Experience with cascade screening: A comprehensive family pedigree analysis of two index patients with Fabry disease.

The wide range of clinical symptoms observed in patients with Fabry disease (FD) often leads to delays in diagnosis and initiation of treatment. Delayed initiation of therapy may result in end-organ damage, such as chronic renal failure, hypertrophic cardiomyopathy, and stroke. Although some tools are available to identify undiagnosed patients, new comprehensive screening methods are needed. In this study, the outcomes of the cascade screening applied to three index cases with FD from 2 familes were investigated. In the pedigree analysis, 280 individuals were included; out of them, 131 individuals underwent genetic testing and cascade screening for FD. During the screening program, a total of 45 individuals were diagnosed, with a diagnostic ratio of 1:15. The average age at diagnosis for all individuals was 30.9 ± 17.7 years, and %25 were pediatric cases (mean age 9.5 ± 5.9 years). Thirty affected relatives were diagnosed from the two index cases in Family 1 and 15 individuals were diagnosed from one index case in Family 2. There were 13 consanguineous marriages observed among 2 pedigres, in two both spouses  were affected, leading to two homozygous affected daughters in one couple. In regions where there is a high prevalence of consanguineous marriages, implementing the cascade screening approach to identify all individuals at risk can be beneficial for patients with FD, specifically women and children.

Vascular access route venture of the chronic hemodialysis patient: A prospective cohort study.

OBJECTIVES: This study aimed to collect evidence to improve the arteriovenous fistula practice by investigating vascular access routes and by identifying the factors influencing the preferred types of vascular access routes for the first-time hemodialysis in our center. METHODS: We performed an epidemiological, prospective, cohort study. The study included 308 patients, who underwent hemodialysis for the first time between March 2023 and August 2023 in our hemodialysis center. We evaluated biochemical parameters, preferred vascular access routes for the first-time hemodialysis, planned/emergency hemodialysis status, the qualifications of the healthcare provider, who inserted the central venous catheter, if applicable, the presence of hypervolemia, anticoagulant use, nephrology follow-up findings, and in-hospital mortality in all patients and in those, who continued with chronic hemodialysis. RESULTS: The number of patients, who continued with chronic hemodialysis, was 167 (54.2%) and a temporary internal jugular central venous catheter was the most commonly preferred vascular access route for the first-time hemodialysis (47.3%). A central venous catheter was most commonly inserted by a nephrologist (53.7%) in chronic hemodialysis patients. Of the patients continuing with chronic hemodialysis, 45.5% were followed up in the nephrology outpatient clinic, 9.6% initiated hemodialysis on a planned basis, and 8.4% initiated hemodialysis with an arteriovenous fistula. A temporary internal jugular central venous catheter was commonly preferred when patients were followed up in the nephrology clinic and when the insertion was performed by a nephrologist; a transient femoral central venous catheter was commonly preferred in case of hypervolemia (p < .001, p < .001, and p = .028, respectively). Age, gender, etiology, anticoagulant use, or biochemical test results did not act on the selection of the access site for the insertion of central venous catheter at the time of the first hemodialysis treatment. The access site for central venous catheter was not associated with in-hospital mortality (p = .644). In the overall patient group, the in-hospital mortality was significantly low in patients followed up in the nephrology clinic (p = .014). CONCLUSION: The use of pre-emptive arteriovenous fistula for the first hemodialysis treatment occurs much less commonly than expected. Hemodialysis initiation rates with pre-emptive arteriovenous fistula lag behind nephrology outpatient follow-up rates.

Characteristics and Survival Results of Peritoneal Dialysis Patients Suffering from COVID-19 in Turkey: A Multicenter National Study.

INTRODUCTION: We aimed to study the characteristics of peritoneal dialysis (PD) patients with coronavirus disease-19 (COVID-19), determine the short-term mortality and other medical complications, and delineate the factors associated with COVID-19 outcome. METHODS: In this multicenter national study, we included PD patients with confirmed COVID-19 from 27 centers. The baseline demographic, clinical, laboratory, and radiological data and outcomes at the end of the first month were recorded. RESULTS: We enrolled 142 COVID-19 patients (median age: 52 years). 58.2% of patients had mild disease at diagnosis. Lung involvement was detected in 60.8% of patients. Eighty-three (58.4%) patients were hospitalized, 31 (21.8%) patients were admitted to intensive care unit and 24 needed mechanical ventilation. Fifteen (10.5%) patients were switched to hemodialysis and hemodiafiltration was performed for four (2.8%) patients. Persisting pulmonary symptoms (n = 27), lower respiratory system infection (n = 12), rehospitalization for any reason (n = 24), malnutrition (n = 6), hypervolemia (n = 13), peritonitis (n = 7), ultrafiltration failure (n = 7), and in PD modality change (n = 8) were reported in survivors. Twenty-six patients (18.31%) died in the first month of diagnosis. The non-survivor group was older, comorbidities were more prevalent. Fever, dyspnea, cough, serious-vital disease at presentation, bilateral pulmonary involvement, and pleural effusion were more frequent among non-survivors. Age (OR: 1.102; 95% CI: 1.032-1.117; p: 0.004), moderate-severe clinical disease at presentation (OR: 26.825; 95% CI: 4.578-157.172; p < 0.001), and baseline CRP (OR: 1.008; 95% CI; 1,000-1.016; p: 0.040) were associated with first-month mortality in multivariate analysis. DISCUSSION/CONCLUSIONS: Early mortality rate and medical complications are quite high in PD patients with COVID-19. Age, clinical severity of COVID-19, and baseline CRP level are the independent parameters associated with mortality.

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.

Medium Cut-Off Membrane Can Be a New Treatment Tool in Amanita phalloides Poisoning.

Mushroom poisoning is a common health problem that can be seen seasonally and geographically. Most mushroom poisoning requiring treatment worldwide is due to Amanita phalloides. Although liver failure and kidney injury are frequent, poisoning can also lead to more serious clinical situations, such as shock, pancreatitis, encephalopathic coma, cardiac failure, disseminated intravascular coagulation, and multiple organ dysfunction syndrome, and may cause death. In addition, when standard treatment approaches fail, extracorporeal treatment methods are often used. We report 2 cases in which hemodialysis with medium cut-off membrane was performed. We observed an improvement in liver and kidney function in both of our cases. The first case recovered, but the second case proved fatal owing to Acinetobacter sepsis, despite an improvement in renal function. Medium cut-off membrane hemodialysis may be an alternative option in the treatment of Amanita phalloides poisoning.

Pregnancy and its outcomes in hemodialysis patients in Turkey.

BACKGROUND/AIM: This study aimed to investigate pregnancy frequency and evaluate the factors affecting live births in hemodialysis (HD) patients. MATERIALS AND METHODS: Female HD patients whose pregnancy was retrospectively reported between January 1, 2014, and December 31, 2019. The duration of HD, primary disease, whether the pregnancy resulted in abortion, stillbirth, or live birth, whether the HD duration was prolonged after diagnosing the pregnancy and whether it accompanied preeclampsia were recorded. RESULTS: In this study, we reached 9038 HD female patients? data in the study. A total of 235 pregnancies were detected in 145 patients. The mean age was 35.42 (35 ± 7.4) years. The mean age at first gestation was 30.8 ± 6.5 years. The average birth week was 32 (28 - 36) weeks. 53.8% (no = 78) of the patients had live birth, 51.7% (no = 70) had at least one abortion in the first 20 weeks, and 13.1% (no = 19) had at least one stillbirth after 20 weeks. The rate of patients' increased numbers of dialysis sessions during pregnancy was 71.7%. The abortion rate was 22.4% in those with increased HD sessions, whereas 79.3% in those not increased HD sessions (p < 0.001). Live birth frequency was 67.2% in the increased HD sessions group and 3.4% in those who did not differ in HD sessions (p < 0.001). CONCLUSION: For the first time, we reported pregnancy outcomes in HD female patients, covering all regions of Turkey. It has been observed that; increasing the number of HD sessions in dialysis patients will decrease fetal and maternal complications and increase live birth rates.

ß2-Microglobulin, Neutrophil Gelatinase-Associated Lipocalin, and Endocan Values in Evaluating Renal Functions in Patients with ß-Thalassemia Major.

Chronic anemia, transfusion-associated iron deposition, and chelating agents lead to renal impairment in ß-thalassemia (ß-thal) patients. The present study aimed to determine the most reliable and practical method in assessing and predicting renal injury in ß-thal major (ß-TM) patients. Therefore, we assessed the predictive values of urine ß2-microglobulin (ß2-MG) and neutrophil gelatinase-associated lipocalin (NGAL) levels, their ratios to urine creatinine, and serum endocan level. Sixty ß-TM patients and 30 healthy controls were included. Renal functions of the patients and controls were evaluated by means of urine protein/creatinine ratio, urine ß2-MG, urine NGAL, and serum endocan level. The ß-TM and control groups were comparable in terms of the demographic characteristics. Of the ß-TM patients, 26.7% had glomerular hyperfiltration and 41.7% had proteinuria. Compared with the control group, the ß-TM group had significantly higher levels of urine protein/creatinine, urine ß2-MG, urine ß2-MG/creatinine, urine NGAL, urine NGAL/creatinine, and serum endocan. These parameters did not differ between the chelating agent subgroups in the patient group. Urine ß2-MG/creatinine and NGAL/creatinine ratios were the parameters with high specificity in predicting proteinuria. There were significant correlations of urine ß2-MG, urine NGAL, and serum endocan levels with serum ferritin concentration. Urine ß2-MG/creatinine, NGAL/creatinine, and protein/creatinine ratios were correlated with each other in the patient group. Positive correlations of urine ß2-MG, urine NGAL, and serum endocan levels with serum ferritin concentration indicated that iron deposition was associated with endothelial damage and renal injury.

Screening Fabry's disease in chronic kidney disease patients not on dialysis: a multicenter study.

OBJECTIVES: Fabry's disease is an X-linked inherited, rare, progressive, lysosomal storage disorder, affecting multiple organs due to the deficient activity of α-galactosidase A (α-Gal A) enzyme. The prevalence has been reported to be 0.15-1% in hemodialysis patients; however, the information on the prevalence in chronic kidney disease not on dialysis is lacking. This study aimed to determine the prevalence of Fabry's disease in chronic kidney disease. METHODS: The patients older than 18 years, enclosing KDIGO 2012 chronic kidney disease definitions, not on dialysis, were enrolled. Dried blood spots on Guthrie papers were used to analyze α-Gal A enzyme and genetic analysis was performed in individuals with enzyme activity ≤1.2 µmol/L/h. RESULTS: A total of 1453 chronic kidney disease patients not on dialysis from seven clinics in Turkey were screened. The mean age of the study population was 59.3 ± 15.9 years. 45.6% of patients were female. The creatinine clearance of 77.3% of patients was below 60 mL/min/1.73 m2, 8.4% had proteinuria, and 2.5% had isolated microscopic hematuria. The mean value of patients' α-Gal A enzyme was detected as 2.93 ± 1.92 µmol/L/h. 152 patients had low levels of α-Gal A enzyme activity (≤1.2 µmol/L/h). In mutation analysis, A143T and D313Y variants were disclosed in three male patients. The prevalence of Fabry's disease in chronic kidney disease not on dialysis was found to be 0.2% (0.4% in male, 0.0% in female). CONCLUSION: Fabry's disease should be considered in the differential diagnosis of chronic kidney disease with unknown etiology even in the absence of symptoms and signs suggestive of Fabry's disease.

MCP-1 and soluble TWEAK levels are independently associated with coronary artery disease severity in patients with chronic kidney disease.

PURPOSE: Patients diagnosed with chronic kidney disease (CKD) have a greater rate of cardiovascular mortality when compared with the general population. The soluble form of TNF-like weak inducer of apoptosis (TWEAK) and monocyte chemoattractan protein 1 (MCP-1) play important roles in cellular proliferation, migration and apoptosis. The current study aimed to analyze whether soluble TWEAK (sTWEAK) and MCP-1 levels are associated with the severity of coronary arterial disease (CAD) in CKD patients. METHODS: Ninety-seven patients diagnosed with CKD stages 2-3 according to their estimated glomerular filtration rate and the presence of kidney injury were included in the study. Plasma sTWEAK and MCP-1 concentrations were determined using commercially available ELISA kits. Coronary angiographies were performed through femoral artery access using the Judkins technique. RESULTS: Correlation analysis of sTWEAK and Gensini scores showed significant association (p < 0.01, r(2) = 0.287). Also significant correlation has been found in MCP-1 levels and Gensini scores (p < 0.01, r(2) = 0.414). When patients were divided into two groups with a limit of 17 according to their Gensini score, sTWEAK levels indicated a statistically significant difference (p < 0.01). CONCLUSIONS: Our findings support a relationship between sTWEAK and MCP-1 levels and CAD in CKD stages 2-3 patients.

Evaluation of bone marrow findings in hemodialysis patients with erythropoietin-resistant anemia.

INTRODUCTION: Treatment with erythropoietin-stimulating agents (ESAs) is widely used in anemia of chronic kidney disease (CKD). Acquired ESA resistance is an important problem. The aim of this study is to examine the bone marrow findings in hemodialysis patients with ESA-resistant anemia. METHODS: The data of 210 patients with acquired ESA resistance were reviewed retrospectively. The patients were divided into groups according to having diagnosis of dysplasia and hematological disease, and survival analysis was performed. RESULTS: A total of 26 patients were included in the study. While dysplasia was present in 10 (38.5%) patients, five of them were diagnosed hematologically. When survival analysis was performed between those with and without a hematological diagnosis, a difference in survival was observed against the group with the diagnosis (24.4 vs. 72 months, p = 0.045). CONCLUSION: Unresponsiveness to ESA treatment in CKD patients or a decrease in one of the other cell lines along with hemoglobin, it would be appropriate to perform early bone marrow examination.

Real-time elastography for distinguishing angiomyolipoma from renal cell carcinoma: preliminary observations.

OBJECTIVE: The objective of this study was to determine the diagnostic performance of sonoelastography for differentiating angiomyolipomas from renal cell carcinomas. SUBJECTS AND METHODS: Twenty-eight angiomyolipomas and 19 renal cell carcinomas were prospectively examined with real-time elastography. Lesions were classified according to four elastographic patterns on the basis of the distribution of the blue area (representing no strain and hardest tissue component). The elasticity patterns and the strain ratios of the angiomyolipomas and renal cell carcinomas were evaluated independently by two observers. Diagnostic performance and interobserver agreement were analyzed. RESULTS: All angiomyolipomas were classified as having a high-strain elastographic pattern (blue areas in < 50% of lesion, considered type 1 or type 2) by both radiologists, whereas 18 of 19 renal cell carcinomas were classified as having a low-strain elastographic pattern (blue areas in ≥ 50% of lesion, considered type 3 or 4) by both radiologists. The respective mean strain ratios measured by two radiologists were 0.15 ± 0.06 and 0.18 ± 0.09 for the angiomyolipomas and 0.64 ± 0.15 and 0.63 ± 0.19 for the renal cell carcinomas. There were significant differences between the elasticity patterns and strain ratios for angiomyolipomas and renal cell carcinomas (p < 0.001). Interobserver agreement was excellent for elasticity patterns and strain ratios, with a weighted kappa coefficient of 0.96 and an intraclass correlation coefficient score of 0.95. CONCLUSION: Our results show that real-time elastography may be useful in differentiating angiomyolipomas from renal cell carcinomas, by use of both elasticity patterns and strain ratios.

The relationship between serum fetuin-A, cystatin-C levels, and microalbuminuria in patients with metabolic syndrome.

BACKGROUND: The metabolic syndrome, syndrome X, is a group of metabolic disorders in which insulin resistance plays a pivotal role. The MS is an important risk factor for subsequent development of type 2 diabetes and cardiovascular disease. Fetuin-A is a liver derived blood protein that acts as effective inhibitor of soft tissue calcification. Cystatin C is a useful marker in measuring glomerular filtration rate. Moreover, recently it has been suggested that cystatin C may be a potential biomarker for detecting microalbuminuria. Microalbuminuria (MA) is a strong indicator of morbidity related to cardiovascular disorders, and is currently considered a novel diagnostic criterion for MS. It has been also demonstrated that the increased serum fetuin-A levels is associated with several parameters of MS. In this study, we attempted to investigate the relationship between serum fetuin-A, cystatin-C levels and microalbuminuria in patients with MS. METHODS: A total of 50 patients with MS and 25 control were included in this study. We defined MS by the NCEP criteria among nondiabetic outpatients. Patients with MS were further divided into two groups based on MA status. Overall 25 of the participants with MS did not have MA (group I), while the remaining 25 had MA (group II). None of the subjects in the healthy control group (group III) had laboratory findings supporting the presence of MA. The serum fetuin-A and cystatin-C levels were measured using ELISA. RESULTS: Age, distributions of sex, BP and LDL cholesterol levels were similar among all groups. BMI, Waist/hip ratio, FBG, HOMA-IR, total cholesterol, trigliserid, CRP levels were significantly higher in group I and group II compared to control. In group II, the cystatin-C and fetuin levels were higher than control. While the cystatin-C levels were higher in group II compared to group I, the fetuin levels did not different. Morever, the fetuin A and cystatin-C concentrations were positively correlated with microalbuminuria (r = 0.26, p = 0.02; r = 0.50, p = 0.0001, respectively). CONCLUSION: In our study, we found that MS patients with microalbuminuria had high levels of fetuin-A and cystatin-C. In conclusion, we suggest that determination of fetuin-A and cystatin C levels could be useful marker as an early indicator of renal injury in patients with MS.

Bone morphogenetic protein-7 and disease progression in renal amyloidosis patients.

INTRODUCTION: Chronic kidney disease (CKD) is an important health care problem with increasing incidence. Early diagnosis, recognition and interventions to avoid the disease progression have great value. Even some risk factors for disease progression have been described; there are still some dark spots. Transforming growth factors (TGFs), particularly bone morphogenetic protein-7 (BMP7) take place in renal fibrosis. Our study aimed to evaluate the association between serum BMP7 levels and the progression of CKD. MATERIALS AND METHODS: Our study has been conducted between January 2008 and December 2010. Decrease in GFR by 10%, doubling of serum creatinine and need for renal replacement therapy have been set as progression end-points. Totally 93 patients (48 female, 45 male) have been included. Baseline and end of follow-up BMP7 levels have been measured. RESULTS: At the end of the follow-up, 46 of 93 patients have been considered as having progressive CKD. Higher levels of serum BMP7 levels have been found to be associated in progressive kidney disease. DISCUSSION: Our results showed that BMP7 levels were higher in patients with progressive CKD, and also BMP7 to be associated with CKD progression. But this relationship was not statistically significant. In patients with progressive CKD, higher levels of proteinuria and blood pressure have been previously described. The effect of BMP7 on kidneys is not still clear, it is hypothesized that TGF-beta1 inhibition may alter renal fibrosis.

Time to Change Our Viewpoints to Assess Renal Risks in Patients with Solitary Kidneys beyond Traditional Approaches?

Solitary functioning kidney (SFK) can be defined as the absence or hypofunction of a kidney due to acquired or congenital reasons. A congenital solitary functioning kidney (cSFK) is more common than is an acquired one (aSFK) and is characterized by the anatomical absence (agenesis) or hypofunction (hypoplasia; hypodysplasia) of one kidney from birth. Among the acquired causes, the most important is nephrectomy (Nx) (due to the donor, trauma or mass resection). Patients with SFK are at risk for the development of chronic kidney disease (CKD) in the long term. This risk potential is also significantly affected by hypertension. The relationship between hypertension and subclinical chronic inflammation is a connection that has not yet been fully clarified pathogenetically, but there are many studies highlighting this association. In recent years, studies examining different fibrosis and inflammation biomarkers in terms of the evaluation and prediction of renal risks have become increasingly popular in the literature. Oxidative stress is known to play an important role in homocysteine-induced endothelial dysfunction and has been associated with hypertension. In our study, we aimed to investigate the relationship between ambulatory blood pressure monitoring (ABPM) and urinary/serum fibrosis and inflammatory markers in patients with SFK. We prospectively investigated the relationship between ABPM results and soluble urokinase plasminogen activator receptor (suPAR), procollagen type III N-terminal peptide (PIIINP), homocysteine and other variables in 85 patients with SFK and compared them between cSFK and aSFK groups. In the etiology of SFK, a congenital or acquired origin may differ in terms of the significance of biomarkers. In particular, the serum homocysteine level may be associated with different clinical outcomes in patients with cSFK and aSFK.

Evaluation of Outcomes of Peritoneal Dialysis Patients in the Post-COVID-19 Period: A National Multicenter Case-Control Study from Turkey.

INTRODUCTION: There are not enough data on the post-CO-VID-19 period for peritoneal dialysis (PD) patients affected from COVID-19. We aimed to compare the clinical and laboratory data of PD patients after COVID-19 with a control PD group. METHODS: This study, supported by the Turkish Society of Nephrology, is a national, multicenter retrospective case-control study involving adult PD patients with confirmed COVID-19, using data collected from April 21, 2021, to June 11, 2021. A control PD group was also formed from each PD unit, from patients with similar characteristics but without COVID-19. Patients in the active period of COVID-19 were not included. Data at the end of the first month and within the first 90 days, as well as other outcomes, including mortality, were investigated. RESULTS: A total of 223 patients (COVID-19 group: 113, control group: 110) from 27 centers were included. The duration of PD in both groups was similar (median [IQR]: 3.0 [1.88-6.0] years and 3.0 [2.0-5.6]), but the patient age in the COVID-19 group was lower than that in the control group (50 [IQR: 40-57] years and 56 [IQR: 46-64] years, p < 0.001). PD characteristics and baseline laboratory data were similar in both groups, except serum albumin and hemoglobin levels on day 28, which were significantly lower in the COVID-19 group. In the COVID-19 group, respiratory symptoms, rehospitalization, lower respiratory tract infection, change in PD modality, UF failure, and hypervolemia were significantly higher on the 28th day. There was no significant difference in laboratory parameters at day 90. Only 1 (0.9%) patient in the COVID-19 group died within 90 days. There was no death in the control group. Respiratory symptoms, malnutrition, and hypervolemia were significantly higher at day 90 in the COVID-19 group. CONCLUSION: Mortality in the first 90 days after COVID-19 in PD patients with COVID-19 was not different from the control PD group. However, some patients continued to experience significant problems, especially respiratory system symptoms, malnutrition, and hypervolemia.

Coexistent findings of renal glomerular disease with Hashimoto's thyroiditis.

AIM AND BACKGROUND: Hashimoto's thyroiditis (HT) is a common autoimmune thyroid disease with a female preponderance. Renal involvement in HT is not uncommon. In the present study, we aimed to define the frequency and characteristics of the glomerular diseases associated with HT and further the understanding of any common pathogenesis between HT and glomerular disease. MATERIALS AND METHODS: We reviewed retrospectively 28 patients with HT who were referred to our Department because of unexplained haematuria, proteinuria or renal impairment from 2007 to 2011. Routine laboratory investigations including blood count, serum biochemistry, urinalysis and 24-h urinary protein excretion were performed on all patients. Renal biopsy was performed in 20 patients with HT, and the specimens were examined by light microscopy and immunofluorescence staining. RESULTS: We detected four cases of focal segmental glomerulosclerosis (FSGS), four membranous glomerulonephritis (MGN), two minimal-change disease (MCD), three immunoglobulin A nephritis (IgAN), three chronic glomerulonephritis (CGN) and one amyloidosis. In three patients, the renal biopsy findings were nonspecific. Daily urinary protein excretion and glomerular filtration rates were found to be independent of the level of thyroid hormone and thyroid-specific autoantibodies. CONCLUSION: Glomerular pathologies associated with HT are similar to those in the general population, the most common lesions being MGN, FSGS and IgA nephritis.

Is conventional hemodialysis enough to manage carbamazepine intoxication?.

Hemodialysis (HD) is a technique that helps in the management of drug intoxications/overdoses - the most important point being the protein-binding rate of the molecules which have to be cleared. In cases where the drug is not bound to the proteins, conventional HD may be effective enough, but if the drug is highly bound to proteins then HD may be insufficient to be effective. Hemoperfusion and albumin dialysis are therefore appropriate alternatives. Herein we report a case of carbamazepine intoxication where conventional HD treatment was performed.

The efficacy of prophylactic antibiotics administration prior to insertion of tunneled catheter in hemodialysis patients.

AIM AND BACKGROUND: Central venous catheter (CVC)-related blood stream infection is a major cause of morbidity and mortality in patients with end-stage renal diseases. However, CVCs are quite frequently required for vascular access in hemodialysis (HD) patients. Tunneled catheters (TCs) are widely used when a catheter is needed for a long period. However, long-term catheter survival is limited by TC-related infections. The purpose of this prospective study was to assess clinical outcomes of prophylactic antibiotics administration prior to insertion of TCs in HD patients. MATERIAL AND METHODS: Sixty uremic patients who required TC insertion due to vascular access failure were included in our study between April 2009 and April 2010. Patients were randomized into two groups: group I and group II. Group I received 1 g of cefazolin sodium intravenously 1 h prior to catheter insertion. Group II received equal amount of saline intravenously 1 h prior to catheter insertion. The primary end points of the study were catheter loss, hospitalization, or mortality due to catheter-related infections (CRIs). The secondary end points included exit-site infection (not requiring hospitalization), tunnel infections (not requiring catheter removal), and bacteremia. RESULTS: During the follow-up period, one patient in group I and three patients in group II reached primary end point (p < 0.05). Catheter loss due to infection was higher in group II than in group I as 6 versus 3, respectively (p < 0.05). Catheter exit-site infections, which does not require hospitalization, have been considered as secondary end points and have been detected in four patients for 7 times in group I and in six patients for 10 times in group II (p < 0.05). Tunnel infection, which does not require removal of the catheter, has been detected in two patients for 3 times in group I and in five patients for 6 times in group II (p < 0.05). CONCLUSION: The prophylactic antibiotic use prior to TC insertion significantly reduced CRIs, bacteremia, and catheter loss.

Additive effectiveness of everolimus plus tamoxifen therapy in treatment of encapsulating peritoneal sclerosis.

Peritoneal dialysis (PD) is one of the commonly used choices of continuous renal replacement therapies. Peritoneal membrane is damaged by using solutions with lower biocompatibility, peritonitis episodes, and vintage of PD therapy. Encapsulating peritoneal sclerosis (EPS) is a rare complication of PD and is presented by progressive fibrosis of the peritoneum. Fibrous tissue entrapment of the intestine, leading to complete intestinal obstruction, is referred to as EPS, the most severe form of sclerosing peritonitis. EPS is irreversible fibrosis of the peritoneal membrane usually associated with high rates of morbidity and mortality. Preventive strategies are the best choice of treatment. Also there is no proven effective therapy for EPS; there are only small-sized trials. Herein we present a case of EPS who improved with everolimus plus tamoxifen therapy.