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

BackgroundMany studies were carried out to investigate the relationship between single nucleotide polymorphisms (SNPs) in vitamin D receptor (VDR) gene with obesity. However, little is known about the role of VDR gene polymorphism with obesity in hemodialysis (HD) patients. Therefore, we aimed to investigate VDR gene TaqI, ApaI and FokI SNPs in overweight/obese HD patients.MethodsSeventy one normal weight and 68 overweight/obese HD patients were included in study. PCR-RFLP method was used for genotyping. Demographic and laboratory data obtained from medical records of patients.ResultsFor all three SNPs, no significant association was found between normal and overweight/obese patients (P>0.05). Lower HDL concentrations and higher levels of triglyceride (TG) and glucose were detected in the obese/overweight patients compared to normal weight (pConclusionsOur study suggest that VDR TaqI, ApaI and FokI polymorphisms are not associated with obesity in HD patients. However, they might be increase the risk of secondary hyperparathyroidism, dyslipidemia, and hyperglycemia, which are among the most common obesity related comorbidities of chronic kidney disease.

Aim: The aim of this study is to determine the prevalence of Chronic renal disease (CKD) in the patients known with gout arthritis. Method:A total of 162 patients with gout arthritis diagnosed between 2014 and 2017 were included in the study. Our work is a retrospective study. Glomerular filtration rate was calculated by Modification of Renal Disease (MDRD) method. Results: The mean age of the patients was found as 59,64 ± 14,54 (18-93). The majority of patients are male. Mean uric acid levels of the patients were found to be 9.07 mg / dL ± 1.75 (4-14.7). Among these individuals with gout arthritis, % 39,5 (64) had CKD stage 3-5, %46,9 (76) had hypertension, %18,5 (30) had diabetes mellitus, %14,2 (23) had coronary artery disease. 33.3% of the patients had CKD stage 2. Nephrolithiasis was detected in 22 of 85 ultrasound patients. The use of diuretics was detected in 36 of the patients. The consultation rate requested by the dietician was 41,4%. Conclusions: Hyperuricemia and hypertension are high in CKD. Diuretics used in the treatment of hypertension and edema may trigger gout arthritis. Similar to other studies in our study, the prevalence of CKD in gout arthritis was found high.

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

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

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

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

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

C3glomerulopati (C3G), kompleman aktivasyonunun anormal kontrolü ile oluşan bir grupböbrek hastalığını kapsayan yakın zamanda tanımlanmış bir hastalıktır. C3Gglomerullerde immunoglobulin birikiminin trace ve yokluğu ile dominant olarak komplemanC3 birikimi ile karakterizedir. C3G C3 glomerulonefrit (C3GN) ve Dens DepositHastalığı (DDD) diye iki grup hastalıktan oluşmaktadır. DDD ve C3GN nadirgörülmektedir ve her iki hastalık hem çocuklarda hemde erişkinlerde görülmektedir.C3G spesifik tedavisinde, plazmaferez, immünsüpresif tedavi ve kompleman inhibitörleriniiçermektedir., C3glomerulopathy is a recently defined disease that encompasses a group of kidneydiseases caused by abnormal control of complement activation. C3G is characterizedbydeposition of dominant complement C3 with absence/traces of immunoglobulinsin the glomeruli. C3G may be classified intodense deposit disease (DDD) and C3 glomerulonephritis (C3GN). DDD andC3GN are rare forms of glomerulonephritis that affect both children and adults.In treatment C3G include on specific treatment, plasma therapy, immunsupressiveand complement inhibition.

WOS: 000412950500025, PubMed ID: 28573371, Primary glomerulopathies are those disorders that affect glomerular structure, function, or both in the absence of a multisystem disorder. We aimed to evaluate the frequency of MEFV gene mutation to show possible coexistence of FMF in patients diagnosed with biopsy-proven primary glomerulonephritis (GN). A total of 64 patients with biopsy-proven primary GN were included in the study. MEFV gene mutations examined retrospectively. The mean age of patients was 39.6 +/- 13.4 (range 18-69), 35 of patients were female and 29 of patients were male. Of the 64 patients, 17 were mesangial proliferative glomerulonephritis (MsPGN), 15 were IgA nephropathy (IgAN), 12 were membranous glomerulonephritis (MGN), 11 were focal segmental glomerulosclerosis (FSGS), three were membranous proliferative glomerulonephritis (MPGN), three were immune complex glomerulonephritis (ICGN), two were minimal change disease (MCD), and one was IgM nephropathy (IgMN). MEFV gene mutation was detected in 35.9% (23) of these patients. The most frequently detected mutations were E148Q and M694V. Twelve cases (18.75% of GN patients) with MEFV gene mutation were diagnosed as FMF phenotype I. The frequency of MEFV gene mutation was detected at a high rate of 35.9%. Further studies with larger populations are needed to clarify the importance of these mutations on clinical progression of glomerulonephritis.

Introduction: Patients’ high levels of health literacy and positive perception of health are effective in their compliance with the treatment. Objectives: The aim of this study was to examine the relationship between health literacy and the perception of health of hemodialysis patients. Patients and Methods: In the study, the sampling method was not performed, but it was performed with patients who received hemodialysis treatment and fulfilled the research criteria within the 3-month period. The descriptive study was conducted with a total of 110 patients in two dialysis centers Sivas province of Turkey. Results: Data were collected using the patient information form, Health Literacy Index (HLI) and perception of health scale (PHS) to determine demographic characteristics. Around 56.4% of the patients had been receiving hemodialysis treatment for 0-4 years. The mean score of patients’ health literacy was 77.40±12.94, and they received the highest score from the appraisal of the subscales. Perception of health mean score was 47.56±4.10. When sociodemographic characteristics were examined, health literacy levels were found to be high in males between the ages of 36-45 years, those with a long duration of chronic kidney disease (CKD) and those with higher education level. Conclusion: According to results patients’ health literacy and disease perception were found to be slightly above the middle level. We found a positive relationship between health literacy level and perception of health, and patients’ health literacy since the perception of health should be increased in order for the hemodialysis treatment process to be effective.

WOS: 000393291900003, OBJECTIVE: The aim of this study was to determine the incidence of peritonitis in patients with continuous ambulatory peritoneal dialysis (CAPD)-related peritonitis, microorganisms causing peritonitis and the clinical course of patients during the 15-year study period. MATERIAL and METHODS: A total of 61 patients (26 male, 35 female) with CAPD-related peritonitis treated in our hospital between 2000-2014 were enrolled into this study. RESULTS: During the study period, 103 episodes of peritonitis were observed, with a mean incidence of 0.29 episodes per patient year. Overall, 58.3% of the episodes were due to gram-positive organisms, 7.8% due to gram-negative organisms and 30.1% were culture negative. The treatment was successful in 96.1% of the episodes, and removal of the Tenckhoff catheter was required in 4 patients. It was observed when the distribution of the causative organisms over the years were examined that the number of CAPD peritonitis and the rate of culture-negative episodes decreased after the year 2008. CONCLUSION: In our center, the main causes of peritonitis were gram-positive microorganisms. During follow-up, the incidence of peritonitis and the rate of culture-negative episodes decreased but there was no change in the gram stain characteristics of organisms. Reducing the incidence of CAPD-related peritonitis could be possible by providing intensive training to patients and strict monitoring.

WOS: 000376969600002, PubMed ID: 29900931, Objectives: This study aims to evaluate the carotid intima-media thickness (CIMT) in patients diagnosed with familial Mediterranean fever (FMF) and investigate whether there is a relationship between glutathione-S-transferase (GST) gene polymorphisms and CIMT. Patients and methods: Sixty FMF patients (17 males, 43 females; mean age: 31.43 +/- 11.36 years; range 18 to 45 years) and 60 healthy controls (22 males, 38 females; mean age: 29.8 +/- 5.82 years; range 18 to 40 years) were enrolled in this study. Polymerase chain reaction-restriction fragment length polymorphism methods were carried out to assess GST polymorphisms. CIMT was measured by carotid ultrasonography. Biochemical parameters were also evaluated using biochemical methods. Results: Right and left CIMT of FMF patients were statistically significantly higher than that of control group (CIMT right p=0.001 and CIMT left: p=0.033). There was no significant association in terms of GST polymorphisms between FMF and control groups. No significant association was observed between GST polymorphisms and CIMT. Low density lipoprotein, erythrocyte sedimentation rate, and fibrinogen levels were significantly higher in the patient group (p0.05). Conclusion: Although no significant association was observed between GST polymorphisms and CIMT in FMF patients and controls, CIMT was statistically significantly higher in FMF patients compared to controls., Scientific Research Project Fund of Cumhuriyet University [T-515], This work is supported by the Scientific Research Project Fund of Cumhuriyet University under the project number "T-515".

Renal tubular acidosis (RTA) is metabolic acidosis disorder with a normal anion gap that occurs resulting from bicarbonate reabsorption or disorder in the hydrogen excretion from the kidney. A variety of tests are required to be administered in a stepwise fashion for the diagnosis and characterization of RTA. Correct diagnosis involves careful evaluation, including exclusion of other entities causing acidosis. The patients were treated with potassium and bicarbonate supplementation. A fifty-one years old female patient presented to the emergency department with quadriparesis dependent on hypokalemia and vitamin D deficiency, was diagnosed with distal renal tubular acidosis (dRTA) combined with Sjogren's Syndrome (SS). We submitted this case in order to draw attention to the presentation of the RTA with SS.

WOS: 000433009400011, PubMed ID: 29544394, Purpose: We investigated the influence of the vitamin D receptor gene Taql (rs731236), Apal (rs7975232), and Fokl (rs2228570) polymorphisms in arteriovenous fistula failure in hemodialysis patients. Methods: This study was carried out with 54 patients who experienced two or more fistula failures in the late period after arteriovenous fistula operation and 58 control patients with no history of arteriovenous fistula failure in 3years or longer. The polymerase chain reaction-restriction fragment length polymorphism method was used to determine the vitamin D receptor Taql, Fokl, and Apal polymorphisms. Results: For vitamin D receptor gene Taql and Fokl polymorphisms, no significant association was found between the two groups (p>0.05). However, a statistically significant association was determined for Apal polymorphism between the two groups (p = 0.02). In patients, Apal AA, AC, and CC genotype frequencies were found as 21 (38.9%), 32 (59.3%), and I (1.8%), respectively. However, genotype frequencies of AA, AC, and CC in the control group were 29 (50%), 22 (37.9%), and 7 (12.1%), respectively. In all three polymorphisms, no significant difference was found between the two groups in terms of allele frequencies (p>0.05). Conclusion: Vitamin D receptor Apal AC genotype may be a possible cardiovascular risk factor for the development of arteriovenous fistula failure.

WOS: 000426502700016, Aim: The purpose is to evaluate the relationship between Restless legs syndrome (RLS) and 25-Hydroxy D [25 (OH) D] vitamin level among hemodialysis patients. Material and Method: Hemodialysis patients, in Cumhuriyet University Faculty of Medicine Department of Nephrology were included in this study. Patients were asked about the diagnosis criteria of the International RLS study group and their laboratory examinations along with 25 (OH) D vitamin levels were assessed. Results: Seventy-five patients, 40 of whom were female, were included in the study. The average age of patients was 57.8 (19-84). Average 25 (OH) vitamin D levels of 75 patients in total were found to be 12.6 +/- 6.27 (3-30). Five patients had 25 (OH) D vitamin insufficiency and 70 had 25 (OH) D vitamin deficiency. Thirty-three patients were diagnosed with RLS and their average vitamin D levels were 10.76 +/- 4.56; the D levels of 42 patients not diagnosed with RLS were found to be 14.18 +/- 7.02. A significant relationship was determined between the patients diagnosed with RLS and their vitamin D levels (p=0.018). Discussion: A significant relationship was determined between RLS and 25 (OH) vitamin D level among hemodialysis patients. The fact that frequency of RLS was higher among hemodialysis patients compared to the general population may be related to low levels of 25 (OH) D vitamin.

WOS: 000444468500015, The Enterobacter cloacae complex, a member of the genus Enterobacter, consists of a group of bacteria that are responsible for serious infections in human beings. A recently identified member of the group, Enterobacter ludwigii sp, is an emerging source of clinically important infections, but, up until now, there has been no report of catheter related bacteremia due to Enterobacter ludwigii sp. in hemodialysis patients. We report a hemodialysis patient with catheter related bacteremia due to Enterobacter ludwigii sp. whose infection improved only partially by antibiotics that were expected to be fully effective, based on antibiotic susceptibility testing; the infection could be cured only after removal of the catheter.

WOS: 000425163400008, OBJECTIVE: Occult hepatitis B (OHB) virus infection is defined as the presence of hepatitis B virus (HBV) DNA in the liver tissue or serum of subjects seronegative for hepatitis B surface antigen. OHB leads to the potential risk of transmission in dialysis service. Routine HBV vaccine in dialysis patients is recommended. However, HBV vaccine response rates are lower than the community. The aim of this study was to determine the prevalence of OHB in hepatitis B vaccinated dialysis patients. MATERIAL and METHODS: This study was performed at the Nephrology Department, Faculty of Medicine, Cumhuriyet University, between 1st January -31st December 2014. Sera from 200 dialysis patients with negative HbsAg were investigated for HBV DNA using the polymerase chain reaction (PCR). RESULTS: The mean age of the patients was 59.57 +/- 14.89 (18-91) years; 179 of them were on hemodialysis and 21 were on peritoneal dialysis. Of the patients included in the study, anti-HBs positivity was present in 135 (67.5%) and anti-HBs negativity in 65 (32.5%). The OHB prevalence was 1.5% (n=3). CONCLUSION: In our study, the OHB prevalence was 1.5%. We assume that HBV infection would be reduced further by routinely applying HBV PCR tests for all patients who start dialysis and by taking precautions against transmission., Cumhuriyet University, The study was carried out with Cumhuriyet University Scientific Research Projects support.

Kalıtımsal hipoürisemiye genellikle ksantinüri ve herediter renal hipoürisemi yol açmaktadır. Hipoürisemi serum ürik asit seviyesinin 2mg/dl’den düşük olması olarak tanımlanmaktadır. Ksantinüri her yaşta, nadir görülen ve otozomal resesif geçen bir hatalıktır. Ksantin ve hipoksantin, enzim veya kofaktör eksikliği sonucu ürik asite dönüşümü olmaz ve kanda birikir, ayrıca idrar ile atılımı artar. Serum ve idrarda ürik asit seviyesi çok düşük saptanmaktadır. Biz bu makalede,hipoürisemi saptanan ve ksantinüri tanısı konulan yetmiş yediyaşında kadınhasta litaratüreşliğinde tartışıldı., Xanthinuria and hereditary renal hypouricemia usually leads to hereditary hypouricemia. Hypouricemia is defined as having serum uric acid level lower than 2 ml/dl. Xanthinuria is an autosomal recessive disease that can be occasionally seen in all ages. As a result of enzyme or cofactor deficiency, xanthine and hypoxanthine do not convert to uric acid and accumulate in the blood and increase the excretion in urine. The level of uric acid is detected very low either in serum or in urine. In this study, a case of a seventy-seven-year-old female patient in which hypouricemia was detected and who had the diagnosis of xanthinuria was discussed.

Xanthinuria and hereditary renal hypouricemia usually leads to hereditary hypouricemia. Hypouricemia is defined as having serum uric acid level lower than 2 ml/dl. Xanthinuria is an autosomal recessive disease that can be occasionally seen in all ages. As a result of enzyme or cofactor deficiency, xanthine and hypoxanthine do not convert to uric acid and accumulate in the blood and increase the excretion in urine. The level of uric acid is detected very low either in serum or in urine. In this study, a case of a seventy-seven-year-old female patient in which hypouricemia was detected and who had the diagnosis of xanthinuria was discussed.

WOS: 000374928100013, Hydatid cyst (CH), which is quite common in the world, mostly transmitted by dog faeces, is a parasitic disease caused by Echinococcus granulosus. CH often infects the liver and lungs. During the clinical course, renal involvement is rarely seen. In this article; due to liver hydatid disease, mezengioproliferatif glomerulonephritis case is presented.

WOS: 000350362400019, PubMed ID: 25655256, Monocyte chemoattractant protein-1 (MCP-1) plays a major role in the pathogenesis and progression of different types of human renal disease. Therefore, in this study, we aimed to investigate the effect of MCP-1 gene -2518 A > G promoter polymorphism in chronic renal failure (CRF) patients requiring long-term hemodialysis. The study population consisted of 201 adult CRF patients requiring long-term hemodialysis and 194 healthy controls. The polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) technique was used for genotyping of MCP-1 -2518 A > G polymorphism in the CRF patients and healthy controls. There were statistically significant differences in terms of genotypic (chi (2) = 12.69, p = 0.02) and allelic (chi (2) = 5.72, p = 0.02) frequencies of MCP-1 -2518 A > G between CRF patients and control subjects. According to our results, in the patient group MCP-1 -2518 AA genotype frequency was significantly higher than that of control group. On the other hand, heterozygous AG genotype frequency in the control group was significantly higher than that of the study group. Three different main disease subgroups of CRF (hypertension, diabetes mellitus, and atherosclerosis) patients were also evaluated, and significant associations were found between hypertension (genotype: chi (2) = 9.28, p = 0.01; allele: chi (2) = 6.00, p = 0.01), atherosclerosis (genotype: chi (2) = 5.37, p = 0.02; allele: chi (2) = 4.13, p = 0.04), and distributions of MCP-1 -2518 A > G genotypes and alleles. However, no significant association was found between diabetes mellitus and distributions of MCP-1 -2518 A > G genotype and allele frequencies (genotype: chi (2) = 2.37, p = 0.3; allele: chi (2) = 1.88, p = 0.17). Current data show that MCP-1 -2518 AA genotype may cause susceptibility to CRF, while G allele may have a protective effect against development of CRF. In addition, MCP-1 -2518 AA genotype seems to associate with CRF originated from hypertension and atherosclerosis in our study population.

WOS: 000376250700009, PubMed ID: 25645282, The cytochrome P450 2D6 (CYP2D6) is a cytochrome P450 enzyme involved in the oxidative biotransformation of the xenobiotics, carcinogens and various clinically important drugs. Patients are evaluated in three sub-groups of extensive (EM), intermediate (IM) and poor metabolizer (PM) phenotypes due to their drug-metabolising ability for the target CYP2D6 gene. Colchicine non-responsive FMF patients were prospectively genotyped for the major CYP2D6 alleles in the current study. Major CYP2D6 alleles of *1, *3, *4, *5, and *6 were genotyped for 30 responsive and 60 non-responsive FMF patients by multiplex PCR-based reverse-hybridization StripAssay and real-time PCR methods. DNA banks isolated from blood-EDTA were retrospectively used in the current patients and results were compared statistically. Increased CYP2D6 *4 and *6 allele frequencies were highly detected in the colchicine non-responsive FMF patients when compared to the responsive group. Results showed the frequencies of major CYP2D6 *1(wild), *3(2637A > delA), *4(G1934A), *5(total gene deletion) and *6(1707T del) alleles in 0.550, 0.042, 0.158, 0.025 and 0.225 for non-responder and 0.880 and 0.120 (CYP2D6*1 and *4) for the responder groups, respectively. Despite small sample size, this study suggests that there is an association between CYP2D6*4 and CYP2D6*6 alleles and drug intoxicants in colchicine non-responder FMF patients.

Pulmonary renal syndrome is a rare but serious complication of systemic vasculitis. The majority the cases are antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis. In this case, a 58 years old female case having aworsening clinical picture of probable urinary infection and acute renal failure was presented. By means of proteinuria, pyuria, hematuria and unexplained acute renal failure, we review the relevant literature on pulmonary renal syndrome associated with granulomatosis with polyangiitis (GPA) with negative serum ANCA serology. This report demonstrates the difficulty of diagnosing granulomatosis with polyangiitis (GPA) until renal biopsy was done while initial diagnostic serological negative ANCA testing.

Amaç: Çalışmamızda, Cumhuriyet Üniversitesi Araştırma ve Uygulama Hastanesi Hemodiyaliz Ünitesi’ne başvuran hastaların HBsAg ve Anti HCV test sonuçlarının incelenerek pozitiflik dağılımlarının belirlenmesi, sonuçların yaş, cinsiyet, yerleşim yeri, hastalık tablosu gibi faktörler açısından değerlendirilerek bu konudaki literatüre katkı sunulması amaçlanmıştır. Gereç ve Yöntemler: Hastanemiz hemodiyaliz ünitesine, 01.01.2002- 31.12.2011 tarihleri arasında başvuran 3023 hastanın HBsAg ve Anti HCV test sonuçları, hemodiyaliz ünitesi ve klinik mikrobiyoloji laboratuvar sonuçları değerlendirilerek geriye dönük olarak incelenmiştir. Bulgular: Çalışmamız süresi içerisinde başvuran, 1696’sı (%56) erkek, 1327’si (%44) kadın, toplam 3023 hasta HBsAg ve Anti HCV testlerinin sonuçları açısından değerlendirilmiştir. Seropozitiflik HBsAg için %3,5, Anti- HCV seropozitifliği ise %5,1 olarak tespit edilmiştir. HBsAg ve Anti HCV’nin birlikte görüldüğü olguların oranı ise %0,2 olarak bulunmuştur. Kronik böbrek yetersizliği olan hastalarda Anti HCV pozitifliğine, akut böbrek yetersizliği olan hastalarda ise HBsAg pozitifliğine daha sık rastlanmıştır. Şehir merkezinde yaşayanlarda Anti HCV, şehir merkezi dışında yaşayanlarda HBsAg’ye daha sık rastlanmıştır. Çalışmamızda HBsAg ve Anti HCV pozitifliğinin yaş gruplarına göre dağılımı istatistiksel açıdan anlamlı bulunmamış; ancak 60-69 yaş aralığında sıklığın arttığı görülmüştür. Sonuç: Çalışmamız süresi içerisinde hastanemiz hemodiyaliz ünitesinde elde edilen HBsAg ve Anti HCV pozitifliklerinin, ülkemiz ortalamasının altında olduğu görülmüştür. Hemodiyaliz merkezlerinin enfeksiyon kontrol önlemlerini etkin bir şekilde uygulaması, sağlık çalışanları, personel, hemodiyaliz hastaları ve yakınlarının düzenli eğitimlerle bu konularda bilgilendirilmesi, seropozitif hastalara bakım veren personel ve ekipmanların ayrılması, hastaların serolojik takip ve aşılanmalarının yapılmasında gereken hassasiyetin gösterilmesi gibi önlemlerle daha düşük HBsAg ve Anti HCV sonuçlarına ulaşılabileceğine inanmaktayız., Objective: The aim of this study is to examine and determine the positive distributions of HBsAg and Anti-HCV test results of the patients who applied to Cumhuriyet University Training and Application Hospital Haemodialysis Unit. We evaluated these results in terms of the factors such as age, gender, settlement and sickness table to make a contribution to the literature. Materials and Methods: The HBsAg and Anti-HCV test results of 3023 patients applying to haemodialysis unit of our hospital between 01.01.2002 and 31.12.2011 were examined retrospectively by evaluating the test results of haemodialysis unit and clinical microbiology laboratory. Results: In our study, total 3023 patient applying to our unit, 1696 male (56%) and 1327 female (44%), were evaluated in terms of the HBsAg and Anti-HCV test results. Seropositivity was 3.5% for HBsAg and 5.1% for Anti-HCV. The proportion of subjects with both HBsAg and Anti-HCV was 0.2%. Anti-HCV positivity was frequently seen in the patients with chronic renal failure and HBsAg was frequently seen in the patients with acute renal failure. While anti-HCV positivity was seen more in in chronic renal failure patients, HBsAg positivity was more frequent in acute renal failure patients. Anti-HCV was frequently seen in the patients living in downtown and HBsAg was frequently seen in the patients living in uptown. In our study, no statistically significant difference was found among the distribution of HBsAg and Anti-HCV positivity by age groups; however, the frequency increased in 60-69 age range. Conclusion: We determined that HBsAg and Anti-HCV positivity obtained from the haemodialysis unit of our hospital were below the country average. We believe that lower HBsAg and Anti-HCV results could be achieved by controls of infection control commitees in haemodialysis centres. Also informing the medical staff, personnel, haemodialysis patients and their relatives about these topics with regular trainings and separating the staff who give care to the seropositive patients and equipment from the others and showing the necessary sensitivity in serologic follow-up and vaccination of the patients can decrease the rate.

Objective: In our study we aimed to determine the frequency of arrhythmias that we believe may affect driving safety. Methods and Results: Two hundred drivers were randomly selected from the heavy vehicle driver population (82 bus and 118 truck drivers, p = 0.08, q = 0.92, N = 1200, α = 0.01, d = 0.045). A questionnaire was completed via face to face interviews with the individuals including questions about their personal socio-demographic characteristics and symptoms for arrhythmias. An electrocardiography (ECG) was taken of the study participants using the Cardioline Delta 3 Plus Digital ECG machine. The cardiologist at the clinic evaluated the questionnaire and ECG for presence of arrhythmias. When indicated, ambulatory electrocardiography (Holter Monitoring) was performed for 24 hours in 133 individuals (71 driver and 62 control). In cases that had Holter examination; ventricular ectopy was identified in 25.4% and 22.6%, and supra-ventricular ectopy in 45.1% and 35.5% in the driver and the control groups; respectively. Ventricular tachycardia was detected in 2 patients. Arrhythmia frequencies were 59.1%, 54.8% and 57.1% in drivers, control and both groups respectively. Statistical differences between drivers and control group for rhythm disorders were not detected. Conclusions: Arrhythmias with lethal and devastating potential; need to be diagnosed and treated in professional drivers with extreme caution. The follow up and screening for heart diseases has a crucial role in preventing accidents and occupational diseases in drivers.

WOS: 000388039500001, Influenza viruses are members of the Orthomyxoviridae family, of which influenza A, B, and C viruses constitute three separate genera. Arterial thrombosis associated with H1N1 influenza A virus infection has rarely been reported. A Turkish man aged 28 years was admitted to our emergency department with dyspnea, bilateral lower extremity insensitivity, and cold. He reported symptoms of fever, myalgia, and cough, which he had had for fifteen days before being admitted to our hospital. The patient was tested for pandemic influenza A (H1N1) virus using polymerase chain reaction (PCR) tests, which were positive. Abdominal computerized tomography with contrast revealed a large occlusive thrombus within the infrarenal aorta.

AMAÇ: Akut dekompanse kalp yetersizliği (ADKY) tedavisinde ultrafiltrasyon ve diüretikleri karşılaştıran çalışmalarda çelişkili sonuçlar elde edilmiştir. Çalışmamızda ADKY nedeniyle yatırılan, sol ventrikül sistolik disfonksiyonu üzerine sağ ventrikül fonksiyon bozukluğunun süperpoze olduğu (biventriküler kalp yetmezliği) hasta grubunda intravenöz diüretik tedavisi ile ultrafiltrasyonu, etkinlik ve güvenilirlik açısından karşılaştırdık.GEREÇ ve YÖNTEMLER: Çalışmamıza ultrafiltrasyon grubuna 10, diüretik grubuna 20 olmak üzere toplam 30 hasta alındı ve hastalar 3 ay takip edildi. BULGULAR: Taburcu olurken; ultrafiltrasyon ve diüretik grupları arasında kilo kaybı, toplam sıvı kaybı ve serum kreatinin düzeyindeki değişiklik açısından istatistiksel olarak anlamlı bir fark bulunmadı. Klinik olarak sağlanan dekonjesyon oranı, her iki grupta benzerdi. Ekokardiyografik parametrelerdeki, diğer biyokimyasal parametrelerdeki değişim, nörohormonal aktivasyonu değerlendirmek için bakılan serum renin ve aldosteron düzeylerindeki değişim de gruplar arasında farklı bulunmadı. İstenmeyen olaylar değerlendirildiğinde; hemodiyalize geçme ultrafiltrasyon grubunda %20, diüretik grubunda %5 oranında, kardiyak arrest ve ölüm ise ultrafiltrasyon grubunda %40, diüretik grubunda %10 oranında görüldü. Hastaların 1 ay ve 3 ay sonraki kilo değişimi, kreatinin ve elektrolit düzeyleri de benzer bulundu.sONUÇ: Sağ ve sol kalp yetersizliğinin birlikte olduğu hastalara uygulanan ultrafiltrasyon ve diüretik tedavilerinin, kilo kaybı, toplam sıvı kaybı, klinik olarak dekonjesyonun sağlanması, böbrek ve kardiyak fonksiyonlarında değişim, renin ve aldosteron düzeylerindeki değişim yönünden birbirine üstünlüğü gösterilemedi. Hemodiyalize geçme, kardiyak arrest ve ölümün ultrafiltrasyon grubunda daha fazla saptanmasına rağmen, istatistiksel değerlendirme yapılamadığından, ultrafiltrasyonun güvenilirliği değerlendirilememiştir. Ultrafiltrasyonun tedavide rutin uygulamaya girmesi için daha kapsamlı çalışmalara ihtiyaç vardır, OBJECTIVE: Studies comparing ultrafiltration and diuretics in management of ADHF have shown controversial results. In this study, we compared the efficacy and safety of intravenous diuretic therapy and ultrafiltration in patients admitted with acute decompensated heart failure (ADHF) who had right ventricular dysfunction superimposed on left ventricular systolic dysfunction. MATERIAL and METHODs: A total of 30 patients of whom 10 were in the ultrafiltration group and 20 were in the diuretic group were enrolled in this study and followed for 3 months. REsULTs: At discharge, there were no significant differences between the ultrafiltration and diuretic groups in terms of weight loss , total fluid loss, and changes in serum creatinine. The clinical decongestion rates were similar in the two groups. Moreover, echocardiographic and biochemical parameters and alterations in renin and aldosterone levels, as measured to assess neurohormonal activation, had overlapping results between the two groups. When unwanted events were analyzed, transition to hemodialysis was seen in 20% of the patients in the ultrafiltration group and 5% of the patients in the diuretic group. The frequency of cardiac arrest and death were 40% in the ultrafiltration group and 10% in the diuretic group. Weight change, creatinine, and electrolyte levels of the patients at 1 and 3 months were also similar.CONCLUsION: Despite the high frequency of hemodialysis transition, cardiac arrest, and death in the ultrafiltration group, safety of ultrafiltration could not be assessed because of inability to perform statistical analyses. Further studies are needed to investigate the practical uses of ultrafiltration in routine clinical practice

AMAÇ: Çalışmada, 15 yıllık çalışma sürecinde, sürekli ayaktan periton diyalizi (SAPD) ilişkili peritonit gelişen hastalarda peritonit sıklığının, peritonite neden olan mikroorganizmaların ve hastalardaki klinik seyrin saptanması amaçlanmıştır.GEREÇ ve YÖNTEMLER: Çalışmaya, üniversite hastanemiz periton diyalizi merkezinde 2000-2014 yılları arasında SAPD uygulanırken peritonit atağı geçiren 61 hasta alındı (26 E, 35 K).BULGULAR: Çalışma süresince 103 SAPD peritonit atağı saptandı. Ortalama insidans ise 0,29/her hasta yılı olarak belirlendi. Atakların % 58,3'ünde Gram-pozitif, % 7,8'inde Gram-negatif etkenler saptanırken, % 30,1'i kültür negatif saptandı.Tedavi atakların % 96,1'inde başarılı oldu, 4 olguda ise Tenckhoff kateterin çıkarılması gerekti. Olguların tamamı tedavi süresi sonunda iyileşti ve peritonit ilişkili ölüm görülmedi. Yıllara göre etkenlerin dağılımı incelendiğinde 2008 yılından sonra SAPD peritonit atak sayısının ve kültür negatif atak oranının azaldığı görüldü.SONUÇ: Merkezimizde SAPD peritonitin en sık etkeni gram-pozitif mikroorganizmalardı. Takip süresince peritonit insidansı ve kültür negatif atak oranı azaldı, ancak etkenlerin gram özelliklerinde değişiklik olmadı. SAPD ilişkili peritonit insidansının azaltılması, hastalara yoğun eğitim verilmesive sıkı takip yapılması ile mümkün olabilir., OBJECTIVE: The aim of this study was to determine the incidence of peritonitis in patients with continuous ambulatory peritoneal dialysis (CAPD)-related peritonitis, microorganisms causing peritonitis and the clinical course of patients during the 15-year study period.MATERIAL and METHODS: A total of 61 patients (26 male, 35 female) with CAPD-related peritonitis treated in our hospital between 2000-2014 were enrolled into this study. RESULTS: During the study period, 103 episodes of peritonitis were observed, with a mean incidence of 0.29 episodes per patient year. Overall, 58.3% of the episodes were due to gram-positive organisms, 7.8% due to gram-negative organisms and 30.1% were culture negative. The treatment was successful in 96.1% of the episodes, and removal of the Tenckhoff catheter was required in 4 patients. It was observed when the distribution of the causative organisms over the years were examined that the number of CAPD peritonitis and the rate of culture-negative episodes decreased after the year 2008.CONCLUSION: In our center, the main causes of peritonitis were gram-positive microorganisms. During follow-up, the incidence of peritonitis and the rate of culture-negative episodes decreased but there was no change in the gram stain characteristics of organisms. Reducing the incidence of CAPDrelated peritonitis could be possible by providing intensive training to patients and strict monitoring.

WOS: 000410618200027, Aim: To evaluate demographic,clinical and laboratory characteristics. causes, MEFV gene mutations, and mortality rates of patients with secondary amyloidosis. Material and Method: 61 patients who had been diagnosed with secondary amyloidosis by renal and rectal biopsy between 2007 and 2013 in the nephrology clinic of Cumhuriyet University. Faculty of Medicine, were included in the study. Demographic characteristics, causes of secondary amyloidosis, MEFV gene mutations, end-stage renal failure (ESRF), renal transplantation, and mortality rates were examined retrospectively. Results: In etiological terms, Familial Mediterranean Fever (FMF) occurrence was 62.2% (38), bronchiectasis and emphysema 9.8% (6), tuberculosis 4.9% (3), coexistence of FMF and ankylosing spondylitis 3.2% (2), coexistence of FMF and rheumatoid arthritis 1.60,6 (1), coexistence of FMF and systemic lupus erythematosus (SLE) 1.6% (1), osteomyelitis 1.6% (1). septic arthritis 1.6% (1), Crohn's disease 1.6% (1), colon cancer 1.6% (1), coexistence of bronchiect-axis and tuberculosis 1.6% (1), rheumatoid arthritis 1.6% (1), and idiopathic cases 6.5% (4). Proteinuria was determined at nephrotic level among 68% (32) of 47 patients who had secondary amyloidosis. MEFV gene mutation of 45 patients with secondary amyloidosis was assessed. Most patients had M694V gene mutation. Surprisingly, we detected heterozygous 61480 mutation in 3 cases. 12 cases died; of these, 9 had ESRF. Five cases with ESRF underwent renal transplantation. Discussion: We found FMF as the most common cause for secondary AA amyloidosis in this study. Further studies should be done with larger or multicenter cohorts.

WOS: 000410618200028, Aim: Recurrent aphthous stomatitis (RAS) is one of the most frequent diseases of the oral mucosa, characterized by chronic, painful, recurrent, and necrotizing ulcerations. The precise etiology and pathogenesis of RAS have not been clarified. Therefore. we aimed to investigate serum levels of VEGF. sVEGFR-1, and endostatin as well as the frequencies of VEGF +936 C/T and -1154 C/A single nucleotide polymorphisms (SNPs) in Turkish patients with recurrent aphthous stomatitis. Material and Method: Forty-two patients with RAS (24 minor RAS and 18 major RAS) and 37 healthy subjects were included in the study. Serum levels of VEGF, sVEC FR-1, and endostatin were measured using the ELISA method. VEGF +936 C/T and-1154 C/A SNPs were determined by the PCR-RFLP method. Results: The mean serum level of VEGF was found higher in bearing CC genotype of +936 CFI SNP compared with CT genotype (639.5 +/- 309.1 vs 442.1 +/- 197.8: p = 0.032). VEGF-1154 GA genotype was found to be more frequent in patients with minor RAS and GG genotype was more frequent in patients with major RAS (p = 0.022). There was a significant difference between minor RAS and major RAS with regard to mean VEGF serum levels (677.1 +/- 316.7 vs 492.9 +/- 242.7; p = 0.032). Discussion: The T allele of +936 C/T SNP is associated with decreased serum VEGF level, and this decrease may have a contributory role in impaired neovascularization and re-epithelialization in the etiology and pathogenesis of RAS. Further studies are needed to determine the role of VEGF in RAS susceptibility and its clinical manifestations., Scientific Research Project Fund of Cumhuriyet University (CUBAP) [T-513], This work was supported by the Scientific Research Project Fund of Cumhuriyet University (CUBAP) under the project number T-513.

WOS: 000312869700007, PubMed ID: 22630818, Mean platelet volume (MPV) is a determinant of platelet activation. Atherogenic index of plasma (AIP), which is defined as the logarithm of the ratio of plasma level of triglycerides to the level of high-density lipoprotein (HDL)-cholesterol (log[TG/HDL-C]), has recently been used as a marker of atherogenicity. This study included 104 normotensive individuals. Mean platelet volume and AIP were measured in all patients. Nondipper individuals (8.6 +/- 0.9 fL, 0.3 +/- 0.2) demonstrated higher values of MPV and AIP compared with dippers (7.9 +/- 1.1 fL, 0.1 +/- 0.2) (P

WOS: 000379533500009, PubMed ID: 27105876, Fabry disease (FD) is a progressive, X-linked inherited disorder of glycosphingolipid metabolism due to deficient or absent lysosomal alpha-galactosidase A (AGALA) activity. FD and familial Mediterranean fever (FMF) have typical clinical similarities, and both diseases may progress to end-stage renal diseases. In this study, we aimed to determine the prevalence of FD in patients with FMF from Central Anatolia of Turkey. The study group consisted of 177 FMF patients, followed up by the Adult and Pediatric Nephrology Clinic of Cumhuriyet University Hospital. Screening for AGALA activity was performed by the dry blood spot method. Mutation analysis for GLA gene was carried out for patients having an AGALA enzyme activity value lower than the normal reference value. Low AGALA activity was detected in 23 (13 %) patients. Heterozygous GLA gene mutation c.[937G > T] p.[D313Y] was detected in one female patient (0.56 %). The patient was a 53-year-old female with proteinuria and who had undergone left nephrectomy; her glomerular filtration rate (GFR) by scintigraphy was found to be 70 ml/min. She had M694V mutation and no clinical manifestation of FD. In our study, the prevalence rate of FD was found as 0.56 % in FMF patients. The similarities between the symptoms of FMF and FD might lead to a diagnostic dilemma in physicians at countries where FMF is observed frequently. Although the prevalence of FD is rare, physicians should keep in mind that FD has an ambiguous symptomology pattern of FMF.

WOS: 000276897900001, PubMed ID: 20408753, Introduction. Plasma carnitine insufficiency has been known to cause muscle weakness. Carnitine levels and pulmonary functions were lower in patients with diabetes. Patients and Methods. To determine whether pulmonary functions are correlated with carnitine levels in patients with type 2 diabetes. In this study, we evaluated pulmonary functions and carnitine concentrations in 49 patients with type 2 diabetes and 34 healthy controls. Results. Carnitine levels were lower in type 2 diabetes group than control group (52.56 +/- 12.38 and 78.96 +/- 10.66 hmol/mL, respectively, p < 0.0001). Pulmonary functions were not significantly different between groups. Carnitine levels were not correlated with age, duration of diabetes, fasting blood glucose levels, and glycemic control (HbA1c%) in patients with type 2 diabetes. However, carnitine levels in patient group were correlated with % forced vital capacity (FVC%) (r = 0.35, p = 0.016), % forced expiratory volume in 1 s (FEV1%) (r = 0.318, p= 0.029), FEV1/FVC (r= 0.302, p= 0.039), inspiratory muscle strength (PImax) (r = 0.407, p = 0.023), and PImax% (r = 0.423, p= 0.018). Conclusion. This study suggests that low carnitine levels may be associated with lower PImax and PImax% in type 2 diabetes.

WOS: 000290102100044, PubMed ID: 20165923, The Familial Mediterranean Fever (FMF) shows an autosomal recessive pattern of inheritance and affects certain ethnic groups. Disease is caused by mutations in MEFV gene and more than 180 mutations have been defined in affected individuals. Current study aimed to determine the frequency-type of the mutations for MEFV gene in Sivas-middle Anatolian city. The cohort was composed of 3340 patients. MEFV gene mutations were studied by multiplex PCR based reverse hybridization stripAssay method. Patients' clinical features were; family history: 68%, erysipelas-like erythema: 17.6%, fever: 89.9%, abdominal pain: 84.2%, peritonitis: 90.2%, arthritis: 33%, pleuritis: 14.2%, parental consanguinity: 21.2%. Current results revealed that M694V is the most frequent mutation (43.12%), followed by E148Q (20.18), M680I(G/C) (15.00%) and V726A (11.32%). The study population has a high rate of carriers and the E148Q mutation frequency was found to be highest when compared to the other regions of Turkey and other Mediterranean groups.

Introduction: Hemolytic uremic syndrome HUS is a clinical syndrome characterized by non-immune microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. In this study, we aimed to evaluate patients diagnosed with HUS in our clinic. Method: Patients who were diagnosed with HUS as clinic and laboratory between August 2012 and October 2017 were included in our nephrology clinic. Biochemistry, haemogram, ANA, anti-Ds DNA, p-ANCA, c-ANCA, anti-GBM antibody, C3, C4 of patients were studied. In some cases, ADAMTS13 was studied and renal biopsy was performed. The demographic features, clinics, and treatments of our cases were reviewed. Results: A total of 18 patients were enrolled in the study, the average age of the patients was 48.3 21-82 and 10 of the cases were female and 8 of the cases were male. Among the etiologic causes of HUS in patients; the complement factor B mutation, complement factor H polymorphism, breast cancer, herbal medicine, infections and idiopathic were detected. Primer glomerulonephritis was detected in 6 cases of renal biopsy. Conclusion: Clinical and laboratory remission was obtained in 13 patients. End-stage renal failure developed in 3 of our patients.

WOS: 000263540800026, Familial Mediterranean fever (FMF) is the most frequent hereditary inflammatory disease characterized by self-limited recurrent attacks of fever and serositis. The aim of the current study is to determine the frequency of the mutations in 365 suspected FMF patients and to reveal whether there is a correlation between genotype and phenotype of these patients. All patients were clinically examined according to Tell-Hashomer FMF criteria and were screened genetically in terms of common 12 Mediterranean fever gene (MEFV) mutations. Various point mutations were detected in 270 (74%) patients. The most frequent mutation was M694V (26.85% of the alleles) and was followed by E148Q (15.55%), M680I (G/C) (9.62%) and V726A (7.96%). Patients who bear M694V homozygous mutation had most severe disease phenotype and high risk for amyloidosis (P = 0.04). Our results indicate that Sivas population has a wide range of heterozygous mutated carriers of MEFV gene and there is a high frequency of E148Q allele when compared to the other Mediterranean groups.