Comparison of homologous and heterologous inactivated and mRNA vaccination programme against SARS-CoV-2 in dialysis patients.

AIM: To evaluate the vaccine response and the effect of the booster dose on COVID-19 positivity in haemodialysis (HD) and peritoneal dialysis (PD) patients who received and did not receive BNT162b2 as a booster dose after two doses of CoronaVac. METHODS: The study included 80 PD and 163 HD patients, who had been administered two doses of the CoronaVac. Antibody levels were measured on Days 42 and 90 after the first dose. Measurements were repeated on Day 181 after the first dose in the patients that received two vaccine doses and on Day 28 after the third dose in those that also received the booster dose. Antibody levels below 50 AU/mL were considered negative. RESULTS: The seropositivity rate was similar in the HD and PD group on Days 42 and 90 (p = 0.212 and 0.720). All patients were seropositive in the booster group. The antibody level was lower in the patients that received CoronaVac as the booster compared to those administered BNT162b2 in HD and PD groups (p < 0.001 and 0.002). COVID-19 positivity was detected in 11 patients (7 = had not received the booster dose, 4 = had received third dose of CoronaVac). The multivariate analysis revealed that as age increased, COVID-19 positivity also increased (OR: 1.080, 95% CI: 1.017 - 1.146, p = 0.012), while booster dose administration decreased this positivity (OR: 0.113, 95% CI: 0.028 - 0.457, p = 0.002). CONCLUSION: Our results may indicate the need for additional vaccination doses in patients with HD and PD. Our findings indicate a higher antibody response in dialysis patients with heterologous BNT162b2 as a booster dose after two doses of CoronaVac compared to homologous CoronaVac.

Pre- and Post-Hemodialysis Cerebral Blood Flow Velocity in Patients With End-Stage Renal Disease.

BACKGROUND: This study assessed the consequences of hemodialysis (HD) on hemodynamic parameters of cerebral circulation by measuring middle cerebral artery blood flow velocities using transcranial Doppler ultrasound before and after a single dialysis session in patients with end-stage renal disease (ESRD). MATERIALS AND METHODS: Fifty clinically stable patients with ESRD undergoing HD and 40 healthy controls were recruited for the study. Blood pressure, heart rate, and body weight were measured. Transcranial Doppler ultrasound evaluations and blood analyses were performed immediately before and after a single dialysis session. RESULTS: The mean cerebral blood flow velocities (CBFVs) in the ESRD patients before HD was 65 ± 17 cm/second and did not differ from that of the normal controls (64 ± 14 cm/s) ( P = 0.735). The postdialysis CBFV also did not differ from that of the controls ( P = 0.054). CONCLUSION: Compensatory cerebral autoregulation and chronic adjustment to therapy may be the reason for the nondeviation of the CBFV values from normality observed in both sessions.

Relationship between Health Literacy and Frailty in Older Adults with Chronic Kidney Disease.

BACKGROUND: Chronic kidney disease (CKD) is associated with an increased risk of frailty, morbidity, and mortality in older adults. Limited health literacy (HL) is a condition that can cause frailty in CKD. Frailty leads to a decreased resistance to stress situations caused by activities of daily living and increased morbidity and mortality. The aim of this study was to investigate the relationship between HL and frailty in older adults with stage 4-5 CKD. METHODS: A total of 109 patients with stage 4-5CKD were included. All patients underwent a comprehensive geriatric assessment. Health literacy was assessed by the Turkish version of the European Health Literacy Questionnaire. Frailty status was measured using the Fried Frailty Index. RESULTS: The median age was 72 (68-80) years and 72 patients (66.1%) had lowHL. Forty-six patients (42.2%) were frail, and frailty was more common in the low HL group. CONCLUSION: The present study demonstrated that low HL level was common in geriatric patients with stage 4-5 CKD and was associated with frailty, as well as a decrease in daily and instrumental life activities, decrease in acceptance of illness scale, and compliance with medical treatment.

Compliance of NKF KDOQI 2020 nutrition guideline recommendations with other guideline recommendations and protein energy wasting criteria in hemodialysis patients.

INTRODUCTION: Nutrition in hemodialysis patients is important in decreasing complications, improving quality of life, and preventing of malnutrition. Recommendations of the guidelines are taken into consideration while prescribing a nutrition therapy plan for patients. However, the recommendations may differ between the guidelines. It was aimed to compare the newly published National Kidney Foundation Kidney Disease Outcomes Quality Initiative (NKF KDOQI) guideline with previous reported two guideline recommendations and protein energy wasting criteria in this study. MATERIALS AND METHODS: Fifty-five maintenance hemodialysis patients between the ages of 18-65 were included in the study. Daily energy intake and daily protein intake of these patients were evaluated by three different nutrition guidelines; NKF KDOQI-2000, 2020 and European Best Practice Guidelines-2007. In addition, protein energy wasting was determined by using anthropometric measurements, biochemical findings and food intake of the patients. RESULTS: When the inadequacy rate in dietary daily energy intake and daily protein intake of the patients evaluated by NKF KDOQI-2000 recommendations, it was found to be higher than the rates in other two recommendations (P<0.05). Based on criteria, protein energy wasting was detected in 29.1% of the patients. While the NKF KDOQI-2020 daily energy intake recommendation was not consistent with other guideline recommendations, it seems highly compatible with protein energy wasting recommendations such as albumin, body mass index, mid-upper arm circumference, energy, and protein intake. While NKF KDOQI-2020 daily protein intake recommendation complies with European Best Practice Guidelines-2007 recommendations, the level of agreement with protein energy wasting criteria is very low. CONCLUSION: Inadequate protein intake is still an ongoing problem in hemodialysis patients. NKF KDOQI-2020 guidelines provide a more suitable and applicable daily energy intake recommendation for patients compared to the previous guidelines.

The Relationship Between Myostatin, Inflammatory Markers, and Sarcopenia in Patients With Chronic Kidney Disease.

OBJECTIVE: To determine the prevalence of sarcopenia in patients with chronic kidney disease (CKD), investigate the relationship of the serum myostatin level with sarcopenia and inflammatory markers. METHODS: The study was conducted with four patient groups: renal transplantation (TX), stage 3-5 non-dialysis-dependent CKD (NDD-CKD), hemodialysis (HD), and peritoneal dialysis (PD). Laboratory parameters, serum myostatin, C-reactive protein, and interleukin-6 levels were studied. Body composition was estimated using a multifrequency bioimpedance analysis. Handgrip strength (HGS) was evaluated with a handgrip dynamometer. The HGS and appendicular skeletal muscle index measurements were used to determine sarcopenia presence. RESULTS: The study included 130 patients (72 [55%] male patients). The patient distribution in groups was as follows: 37 in HD, 28 in PD, 37 in renal TX, and 28 in NDD-CKD. The highest level of myostatin was measured in the HD group, and the lowest in the TX group (P < .001). The HGS measurement in the PD group was significantly lower than that in the TX group (P = .025). The myostatin was negatively correlated with HGS, albumin, estimated glomerular filtration rate, and Kt/Vurea. However, myostatin had no correlation with inflammatory markers or appendicular skeletal muscle index. Sarcopenia was present in 37 (29%) patients: 15 (40%) in the HD group, nine (32%) in NDD-CKD, seven (25%) in PD, and six (16%) in TX. When the patients with and without sarcopenia were compared, only myostatin was higher in the former (P = .045). As a result of multivariate analysis, myostatin was the only independent factor which predicts sarcopenia (odds ratio: 1.002, 95% confidence interval: 1.001-1.005, P = .048). CONCLUSION: To prevent devastating events associated with sarcopenia in patients with CKD, renal transplantation seems to be the best treatment solution. For the early recognition of sarcopenia, the measurement of the serum myostatin level may be a promising diagnostic approach.

Discontinuation of Eculizumab treatment after hematological remission in patients with atypical and drug-induced hemolytic uremic syndrome.

Introduction. The aim was to evaluate the effect of therapeutic plasma exchange (TPE) and eculizumab on hematological and renal survival in atypical hemolytic uremic syndrome (aHUS), and additionally, to examine the reliability of discontinuation of eculizumab treatment.Methods. This was an observational and retrospective study of 18 patients diagnosed with aHUS.Results. The median age of the study population was 30 (22-66) years. Four of 18 patients achieved hematological remission with the TPE alone. However, one patient died after three sessions of TPE. Eculizumab was used in 13 patients and no death was observed. One year after treatment, improved kidney function was observed in 2 of 3 (66%) patients for TPE and 5 of 9 (56%) patients for Eculizumab. We discontinued eculizumab treatment in 9 patients. One of the patients who had a C3 gene mutation experienced disease relapse after Eculizumab discontinuation. None of the patients who had drug associated aHUS developed disease relapse after Eculizumab discontinuation.Conclusion. Eculizumab treatment is a life-saving therapy in aHUS. Treatment discontinuation may be considered at least six months after hematologic remission in patients who had stable renal function or no expectancy for renal survival. Moreover, drug-associated cases seem to tend not to develop disease relapse in the long term.

Tuberculosis presenting as proliferative glomerulonephritis with monoclonal immunoglobulin deposits / Tuberculosis que se presenta como glomerulonefritis proliferativa con depósitos de inmunoglobulina monoclonal

Abstract Proliferative glomerulonephritis with monoclonal immunoglobulin deposits is defined as membranoproliferative glomerulonephritis like injury with monotypic Ig deposits restricted to a single light chain isotype.Here we present a patient who presented with hypocomplementemia and nephrotic syndrome, who was initially diagnosed with proliferative glomerulonephritis with monoclonal immunoglobulin deposits. He developed disseminated tuberculosis after a brief course of immunosuppression. Successful treatment of tuberculosis resulted in the complete remission of glomerular disease and the disappearance of monoclonal protein. Hence, we believe he had Tuberculosis-related proliferative glomerulonephritis with monoclonal immunoglobulin deposits. Treatment strategies have not been structured due to the rarity of the condition and lack of randomized trials. However, expert opinion suggests clone-based therapy. proliferative glomerulonephritis with monoclonal immunoglobulin deposits with a benign course without clone-based therapy has been reported. Patients seldom respond to classic immunosuppressants. Even some cases experience slowly progressive disease under angiotensin converting enzyme inhibition alone. There are also cases secondary to viral infections. Our case and the particular "benign" cases lead us to an intriguing proposition that proliferative glomerulonephritis with monoclonal immunoglobulin deposits might not be a single disease. A subset of patients may be experiencing infection-related or post-infectious glomerulonephritis presenting as proliferative glomerulonephritis with monoclonal immunoglobulin deposits.

Resumen La lesión similar a la glomerulonefritis membranoproliferativa con depósitos de Ig monotípicos restringidos a un isotipo de cadena ligera única se conoce actualmente como glomerulonefritis proliferativa con depósitos de inmunoglobulina monoclonal. A continuación presentamos a un paciente que presentó hipocomplementemia y síndrome nefrótico, al que inicialmente se le diagnosticó glomerulonefritis proliferativa con depósitos de inmunoglobulina monoclonal. Desarrolló tuberculosis diseminada después de un breve curso de inmunosupresión. El tratamiento exitoso de la tuberculosis dio como resultado la remisión completa de la enfermedad glomerular y la desaparición de la proteína monoclonal. Por lo tanto, creemos que tenía glomerulonefritis proliferativa con depósitos de inmunoglobulina monoclonal relacionada con tuberculosis diseminada. Las estrategias de tratamiento no se han estructurado debido a la rareza de la afección y la falta de ensayos aleatorios. Sin embargo, la opinión de los expertos sugiere una terapia basada en clones. Se ha informado de glomerulonefritis proliferativa con depósitos de inmunoglobulina monoclonal con un curso benigno sin terapia basada en clones. Los pacientes rara vez responden a los inmunosupresores clásicos. Incluso algunos casos experimentan una enfermedad de progresión lenta solo con la inhibición de la enzima convertidora de angiotensina. También hay casos secundarios a infecciones virales. Nuestro caso y los casos "benignos" particulares nos llevan a la propuesta intrigante de que la glomerulonefritis proliferativa con depósitos de inmunoglobulina monoclonal podría no ser una sola enfermedad. Un subgrupo de pacientes puede estar experimentando glomerulonefritis postinfecciosa o relacionada con una infección que se presenta como glomerulonefritis proliferativa con depósitos de inmunoglobulina monoclonal.

Obstetric and long-term graft outcomes in pregnant kidney transplant recipients: A single-center experience.

BACKGROUND: Kidney transplantation (KT) is the best option for many women with end-stage renal disease desiring pregnancy. The aim of this study was to investigate obstetric and graft outcomes among KT recipient women in our center. METHODS: Maternal and fetal data were assessed in 29 pregnancies of 18 female KT recipients. Each patient was matched with two controls without pregnancy history for factors known to affect graft function. According to pre-pregnancy levels, serum creatinine and eGFR slope in the gestational and postpartum periods were calculated as percentages. RESULTS: The main maternal and fetal complications were preeclampsia (38%) and preterm births (38%), respectively. Pregnancy (odds ratio [OR]: 5.09; p = .02), proteinuria in the third trimester (OR: 5.52; p = .02), proteinuria in postpartum third months (OR: 7.4; p = .008) and stable creatinine levels in the first 6 months of pregnancy (OR: 11.25 p = .03) were associated with graft dysfunction. Postpartum first year eGFR decline (-16.8% vs. -6.7%; p = .04) and second-year eGFR decline (-18.5% vs. -8.3%; p = .04) were significantly higher in the pregnancy group than those matched controls. CONCLUSION: Pregnancy after KT is associated with high rates of maternal and fetal complications. The sustained decline of eGFR may suggest an increased risk of graft loss compared to recipients with similar clinical characteristics.

A Comparison of the Indirect Calorimetry and Different Energy Equations for the Determination of Resting Energy Expenditure of Patients With Renal Transplantation.

OBJECTIVE: We aimed to evaluate the agreement between the resting energy expenditure (REE) obtained by indirect calorimetry and eight prediction equations in adult patients with renal transplantation and a newly developed REE prediction equation for use in patients with renal transplantation in the clinic. METHODS: A total of 51 patients (30 males and 21 females) were involved in the study. The REE was measured by indirect calorimetry and compared with the previous prediction equations. The agreement was assessed by the interclass correlation coefficient and by Bland-Altman plot analysis. RESULTS: No significant difference was found in terms of age and body mass index between the genders. Differences between the predicted and measured REEs were maximum in the Bernstein equation (-478 kcal) and minimum in the Cunningham equation (-69 kcal). It was found that underprediction values varied from 27.5% (chronic kidney disease equation) to 98.0% (Bernstein equation). The highest overprediction value was found in the Schofield equation (17.7%). The Cunningham equation and the new equation had the lowest root mean square error (265 kcal/day). In this study, fat-free mass (FFM) was found to be the most significant variable in multiple regression analysis (r2: 0.55). The new specific equation based on FFM was generated as 424.2 + 24.7∗FFM (kg). Besides that, it was found that the new equation and Cunningham equation were distributed randomly according to Bland-Altman analysis. A supplementary new equation based on available anthropometric measurements was developed as -1996.8 + 19.1∗height (cm) + 7.2∗body weight (kg). CONCLUSION: This study showed that most of the predictive equations significantly underestimated REE. In patients with renal transplantation, if the REE is not measurable by indirect calorimetry, the use of the proposed equations will be more accurate.

Comparison Between RIFLE, AKIN, and KDIGO: Acute Kidney Injury Definition Criteria for Prediction of In-hospital Mortality in Critically Ill Patients.

INTRODUCTION: Acute kidney injury (AKI) is an important lifethreatening complication in patients hospitalized in intensive care units (ICU). This study was conducted to determine the incidence of AKI in the medical intensive care unit of a tertiary university hospital and to compare the predictive performance of three different AKI criteria (RIFLE, AKIN, and KDIGO) for in-hospital mortality. METHODS: The data of all consecutive patients were evaluated from their hospitalization to ICU until discharge or death, retrospectively. Patients with end-stage renal disease, history of kidney transplantation, those who stayed in the ICU for less than 72 hours, who underwent dialysis before admission to the ICU, and those with incomplete medical records were excluded. AKI was defined using serum creatinine criteria of RIFLE, AKIN, and KDIGO. RESULTS: 303 patients were included in this study. According to RIFLE, AKIN, and KDIGO criteria the incidence of AKI were 47.9 %, 44.6%, and 50.2%; respectively. In-hospital mortality rates were higher in AKI patients (P < .05 according to all three criteria). Regression analysis revealed that AKI was a predictor of in-hospital mortality (P < .05, for all). The ROC analyses showed that each of these criteria had similar abilities to predict in-hospital mortality (area under (Au) ROC for RIFLE = 0.76, AuROC for AKIN = 0.72, and AuROC for KDIGO = 0.76). CONCLUSION: The incidence of AKI was higher with KDIGO criteria. In-hospital mortality rates were higher in patients with AKI. Each criteria had similar abilities to predict in-hospital mortality.

Relationship Among Denture Status, Remaining Teeth Number, and Malnutrition in Patients With Chronic Kidney Disease.

The purpose of the present study was to evaluate the relationship among the denture status, number of remaining teeth, and malnutrition in patients with chronic kidney disease (CKD). Seventy-three patients (43 men/30 women) who required hemodialysis were included in the study. Weight and height, Kt/V, urea reduction ratio, midweek interdialytic weight gain, ultrafiltration volume, and erythropoietin dosage were determined for all patients. Laboratory measurements and predialysis blood samples for biomarkers were collected within the monthly routine tests. Dental examination focused on the denture status and total number of remaining teeth. Denture status were classified into two groups as prosthesis group (patients have prosthesis) and no prosthesis group (patients do not have any kind of prosthesis). And also, patients were assigned to one of four categories based on the number of remaining natural teeth: (i) edentulous, (ii) 1-7 teeth, (iii) 8-19 teeth, and (iv) >20 teeth. The data were statistically analyzed (a = 0.05). Body mass index values were increased in the prosthesis patients. Serum albumin levels of the CKD patients were under the reference value (3.8 g/dL) both in prosthesis and no prosthesis groups. Patients who have eight and more teeth showed higher serum albumin levels, and patients who had 1-7 teeth showed lower serum albumin levels in prosthesis group. CKD patients' nutritional habits were affected by denture status (having prosthesis or not). The number of the remaining teeth in prosthesis group affected the serum albumin levels of CKD patients.

Early Diagnostic Markers for Detection of Acute Kidney Injury in Allogeneic Hematopoietic Stem Cell Transplant Recipients.

OBJECTIVES: Acute kidney injury is a relatively frequent complication of allogenic hematopoietic stem cell transplant, resulting in increased risk of morbidity and mortality. Early diagnosis and management of acute kidney injury is of great importance for prevention of poor outcomes in these transplant recipients. MATERIALS AND METHODS: Fifty consecutive patients, hospitalized for allogenic hematopoietic stem cell transplant at the Bone Marrow Transplantation Unit of Gazi University Faculty of Medicine, were included in this prospective study. Serial measurements of serum creatinine and creatinine clearance were obtained before administration of conditioning regimen and at 0, 7, 14, 21, and 28 days after start of conditioning. Blood and urine samples were also obtained for the measurement of serum cystatin C and urine neutrophil gelatinase-associated lipocalin levels before conditioning and 24 hours before each serum creatinine measurement. RESULTS: During the median 25 days of follow-up, acute kidney injury developed in 19 patients: 10 patients had stage 1, 7 had stage 2, and 2 had stage 3 acute kidney injury according to the Acute Kidney Injury Network classification. There were significant positive correlations between serum cystatin C levels and serum creatinine levels and negative correlations with creatinine clearance levels at each time point (P < .001), whereas no statistically significant associations were observed with urinary neutrophil gelatinase-associated lipocalin levels. Both univariate and multivariate Cox regression models showed a statistically significant association between serum cystatin C levels and development of acute kidney injury, whereas urine neutrophil gelatinase-associated lipocalin levels did not show any significant associations. CONCLUSIONS: Serum cystatin C levels might be a useful marker for early detection of acute kidney injury in adult allogenic hematopoietic stem cell transplant recipients. Close monitoring of kidney function by sensitive biomarkers might provide early recognition and timely management of acute kidney injury in high-risk patient populations.

Could drug burden be associated with severe periodontitis in patients receiving haemodialysis?

BACKGROUND: Periodontitis increases the risk of cardiovascular disease in the general population by triggering systemic inflammation. AIM: To investigate the relationship between systemic inflammation and periodontitis, and clarify any association between severe periodontitis and the medications used by patients receiving haemodialysis. DESIGN: A cross-sectional study. PARTICIPANTS: The study was undertaken with 56 patients receiving haemodialysis. MEASUREMENTS: Demographic and laboratory data and prescribed drugs regularly used by patients were recorded from hospital records. During the dialysis session, a validated Xerostomia Inventory score was completed. A complete dental/periodontal examination was also undertaken on all patients by the same periodontist. RESULTS: In the study population, stage I periodontitis was determined in 41%, stage II periodontitis in 17%, stage III periodontitis in 21%, and stage IV periodontitis in 21%. Male gender, hypertension, coronary artery disease, ß antagonists, calcium channel blockers, sodium polystyrene sulphonate, teeth brushing less than twice a day and high sensitive C-reactive protein > 8 mg/l were significantly associated with severe periodontitis. CONCLUSION: Drugs, including ß antagonists, calcium channel blockers, polystyrene sulphonate, co-morbid conditions and poor or insufficient oral care could facilitate an increase in the severity of periodontitis in patients receiving haemodialysis. Severe periodontitis also seems to be associated with cardiovascular disease and inflammation in patients with chronic renal disease.

Oral Candida Colonization as a Risk Factor for Chronic Inflammation and Atherosclerosis in Hemodialysis Patients.

The purpose of this study was to determine the prevalence of oral Candida spp. in HD patients and to investigate its relation with systemic inflammation and atherosclerosis. Microbiological samples were taken from buccal mucosa, palate, and dental prosthesis with a cotton swab. High-sensitivity CRP (hsCRP) and IL-6 were measured as inflammation markers. A total of 69 patients (58% male and median age 62 years) were enrolled in this study; 53.6% of total patients had oral Candida colonization. HsCRP and IL-6 levels were found to be significantly higher in the oral Candida colonization positive group than in the Candida colonization negative group (P = 0.002 and P = 0.01, respectively). HDL levels were significantly lower in the Candida colonization positive group (P = 0.03). Peripheral artery disease (P = 0.05) and oral Candida colonization (P = 0.002) were significantly associated with inflammation. In addition to conventional risk factors such as age (P = 0.03), diabetes (P = 0.001), and peripheral artery disease (P = 0.002), oral Candida colonization is associated with coronary artery disease (P = 0.04). Oral Candida colonization might be associated with chronic inflammation and development of atherosclerosis in HD patients.

Hypothalamic Energy Regulatory Peptides in Chronic Kidney Disease.

Loss of appetite affects one-third of patients with CKD and is the leading cause of malnutrition in this population. Orexigenic Agouti-related peptide (AgRP) with neuropeptide-Y (NPY) and anorexigenic melanocyte-stimulating hormone-α (MSH-α) with cocaine- and amphetamine-regulated transcript (CART) are known to regulate appetite. In this study, we aimed to evaluate the levels of these peptides in CKD patients compared to healthy subjects and demonstrate the effects of dialysis treatment and erythropoiesis-stimulating agent (ESA) therapy. The cross-sectional study is composed of consecutive inclusion of 20 healthy individuals, 20 predialysis CKD patients, 20 HD, and 20 peritoneal dialysis (PD) patients. Exclusion criteria were an active infection, history of malignancy, hypo- or hyperthyroidism, and diabetes. Patients on dialysis had targeted Kt/Vs. Demographic features and BMIs of the four groups were similar. Levels of AgRP, NPY, AMSH, and CART were significantly different between groups. Nondialysis CKD patients had significantly lower hypothalamic hormones compared to healthy individuals, HD and PD patients (P = 0.02, P = 0.03, and P = 0.07 for AgRP; P = 0.02, P = 0.01, and P = 0.09 for NPY; P = 0.02, P = 0.02, and P = 0.03 for AMSH; P = 0.02, P = 0.005, and P = 0.030 for CART). Dialysis patients with or without ESA treatment had similar hormone levels (P = 0.13 for AgRP; P = 0.11 for NPY; P = 0.23 for AMSH, and P = 019 for CART). Predialysis CKD patients have lower orexigenic and presumably indirectly lower anorexigenic peptides compared to healthy subjects and dialysis patients. ESA treatment does not affect these hypothalamic peptides in dialysis patients.

High incidence of co-existing factors significantly modifying the phenotype in patients with Fabry disease.

Fabry disease results from deficiency of the lysosomal enzyme alpha-galactosidase A. The families of 11 index cases were screened by enzyme and molecular assays. Further clinical and laboratory investigations were carried out in all cases. Including 33 new patients, a total of 28 females (Age 25,82 ±â€¯12,1 Range 8-46) and 16 males (Age 24,56 ±â€¯15,04 Range 2-48) were investigated. Ten different disease-causing variants were found two of them being novel. One patient had co-existing familial mediteranian fever, one had celiac disease and three had rheumatological disorders. Lipoprotein (a) levels were elevated in 17,6%, homocysteine in 22,2%, total and low density cholesterol in 12% and antithrombin 3 levels were elevated in 13,3%. One patient was found to be heterozygous for prothrombin p.G20210A disease-causing variant (5,8%) and two for factor V Leiden disease-causing variant (11,7%). Anticardiolipin IgM antibody was found to be positive in 11,7%. The patients with abnormal cranial imaging were also noticed to have additional risk factors for thrombosis. This study provides the largest data about Fabry patients from Turkey and implies that co-existing risk factors unrelated to Fabry Disease have significant association with the presence of clinical symptoms in females and might cause an early and severe clinical course in males.

Association of Malnutrition Inflammation Score With Anthropometric Parameters, Depression, and Quality of Life in Hemodialysis Patients.

Objective: Hemodialysis patients may be at risk for malnutrition due to catabolic effects caused by dialysis, loss of amino acids, inadequate nutrient intake, acidosis, and inflammation. Malnutrition may have negative effects on quality of life, mortality, and disease prognosis. This study was conducted to determine the relationship between anthropometric parameters, depression, and quality of life with Malnutrition Inflammation Score (MIS) on 55 patients aged between 18 and 65 years (36 males and 19 females) who have dialysis treatment for 3 days per week for at least 3 months due to end-stage renal disease. Methods: Patients were evaluated with MIS, Subjective Global Assessment (SGA), Beck Depression Inventory (BDI), and Satisfaction with Life Scale (SWLS). In addition, anthropometric (body weight, height, mid-upper arm circumference [MUAC]) and body composition measurements of patients were taken, body mass index (BMI) values were calculated, and biochemical parameters (albumin, C-reactive protein [CRP], and total iron binding capacity [TIBC]) were analyzed. Results: At the end of the study, 14.5% of the patients according to SGA, were classified as malnourished. There was a statistically significant positive correlation of BDI, duration of dialysis, and CRP with MIS. However, MIS had a statistically significant negative correlation with SWLS, body weight, and MUAC (p < 0.05). Conclusion: MIS is an effective screening tool for assessing malnutrition and quality of life in hemodialysis patients. However, there is a need for studies to identify cutoff points of MIS.

Predictive value of neutrophil/lymphocyte ratio in renal prognosis of patients with granulomatosis with polyangiitis.

INTRODUCTION: Granulomatosis with polyangiitis (GPA) is a rare necrotizing vasculitis, which usually involves the upper and lower respiratory systems and kidneys and often have a relapsing course. Neutrophil/lymphocyte ratio (NLR) has been shown to be a useful marker predicting not only progressive disease, but also mortality in various inflammatory diseases. We aimed to investigate the roles of NLR in predicting the extend of clinical involvement and prognosis of patients with GPA. MATERIALS AND METHODS: Consecutive newly diagnosed GPA patients who had follow-up for at least 6 months between 2010 and 2016 at Gazi University Internal Medicine-Rheumatology clinic were retrospectively analyzed. RESULTS: Fifty-three newly diagnosed GPA patients were studied. NLR was significantly higher in the GPA group compared with the control group (4.50 [min-max: 0.07-34.81] vs 1.77 [min-max: 1.04-2.90], respectively, p < .001). NLR significantly correlated with ESR and CRP levels (r = .40 and r = .48, respectively, p < .001 for both). DISCUSSION: GPA is a vasculitis with a significant morbidity and mortality (REF). Renal involvement usually presents with crescentric glomerulonephritis, resulting in significant and permanent loss of renal functions and end-stage kidney disease. Higher NLR at baseline is associated with worse renal outcome. Our findings suggest that baseline NLR could have a predictive value for renal prognosis. We have also demonstrated a significant correlation between NLR and BVAS activity scores. Our data suggest that GPA patients with a significantly high NLR at baseline might need closer follow-up for persistent disease activity.

The effect of systemic erythropoietin treatment on retinal nerve fiber layer parameters in patients with chronic renal failure undergoing peritoneal dialysis.

PURPOSE: To evaluate the effect of erythropoietin (EPO) treatment on retinal nerve fiber layer (RNFL) parameters in patients with chronic renal failure (CRF) undergoing peritoneal dialysis (PD). METHODS: Fifty-eight eyes of 29 patients with CRF undergoing PD were evaluated. Fifteen patients have been treated with EPO (group 1), 14 patients without EPO treatment (group 2), and 30 eyes of 15 age-matched normal control subjects were assessed in group 3. A complete ophthalmologic examination and RNFL measurements were performed for each patient after PD. Anemia parameters were also measured. RNFL thickness protocol was used to acquire circular scans of 3.4 mm in diameter around optic nerve. RNFL thicknesses were evaluated in 4 quadrants. Only the left eyes were recruited for statistical analysis. The mean and quadrantal RNFL thickness values in group 1 were compared with those of groups 2 and 3. RESULTS: The mean RNFL thickness values in patients undergoing PD were statistically lower than the control group at superior, inferior, nasal, and temporal quadrant, respectively (P=0.03, 0.04, 0.04, and 0.03). Differences between the RNFL thickness values in group 1 and group 2 were statistically significant only in the temporal quadrant (P=0.02). CONCLUSIONS: In patients with CRF undergoing PD, RNFL thickness parameters were found to be significantly reduced. The effect of EPO on RNFL parameters was statistically significant only in the temporal quadrant.

The effect of dietary salt restriction on hypertension in peritoneal dialysis patients.

BACKGROUND/AIM: To investigate the effect of dietary salt restriction on blood pressure levels, total sodium removal, and hydration status of peritoneal dialysis (PD) patients. MATERIALS AND METHODS: Thirty-one stable PD patients who consulted a renal dietitian monthly for dietary recommendations, including restricted salt intake <5 g/day, and education about hypertension and hypervolemia were included in this study. Baseline and third month clinical and laboratory findings, bioelectrical impedance analysis results, and urinary and peritoneal sodium removal values were recorded. RESULTS: The mean age of the patients was 47.6 years and the mean time on PD was 39.6 months. The mean total sodium removal decreased slightly from 139.4 ± 69.1 to 136.2 ± 64.8 mmol/day (P > 0.05) for the whole sample, and from 164.3 ± 70.9 to 154.2 ± 72.3 mmol/day (P > 0.05) for the hypertensive subgroup (n: 17). Systolic blood pressure (from 134.3 ± 20.1 to 127.2 ± 19.5 mmHg, P: 0.01), diastolic blood pressure (from 83.2 ± 12.0 to 77.4 ± 10.5 mmHg, P: 0.01) and total body water (from 39.2 ± 10.9 to 38.3 ± 9.3 L, P: 0.04) decreased significantly. CONCLUSION: We demonstrated that even a little reduction in daily dietary sodium intake caused significant decreases in blood pressure levels and fluid overload.