Evaluation of the Renal and Cardiovascular Effects of Long-Term Tolvaptan Treatment in Autosomal Dominant Polycystic Kidney Disease.

INTRODUCTION: Cardiovascular diseases constitute a significant cause of morbidity and mortality in individuals with autosomal dominant polycystic kidney disease (ADPKD). This study aimed to assess the long-term effects of tolvaptan on the kidneys and heart in rapidly progressing ADPKD. METHODS: Among 354 patients diagnosed with ADPKD, 58 meeting the eligibility criteria for tolvaptan were included in the study. The study comprised two groups with similar demographic and clinical characteristics: 29 patients receiving tolvaptan treatment and 29 in the control group. Several included genetic analysis, magnetic resonance imaging, and echocardiography. Clinical and cardiac changes were recorded in both groups after a 3-year follow-up. RESULTS: Tolvaptan treatment demonstrated a significant reduction in the rate of eGFR decline compared to the control group. Furthermore, it was observed that tolvaptan could prevent the development of cardiac arrhythmias by inhibiting an increase in QTc interval and heart rate. CONCLUSION: These findings suggest that, in addition to slowing kidney progression in ADPKD management, tolvaptan may potentially benefit in preventing cardiac complications.

The refractory secondary hyperparathyroidism presenting with retro-orbital brown tumor: a case report.

BACKGROUND: Tertiary hyperparathyroidism describes the autonomous and excessive secretion of parathyroid hormone (PTH) by the parathyroid glands after longstanding secondary hyperparathyroidism in chronic kidney disease. Brown tumors are a sign of uncontrolled hyperparathyroidism. In this case, we have reported a refractory and destructive hyperparathyroidism storm. Also, it presented with atypical onset and unexpected adenoma location. CASE PRESENTATION: A 37-year-old man was diagnosed with end-stage kidney disease 22 years ago. He has been undergoing dialysis treatment since that time. Recently, he was admitted to the ophthalmology department due to the unilateral anterior bulging of the right eye and drooping of the eyelid. Magnetic resonance imaging exhibited an extraconal mass lesion located in the right orbital posterior superolateral position. Computerized tomography scans considered expansile bone lesion with peripheral calcification and originating from the sphenoid wing. The bone mass lesion was resected via craniotomy due to the compressive effect. The pathological findings were consistent with brown tumors. Plasma intact PTH level was 4557 pg/mL. The patient informed that he underwent parathyroidectomy and two leg fractures operation in a medical query. Parathyroid scintigraphy determined three distinct foci consistent with adenomas and one of them was in mediastenum. Second parathyroidectomy was recommended to the patient but the patient refused surgery. Despite his medication and dialysis regimen being revised, PTH levels were maintained at higher levels in follow-up. CONCLUSIONS: We presented a hyperparathyroidism case that was resistant to all treatments and exhibited all the severe complications in a long-term dialysis patient. Furthermore, this case has revealed the importance and difficulty of secondary hyperparathyroidism management.

Evaluation of the causes affecting the development of pruritus in patients with peritoneal dialysis.

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.

Management of Hyperkalemia in Heart Failure.

Hyperkalemia is a common electrolyte abnormality in heart failure (HF) that can cause potentially life-threatening cardiac arrhythmias and sudden cardiac death. HF patients with diabetes, chronic kidney disease and older age are at higher risk of hyperkalemia. Moreover, hyperkalemia is also often associated with the use of renin-angiotensin-aldosterone system inhibitors (RAASi) including angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, mineralocorticoid receptor antagonists and sacubitril-valsartan. In clinical practice, the occurrence of hyperkalemia is a major concern among the clinicians and often limits RAASi use and/or lead to dose reduction or discontinuation, thereby reducing their potential benefits for HF. Furthermore, recurrent hyperkalemia is frequent in the long-term and is associated with an increase in hyperkalemia-related hospitalizations. Therefore, management of hyperkalemia has a special importance in HF patients. However, treatment options in chronic management are currently limited. Dietary restriction of potassium is usually ineffective with variable adherence. Sodium polystyrene sulfonate is commonly used, but its effectiveness is uncertain and reported to be associated with intestinal toxicity. New therapeutic options such as potassium binders have been suggested as potentially beneficial agents in the management of hyperkalemia. This document discusses prevalence, predictors and management of hyperkalemia in HF, emphasizing the importance of careful patient selection for medical treatment, uptitration of the doses of RAASi, regular surveillance of potassium and treatment options of hyperkalemia.

Characteristics and outcomes of acute kidney injury in hospitalized COVID-19 patients: A multicenter study by the Turkish society of nephrology.

BACKGROUND: Acute kidney injury (AKI) is common in coronavirus disease-2019 (COVID-19) and the severity of AKI is linked to adverse outcomes. In this study, we investigated the factors associated with in-hospital outcomes among hospitalized patients with COVID-19 and AKI. METHODS: In this multicenter retrospective observational study, we evaluated the characteristics and in-hospital renal and patient outcomes of 578 patients with confirmed COVID-19 and AKI. Data were collected from 34 hospitals in Turkey from March 11 to June 30, 2020. AKI definition and staging were based on the Kidney Disease Improving Global Outcomes criteria. Patients with end-stage kidney disease or with a kidney transplant were excluded. Renal outcomes were identified only in discharged patients. RESULTS: The median age of the patients was 69 years, and 60.9% were males. The most frequent comorbid conditions were hypertension (70.5%), diabetes mellitus (43.8%), and chronic kidney disease (CKD) (37.6%). The proportions of AKI stages 1, 2, and 3 were 54.0%, 24.7%, and 21.3%, respectively. 291 patients (50.3%) were admitted to the intensive care unit. Renal improvement was complete in 81.7% and partial in 17.2% of the patients who were discharged. Renal outcomes were worse in patients with AKI stage 3 or baseline CKD. The overall in-hospital mortality in patients with AKI was 38.9%. In-hospital mortality rate was not different in patients with preexisting non-dialysis CKD compared to patients without CKD (34.4 versus 34.0%, p = 0.924). By multivariate Cox regression analysis, age (hazard ratio [HR] [95% confidence interval (95%CI)]: 1.01 [1.0-1.03], p = 0.035], male gender (HR [95%CI]: 1.47 [1.04-2.09], p = 0.029), diabetes mellitus (HR [95%CI]: 1.51 [1.06-2.17], p = 0.022) and cerebrovascular disease (HR [95%CI]: 1.82 [1.08-3.07], p = 0.023), serum lactate dehydrogenase (greater than two-fold increase) (HR [95%CI]: 1.55 [1.05-2.30], p = 0.027) and AKI stage 2 (HR [95%CI]: 1.98 [1.25-3.14], p = 0.003) and stage 3 (HR [95%CI]: 2.25 [1.44-3.51], p = 0.0001) were independent predictors of in-hospital mortality. CONCLUSIONS: Advanced-stage AKI is associated with extremely high mortality among hospitalized COVID-19 patients. Age, male gender, comorbidities, which are risk factors for mortality in patients with COVID-19 in the general population, are also related to in-hospital mortality in patients with AKI. However, preexisting non-dialysis CKD did not increase in-hospital mortality rate among AKI patients. Renal problems continue in a significant portion of the patients who were discharged.

Characteristics and outcomes of hospitalised older patients with chronic kidney disease and COVID-19: A multicenter nationwide controlled study.

OBJECTIVE: Older adults with co-morbidities have been reported to be at higher risk for adverse outcomes of coronavirus disease 2019 (COVID-19). The characteristics of COVID-19 in older patients and its clinical outcomes in different kidney disease groups are not well known. METHODS: Data were retrieved from a national multicentric database supported by Turkish Society of Nephrology, which consists of retrospectively collected data between 17 April 2020 and 31 December 2020. Hospitalised patients aged 18 years or older with confirmed COVID-19 diagnosis suffering from stage 3-5 chronic kidney disease (CKD) or on maintenance haemodialysis (HD) treatment were included in the database. Non-uraemic hospitalised patients with COVID-19 were also included as the control group. RESULTS: We included 879 patients [388 (44.1%) female, median age: 63 (IQR: 50-73) years]. The percentage of older patients in the CKD group was 68.8% (n = 188/273), in the HD group was 49.0% (n = 150/306) and in the control group was 30.4% (n = 70/300). Co-morbidities were higher in the CKD and HD groups. The rate of presentation with severe-critical disease was higher in the older CKD and HD groups (43.6%, 55.3% and 16.1%, respectively). Among older patients, the intensive care unit (ICU) admission rate was significantly higher in the CKD and HD groups than in the control group (38.8%, 37.3% and 15.7%, respectively). In-hospital mortality or death and/or ICU admission rates in the older group were significantly higher in the CKD (29.3% and 39.4%) and HD groups (26.7% and 30.1%) compared with the control group (8.6% and 17.1%). In the multivariate analysis, in-hospital mortality rates in CKD and HD groups were higher than control group [hazard ratio (HR): 4.33 (95% confidence interval [CI]: 1.53-12.26) and HR: 3.09 (95% CI: 1.04-9.17), respectively]. CONCLUSION: Among older COVID-19 patients, in-hospital mortality is significantly higher in those with stage 3-5 CKD and on maintenance HD than older patients without CKD regardless of demographic characteristics, co-morbidities, clinical and laboratory data on admission.

Predicting the outcome of COVID-19 infection in kidney transplant recipients.

BACKGROUND: We aimed to present the demographic characteristics, clinical presentation, and outcomes of our multicenter cohort of adult KTx recipients with COVID-19. METHODS: We conducted a multicenter, retrospective study using data of patients hospitalized for COVID-19 collected from 34 centers in Turkey. Demographic characteristics, clinical findings, laboratory parameters (hemogram, CRP, AST, ALT, LDH, and ferritin) at admission and follow-up, and treatment strategies were reviewed. Predictors of poor clinical outcomes were analyzed. The primary outcomes were in-hospital mortality and the need for ICU admission. The secondary outcome was composite in-hospital mortality and/or ICU admission. RESULTS: One hundred nine patients (male/female: 63/46, mean age: 48.4 ± 12.4 years) were included in the study. Acute kidney injury (AKI) developed in 46 (42.2%) patients, and 4 (3.7%) of the patients required renal replacement therapy (RRT). A total of 22 (20.2%) patients were admitted in the ICU, and 19 (17.4%) patients required invasive mechanical ventilation. 14 (12.8%) of the patients died. Patients who were admitted in the ICU were significantly older (age over 60 years) (38.1% vs 14.9%, p = 0.016). 23 (21.1%) patients reached to composite outcome and these patients were significantly older (age over 60 years) (39.1% vs. 13.9%; p = 0.004), and had lower serum albumin (3.4 g/dl [2.9-3.8] vs. 3.8 g/dl [3.5-4.1], p = 0.002), higher serum ferritin (679 µg/L [184-2260] vs. 331 µg/L [128-839], p = 0.048), and lower lymphocyte counts (700/µl [460-950] vs. 860 /µl [545-1385], p = 0.018). Multivariable analysis identified presence of ischemic heart disease and initial serum creatinine levels as independent risk factors for mortality, whereas age over 60 years and initial serum creatinine levels were independently associated with ICU admission. On analysis for predicting secondary outcome, age above 60 and initial lymphocyte count were found to be independent variables in multivariable analysis. CONCLUSION: Over the age of 60, ischemic heart disease, lymphopenia, poor graft function were independent risk factors for severe COVID-19 in this patient group. Whereas presence of ischemic heart disease and poor graft function were independently associated with mortality.

Effect of Lavender Aromatherapy On Arteriovenous Fistula Puncture Pain and the Level of State and Trait Anxiety in Hemodialysis Patients: A Randomized Controlled Trial.

BACKGROUND: Patients undergoing haemodialysis suffer from anxiety and pain due to the insertion of haemodialysis needles, estimated totally 320 times per year. AIMS: This randomized controlled trial was conducted in order to determine the effect of lavender aromatherapy, performed through inhalation for five minutes three times a week, on pain and anxiety experienced by patients undergoing haemodialysis after needle insertion into a fistula. DESIGN: Patient information form, Numeric Rating Scale (NRS) and State-Trait Anxiety Inventory (STAI) were applied to patients in the intervention group at the first follow up (the first interview before aromatherapy administration). SETTINGS: The researcher completed the data collection forms by conducting face-to-face interview with the patients and applied lavender oil to all the patients in the intervention group at the last hour of every dialysis session. PARTICIPANTS/SUBJECTS: Totally three sessions were carried out as three times for one week in accordance with protocol of aromatherapy inhalation. METHODS: It took 5 minutes to complete each session. Olive oil was applied to the placebo group since it has no therapeutic value. At the end of the application, NRS and STAI were applied to the patients again. RESULTS: The use of lavander aromatherapy significantly reduced the scores of arteriovenous fistula (AVF) puncture-related pain, which was 6.24±1.24 in the first session, to 3.56±1.28 in the second session (p=0.002). It was found that scores of STAI obtained at the first follow-up) were higher in the intervention group than the placebo group but the difference between them was not statistically significant (p>0.05). The STAI mean scores of the patients in the intervention group after aromatherapy were 39.12±6.71 in the state anxiety subscale and30.04±1.39 in the trait anxiety subscale. All subscale scores of STAI decreased significantly in the intervention group in the second follow-up compared to the first follow-up (p<0.05). CONCLUSIONS: Lavender inhalation could be used as a safe, simple, and low-cost method in nursing care if it is proved to be effective in reducing anxiety and relieving pain after needle insertion into a fistula in patients undergoing haemodialysis.

Impact of hospital-acquired acute kidney injury on Covid-19 outcomes in patients with and without chronic kidney disease: a multicenter retrospective cohort study

Background/aim: Hospital-acquired acute kidney injury (HA-AKI) may commonly develop in Covid-19 patients and is expected to have higher mortality. There is little comparative data investigating the effect of HA-AKI on mortality of chronic kidney disease (CKD) patients and a control group of general population suffering from Covid-19. Materials and methods: HA-AKI development was assessed in a group of stage 3­5 CKD patients and control group without CKD among adult patients hospitalized for Covid-19. The role of AKI development on the outcome (in-hospital mortality and admission to the intensive care unit [ICU]) of patients with and without CKD was compared. Results: Among 621 hospitalized patients (age 60 [IQR: 47­73]), women: 44.1%), AKI developed in 32.5% of the patients, as stage 1 in 84.2%, stage 2 in 8.4%, and stage 3 in 7.4%. AKI developed in 48.0 % of CKD patients, whereas it developed in 17.6% of patients without CKD. CKD patients with HA-AKI had the highest mortality rate of 41.1% compared to 14.3% of patients with HA-AKI but no CKD (p < 0.001). However, patients with AKI+non-CKD had similar rates of ICU admission, mechanical ventilation, and death rate to patients with CKD without AKI. Adjusted mortality risks of the AKI+non-CKD group (HR: 9.0, 95% CI: 1.9­44.2) and AKI+CKD group (HR: 7.9, 95% CI: 1.9­33.3) were significantly higher than that of the non-AKI+non-CKD group. Conclusion: AKI frequently develops in hospitalized patients due to Covid-19 and is associated with high mortality. HA-AKI has worse outcomes whether it develops in patients with or without CKD, but the worst outcome was seen in AKI+CKD patients.

Determinants of mortality in a large group of hemodialysis patients hospitalized for COVID-19.

BACKGROUND: Maintenance hemodialysis (MHD) patients are at increased risk for coronavirus disease 2019 (COVID-19). The aim of this study was to describe clinical, laboratory, and radiologic characteristics and determinants of mortality in a large group of MHD patients hospitalized for COVID-19. METHODS: This multicenter, retrospective, observational study collected data from 47 nephrology clinics in Turkey. Baseline clinical, laboratory and radiological characteristics, and COVID-19 treatments during hospitalization, need for intensive care and mechanical ventilation were recorded. The main study outcome was in-hospital mortality and the determinants were analyzed by Cox regression survival analysis. RESULTS: Of 567 MHD patients, 93 (16.3%) patients died, 134 (23.6%) patients admitted to intensive care unit (ICU) and 91 of the ones in ICU (67.9%) needed mechanical ventilation. Patients who died were older (median age, 66 [57-74] vs. 63 [52-71] years, p = 0.019), had more congestive heart failure (34.9% versus 20.7%, p = 0.004) and chronic obstructive pulmonary disease (23.6% versus 12.7%, p = 0.008) compared to the discharged patients. Most patients (89.6%) had radiological manifestations compatible with COVID-19 pulmonary involvement. Median platelet (166 × 103 per mm3 versus 192 × 103 per mm3, p = 0.011) and lymphocyte (800 per mm3 versus 1000 per mm3, p < 0.001) counts and albumin levels (median, 3.2 g/dl versus 3.5 g/dl, p = 0.001) on admission were lower in patients who died. Age (HR: 1.022 [95% CI, 1.003-1.041], p = 0.025), severe-critical disease clinical presentation at the time of diagnosis (HR: 6.223 [95% CI, 2.168-17.863], p < 0.001), presence of congestive heart failure (HR: 2.247 [95% CI, 1.228-4.111], p = 0.009), ferritin levels on admission (HR; 1.057 [95% CI, 1.006-1.111], p = 0.028), elevation of aspartate aminotransferase (AST) (HR; 3.909 [95% CI, 2.143-7.132], p < 0.001) and low platelet count (< 150 × 103 per mm3) during hospitalization (HR; 1.864 [95% CI, 1.025-3.390], p = 0.041) were risk factors for mortality. CONCLUSION: Hospitalized MHD patients with COVID-19 had a high mortality rate. Older age, presence of heart failure, clinical severity of the disease at presentation, ferritin level on admission, decrease in platelet count and increase in AST level during hospitalization may be used to predict the mortality risk of these patients.

Soluble vascular endothelial growth factor receptor-1 as a novel marker of arteriovenous fistula stenosis in hemodialysis patients.

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 < 0.001]. According to body mass index (BMI) categories, obese patients (BMI > 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 (<6093.07 pg/mL) had longer cumulative stenosis-free survival than patients with sVEGFR-1 levels above the median value (P < 0.001). DISCUSSION: Increased levels of sVEGFR-1 and obesity were found to be associated with AVF stenosis in hemodialysis patients.

A rare complication following internal jugular vein catheterization to malposition: acute Budd Chiari syndrome.

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.

The effect of sodium exchange and dialytic biochemical parameters on blood pressure, arterial stiffness, and endothelial functions in patients with peritoneal dialysis.

OBJECTIVE: 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. METHOD: 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. RESULTS: 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). DISCUSSION AND CONCLUSION: 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.

Hypoxia-inducible factors in arteriovenous fistula maturation: A prospective cohort study.

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-2α) 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-2α measurement. HIF-2α levels were measured by the ELISA method. RESULTS: Compared with healthy subjects, ESKD patients had a significantly higher level of HIF-2α. [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-2α 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-2α independently predicted AVF maturation. The ROC curve analysis showed that HIF-2α > 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-α levels were higher in ESKD patients than healthy subjects. HIF-2-α could be a marker of AVF maturation failure.

Association of OSR-1 With Vascular Dysfunction and Hypertension in Polycystic Kidney Disease.

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.

Successful treatment of tubulointerstitial nephritis in immunoglobulin G4-related disease with rituximab: A case report.

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.

Systemic Succinate, Hypoxia-Inducible Factor-1 Alpha, and IL-1ß Gene Expression in Autosomal Dominant Polycystic Kidney Disease with and without Hypertension.

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-1ß 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-1ßgene 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-1ß geneexpression by using quantitative real-time PCR. RESULTS: There were significant differences in whole blood HIF-1α, IL-1ß geneexpression, and serumsuccinate levels between the ADPKD patients and the control subjects. Whole blood HIF-1αgene expression, IL-1ß 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-1ß 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-1ß 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.

Multiple urinary tract infections are associated with genotype and phenotype in adult polycystic kidney disease.

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 cm3) 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.

Dysmetabolic markers predict outcomes in autosomal dominant polycystic kidney disease.

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-1ß, IL-6, TNF-α 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-1ß (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.

Brown tumor of the thoracic spine presenting with paraplegia in a patient with peritoneal dialysis.

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.