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Background: Severe acute problems, which are related to different systems, could be reasons for applying to the emergency department in patients with peritoneal dialysis. In this study, the aim was to evaluate the reasons and treatment of applications to the emergency department in these patients after office hours. Methods: This study included followed up CAPD patients, who applied to the Emergency Department between January 2017 and May 2020 at our University. Demographical, biochemical, and clinical data, such as, renal, peritoneal, and weekly Kt/V, normalized protein catabolic rate (nPCR), peritoneal UF, and GFR were recorded from the outpatient’s department data. In addition, reasons for application to emergency services and hospitalization were evaluated. Results: 38 peritoneal dialysis patients applied 88 times to the emergency department and were hospitalized between 2017 and 2020. Patients, who applied after office hours were included in the study. Around 130 patients were regularly followed-up in our CAPD outpatient clinic during this period. While the most important peritoneal reasons for admission were exit site infection and catheter dysfunction, extra peritoneal complications were various. Patients were hospitalized in the Cardiology and General Surgery departments with a high rate of acute cardiovascular events and acute abdominal pathologies. Conclusion: Although the first approach to these patients in the emergency department is usually performed by a consulted nephrologist, effective and priority treatment could only be given by an emergency doctor, who has knowledge about the special problems of dialysis patients.

Background: Peritoneal dialysis is a frequently used renal replacement treatment option in patients with end-stage renalfailure, and it is emphasized several times that it should be started as the first treatment in the literature. However, for manydifferent reasons, sometimes the treatment is stopped and switched to hemodialysis. In this study, we aimed to examinethe reasons and factors affecting the transition to hemodialysis in our unit.Methods: Fifty-five patients who were – transferred to hemodialysis – regularly attending the Peritoneal Dialysis Unit ofErciyes University Nephrology Department were included in our study. Biochemical analysis, peritoneal equalization test(PET), dialysis adequacy (kt/V), and creatinine clearance of these patients were recorded regularly. Regular cardiac exami-nations and ambulatory blood pressure measurements were also performed. The reasons for the transition from perito-neal dialysis to hemodialysis were also noted in detail.Results: The mean age of the patients was 54.02 ± 11.41 years, and 27 (62.8%) of the patients were male. While the shortestperiod spent on peritoneal dialysis was 13 months, the longest was 191 months. The most common type of permeabilityin PET analysis of the patients was observed as high-average. The most common reason for the transition to hemodialysiswas inadequate dialysis. Using univariate and multiple regression models, factors that predict the duration of stay in peri-toneal dialysis were examined. We found that the urine volume, total kt/V, and the number of peritonitis could be effectivein predicting this period.Conclusion: Peritoneal dialysis is a renal replacement option that has advantages such as patient comfort, preservation ofthe kidney’s remaining work, and social life. The reasons for the transition should be examined in detail, and the necessaryinterventions to prolong the time in peritoneal dialysis should be evaluated.

Sodium phosphate-based enema preparations are low-volume, hyperosmotic agents commonly used in bowel preparation for sigmoidoscopy/colonoscopy and surgical operation. Although generally safe and well tolerated in adults, severe metabolic complications have been reported with use. The patient in this case report was admitted to the general surgery department with complaints of nausea, vomiting, and abdominal pain after oral sodium phosphate solution. After clinical evaluation, hemodialysis was started for acute kidney injury (AKI). Clinicians should be alert using sodium phosphate solution for bowel cleansing before sigmoidoscopy/colonoscopy and surgical operation, in terms of acute kidney damage and electrolyte imbalance.

Aim: There has been a steady increase in the number of elderly patients with end-stage renal disease (ESRD) receiving renal replacement therapy (RRT). We aimed to compare the efficiency and complications between individual and family member-assisted PD in a patient group with geriatric ESRD. Method: This retrospective study included 50 patients > 65 years of age who received CAPD treatment between 2017-2019 in the peritoneal dialysis unit. Before dialysis modality selection, patients' functional status was evaluated with the 6-item activities of daily living (ADL) and 8-item instrumental daily living activities (IADL). Their three years’ follow-up results were evaluated in terms of dialysis efficiency parameters and dialysis complications. Results: There was no statistical difference between the two groups regarding their age, gender, and biochemical data. There was also no difference between edema, exit-site infection, and leakage; however, a significant difference was observed between the total number of peritonitis and the time until the first peritonitis attack. The time until the first peritonitis attack was shorter in the APD group than in the other group. Besides, the number of total peritonitis attacks was higher in the assisted PD group. Conclusion: The elderly ESRD population is increasing, and PD use is declining worldwide. However, if APD is not done well, it may not be an effective strategy to reverse this decline and provide safe and successful treatment to many frail, elderly patients.

Background Tunneled catheters can be used as an alternative vascular access in patients with limited health expectancy,vascular access problems and several comorbidities. We aimed to present a patient with venous stenosis related- reversible acute Budd-Chiari syndrome after catheter malposition. Case presentation After changing of tunneled catheter insertion, 36-year old man was admitted to our hospital with sudden onset of nausea, fever, chills and worsening general condition In computed tomography (CT) imaging, a hypodense thrombus was observed in which the distal end of the catheter is at the level of drainage of the hepatic veins in the inferior vena cava and that blocked hepatic vein drainage around the catheter. The catheter was removed and a new catheter was inserted in the same session. Because patient’s general condition was good and without fever, he was discharged with advices on the 9th day of hospitalization. Conclusion Although catheter malposition and thrombosis are not a common complication, clinicians should be alert of these complications.

IgA nephropathy (IgA-N) is the most common glomerulonephritis type globally. IgA-N is usually accepted as a kidney disease; however, IgA-N has been reported with infections, autoimmune diseases, and malignancies in the literature. In this report, we present a case of a 33-year-old man diagnosed with IgA nephropathy first and then with B-cell acute lymphoblastic leukemia (B-ALL). He was admitted to our hospital with complaints of nausea, vomiting, fatigue, and headache. Laboratory investigations revealed increased levels of blood urea nitrogen and creatinine, hypercalcemia, anemia, and thrombocytopenia. Kidney biopsy was performed, and IgA nephropathy was detected. Atypical lymphocytes and erythroblasts were present in the peripheral blood smear. Bone marrow biopsy was performed and demonstrated B-ALL. Kidney function tests normalized after 1 month of chemotherapy, including steroid treatment with intravenous fluid administration. In conclusion, it has been speculated that IgA nephropathy is associated with B-ALL.

Background: Although several renal biopsy registry reports have been published worldwide, there are no data on primary glomerular disease trends in Turkey. Methods: Three thousand eight-hundred fifty-eight native kidney biopsy records were assessed in the Turkish Society of Nephrology Primary Glomerulopathy Working Group (TSN-GOLD) Registry. Secondary disease and transplant biopsies were not recorded in the registry. These records were divided into four periods, before 2009, 2009 to 2013, 2013–2017, and 2017–current. Results: A total of 3858 patients (43.6% female, 6.8% elderly) were examined. Nephrotic syndrome was the most common biopsy indication in all periods (58.6%, 53%, 44.1%, 51.6%, respectively). In the whole cohort, IgA nephropathy (IgAN) (25.7%) was the most common PGN with male predominance (62.7%), and IgAN frequency steadily increased through the periods (× 2 = 198, p < 0.001). MGN was the most common nephropathy in the elderly (> 65 years), and there was no trend in this age group. An increasing trend was seen in the frequency of overweight patients (× 2 = 37, p < 0.0001). Although the biopsy rate performed with interventional radiology gradually increased, the mean glomeruli count in the samples did not change over the periods. Conclusions: In Turkey, IgAN is the most common primary glomerulonephritis, and the frequency of this is increasing. © 2022, The Author(s), under exclusive licence to Springer Nature B.V.

Secondary and tertiary hyperparathyroidism is an important problem of chronic kidney disease. Brown tumor is a benign, unusual, reactive lesion as a result of disturbed bone remodeling, from long-standing increase in parathyroid hormone level. Brown tumors may cause morbidity due to pressure symptoms on neural structures and spontaneous bone fractures. Herein, we presented a peritoneal dialysis patient with tertiary hyperparathyroidism under calcand calcitriol treatment for 4 years due to refusing of the parathyroidectomy operation. She admitted to hospital for sudden onset back pain with difficulty in gait and walking, and imaging studies showed an expansile mass lesion in the thoracic spine. She was operated for mass and diagnosed with brown tumor. After operation, she lost the ability of walking than become paraplegic and she underwent rehabilitation program. Preventive measures including calcitriol and cinacalcet may cause a modest decrease in parathyroid hormone levels but it should be remembered for the development of bone complications such as brown tumor formation in patients with moderate elevated PTH levels, especially those with tertiary hyperparathyroidism. Parathyroidectomy should be performed without delay in these cases.

Background and Objectives: Cyst pressure induces renin-angiotensin-aldosterone system activation and kidney hypoxia in autosomal dominant polycystic kidney disease (ADPKD). Lipopolysaccharide-induced Toll-like receptor activation causes metabolic disturbances that are triggered by increased succinate levels and hypoxia inducible factors, which results in inflammation via IL-1β activation. Since we aimed to investigate the role of both inflammation and hypoxia in the clinical course of ADPKD, via succinate levels from sera samples, HIF-1α gene expression from whole blood and urine samples and IL-1βgene expression from whole blood were measured. Methods: One hundred ADPKD patients and 100 matched healthy controls were enrolled to this cross-sectional study. Twenty-four-hour ambulatory blood pressure monitoring was conducted in all participants. Blood, serum, and urine samples were taken after 12-h fasting for the measurement of biochemical parameters and succinate levels. Whole blood and urine samples were used for HIF-1α and IL-1β geneexpression by using quantitative real-time PCR. Results: There were significant differences in whole blood HIF-1α, IL-1β geneexpression, and serumsuccinate levels between the ADPKD patients and the control subjects. Whole blood HIF-1αgene expression, IL-1β geneexpression, and serumsuccinate levels were also significantly different in ADPKD patients with hypertension in comparison with normotensive ones (p < 0.05). Serum succinate levels and blood IL-1β geneexpression were increased in ADPKD patients with high levels of HIF-1α geneexpression (p = 0.018 and p = 0.029, respectively). Conclusions: Increased age,low eGFR, and HIF-1α and IL-1β geneexpressions were also independently associated with hypertension in ADPKD patients. Inflammation and hypoxia are both relevant factors that might be associated with hypertension in ADPKD.

Background Several factors play a role in the pathogenesis of pruritus in uremic patients. The pathophysiology is complex and many factors have been identified in these patients. The aim of this study was to investigate the presence, severity, and possible causes of pruritus in patients with peritoneal dialysis (PD) . Methods Eighty patients, who received continuous ambulatory peritoneal dialysis (CAPD) treatment, were included in this study. Biochemical measurements, parathormone, C-reactive protein (CRP), and vitamin B12 levels of all the patients were recorded. Furthermore, substance P (SP) levels were measured by ELISA methods. Patients were examined by a dermatologist and pruritus degrees were queried using the visual analog score (VAS) with skin dryness. Results In generalized linear model analysis, total urea clearance and SP independently predicted VAS scores. SP was significantly predictive in ROC analysis in identifying the VAS score in patients with peritoneal dialysis. The sensitivity and specificity of SP were 80% and 67% (cut-off > 364), respectively, with an area under the ROC curve of 0.757 (95% CI 0.650-0.865, p < 0.001). SP also was significantly predictive in ROC analysis in identifying xerosis in PD patients. Conclusion Pruritus was proportional to the amount of substance P and total urea clearance was another reason affecting pruritus in peritoneal dialysis patients.

Objective: Coronavirus disease 2019 (COVID-19) has been declared a pandemic by the World Health Organization. Many studies have examined their psychological effects. However, during these periods when the new case of COVID-19 patients decreased, their psychological effects were not sufficiently reported. Our aim was to investigate the anxiety and depressive symptom levels of healthcare workers (HCW) during the periods when the increase in the number of cases slowed down in the COVID-19 epidemic. Materials and Methods: A cross-sectional a questionnaire consisted of Beck Depression Inventory and Beck Anxiety Inventory was applied to 143 people working in a university hospital. General information such as age, gender, marital status, study history in the COVID-19 pandemic, history of chronic disease, and whether there are other HCW at home was collected and compared. Results: The median value of beck anxiety score was 6. The median value of Beck depression score varies by gender (p=0.015). Median value of Beck depression score varies according to marital status (p=0.011). The median value of Beck anxiety score varies according to gender (p=0.008). The median value of Beck anxiety score varies according to occupational groups (p=0.003). A significant link was obtained between Beck depression groups and marital status (p

INTRODUCTION Arteriovenous fistula (AVF) stenosis is one of the most important clinical problems in hemodialysis patients. The histopathological findings of neointimal hyperplasia and impaired angiogenesis have been well established in stenotic AVFs. Soluble vascular endothelial growth factor receptor-1 (sVEGFR-1) has been implicated in pathological angiogenesis. Thus, we aimed to investigate the association between sVEGFR-1 and AVF stenosis in hemodialysis patients. METHODS This prospective cohort study included 70 patients with end-stage renal disease. Forty-five patients were included in the final analysis, and the median follow-up period was 36 months. Venous stenosis was detected by physical examination and documented by fistulography. Blood samples were analyzed a day before the fistula operation, and serum levels of sVEGFR-1 were measured. FINDINGS The median sVEGFR-1 level was higher in the stenosis group than in the nonstenosis group (17 pg/mL [89.5%] vs. 5 pg/mL [19.2%], respectively; P 30 kg/m2 ) had the shortest stenosis-free survival (20 months [9.35-30.65]). Multivariate Cox analysis showed that sVEGFR-1, serum creatinine, and parathyroid hormone levels were associated with AVF stenosis risk. Kaplan-Meier survival curves showed that patients with less than the median value of sVEGFR-1 (

Background: We aimed to investigate the role of HIF-2α on chronic allograft nephropathy, which is a consequence of chronic inflammation and fibrosis. Method: The study was conducted between November 2019 and January 2021, patients who had a kidney biopsy in our university in the last two years were included in the study. 15 patients with a diagnosis of chronic allograft nephropathy proven by biopsy, 15 patients with an eGFR below 60 ml/dk for other reasons as non-CAN group. Also, 15 patients with an eGFR above 60 ml/dk were included in the study as a control group. Serum HIF-2α levels were detected by a commercial kit using the quantitative sandwich enzyme immunoassay technique. Results: HIF-2α was higher in the CAN group compared to other groups. HIF-2α was significantly predictive in ROC analysis in identifying renal transplantation patients with CAN. The sensitivity and specificity of HIF-2α were 100% and 76% (cut off >0.2) with an area of under the ROC curve of 0.941 (95% CI 0. 850-1.000), p < 0.001). Conclusion: Serum HIF-2α may usefulness as potential simple biomarkers for detecting allograft dysfunction after kidney transplantation and it can be also a guide in determining these treatment strategies.

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.

This study aimed to investigate the relationship between fluid and sodium excretion and blood pressure, echocardiographic parameters, and arterial stiffness in peritoneal dialysis (PD) patients and to evaluate the effect of sodium excretion on patients’ survival. This study was conducted as a single-centered, prospective study in the Department of Nephrology in Erciyes University. The patients included in the study were followed up for 3 years. Seventy PD patients were included in the study. We recorded demographic characteristics, biochemical parameters, urine and peritoneal ultrafiltration volumes, peritoneal equalization tests, ambulatory blood pressure measurements, and echocardiographic measurements. We measured the amount of total sodium excretion of the patients and arterial stiffness using pulse wave velocity (PWV). Patients were divided into two groups based on the amount of total sodium excretion: low group and high group. We compared these groups in terms of cardiac and biochemical parameters. When demographic data and biochemical values were compared, there was no significant difference between the two groups. NT-proBNP level, systolic blood pressure, and mean arterial pressure were significantly higher in the low group (p: 0.02, p: 0.031, p: 0.05, respectively). Net ultrafiltration was significantly higher in the high group (p: 0.03), was also found to be high in patients with high sodium excretion (p: 0.001). Negative correlations were found between sodium excretion and net ultrafiltration, NT-Pro BNP, and PWV. At the end of the 3-year follow-up, the survival rate was shorter and the mortality rate was higher in the low group (p: 0.042). Fluid status in PD patients can affect arterial stiffness both directly and through hypertension. Correction of hypervolemia has the potential to not only prevent hypertension and left ventricular hypertrophy, but also to improve arterial stiffness, a well-known cardiovascular risk factor. The mortality rate was higher in PD patients with low total sodium excretion. Therefore, these patients should be followed more closely to ensure volume control.

Background Neointimal hyperplasia is the main cause of arteriovenous fistula (AVF) failure. Hypoxia-inducible factors (HIFs) factors are associated with neointimal hyperplasia. Thus, we investigated the association between HIF-2 alpha (HIF-2 alpha) and AVF maturation in end-stage kidney disease (ESKD) patients. Methods This prospective cohort study was conducted in 21 voluntary healthy subjects and 50 patients with ESKD who were eligible for AVF creation. Inclusion criteria were being ESKD patients without a history of AVF surgery and dialysis. Eight patients excluded from the study due to having unavailable veins six patients were excluded due to acute thrombosis after surgery. One patient lost to follow-up. A total of 35 patients were included in final analysis. The blood samples were collected a day before the AVF surgery for biochemical parameters and HIF-2 alpha measurement. HIF-2 alpha levels were measured by the ELISA method. Results Compared with healthy subjects, ESKD patients had a significantly higher level of HIF-2 alpha. [1.3 (1.0-1.9) vs 2.2 (1.6-3.0)] (P = .002). Patients were divided into two groups after the evaluation of AVF maturation, as the mature group (n = 19) and the failure group (n = 16). Serum HIF-2 alpha level was 1.7 (1.1-1.8) in the mature group; however, it was 3.1 (2.8-3.3 in failure group (P .001). Multiple logistic regression analyses showed that HIF-2 alpha independently predicted AVF maturation. The ROC curve analysis showed that HIF-2 alpha > 2.65 predicted AVF maturation failure with the 87% sensitivity and 94% specificity [AUC:0.947, 95% CI (0.815-0.994),P .001]. Conclusions HIF-2-alpha levels were higher in ESKD patients than healthy subjects. HIF-2-alpha could be a marker of AVF maturation failure.

Autosomal-dominant polycystic kidney disease (ADPKD) is associated with oxidative stress and hypertension development before renal function decline and cardiovascular disease development. Oxidative stress-responsive kinase-1 (OSR-1) participates in the signaling regulating Na+ transport during oxidative stress and also plays a role in the regulation of cell volume and blood pressure. Therefore, we aimed to investigate the potential role of OSR-1 in ADPKD patients. Eighty ADPKD patients, 80 healthy controls, and 80 non-ADPKD patients with hypertension were enrolled in this cross-sectional study. Twenty-four-hour ambulatory blood pressure monitoring was conducted in all participants. Blood samples were taken after 12-h fasting for the measurement of biochemical parameters and OSR-1 gene expression. Vascular dysfunction was assessed using ischemia-induced forearm flow-mediated vasodilation (FMD). Briefly, of the 80 ADPKD patients, 41(51%) were male, and 53(66%) of them were hypertensive. The mean age of the 80 controls was 35.3 +/- 12.6 years, and 37(46%) of them were male. The mean age of the 80 non-ADPKD patients with hypertension was 44.6 +/- 11.9 years, and 38(47.5) of them were male. There were significant differences in serum OSR-1 gene expression between the ADPKD patients and the control subjects. Serum OSR-1 gene expression was also significantly increased in hypertensive ADPKD patients in comparison with both normotensive ADPKD counterparts and non-ADPKD hypertensive subjects. Serum OSR-1 gene expression was increased in patients with ADPKD than healthy subjects. Low estimated glomerular filtration rate (eGFR), OSR-1 gene expression, total kidney volume (TKV), and high-density lipoprotein (HDL) were also independently associated with hypertension in ADPKD patients.

Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by multisystem involvement. The renal involvement of SLE may present with hematuria, proteinuria or acute kidney dysfunction. It is well established that pregnancy may trigger the disease activation in patients with SLE. Lupus nephritis (LN) may be diagnosed during pregnancy in very few patients. Preeclampsia is a pregnancy-related disorder characterized by maternal hypertension, proteinuria, and edema and is sometimes complicated by renal dysfunction, which usually occurs in the last trimester of pregnancy. However preeclampsia rarely occurs within 48 h of postpartum. In this report, we present the case of a 20-year-old patient with oliguria, proteinuria, edema, and hypertension who was diagnosed with preeclampsia starting at the 35th week of her first pregnancy. Acute kidney failure developed in the postpartum period after emergency cesarian delivery. Crescentic and diffuse LN was revealed by renal biopsy. While SLE is a risk factor for preeclampsia, LN should be considered in kidney failure in the third trimester or postpartum period.

Renal amyloid A (AA) amyloidosis is one of the systemic etiologies of nephrotic syndrome (NS) in adults. Chronic inflammatory conditions including rheumatoid arthritis, familial periodic fever syndromes, spondyloarthropathies, and chronic bacterial infections such as tuberculosis and chronic osteomyelitis are the common causes of secondary AA amyloidosis. In this case report, we reported a patient presented with nephrotic syndrome and secondary AA amyloidosis due to chronic hepatitis B infection in the absence of any glomerulonephritis.

OBJECTIVE: Metastatic infection is a rare but serious complication in patients undergoing hemodialysis (HD). In this retrospective study, we aimed to analyze the clinical aspects, responsible microorganisms, treatments, and clinical outcomes in HD patients with metastatic infection.

Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary cause of chronic kidney disease. The intriguing role of innate immune system and inflammation become a target for potential therapeutic approach to slow progression. When toll-like receptors (TLRs) signaling and their receptors activate, they start a cascade of intracellular signaling that induces the production of the inflammatory cytokines and chemokines. Thus, we aim to investigate the association of TLRs between progression of ADPKD. Ninety ADPKD patients and ninety matched controls were enrolled this prospective study and were followed during 3 years. TLR-2 and TLR-4 gene polymorphisms and expressions were measured. Hypertension was diagnosed with ambulatory blood pressure monitoring. Rapid progression was defined as sustained decline in estimated glomerular filtration rate (eGFR) of more than 5 mL/min per 1.73 m2 per year. TLR-4Asp299Gly polymorphisms were significantly different between patient and control group (P

BACKGROUND Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated condition that consisted of disorders that share particular clinical, serologic and pathologic properties. The common presentation of disease includes tumor-like swelling of involved organs and the histopathological findings are a lymphoplasmacytic infiltrate enriched with IgG4-positive plasma cells, and a variable degree of fibrosis that has a characteristic "storiform" pattern in biopsy specimens of tumor-like masses. Major presentations of this disease, which often affects more than one organ, include autoimmune pancreatitis, salivary gland disease (sialadenitis), orbital disease and retroperitoneal fibrosis. The steroid treatment is essential for the treatment of the disease however, other immunosuppressive drugs including cyclophosphamide or rituximab could be an option in resistant cases. CASE SUMMARY Herein, we reported a 34-year-old woman whom previously had diagnosed with asthma, rheumatoid arthritis and Sjögren’s syndrome (SS) referred our nephrology department due to acute kidney failure development at the last rheumatology visit. After kidney biopsy she has been diagnosed with IgG4-RD and tubuluointerstitial nephritis. She had been accepted resistant to steroid, mycophenolate mofetil, methotrexate and azathioprine therapies due to receiving in last two years. She refused to receive cyclophosphamide due to potential gonadotoxicity of the drug. Thus, rituximab therapy was considered. She received 1000 mg infusion, 15 d apart and 6 mo later it has been administered same protocol. After one year from the last rituximab dose serum creatinine decreased from 4.4 mg/dL to 1.6 mg/dL, erythrocyte sedimentation rate decreased from 109 mm/h to 13 mm/h [reference range (RR) 0-20], and C-reactive protein decreased from 55.6 mg/L to 5 mg/L (RR 0–6). All pathologic lymph nodes and masses were also disappeared. CONCLUSION Patients with IgG4-RD usually misdiagnosed with rheumatologic diseases including systemic lupus erythematous or SS and also they were screened for the presence of malignancy. Rituximab could be an important treatment option in cases with steroid resistant tubulointerstitial nephritis in IgG4-RD.

Background Urinary tract infections (UTI) are one of the important clinical presentations in patients with autosomal dominant polycystic kidney disease (ADPKD). The association between UTI among genotypic and phonotypic properties of ADPKD patients is still obscure. Thus, we investigated the relationship between UTI and polycystin gene mutation with total kidney volume. Methods Forty patients with ADPKD patients with a history of more than two UTI and age-gender-matched 40 ADPKD patients without UTI history enrolled in the study. Ambulatory blood pressure monitoring was performed in all participants. Magnetic resonance imaging (MRI) was performed with a 1.5-T system, and total kidney volumes were calculated using mid-slice technique. To determine PKD1 and PKD2 genotype, we performed molecular and genetic tests involving the following steps: DNA isolation, next-generation sequencing (NGS) and data analysis. Results ADPKD patients with UTI had lower eGFR values than those without UTI [64.9 (32.2-100.8) vs 89.5 (59.0-110.0) (p = 0.041)]. In addition, patients with UTI had significantly increased height-adjusted total kidney volume than patients without UTI [950 (290-1350) vs 345 (243-780.0) (p = 0.005)]. Multiple logistic regression analysis showed that the PKD1-truncating mutation and hTKV independently predicted UTI. The sensitivity and specificity of hTKV were 65% and 77% (cutoff > 727 cm(3)) with an area of under the ROC curve of 0.70 (95% CI 0.56-0.85, p = 005). Conclusions ADPKD patients with larger kidneys and PKD1 mutation are susceptible to increased risk of multiple UTI. Additionally, renal function decreased in ADPKD patients with multiple UTI history.

Background Overweight and obesity were recently associated with a poor prognosis in patients with autosomal dominant polycystic kidney disease (ADPKD). Whether the metabolic consequences of obesity as defined by the metabolic syndrome (MS) are also linked with disease progression remains untested. Methods Eligible ADPKD patients with different stages of CKD (n = 105) and 105 non-diabetic controls matched for CKD stage were enrolled in the study. Groups were evaluated at baseline for presence of MS, blood markers of metabolism, homeostasis model assessment of insulin resistance (HOMA-IR) score, and biochemical markers of inflammation (hs-CRP, IL-1 beta, IL-6, TNF-alpha and PON-1). MS was defined according to the National Cholesterol Education Program-Adult Treatment Panel III (NCEP-ATP III). Patients were followed for 12 months and progression defined as a decrease in baseline eGFR > 10%. Results MS and hypertension were more prevalent amongst ADPKD patients than in the control group. Meanwhile, markers of inflammation such as hs-CRP (3.63 [3.45-5.17] vs. 4.2 [3.45-8.99] mg/dL; p = 0.014), IL-6 (21.65 [14.1-27.49] vs. 24.9 [16.23-39.4] pg/mL; p = 0.004) and IL-1 beta (21.33 [15.8-26.4] vs. 26.78 [18.22-35] pg/mL; p < 0.001) levels were all more elevated in ADPKD patients than in non-diabetic CKD subjects. In multivariate analysis having a truncating PKD1 mutation predicted (OR 1.25 [1.09-1.43]; p = 0.002) fulfilling the MS criteria. Finally, ADPKD patients fulfilling MS criteria had a significantly more rapid progression during 12 months of follow-up than did those that did not (OR 3.28 [1.09-9.87]; p = 0.035). Conclusions Our data supports the notion that dysmetabolisms part of the ADPKD phenotype and associated with a poor outcome, especially in patients with a truncating PKD1 mutation.

Familial Mediterranean fever (FMF) is an autosomal recessive hereditary disease characterized by recurrent attacks of fever, usually accompanied by sterile polyserositis. Although colchicine is the main medical treatment option for FMF, potential adverse effects and drug interactions should be considered during the follow-up of these patients. Herein, we presented a case of a hemodialysis patient with FMF, who developed rhabdomyolysis due to concomitant use of colchicine and clarithromycin for pneumonia treatment.

Peritoneal dialysis (PD) patients have different peritoneal membrane permeability (transport) characteristics. High peritoneal membrane permeability is associated with increased mortality risk in the patient population. In this study, we aimed to investigate possible risk factor(s) related to high peritoneal membrane permeability.The study included 475 PD patients (46.1 ± 14.5 years of mean age; 198 female and 277 male). The patients were divided two groups according to peritoneal equilibration test (PET) result: high-permeability group (high and high-average) and low- permeability group (low-average and low).In both the univariate and multivariate logistic regression analyses, it was found that diabetes mellitus and hypoalbuminemia was significantly associated with high peritoneal membrane permeability [relative risk (RR): 1.90, 95% confidence interval (CI): 1.26-2.86, p: 0.002 and RR: 2.14, 95% CI: 1.44-3.18, p0.001, respectively].Diabetes mellitus and hypoalbuminemia were closely associated with high peritoneal membrane permeability. Diabetic patients had 1.9 times the likelihood of having high permeability. However, the relationship between hypoalbuminemia and high peritoneal permeability appears to be a result rather than cause.

The prominent features of autosomal dominant polycystic kidney disease (ADPKD) are early development of hypertension, chronic kidney disease and cardiovascular problems. Thus, we aimed to investigate the role of endothelin, a vascular biomarker, in the clinical course of ADPKD, including renal and cardiovascular survival. In 138 patients with ADPKD and 28 healthy controls, we measured serum endothelin-1 (ET-1) levels by enzyme-linked immunosorbent assay (ELISA). Endothelium-dependent vasodilatation (flow-mediated dilatation, FMD) and endothelium-independent vasodilatation (nitroglycerin-mediated dilatation, NMD) of the brachial artery were assessed non-invasively with high-resolution ultrasound. Magnetic resonance imaging (MRI) was performed with a 1.5-T system, and total kidney volumes were calculated using mid-slice technique. To determine PKD1 and PKD2 genotype, we performed molecular and genetic tests involving the following steps: DNA isolation, next-generation sequencing (NGS) and data analysis. Endothelin levels and height-adjusted total kidney volumes (hTKV) significantly increased while the estimated glomerular filtration rate (eGFR) decreased across CKD stages 1–4. Hypertension was more frequent in ADPKD patients with high serum endothelin. At multivariate Cox analysis, endothelin level, PKD1 truncating mutation, hTKV, high-sensitive C reactive protein (hs-CRP) level and the presence of diabetes mellitus were associated with the risk of overall survival. Moreover, endothelin level, PKD1 truncating mutation, hTKV, age and presence of hypertension were associated with the risk of renal survival. Additionally, body mass index (BMI), FMD, PKD1 truncating mutation, endothelin and triglyceride levels were independently associated with hypertension. Increased serum endothelin levels independently predict hypertension in ADPKD. Serum endothelin levels are also associated with both renal and overall survival in patients with ADPKD.

OBJECTIVE: We aimed to investigate whether there is a relationship between neutrophil/lymphocyte (N/L) ratio and albuminuria in diabetic patients.

Aim The aims of this study were to report the prevalence of metabolic syndrome (MS) in a cohort of Turkish kidney transplant recipients and to define the relationships between MS, microalbuminuria and C-reactive protein (CRP), which are cardiovascular risk factors, in kidney transplant setting. Methods This cross sectional study included 170 adult renal transplantation recipients with a mean follow-up of 53.1 ± 49.9 months. The diagnosis of MS was made according to the National Cholesterol Education Program-Adult Treatment Panel III (NCEP-ATP III) criteria. Microalbuminuria was defined as a urinary albumin/creatinine ratio of 30–300 mg/g. CRP levels ≥6.0 were classified as high CRP. Results Mean age was 39.3 ± 11 years. The prevalence of MS was 45.8% (n = 78). The prevalence of microalbuminuria was not different in patients with MS compared to those without MS (39.7% vs 37%, P = .428). In multivariate logistic regression analyses, systolic blood pressure (SBP) (odds ratio 1.68; 95% confidence interval [CI] 1.12–2.52; P = .011) and high fasting glucose (odds ratio 2.82; 95% confidence interval [CI] 1.16–6.86; P = .022) were significantly associated with microalbuminuria. When patients with MS and high CRP were compared with patients with normal CRP and without MS, microalbuminuria did not differ between the groups ( P = .213). Conclusion The prevalence of MS in our kidney recipient cohort was found to be increased compared to general population. MS was not related to increased prevalence of microalbuminuria, even when combined with high CRP. Microalbuminuria was associated with elevated SBP and hyperglycemic status.

Background The presence of cardiac valvular calcification in patients with chronic kidney disease has become important. We aimed to evaluate the prevalence of cardiac valvular calcification and its association with serum Fetuin-A levels in kidney transplant recipients. Methods The cardiac valvular calcification was assessed by echocardiography in 89 kidney transplant recipients. Serum Fetuin-A levels were measured by use of the enzyme-linked immunosorbent assay method. Results Patients were divided into 3 groups: patients without cardiac valvular calcification (n = 14), patients with aortic valve calcification (n = 51), and patients with both aortic and mitral valve calcification (n = 24). Aortic calcification area and number of aortic calcifications were significantly increased in the group with aortic and mitral calcification group compared with the other two groups. These two parameters were also significantly increased in patients with cardiac valvular calcification compared with patients without cardiac valvular calcification. Serum Fetuin-A levels were significantly increased in patients with aortic valve calcification compared with the other two groups, whereas there were no significant differences between these two groups. Serum Fetuin-A levels were positively correlated with blood urea nitrogen level (r = .241, P = .025) and serum creatinine level (r = .262, P = .014), whereas it was negatively correlated with serum phosphorus level (r = −.409, P Conclusions Serum Fetuin-A levels can be taken into consideration for the assessment of patients in terms of cardiac valvular calcification, depending on the relationship between serum Fetuin-A levels and cardiac valvular calcification during follow-up after kidney transplantation.

Aim: The aim of this study is to investigate whether there is a relationship between inflammation and volume status in patients underwent peritoneal dialysis (PD). Patients and method: This cross-sectional study included 159 PD patients. The median duration of PD was 17 (range, 1-151) months. All patients were examined using bioelectrical impedance analysis to estimate the ratio of extracellular water to total body water (ECW/TBW), which was used to assess their volume status. The patients were categorized as having one of the following three volume statuses: hypervolemic (above + 2 SD from the mean, which was obtained from healthy controls), normovolemic (between + 2 SD and -2 SD), or hypovolemic (below -2 SD from the mean). Five patients with hypovolemia were excluded from the study. Fifty-six patients were hypervolemic whereas 98 patients were euvolemic. High-sensitive C-reactive protein (hs-CRP) levels were measured to evaluate inflammation in all patients. Results: hs-CRP value levels were significantly higher in hypervolemic patients compared with euvolemic patients [7.1 (3.1-44.0) mg/L vs. 4.3 (3.1-39.6), p: 0.015, respectively]. Left ventricular hypertrophy was more frequent in hypervolemic patients compared with euvolemic patients (53.6% vs. 30.6%, p: 0.004, respectively). ECW/TBW ratio positively correlated with hs-CRP (r: 0.166, p: 0.039). Gender, hs-CRP, and residual Kt/V urea were found to be independent risk factors for hypervolemia in multivariate analysis. Conclusion: Inflammation is associated with hypervolemia in PD patients. Residual renal functions play an important role to maintain euvolemia in PD patients.

AIM Nephrotic syndrome (NS) is associated with an increased rate of cardiovascular events. The YKL-40 level is associated with atherosclerosis, endothelial dysfunction and proteinuria in renal and non-renal populations. The aim of this study was to investigate the relationships between the YKL-40 level and both vascular injury and proteinuria in NS patients. METHODS Sixty-nine NS patients and 20 healthy subjects were enrolled in the present study. The endothelial function was assessed according to the flow mediated dilatation (FMD) and the degree of arterial stiffness was determined based on the pulse wave velocity (PWV). The serum YKL-40 levels were measured using ELISA. RESULTS The YKL-40 levels and PWV values were higher and the FMD values were lower in the NS patients than in the healthy controls. However, the CA-IMT and LVEF levels were not statistically different between the two groups. The patients were divided into three groups with respect to the extent of proteinuria: the normoproteinuria group (n:18), non-nephrotic proteinuria group (n:33) and nephrotic proteinuria group (n:18). Consequently, the YKL-40 levels and PWV values were significantly increased and the FMD values were decreased in the nephrotic proteinuria group compared to that observed in both the non-nephrotic proteinuria and normoproteinuria groups. Furthermore, the YKL-40 level correlated with the FMD and PWV values in the NS patients. In addition, proteinuria correlated with the YKL-40, FMD, PWV, eGFR and fasting LDL cholesterol values in this patient group. Multivariate linear regression analyses showed that the YKL-40 and eGFR values were effective in predicting proteinuria in the NS patients. CONCLUSIONS The serum YKL-40 level is associated with endothelial dysfunction and increased arterial stiffness in NS patients and may be an indicator of the level of proteinuria in this patient population.

Background The pathogenesis of post-transplantation erythrocytosis (PTE) is not well understood and appears to be multifactorial. Our hypothesis in this study was that several factors, including toxicity of calcineurin inhibitor, immunologic factors, and chronic allograft nephropathy, can trigger local tissue hypoxia in peritubular interstitium, which is where production of erythropoietin (EPO) takes place. This local interstitial tissue hypoxia can cause an increase in renal EPO production, which induces the development of PTE. Methods This cross-sectional study included 15 renal transplant recipients, in whom polycythemia developed after kidney transplantation, with elevated hematocrit level to >51%. Forty-eight age- and gender-matched renal transplant recipients with normal hematocrit level were included as the renal transplant control group. In addition, 13 age- and gender-matched healthy subjects were also included as the healthy control group. We used urine hypoxia-inducible factor-2 alpha (HIF-2α) levels to evaluate whether there is local tissue hypoxia in renal allograft. HIF-2α levels were measured by double antibody sandwich enzyme-linked immunosorbent assay (ELISA). Serum EPO and insulin-like growth factor-1 (IGF-1) levels were also measured. Results HIF-2α levels were significantly lower in the polycythemia group than the other two groups, but there was no significant difference between the healthy control group and the renal transplant control group with regard to HIF-2α levels. There was no significant difference among the 3 study groups in terms of levels of serum EPO and IGF-1. Conclusion Local tissue hypoxia in renal allograft does not seem to play an important role in the development of PTE.

Background Autosomal dominant polycystic kidney disease (ADPKD) is a common hereditary disorder with unclear disease mechanism. Currently, overt hypertension and increased renal volume are the best predictors of renal function. In this study, we assessed the usefulness of selected circulating microRNAs (miRs) to predict disease progress in a cohort with ADPKD. Methods Eighty ADPKD patients (44.6 ± 12.7 years, 40% female, 65% hypertensive) and 50 healthy subjects (HS; 45.4 ± 12.7, 44% female) were enrolled in the study. Serum levels of 384 miRs were determined by Biomark Real Time PCR. Groups were compared using the limma method with multiple-testing correction as proposed by Smyth (corrected p 10% loss of GFR over the following 12 months (cut-off >2.2 AU, sensitivity 83%, specificity 78%, area 0.872 [95% CI: 0.790–0.953, p

Castleman's disease (CD), also known as angiofolicular lymph node hyperplasia, is a rare heterogenous group of lymphoproliferative disorders. Histologically, it can be classified as hyaline vascular type, plasma cell type, or mixed type. Clinically two different subtypes of the CD are present: Unicentric and multicentric. Unicentric CD is generally asymptomatic and associated with hyaline vascular type, and its diagnoses depend on the localized lymphadenopathy on examination or imaging studies. However, multicentric CD presents with generalized lym-phadenopathy and systemic symptoms including malaise, fever, night sweats, weight loss, and it is associated with the plasma cell type and mix type. Herein, we report a patient with unicentric CD of the plasma cell type without systemic symptoms, who developed end stage renal failure caused by amyloidosis 6 years after onset of CD.

reSultS: The mean age was 47 ± 13 years and 38 (74.5%) of the patients were male. No significant difference was found in echocardiographic parameters including ejection fraction, fractional shortening, left ventricular mass, left ventricular mass index, left ventricular posterior wall thickness, inter ventricular septal thickness, left atrial diameter, early diastolic filling/late diastolic filling ratio before and after the period of PD. Left ventricular end-systolic diameter and left ventricular end-diastolic diameter values were significantly lower found in the period after PD.