to 2013, 2013 to 2017, and 2017 to current. Results A total of 3858 patients (M = 2173, K = 1685) were examined. There was no difference in the distribution of number of patients according to periods (Q1 = 968, Q2 = 960, Q3 = 968, Q4 = 962). Nephrotic syndrome was the most common biopsy indication in all quarters (58.6%, 53%, 44.1%, 51.6%, respectively). Glomerulopathy types; Membranous GN (29.9%, n = 290) was the first in Q1 and IgA nephropathy (17.7%, n = 172) was the second. IgA nephropathy (28.5%, n = 274) was the most common glomerulopathy in Q2, while Membranous GN (25.5%, n = 245) was the second most common. IgA nephropathy (29.9%, n = 285) was the most common glomerulopathy in Q3, followed by FSGS (22.9%, n = 222) and Membranous GN. In Q4, FSGS (26.8, n = 258) was the most common glomerulopathy, followed by IgA nephropathy (26.7%, n = 257) and Membranous GN (24.5%, n = 236) (Figure 1). Conclusion While biopsy indications did not change over the years, the incidence of FSGS and IgA nephropathy gradually increased.

Background and Aims The combination of new onset haematuria, proteinuria and declining GFR, often referred to as the nephritic syndrome, constitute a strong indication for renal biopsy. Several different disease entities can underlie these findings, many of them requiring prompt treatment. Epidemiological studies of glomerular diseases are in general hampered by the strong influence of differences in biopsy practices. However, there is reason to believe that such differences are less pronounced in cases of acute/subacute nephritic syndrome. The aim of the present study is to compare the frequencies of diseases causing the nephritic syndrome in Japan and Europe using data from renal biopsy registries. Method Data were retrieved from national or regional renal biopsy registries in Japan, Sweden, Norway, Scotland, Austria, Croatia and Turkey from 2007-01-01 to 2018-12-31. Only patients biopsied due to acute or subacute nephritic syndrome, defined as a combination of new onset haematuria, sub-nephrotic proteinuria and reduced GFR, were included. Demographic, clinical and laboratory data were collected from the time of renal biopsy. Results A total number of 2789 patients from Japan and 1202 patients from Europe were included. Among the Japanese patients, 46% were women, median age was 67 years (IQR 54-74) and median creatinine concentration at the time of renal biopsy was 198 µmol/L (IQR 109-343). In Europe, 43% were women, median age was 58 years (IQR 42-70) and median creatinine concentration was 210 µmol/L (IQR 127-390). Figures from the different European countries are presented in Table 1. The most common diagnosis in both Japan and Europe was ANCA-associated nephritis (44% in Japan, 34% in Europe), followed by IgA nephritis (8.5% in Japan, 18.6% in Europe) and acute tubulointerstitial nephritis (6.2% in Japan and 7.1% in Europe). Conclusion We describe preliminary data from a large multicenter cohort of patients presenting with the nephritic syndrome. This clinical presentation is most often encountered in the 6th and 7th decade of life and the distribution between men and women is fairly equal. The most common underlying cause of the nephritic syndrome in both Japan and Europe is ANCA-associated nephritis.

Background and Aims Hematuria is one of the most common laboratory findings in nephrology practice. In different regions of the world, the etiologic causes differ. To date, there is no enough data regarding the clinical and histopathologic characteristics of primary glomerular diseases (PGD) patients with hematuria in our country. Method Data were obtained from national multicenter (47 centers) data entered into the Turkish Society of Nephrology Glomerular Diseases (TSN-GOLD) database between May 2009 and June 2019. The data of all PGD patients over the age of 16 years who were diagnosed with renal biopsy and had hematuria data were included in the study. The biopsy samples were processed using a light microscopy and immunofluorescence examination. Demographic characteristics such as age, sex, indications for biopsy, primary glomerular diseases, comorbidities, laboratory and biopsy findings of all patients were also recorded. Hematuria was defined as the presence of at least 5 red blood cells/hpf. Results Data of 3394 patients were included to the study after the exclusion of patients with secondary glomerulonephritis and patients with missing biopsy findings. While 1699 (50.1%) patients had hematuria, 1695 (49.9%) patients did not have hematuria. Demographic, laboratory, and histopathological characteristics of patients with and without hematuria are given in Table. Patients with hematuria had statistically higher systolic blood pressure (SBP), serum blood urea nitrogen, creatinine, albumin, levels and urine pyuria, however, these patients had statistically lower age, body mass index, presence of hypertension and diabetes, eGFR, 24-hour proteinuria, serum total, HDL and LDL-cholesterol and C3 levels when compared with patients without hematuria. Figure depicted the etiologic causes of patients with and without hematuria. According to histopathological findings, number of global sclerotic glomeruli, cellular and fibrocellular crescents, the levels of mesangial proliferation, endocapillary proliferation, exudative changes in glomeruli, severe tubular atrophy, interstitial inflammation, subendothelial deposition, moderate and severe IgA and C3 deposition were found to be significantly higher and the levels of basal membrane thickening, interstitial fibrosis, subepithelial deposition, severe IgG staining were found to be significantly lower in patients with hematuria. Conclusion This is the first multicenter national report regarding the demographic and histopathologic data of PGD patients with or without hematuria. Hematuria, a feature of nephritic syndrome, was found at a higher than expected in the PGDs presenting with nephrotic syndrome in our national database.

Background and Aims According to the data of the Turkish Society of Nephrology-Glomerular Diseases Working Group (TSN-GOLD Working Group), IgA nephropathy is the most common primary glomerular disease in Turkey. The purpose of this study was to investigate the epidemiological and clinical data of IgA nephropathy patients in Turkey. Method 4399 patients with primary glomerular diseases from 47 centers who were followed up between May 2009 and May 2019 were included in the study conducted by TSN-GOLD Working Group. 524 patients were excluded due to lack of pathological data. Among the remaining patients, demographic, clinical and laboratory data of 994 patients with IgA nephropathy were analyzed. Results The median age of the patients was 37 (28-47) years, and 37.3% of them were female. The laboratory and clinical data at the time of diagnosis is presented in Figure-1, and biopsy indications are described in Figure-2. The median number of glomeruli was 16 (IQR: 3.5-4.3), sclerotic glomeruli was 2 (IQR: 1-5), and segmental sclerotic glomeruli was 1 (IQR: 1-2). Exudative changes, subendothelial and subepithelial deposition were present in 566 patients (56.9%), 46 patients (4.6%) and in 38 patients (3.8%), respectively. 662 (66.1%) and 611 of the patients (61.4%) had tubular atrophy and interstitial fibrosis in varying degrees, respectively. 672 (%67.6) and 416 patients (%41.9) had interstitial inflammation and vascular changes, respectively. In immunofluorescence staining, 18%, 30.1%, 4.4%, 68% of the patients had IgG, IgM, C1q and C3 positivity, respectively. Crescentic glomeruli were detected in 227 patients (3.3 ± 3.1 glomeruli). Patients with crescentic glomeruli had significantly higher proteinuria and lower eGFR than the patients without [2203 mg/day (15-26078) vs 1807 mg/day (15-29112); p=0.001; 55.3 ml/min/1.73 m2 (3.72-141.9) vs 72 ml/min/1.73 m2 (3.84-150.81); p Conclusion In this study we found that, the most common presentation of IgA nephropathy patients in our country was asymptomatic urinary abnormalities followed by nephritic and nephrotic syndrome. Higher proteinuria and lower eGFR values in patients with crescentic glomeruli, support the adoption of crescentic lesions in the new Oxford classification (MEST-C) to predict more precise outcome of IgA nephropathy patients. The high number of patients to whom the Oxford classification was applied provided us with the opportunity to examine the clinical reflections of pathological features. Evaluation of the follow-up data of the patients will give us the possibility to reveal the effect of initial clinical and pathological features on clinical findings and renal outcome.

Background and Aims In IgA nephropathy (IgAN), which is characterized by mesangial IgA accumulation, there is the formation of circulating autoantibodies against galactose deficient IgA1s (Gd-IgA1). IgG / Gd-IgA1 immunocomplexes accumulate in the glomerular mesangium and play a role in the pathogenesis of IgAN. Recent studies have suggested a relationship between glomerular IgG deposition and the severity of glomerular inflammation. However, detection of the presence and severity of IgG in routine immunofluorescence microscopy (IFM) may fail. This study aims to investigate whether IgG positivity detected by IFM is associated with poor renal prognostic indicators and whether renal prognosis can be predicted according to IgG positivity. Method 4399 patients who were enrolled between May 2009-June 2019 in database of Turkish Society of Nephrology, Glomerular Diseases Working Group (TSN-GOLD) including 44 centers were evaluated. After exclusion criteria, 994 primary IgAN patients were included in the study. Glomerular IgG negative and positive patients were compared by means of Oxford classification scores, histopathological evaluations, proteinuria, creatinine, albumin, blood pressures. IgG positive patients were divided into subgroups according to the grade of the IFM positivity. The relationship between IgG positivity and poor prognosis criteria were evaluated. Results Demographic and biochemical findings of glomerular IgG positive and negative patients at the time of biopsy are shown in Table 1. No difference was found between the groups. There was no difference in the demographic and biochemical findings at the time of biopsy in IgG subgroup analyses (Table 2). There was no difference between the histopathological and Oxford MEST scores of the subgroups. Glomerular IgG positivity was not associated with diastolic blood pressure, systolic blood pressure, urea, uric acid, age, eGFR, albumin, proteinuria (p> 0.05 for all, r= -0.084, r= -0.102, r= -0.006, r=0.062, r= 0.014, r= -0.044, r= -0.061, r= -0.066, r= 0.150, respectively). Conclusion Glomerular IgG positivity detected by routine IFM in IgAN is not associated with poor renal prognostic indicators. It is difficult to predict renal prognosis by looking at the severity of IgG positivity at the baseline evaluation.

Background and Aims Rapidly progressive glomerulonephritis (RPGN) is a clinical condition that develops due to different etiologic causes, characterized by a rapid and progressive decrease in renal function and progresses to end-stage renal failure in weeks to months if not treated. In our study, diagnostic and demographic characteristics of patients diagnosed with RPGN by biopsy, clinical and laboratory findings in our country were investigated. Method Data were obtained from national multicenter (47 centers) data entered into the Turkish Society of Nephrology Glomerular Diseases (TSN-GOLD) Working Group database between May 2009 and June 2019. Demographic characteristics such as age, sex, indications for biopsy, diagnosis of the glomerular diseases, comorbidities, laboratory and biopsy findings of all patients were recorded. The data presented is cross-sectional and includes application data for the biopsy period. According to their types, RPGN patients were classified as type 1 (anti-GBM related), type 2 (immunocomplex related) and type 3 (immune-negative; “pauci-immune”). Results After exclusion of 46 patients with missing data, 200 patients (mean age 47.9 ± 16.7 years, 44% female) were included in the study which constitutes 5.2% of the total glomerulonephritis database (total number of patients: 3875). Hypertension was present in 62 patients (31.0%) and diabetes was present in 18 patients (9.0%). Renal biopsy was performed in 147 (73.5%) patients due to nephritic syndrome (RPGN included). 80.2% of the patients' biopsies were performed in nephrology clinics and 19.8% of them were performed in radiology clinics. ANCA positivity was found in 121 (60.5%) patients (proteinase 3-ANCA was positive in 55 and myeloperoxidase-ANCA positive in 66 patients). Type 1 RPGN was detected in 11 (5.5%), type 2 RPGN in 42 (21%) and type 3 RPGN in 147 (73.5%) patients. In 21 patients (10.5%), biopsy revealed RPGN with advanced chronic changes (fibrous global sclerotic glomeruli, advanced interstitial fibrosis and tubular atrophy). Mean serum creatinine was 4.2 ± 3.4 mg/dl, median glomerular filtration rate was 18 (10-37) ml/min and proteinuria 2100 (1229-3526) mg/day according to CKD-EPI formula. The mean number of glomeruli in the biopsies was 18.8 ± 10.6 and the number of crescentic glomeruli was 9.9 ± 7.7 (ratio: 52.7%) (Figure). The patients were divided into 3 groups according to their crescentic glomeruli ratios. The proportion of crescentic glomeruli is 10-50% in group 1, 50-80% in group 2, and >80% in group 3. The demographic, laboratory and histopathological characteristics of the groups are given in Figure. It was observed that urea and creatinine increased and calcium and hemoglobin decreased with increasing crescentic glomerular ratio. Conclusion Our study provides valuable demographic, clinical, laboratory and histopathological data about RPGN in our country. Our data are generally compatible with the literature. In our study, advanced chronic histopathological findings were prominent in the biopsy of 21 patients. Early biopsy should be performed to confirm the diagnosis of RPGN and to avoid unnecessary intensive immunosuppressive therapy. In addition to the treatments applied, detailed data, including patient and renal survival are needed.

Background and Aims Fabry Disease (FD) is a lysosomal glycosphingolipid storage disorder. Kidneys are affected in FD. Despite enzyme replacement theraphy (ERT), some patient still have an ongoing deterioration of renal functions probably secondary to inappropriate immune responses. There is no data regarding NK cell subtypes and the exact relation with B-T lymphocytes and NK cells in patients with FD. We aimed to analyze subtypes of NK cells in patients with FD and compared these results with healthy subjects. Additionally, we analyzed the renal biopsy (RBx) materials of our patients prior to ERT in terms of B, T and NK cell infiltration and compared these cells in some of our FD patients after 4 years of ERT. Method 15 patients with FD and 10 healthy controls were included in the study. Of 15 patients, 8 patients were receiving agalsidase alfa or beta. Blood samples obtained from both group were taken into 2 ml EDTA tube to evaluate peripheral NK cell subgroups according to CD56 and CD16 expression and CD3, CD4, CD8 expression to determine subtypes T cells. These cells were evaluated by flow cytometry technique. The RBx of 10 patients with FD were evaluated before ERT and 3 were reevaluated after 4 years of ERT in terms of T-B, NK and plasma cells infiltration of kidney. The paraffin blocks of specimens were examined with HE, PAS, Masson-trichrome and toluidine blue staining and frozen blocks were examined with red-oil 10 staining Results The characteristics of patients with FD and healthy controls are depicted in Figure 1. Except proteinuria, there were no significant differences in terms of biochemical parameters between two groups. According to flow-cytometric analyses, total percentage of NK and T helper 1, 2, 17 and Treg cells of FD patients are similar to controls (Fig 1). When we analyzed subgroups of NK cells and determined that CD56dimCD16dimNK cells were increased, however, CD56dimCD16brightNK cells were decreased in patients with FD compared to controls (Fig1). We performed RBx of 10 patients. The percentages of CD3, 4, 8 positive Tcells, CD19, CD20 positive B cells, CD 16, 56 positive NK cells and CD 138 positive plasma cell infiltration are shown in Fig 2. According to RBx findings prior to ERT, all patients had interstistial fibrosis (IF), podocyte vacuoles (PV), podocyte inclusion (PI), CD3, CD4, CD16, CD56 positivity at different levels. In contrast, almost none of the patients had CD19, CD20, CD138 positivity. We rebiopsied 3 patients after 4 years of ERT and reevaluated specimens in terms of IF, global and segmental sclerosis (SS), PV, PI and the percentages of CD3, CD4, CD16, CD56, CD 19, CD 20, CD 138 positivity. 2 rebiopsied patients from the same family had increased serum creatinine and proteinuria despite 4 years of regular ERT. In the second biopsy of these two patients, we found that global sclerosis (GS), IF and PI are increased and abundant CD19, CD20 and CD 138 positive cells were seen which were almost absent in the first biopsy. The other patient from another family, had mildly increased proteinuria and had no creatinine increment after 4 years of ERT. In this patient, we found that GS, SS, IF, PI and arterial sclerosis were increased, however CD 3, 4, 8 positive T cells were decreased and no changes in the percentage of CD16, 56 positive NK cells. We could not demonstrate CD 19, 20, 138 positivity in the latter patient. Conclusion Our group, for the first time, demonstrated that CD56dimCD16dimsubtype of NK cells is increased in FD which is closely associated with cellular cytotoxity in normal population. Additionaly, CD 19 and CD 20 positive B cell infiltration are increased and glomerular and interstitial changes are advanced in some patients with ongoing kidney function deterioration despite 4 years of regular ERT. Hence, infiltration of Bcells in the kidneys of patients under ERT might change our therapeutic approaches in the near future. Further studies are needed to clarify the exact roles of B T and NK cells in FD.

Background and Aims Therapeutic plasma exchange (PEX) has an increasingly long list of indications in recent years, in addition to immunosuppressive therapies in many life-threatening immune activations, it provides significant improvements in mortality and morbidity. In this study, we aimed to investigate the laboratory and clinical effects of PEX performed with nephrologic indication in our clinic. Method The records of 67 patients (36 females, 31 males; mean age, 45.7±15.8 years) who underwent PEX with nephrological indication between 2012 and 2017 in our clinic were retrospectively reviewed. Characteristics of the patients such as, indications of PEX, laboratory values and number of PEX sessions were recorded. In addition, clinical responses were also evaluated. Results When a total 398 PEX sessions were examined, the most common indication (40.3%) was acute humoral rejection after renal transplantation, followed by granulomatosis with polyangiitis (19.4%) and thrombotic microangiopathy (9%), respectively. The average of the PEX sessions was 5.94. There was a statistically significant increase in the bicarbonate value when the arterial blood gas of the patients was evaluated after PEX (p=0.002). However, no significant difference was observed in the pH and ionized calcium values (p=0.135, p=0.969, respectively) (Table 1). When all the patients were evaluated, there was no significant change in hemoglobin values (p=0.174), but platelet values decreased significantly (p=0.011) after PEX. However, when the patient group admitted with thrombotic microangiopathy was examined, it was observed that platelet count increased and LDH level decreased significantly (p=0.063, p=0.028, respectively). When the serum creatinine values of all patients were evaluated, a significant decrease was observed in the serum creatinine values after PEX (p=0.001). In addition, after 2 years following PEX treatment, 70.1% of patients were still alive and 12.5% of patients undergoing PEX for acute humoral rejection were undergoing on hemodialysis treatment. Conclusion According to the results of our study, patients should be followed up especially for acid-base and electrolyte changes after PEX. In addition, therapeutic PEX might be effective in terms of improving morbidity and 2 year-mortality of these patients.

Background and Aims The largest data on the epidemiology of primary glomerular diseases (PGD) are obtained from the databases of countries or centers. Here, we presented the extended results of the Primary Glomerular Diseases Study of the Turkish Society of Nephrology Glomerular Diseases (TSN-GOLD)Working Group. Method The data of patients who underwent renal biopsy and diagnosed as primary glomerular disease were recorded in the database prepared for the study. Between May 2009 and May 2019, a total of 4399 patients from 47 centers were evaluated. Basal data of 3875 patients were analyzed after exclusion of those lacking light microscopy and immunofluorescence findings. Results The mean age was 41.5 ± 14.9 years. Of the patients, 1690 were female (43.6%) and 2180 (56.3%) were male. Nephrotic syndrome was the most common biopsy indication (51.7%). This was followed by asymptomatic urinary abnormalities (18.3%) and nephritic syndrome (17.8%). The most common PGH was IgA nephropathy (25.7%), followed by membranous nephropathy (25.6%) and FSGS (21.9%). The mean total number of glomeruli per biopsy was 17 ± 10. Mean baseline systolic blood pressure was 130 ± 20 mmHg and diastolic blood pressure was 81 ± 12 mmHg. Median proteinuria was 3300 (IQR: 1467-6307) mg / day, mean serum creatinine, estimated GFR and albumin values were 1.4 ± 1.5 mg / dl, 80.7 ± 39.1 ml / min and 3.2 ± 0.9 g / dl, respectively. Conclusion In Turkey, the incidence of IgA nephropathy patients have become more common than membranous nephropathy among PGD patients diagnosed with renal biopsy.

The coronavirus disease 2019 (COVID-19) pandemic has created major challenges for all countries around the globe. Retrospective studies have identified hypertension, cardiovascular disease, diabetes and older age as risk factors for high morbidity and mortality from COVID-19. There is a general concern that patients with immune-mediated kidney diseases, namely those on immunosuppressive therapies and/or those with more advanced kidney failure, could particularly be at risk for adverse outcomes due to a compromised antiviral immunity. Uncertainties exist on how management routines should be reorganized to minimize the risk of severe acute respiratory syndrome coronavirus 2 infection and what measures are necessary for infected patients. The aim of the present review of the Immunonephrology Working Group of the European Renal Association–European Dialysis and Transplant Association is to provide recommendations for the management of patients with immune-mediated kidney diseases based on the available evidence, similar circumstances with other infectious organisms and expert opinions from across Europe. Such recommendations may help to minimize the risk of encountering COVID-19 or developing complications during COVID-19 in patients with immune-mediated kidney disease.

Background The largest data on the epidemiology of primary glomerular diseases (PGDs) are obtained from the databases of countries or centers. Here, we present the extended results of the Primary Glomerular Diseases Study of the Turkish Society of Nephrology Glomerular Diseases (TSN-GOLD) Working Group. Methods Data of patients who underwent renal biopsy and received the diagnosis of PGD were recorded in the database prepared for the study. A total of 4399 patients from 47 centers were evaluated between May 2009 and May 2019. The data obtained at the time of kidney biopsy were analyzed. After the exclusion of patients without light microscopy and immunofluorescence microscopy findings, a total of 3875 patients were included in the study. Results The mean age was 41.5 ± 14.9 years. 1690 patients were female (43.6%) and 2185 (56.3%) were male. Nephrotic syndrome was the most common biopsy indication (51.7%). This was followed by asymptomatic urinary abnormalities (18.3%) and nephritic syndrome (17.8%). The most common PGD was IgA nephropathy (25.7%) followed by membranous nephropathy (25.6%) and focal segmental glomerulosclerosis (21.9%). The mean total number of glomeruli per biopsy was 17 ± 10. The mean baseline systolic blood pressure was 130 ± 20 mmHg and diastolic blood pressure was 81 ± 12 mmHg. The median proteinuria, serum creatinine, estimated GFR, and mean albumin values were 3300 (IQR: 1467–6307) mg/day, 1.0 (IQR: 0.7–1.6) mg/dL, 82.9 (IQR: 47.0–113.0) mL/min and 3.2 ± 0.9 g/dL, respectively. Conclusions The distribution of PGDs in Turkey has become similar to that in other European countries. IgA nephropathy diagnosed via renal biopsy has become more prevalent compared to membranous nephropathy.

Background: In our study, diagnostic and demographic characteristics of patients diagnosed with RPGN by biopsy, clinical and laboratory findings in our country were investigated. Methods: Data were obtained from the Turkish Society of Nephrology Glomerular Diseases (TSN-GOLD) Working Group database. Demographic characteristics, indications for biopsy, diagnosis of the glomerular diseases, comorbidities, laboratory and biopsy findings of all patients were recorded. According to their types, RPGN patients were classified as type 1 (anti-GBM related), type 2 (immuncomplex related) and type 3 (pauci-immune). Results: Of 3875 patients, 200 patients with RPGN (mean age 47.9 ± 16.7 years) were included in the study which constitutes 5.2% of the total glomerulonephritis database. Renal biopsy was performed in 147 (73.5%) patients due to nephritic syndrome. ANCA positivity was found in 121 (60.5%) patients. Type 1 RPGN was detected in 11 (5.5%), type 2 RPGN in 42 (21%) and type 3 RPGN in 147 (73.5%) patients. Median serum creatinine was 3.4 (1.9–5.7) mg/dl, glomerular filtration rate was 18 (10–37) ml/min/1.73m2 and proteinuria 2100 (1229–3526) mg/day. The number of crescentic glomeruli ratio was ratio 52.7%. It was observed that urea and creatinine increased and calcium and hemoglobin decreased with increasing crescentic glomerular ratio. Conclusions: Our data are generally compatible with the literature. Advanced chronic histopathological findings were prominent in the biopsy of 21 patients. Early biopsy should be performed to confirm the diagnosis of RPGN and to avoid unnecessary intensive immunosuppressive therapy. In addition to the treatments applied, detailed data, including patient and renal survival, are needed. © 2020, Japanese Society of Nephrology.

Renal transplantation is the most effective treatment modality for end-stage renal failure. According to the Ministry of Health Organ Transplant Registration System, despite the presence of 14,936 renal transplant recipients in Turkey, there are not enough data about the prognosis of these patients. Therefore, we aimed to ascertain the rate of patients returning to hemodialysis (HD) due to nonfunctioning graft in our country. One thousand four hundred and ninety-eight (males: 826, females: 672) HD patients who undergo HD at 22 HD centers in total, from different geographical regions to represent our country were examined retrospectively. The informations were obtained from patient registry files and anamnesis which were in HD centers. The number of patients returning to HD due to the loss of graft function was 77 (males: 56, females: 21). Eleven of the patients had transplantation from cadavers (14%) and 66 from living donors (86%). Prevelance of patients, who return to HD after the failure of renal transplantation, between HD patients was 5.1. The mean duration of return to HD after renal transplantation was 6.7 ± 5.9 years for all patients. There was no significant difference in the duration without HD after transplantation between two groups when cadaveric and living donor transplants were compared (P = 0.759). There was no statistically significant difference in duration without HD after transplantation between patients receiving HD treatment before transplantation and preemptive transplant (P = 0.212). The prevelance of patients, who return to HD due to nonfunctioning graft among HD patients was 5.1. The duration without HD were similar after transplantation from both cadavers and living donors. The duration without HD was found longer among those who were operated before 2000.

Hemodialysis patients have extremely increased cardiovascular mortality. The coronary artery calcification score (CACS) in uremic patients receiving hemodialysis reflects the severity of atherosclerotic vascular disease and predicts the cardiovascular events. In cardiac conditions, left atrial (LA) size has a prognostic importance. In this study, relationship between coronary artery calcification and left atrial size was investigated. This was a cross-sectional study involving 32 hemodialysis patients (16 females, 16 males; mean age, 52.4 ± 14.1 years) receiving HD for ≥6 months. Coronary artery calcium scoring was performed by a 16-MDCT scanner, and CACS was calculated by Agatston score. A calcification was defined as a minimum of two adjacent pixels (>0.52 mm2) with a density over 130 Hounsfield units. Patients were divided into two subgroups (group 1: CACS ≤ 45.85, n = 16 and group 2: CACS > 45.85, n = 16) according to median CACS value. Mean CACS value of 32 hemodialysis patients was 245.57 ± 373.91. LA size was significantly higher in patients with CACS > 45.85 (group 2) than in patients with CACS ≤ 45.85 (group 1). In the bivariate correlation analysis, total CACS was positively correlated with left atrium size (r = 0.47, p = 0.006). Total CACS was positively correlated with age (r = 0.43, p = 0.014). LA size was positively correlated with diastolic blood pressure (r = 0.42, p = 0.016) and negatively correlated with ejection fraction (r = −0.42, p = 0.016). The clinical parameters such as BMI, duration of dialysis, blood pressure, ejection fraction, serum levels of calcium, phosphorus, uric acid, albumin, CRP, triglyceride, cholesterol, hemoglobin and ferritin were not independently associated with total CACS. We found a positive relationship between the CACS and LA size measured by echocardiography in hemodialysis patients. Therefore; echocardiography, which is cheaper and non-invasive than tomographic examinations, might be considered for the risk stratification of coronary artery disease in hemodialysis patients.

Diabetic kidney disease (DKD) can occur in approximately 30-40% of both type 1 and type 2 diabetic patients. The well-established features of DKD include increased serum glucose levels along with chronic low-grade inflammation, OxS, increased advanced glycation end products, sorbitol accumulation, increased hexosamine, and protein kinase C pathway activation. On the other hand, accumulating evidence suggests that novel pathways including apoptosis and autophagy might also play important roles in the pathogenesis and progression of DKD. In this review, the integrated mechanisms of inflammation, oxidative stress, apoptosis, and autophagy are discussed in the pathogenesis as well as progression of DM and DKD.

The most important cause of anemia in CKD is relative deficiency of erythropoietin (EPO) secretion from the diseased kidney and EPO therapy has become the standard treatment for anemia of CKD. However, some patients do not respond well to erythropoiesis stimulating agent (ESA), so-called ESA resistance. One of the most important causes of ESA resistance is chronic inflammation in hemodialysis (HD) patients. ESA hyporesponsiveness index (EHRI), calculated as the weekly dose of EPO divided by kilograms of body weight divided by the hemoglobin level, and has been considered useful to assess the EPO resistance. Neutrophil/lymphocyte (NLR) ratio and platelet/lymphocyte ratio (PLR) were also found to be associated with inflammation in HD patients. However, the relationship between NLR, PLR and EHRI has not been investigated before. HD patients underwent medical history taking, physical examination, calculation of dialysis adequacy and biochemical analysis and calculation of EHRI. Logarithmically converted EHRI (logEHRI) was correlated only with hemoglobin (r -0.381, P < 0.0001) and PLR (r = 0.227, P = 0.021) but not with NLR. Comparison of PLR among 25th, 50th and 75th percentile of EHRI showed that PLR levels increased going from the 25th to 75(th) percentile (P = 0.032). Posthoc analysis revealed that 25-75th percentile (P = 0.014) and 50-75th percentile (P = 0.033) were different with respect to PLR. In linear regression analysis, PLR (standardized β = 0.296, confidence interval: 0.000-0.001, P = 0.003) was independently associated with logEHRI. We found that PLR was independently associated with EHRI in HD patients. PLR, which is quite a simple and cheap method, may guide clinicians for detecting EPO resistance.

Left atrium (LA) mechanical functions and atrial electromechanical delay (AEMD) times were considered independent predictors of cardiovascular morbidity in general population. Data are scant about these parameters in end-stage renal disease (ESRD) patients receiving hemodialysis (HD) and peritoneal dialysis (PD). We aimed to evaluate AEMD times and LA mechanical functions and associated risk factors in HD and PD patients. Forty-four healthy individuals, 62 HD and 50 PD patients were enrolled in the study. Echocardiography was performed before midweek dialysis session for HD patients and on admission for PD patients. Data were expressed as mean ± SD. Spearman’s test was used to assess linear associations. Predictors of left intra-atrial EMD time and LA active emptying volume (LAaeV) were assessed by regression analysis. Left intra-atrial-AEMD times were significantly longer in HD patients compared to PD patients. LAaeV was positively correlated with inter-atrial time, left intra-atrial time, systolic and diastolic BP, calcium and neutrophil-to-lymphocyte ratio (NLR) (r 0.22, p 0.016; r 0.28, p 0.002; r 0.34, p

WOS: 000392937800028 PubMed ID: 28006774 Background/Aims: Fabry disease is a treatable cause of chronic kidney disease (CKD) characterized by a genetic deficiency of a-galactosidase A. European Renal Best Practice (ERBP) recommends screening for Fabry disease in CKD patients. However, this is based on expert opinion and there are no reports of the prevalence of Fabry disease in stage 1-5 CKD. Hence, we investigated the prevalence of Fabry disease in CKD patients not receiving renal replacement therapy. Methods: This prospective study assessed a-galactosidase activity in dried blood spots in 313 stage 1-5 CKD patients, 167 males, between ages of 18-70 years whose etiology of CKD was unknown and were not receiving renal replacement therapy. The diagnosis was confirmed by GLA gene mutation analysis. Results: Three (all males) of 313 CKD patients (0.95%) were diagnosed of Fabry disease, for a prevalence in males of 1.80%. Family screening identified 8 aditional Fabry patients with CKD. Of a total of 11 Fabry patients, 7 were male and started enzyme replacement therapy and 4 were female. The most frequent manifestations in male patients were fatigue (100%), tinnitus, vertigo, acroparesthesia, hypohidrosis, cornea verticillata and angiokeratoma (all 85%), heat intolerance (71%), and abdominal pain (57%). The most frequent manifestations in female patients were fatigue and cornea verticillata (50%), and tinnitus, vertigo and angiokeratoma (25%). Three patients had severe episodic abdominal pain attacks and proteinuria, and were misdiagnosed as familial Mediterranean fever. Conclusions: The prevalence of Fa bry disease in selected CKD patients is in the range found among renal replacement therapy patients, but the disease is diagnosed at an earlier, treatable stage. These data support the ERBP recommendation to screen for Fabry disease in patients with CKD of unknown origin. (C) 2016 The Author(s) Published by S. Karger AG, Basel Sanofi Genzyme Corporation; Intensificacion ISCIII and REDINREN [RD012/0021] We are indebted to our patients and their families for volunteering to contribute their medical information to the physicians who are dedicated to their care. This work was supported by grants from Sanofi Genzyme Corporation. The lead investigator had full access to all data in the study. All investigators take responsibility for the accuracy of the individual data that they entered in the system provided by FMF Arthritis Vasculitis and Orphan disease Research (FAVOR). AO was supported by Intensificacion ISCIII and REDINREN RD012/0021.

Background: Diabetic nephropathy is the most common cause of end-stage renal disease. Emerging evidences indicate that many mechanistic pathways including apoptosis play an important role in the pathogenesis and progression of macrovascular and microvascular complications of diabetes mellitus. The aim of the present study is to show the effects of grape seed extract (GSE) on oxidative stress and apoptosis in the kidney of streptozotocin-induced diabetic rats. Materials and methods: The study included control group, diabetic group without treatment and diabetic group treated with GSE (n=7) group. GSE was given orally (100 mg/kg/day) for six weeks. Following parameters were evaluated; oxidative stress index, caspase 1, IL1-alpha, caspase 2, IL1-beta, BCL2-associated agonist of cell death (BAD), X-linked inhibitor of apoptosis (XIAP), DNA fragmentation factor, alpha subunit and beta bubunit (DFFA, DFFB), BH3 interacting domain death agonist (BID), caspase 6, Bcl2-like 1 (BCL-XL), caspase 8, tumor necrosis factor receptor superfamily, member 1 b (TNFRSF1B) and IAP-binding mitochondrial protein (DIABLO). Results: Oxidative stress index levels were significantly increased in the kidney of diabetic group without treatment compared to control group, and decreased in diabetic + GSE group compared to diabetic group without treatment. In the kidney of diabetic group without treatment, caspase 1, IL-1 alpha, BAD, DFFA, DFFB and caspase-6 gene expressions were significantly higher compared to control group. In diabetic + GSE group caspase 1, caspase 2, XIAP, DFFA, BID, BCL-XL and TNFRSF1B genes were significantly decreased compared to control group. Conclusions: Grape seed reduces oxidative stress and apoptosis gene expression suggesting the protective effect on diabetic nephropathy. © 2015 Informa Healthcare USA, Inc. All rights reserved: reproduction in whole or part not permitted

Background: Atrial electromechanical delay (AEMD) times were considered independent predictors of cardiovascular morbidity among the general population. We aimed at evaluating AEMD times and other risk factors associated with 2-year combined cardiovascular (CV) events in HD patients. Material and Methods: Sixty hemodialysis (HD) and 44 healthy individuals were enrolled in this prospective study. Echocardiography was performed before the mid-week dialysis session for HD patients. Data were expressed as mean ± SD. Spearman test was used to assess linear associations. Survival was examined with the Kaplan-Meier method. Multivariate Cox regression analysis was used to determine the predictors of combined CV events in this cohort. Results: At the beginning of the study, left intra-atrial-AEMD times were significantly longer in HD patients compared to the left intra-atrial-AEMD times in healthy individuals. After 24 months, 41 patients were still on HD treatment and 19 (31.6%) had died. Serum triglyceride, total cholesterol and albumin were found to be higher and C-reactive protein (CRP) levels, left intra-atrial EMD time (LIAT) and interatrial EMD times were found to be lower in survived HD patients. With the cut-off median values of 3.5 g/dl for albumin, 0.87 mg/dl for CRP, 157 mg/dl for total cholesterol and 151 mg/dl for triglyceride, the Kaplan-Meier curves demonstrated significant differences in terms of all-cause mortality. We also demonstrated the Kaplan-Meier survival curves of HD patients according to tertile values of LIAT. Cox regression analysis revealed that increased CRP and higher LIAT were found to be independent predictors of combined CV events. Conclusions: Increased LIAT and inflammation were found to be closely associated with 2 years combined CV events and all-cause mortality in HD patients.

Acute kidney injury (AKI) is a common complication of cardiac surgery developing in 25-35% cases. Recently, neutrophil gelatinase-associated lipocalin (NGAL) was shown to predict AKI development earlier than serum creatinine. Some studies demonstrated the predictive role of post-operative serum uric acid (SUA) as an early marker of AKI. We aimed to study the role of serum and urine NGAL as well as SUA to predict progression of AKI.This is a prospective observational study of patients undergoing cardiac surgery. Blood and urine samples for measurement of uric acid, serum and urine NGAL levels were collected prior to cardiac surgery (0 h), and in the time course at 2nd and 24th hours after surgery. Patients who developed AKI were divided into two subgroups as progressing and non-progressing AKI.Sixty patients (42 males, 18 females) were included. After cardiac surgery, 40 patients developed AKI, 20 of whom non-progressing AKI, and 20 progressing AKI. All of the markers significantly increased in AKI patients. A receiver operator characteristics (ROC) curve analysis showed higher predictive ability of SUA for progressing AKI compared with serum and urine NGAL. When compared markers obtained at the second hour after surgery, UA had significantly large AUC than NGAL to predict AKI developed at 24 and 48 h, particularly in patients, who require renal replacement therapy (RRT).Uric acid seems to predict the progression of AKI and RRT requirement in patients underwent cardiac surgery better than NGAL.

Chronic inflammation was found to be correlated with coronary (CAC) and thoracic peri-aortic calcification (TAC) in end-stage renal disease (ESRD) patients. Neutrophil-to-lymphocyte ratio (NLR) was introduced as a potential marker to determine inflammation in cardiac and noncardiac disorders. Data regarding NLR and its association with TAC and CAC are lacking. We aimed to determine the relationship between NLR and vascular calcification in ESRD patients. This was a cross-sectional study involving 56 ESRD patients (22 females, 34 males; mean age, 49.9 ± 14.2 years) receiving peritoneal dialysis or hemodialysis for ≥6 months in the Dialysis Unit of Necmettin Erbakan University. TAC and CAC scores were measured by using an electrocardiogram-gated 64-multidetector computed tomography. NLR was calculated as the ratio of the neutrophils and lymphocytes. There was a statistically significant correlation between NLR, TACS and CACS in ESRD patients (r = 0.43, P = 0.001 and r = 0.30, P = 0.02, respectively). The stepwise linear regression analysis revealed that age, as well as NLR were independent predictors of TACS. However, increased age was the only independent predictor of CACS according to linear regression analysis. Simple calculation of NLR can predict vascular calcification in ESRD patients.

Neutrophil-to-lymphocyte ratio (NLR) was introduced as a potential marker to determine inflammation in end-stage renal disease (ESRD) patients. Recently, platelet-to-lymphocyte ratio (PLR) and NLR were found to positively correlated with inflammatory markers including tumor necrosis factor-α (TNF-α) and interleukin (IL)-6 in cardiac and noncardiac patients. Data regarding PLR and its association with inflammation are lacking in hemodialysis (HD) and peritoneal dialysis (PD) patients. Hence, we aimed to determine the relationship between PLR, NLR, and inflammation in ESRD patients. This was a cross-sectional study involving 62 ESRD patients (29 females, 33 males; mean age, 49.6 ± 14.6 years) receiving PD or HD for ≥6 months in the Dialysis Unit of Necmettin Erbakan University. PLR, NLR, C-reactive protein, TNF-α, IL-6 levels were measured. PLR, NLR, serum high sensitive C-reactive protein, IL-6, and TNF-α levels were significantly higher in PD patients when compared with HD patients. ESRD patients with PLR ≥ 140 had significantly higher NLR, IL-6, and TNF-α levels when compared to patients with PLR

Patients with diabetic kidney disease (DKD) are more prone to contrast-induced nephropathy (CN). Apoptosis and autophagy were found to be essential in the pathogenesis of DKD. Interleukin-33 (IL-33) is a cytokine, but its role in DKD and CN is unknown. As IL-33 is modulated by apoptosis, we aimed to determine the relationship between IL-33 apoptosis and autophagy in DKD with CN.Thirty male Sprague-Dawley rats were enrolled and randomly allocated into three groups. The first group was comprised of healthy rats (HRs), whereas the other two groups were made up of diabetic rats (DRs) and diabetic rats with CN (DRs + CN). All groups except the HRs received 50 mg/kg/day of streptozotocin (STZ). The DRs + CN group was induced by administering 1.5 mg/kg of intravenous radiocontrast dye on the 35th day.We observed increased IL-33 in the kidney tissue following induction of CN in the DRs. The DRs showed moderate immunopositivity, and the DRs + CN showed severe immunopositivity for caspase-3, cleaved caspase-3, caspase-8, caspase-9, LC3B, and Beclin-1 in tubular cells and glomeruli. The DRs also showed moderate immunopositivity in tubular cells, and the DRs + CN group showed severe immunopositivity for IL-33 in tubular cells. Increased caspase-3 was found in both glomeruli and tubuli; however, we could not demonstrate IL-33 in glomeruli. This could be secondary to inactivation of IL-33 via increased caspase-3 activity.The release of IL-33 from necrotic cells might induce autophagy, which can further balance the effects of increased apoptosis secondary to CN in DKD.

Thoracic peri-aortic fat tissue (PFT) is considered as a metabolically active organ in atherosclerosis. Malnutrition, inflammation and atherosclerosis/calcification (MIAC) are the most commonly encountered risk factors of cardiovascular disease in end-stage renal disease (ESRD) patients. Calcification of the aorta was found to be an important cardiovascular risk marker predicting future events, morbidity and mortality in this population. We aimed to investigate the relationship between PFT, MIAC syndrome and thoracic aortic calcification (TAC) in ESRD patients.Seventy-nine ESRD patients receiving hemodialysis (HD) or peritoneal dialysis (PD) and 20 control subjects were enrolled in this cross-sectional study. PFT and TAC were assessed using a 64-MDCT scanner. Patients with serum albumin3.5 g/dL were defined as patients with malnutrition; those with serum C-reactive protein level10 mg/L had inflammation, and those with coronary artery calcification score (CACS)10 had atherosclerosis/calcification.TAC and PFT were significantly higher in ESRD patients compared with control subjects. There was a statistically significant relationship between PFT and TAC in ESRD patients (r = 0.458, p0.0001). PFT was found to be significantly increased when the MIAC components increased. PFT was positively associated with age, BMI, uric acid, hemoglobin and CAC. The multivariate analysis revealed that age and uric acid were independent predictors of increased PFT. Twenty-four (30.4 %) patients had none, 30 (37.9 %) had one component, 17 (21.5 %) had two components, and 8 (10.2 %) had all MIAC components. PFT was highest among patients having all three components (28.6 cm(3)) and lowest among those who do not have the MIAC syndrome (8.54 cm(3)). TAC was highest among patients having all three components (179.2 HU) and lowest among those who do not have the MIAC syndrome (0 HU).We found a relationship between PFT and MIAC syndrome in ESRD patients.

Amyloid fibrils can affect vascular structure through deposition and by causing nitric oxide depletion and increase of asymmetric dimethyl arginine. Patients with amyloidosis are prone to development of hypotension. Hypotension may also affect the maturation of arteriovenous fistula (AVF) and may set the stage for formation of thrombosis and fistula failure. Thus, we aimed to evaluate effects of secondary amyloidosis on AVF outcomes and intradialytic hypotension. This is a case-control study which included 20 hemodialysis patients with amyloidosis and 20 hemodialysis patients without amyloidosis as control group. All patients underwent Doppler ultrasound of AVF. A thorough fistula history and baseline laboratory values along with episodes of intradialytic hypotension and blood pressure measurements were recorded. There was no difference between the groups regarding age, gender, body mass index, presence of comorbidities, hypertension, and drug use. Systolic and diastolic blood pressures were similar (119 ± 28/75 ± 17 and 120 ± 14/75 ± 10 mmHg for patients with and without amyloidosis, respectively). Intradialytic hypotension episodes were also similar. Patients with amyloidosis had significantly lower serum albumin and higher C-reactive protein values compared to control hemodialysis patients. AVF sites and total number of created fistulas were similar in both groups. Flow rates of current functional AVFs were not different between the groups (1084 ± 875 and 845 ± 466 mL/minute for patients with and without amyloidosis, respectively, p:0.67). Patency duration of first AVF was not different between the groups. Clinical fistula outcomes and rate of intradialytic hypotension episodes were not significantly different between patients with and without secondary systemic amyloidosis.

Health-related quality of life (HRQoL) and sleep quality (SQ) were impaired in patients with end-stage renal disease (ESRD). The impairment of both HRQoL and SQ and being in a depressive mood were found to be associated with increased morbidity and mortality in dialysis patients. We aimed to investigate the association between SQ, HRQoL, and depression, and to define independent predictors of SQ and depression in peritoneal dialysis (PD) and hemodialysis (HD) patients. Ninety HD patients (41 females, 49 males with mean age 50 ± 15.7 years) and 64 PD patients (27 females, 37 males with mean age 52.4 ± 15.3 years) receiving renal replacement therapy for at least 3 months were screened for the assessment of SQ, HRQoL, and depression in this cross-sectional study. A modified postsleep inventory, Short Form of Medical Outcomes Study (SF-36) and Beck depression inventory (BDI) were applied to all patients for evaluating SQ, HRQoL, and depression, respectively. HD and PD patients had similar total SQ scores. Physical and mental component scale of HRQoL were found to be significantly higher in HD patients (p < 0.001). PD patients were found to be much more in depressive mood when compared with HD patients (p < 0.001). Independent predictors of depression in patients were mental component scale of HRQoL, gender (being female), and dialysis modality (being PD patient). Physical component scale was also found to be an independent predictor of SQ. This study showed that despite similar SQ scores between two groups, HD patients had better HRQoL and less depression than PD patients.

A few studies investigated renal size using computed tomography (CT) in middle-aged and elderly patients while taking renal function into account. Thus, we aimed to assess kidney dimensions and their predictors in patients without known renal disease by multi-detector computed tomography (MDCT).This is a 6-month-long prospective observational study. Estimated glomerular filtration rate (eGFR) was calculated with Cockcroft-Gault (CG) and modification of diet in renal disease (MDRD) formulas. Pole-to-pole kidney length (R1) was determined from coronal and sagittal oblique images. Kidney length (R2), width (R3), and parenchymal thickness were measured in axial slices.The data of the 930 patients were included in this study. CG-eGFR was more closely correlated with R1, R2, and R3 than MDRD-eGFR. CG-eGFR, female gender, and presence of diabetes mellitus were independent predictors of R1 size in logistic regression analyses.Kidney sizes diminish with advancing age. CG-eGFR shows a better correlation with kidney dimensions compared with MDRD. Overall, age was a stronger determinant of eGFR than renal dimensions.

Cardiovascular problems are a major cause of morbidity and mortality in patients with autosomal dominant polycystic kidney disease. Endothelial dysfunction, an early and reversible feature in the pathogenesis of atherosclerosis, is associated with increased vascular smooth muscle tone, arterial stiffening, and increased intima-media thickness. Coronary flow velocity reserve is a noninvasive test showing endothelial function of epicardial coronary arteries and coronary microcirculatory function. The aim of the study was to investigate the carotid intima-media thickness and coronary flow velocity reserve in patients with autosomal dominant polycystic kidney disease.Thirty normotensive patients with autosomal dominant polycystic kidney disease (10 male, 20 female) with well-preserved renal function and 30 healthy subjects (12 male, 18 female) were included in the study. Coronary flow velocity reserve was measured at baseline and after dipyridamole infusion by echocardiography. Coronary flow velocity reserve was calculated as the ratio of hyperemic to baseline diastolic peak velocities.Carotid intima-media thickness was significantly higher in patients than in control subjects (0.80 +/- 0.29 versus 0.54 +/- 0.14 mm, respectively; P0.001). Moreover, coronary flow velocity reserve was significantly lower in patients than in control subjects (1.84 +/- 0.39 versus 2.65 +/- 0.68, respectively; P0.001).Normotensive patients with autosomal dominant polycystic kidney disease with well-preserved renal function have significantly increased carotid intima-media thickness and significantly decreased coronary flow velocity reserve compared with healthy subjects. These findings suggest that atherosclerosis starts at an early stage in the course of their disease in patients with autosomal dominant polycystic kidney disease.

Chronic kidney disease and need for renal replacement therapy in hemophiliacs are relatively rare occurrences. Successful hemodialysis and peritoneal dialysis applications have been reported in the literature. We report a difficult-to-manage patient with hemophilia A plus factor VIII inhibitor who presented with gastrointestinal bleeding complicated by uremia. We admitted this hemophilia A patient with gastrointestinal bleeding who did not take regular factor infusions. He also had chronic kidney disease due to urinary stone disease. Since uremia might have contributed to bleeding, we chose hemodialysis along with factor VIII supplementation. His factor VIII and factor VIII inhibitor levels were 4% and 5 Bethesda units respectively. In order to bypass the inhibitor, we applied factor VIIA and prothrombin complex concentrate. After cessation of the hemorrhage, we placed a Tenckhoff catheter under prothrombin complex concentrate infusion. We did not observe any perioperative complication. To our knowledge, this is the first report of successful peritoneal dialysis in a hemophilia A patient who had factor VIII inhibitors.

Despite the improvements in diagnostic tools and medical applications, cardiovascular diseases (CVD), especially coronary artery disease (CAD), remain the most common cause of morbidity and mortality in patients with chronic kidney disease (CKD). The main factors for the heightened risk in this population, beside advanced age and a high proportion of diabetes and hypertension, are malnutrition, chronic inflammation, accelerated atherosclerosis, endothelial dysfunction, coronary artery calcification, left ventricular structural and functional abnormalities, and bone mineral disorders. Chronic kidney disease is now recognized as an independent risk factor for CAD. In community-based studies, decreased glomerular filtration rate (GFR) and proteinuria were both found to be independently associated with CAD. This paper will discuss classical and recent epidemiologic, pathophysiologic, and clinical aspects of CAD in CKD patients.