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Objective To create an efficient and robust mass spectrometric method for the simultaneous quantitation of podocin and podocalyxin in urine samples and to evaluate urinary podocin and podocalyxin levels in patients with nephrotic syndrome (NS). Methods A mass spectrometric method was generated for the measurement of tryptic peptides in urine sediment. Separation of peptides was achieved via liquid chromatography, and mass spectrometric analyses were conducted by electrospray ionization triple-quadrupole mass spectrometry in the multiple reaction monitoring mode. Results Intra- and interassay precision values were below 12% and accuracies ranged from 87% to 111% for both of peptides. The validated method was successfully applied to detect these peptides in patients with NS. Urine podocin and podocalyxin levels were significantly higher in patients with NS compared to healthy controls. Conclusions This proposed mass spectrometric method provides technological evidence that will benefit the clinical field in the early diagnosis and follow-up of NS.

Encapsulated peritoneal sclerosis (EPS) is a rare, but frequently fatal, long-term complication of peritoneal dialysis. Endometriosis is a common gynecological problem but hemoperitoneum due to endometriosis has been reported to be extremely rare in hemodialysis (HD) patients. A 25-year-old female HD patient was admitted to our clinic with nausea, vomiting, abdominal pain, and weight loss for last 3 months. Candida tropicalis and Candida glabrata were isolated in the fungal cultures from peritoneal fluid. Her abdominal computerized tomography scan has shown irregular peritoneal calcifications, diffuse peritoneal thickening, dilatation of the small bowel loops, and cocoon formation which all were typical for EPS. Hemoperitoneum was reported to recur for four times with intervals suggesting menstrual cycles. Her peritoneal biopsy, along with the signs of EPS, has also revealed the presence of endometriosis. The patient died with symptoms of septic shock in the first year of EPS diagnosis.

Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease that may progress to end-stage renal disease, characterized by increased kidney volume due to cystic formations. In this study, we aimed to investigate the relationship between serum uromodulin levels, total kidney volume and estimated glomerular filtration rate (eGFR) in patients with ADPKD. This study included a total of 54 ADPKD patients and 18 healthy volunteers (control group). Total kidney volumes were calculated through magnetic resonance images using ellipsoid method. Serum uromodulin measurements were measured using an ELISA method. Serum uromodulin levels were lower in patients compared with the control group (2.47 ± 0.16 vs 2.6 ± 0.28, p = 0.021). There was no significant difference in uromodulin values among the patients in chronic kidney disease (CKD) stages 1–2, 3 and 4–5. TKV measurements of CKD stage 4–5 patients were significantly higher than the stage 1–2 patients (p = 0.015). A negative correlation was observed between TKV and eGFR (r = − 0.433, p = 0.001). A positive correlation was observed between uromodulin and eGFR (r = 0.274, p = 0.02). When the serum levels of uromodulin and the level of eGFR were evaluated using simple linear regression analysis, R2 value was found to be 0.075, suggesting that 7.5% change in serum uromodulin values corresponds with the change in eGFR value. These findings are consistent with previous studies that reported that serum uromodulin may be a good biomarker for demonstrating renal function in the early stages of CKD, before eGFR levels deteriorate. Serum uromodulin level may be useful in demonstrating renal functions in the follow-up of individuals with ADPKD.

Aim The aim of our study was to determine the factors effecting disease recurrence and graft survival in patients who developed end-stage renal disease (ESRD) due to focal segmental glomerulosclerosis (FSGS) and underwent renal transplantation (Rtx). Methods A total of 37 patients with FSGS (female/male: 10/27) who underwent Rtx in our transplant center between 2001 and 2014 were included in the study. The patients were diagnosed with FSGS by biopsy. Comparative analyses were performed in order to determine the factors effecting disease recurrence and graft survival. Plasmapheresis was performed with 40 mL/kg plasma. The diagnosis of the recurrence of FSGS and the acute rejections were also confirmed by biopsy. Results Statistical analyses revealed that, recurrence rates were higher in Rtx recipients from deceased donor [deceased donor versus living donor, 2 (50.0%) versus 3 (9.1%), P = 0.024]. However, no correlation was found between recurrence and renal replacement treatment (RRT) methods, duration of RRT, preoperative or postoperative prophylactic plasmapheresis, the presence of preoperative nephrotic proteinuria, donor's or recipient's age or gender, kinship with donor, time interval between development of FSGS and ESRD, or performing native nephrectomy. Graft survival rates were higher in Rtx patients that were transplanted from living donor, first-degree relatives, and in patients without recurrence. Conclusion In countries where organ donation is insufficient, living donors can be used with a low risk of recurrence for Rtx candidates with FSGS. Also, grafts from living donors, particularly from first-degree relatives, have higher survival rates.

Our aim was to compare the short- and long-term clinical outcomes of hepatitis B surface antigen–positive (HbsAg+) renal transplant recipients with HbsAg− recipients. A total of 204 patients who underwent renal transplantation in our center between 2001 and 2014 were included in the study. The patients were divided into 2 groups. Group 1 was the HbsAg− group (n = 136), and group 2 was the HbsAg+ group (n = 68). There was no significant difference between the groups in terms of lymphocyte crossmatches, numbers of mismatches, immunosuppressive treatment protocols, and induction treatments. In the HbsAg+ group, 51 patients were hepatitis B virus DNA+, 64 patients were HbeAg−, and 4 patients were HbeAg+. A total of 57 patients (83.8%) were treated with lamivudine, 4 patients (5.9%) with entecavir, and 7 patients (10.3%) with tenofovir for hepatitis B infection. Graft and patient survival rates, graft functions, acute hepatitis rates, acute rejection rates, and other clinical outcomes of the groups were compared. Demographic data and immunologic risk profiles of the groups were similar. Acute rejection rates, graft survival rates, and patient survival rates were similar. Acute hepatitis rates, glomerular filtration rates on the last controls, and delayed graft function rates were higher in group 2, whereas chronic allograft dysfunction and new-onset diabetes mellitus after transplantation rates were similar between the groups. Our study revealed that graft and patient survival, and acute rejection rates were similar between HbsAg+ and HbsAg− recipients, whereas acute hepatitis rate was higher in HbsAg+ recipients.

Aim The aim of this study was to determine the effects of hepatitis B surface antigen (HBsAg) positivity of the donors on graft survival and liver complications in HBsAg(+) renal transplant recipients. Patients and method A group of 55 patients who underwent renal transplantation (RTx) in our hospital between 2001 and 2012 were included in the study. Patients were divided into 2 groups. Group 1 (n = 50) consisted of HBsAg(+) renal transplant recipients (RTR) whose donors were HBsAg(-). In Group 2 (n = 5), RTR and donors were both HBsAg(+). Lymphocyte cross matches, number of mismatches, donor types, renal replacement treatment modalities, drugs of induction treatment, and preoperative hepatitis B virus DNA titers of the groups were similar. In Group 1, 42 patients were taking lamivudine, 3 patients were taking entecavir, and 5 patients were taking tenofovir. All of the patients in Group 2 were taking lamivudine. Patient and graft survival rates, graft functions, acute hepatitis rates, acute rejection rates, and other clinical outcomes of the groups were compared. Results Demographic data of the groups were similar. Acute rejection rates (P = 0.458), graft survival rates (P = 0.515), and patient survival rates (P = 0.803) were also similar. No significant difference was found between the groups in terms of acute hepatitis rate (P = 0.511), glomerular filtration rate (calculated by Modification of Diet in Renal Disease formula) in the last follow-up (P = 0.988), alanine aminotransferase levels (P = 0.069), or delayed graft function rate (P = 0.973). Rates of chronic allograft dysfunction and new onset diabetes mellitus after transplantation were similar. Conclusion Our study revealed that, RTx from HBsAg(+) donors to HBsAg(+) recipients is safe with antiviral treatment.

Diabetic nephropathy (DN) is one of the most important causes of the end-stage renal failure and its prevalence is found to be increasing. The presence of hypertension and progressive proteinuria is among the important findings. In this study, the effects of double and triple combinations of trandolapril, telmisartan, and verapamil on proteinuria were investigated in diabetic patients with nephropathy. Seventy-eight patients (mean age: 56.11 ± 11.26 years; 47 females and 31 males) with overt proteinuria and DN were included in this study. The patients were divided into four groups: Group I (n: 18, trandolapril + telmisartan), Group II (n: 20, trando- lapril + verapamil), Group III (n: 20, trandolapril +telmisartan + verapamil), and Group IV (n: 20, telmisartan + verapamil). At the end of a three-month therapy, within and between group compa- risons were done about the effects of the use of double or triple drug combinations on proteinuria, glomerular filtration rate (GFR), electrolytes, serum albumin, low-density lipoprotein (LDL)- cholesterol, and HbA1C. There was no significant difference among groups in terms of age, gender, diabetes duration, body mass index, and retinopathy frequency. The decreases in protei- nuria and mean arterial blood pressure (MABP) were significant in all groups. The decrease in proteinuria was independent of the decrease in MABP [the reduction rate in proteinuria was 39% (P

CD200 is a novel immune-effective molecule, existing in a cell membrane-bound form, as well as in a soluble form in serum, which performs to modulate inflammatory and acquired immune responses. Autosomal dominant polycystic kidney disease (ADPKD) is characterized by the development of large renal cysts and progressive loss of renal function. As defects in cell cycle arrest and apoptosis of renal tubular epithelial cells occur in ADPKD, we asked whether serum soluble CD200 might underlie and effect on ADPKD. Serum soluble CD200 levels were measured in 44 patients with ADPKD and 24 healthy volunteers. Concentrations of soluble CD200 in the serum samples were quantified using an ELISA kit. The mean serum soluble CD200 levels were higher in patients with ADPKD than in the control group (71.4±29.2 and 21.4±5.6 pg/mL, p0.05). Our results show that patients with ADPKD have activated soluble CD200 levels which were related to renal function and inflammation.

Focal segmental glomerulosclerosis (FSGS) and other glomerulonephritis due to the use of 5-aminosalicylic acid derivatives have been reported in the literature. A 38-year-old male who had been using mesalazine for four years because of ulcerative colitis applied to doctor due to swelling in the lower extremities. The patient was diagnosed with nephrotic syndrome (NS). Renal biopsy was performed, and FSGS was diagnosed. Antiproteinuric treatments were initiated with steroid therapy. The patient has been followed with the normal renal function of the after treatment. 5-aminosalicylic acid derivatives affect renal functions at different levels and caused in NS. © 2020 Saudi Center for Organ Transplantation. WOS:000568241100028 32394929

Objective We aimed to determine the prevalence of sexual dysfunction and clarify the relationship between sexual dysfunction and depressive mood state, drugs, and disease activities in patients with predialytic chronic kidney disease (CKD). Materials and Methods In total, 150 patients with CKD who had an estimated glomerular filtration rate of 15-60 mL/min were included; 65 healthy controls were selected. A detailed medical and sexual medical history was taken from individuals in the control and patient groups by applying the Golombok-Rust Inventory of Sexual Satisfaction and Hospital Anxiety and Depression Scale. Results Sexual frequency (p=0.027), impotence (p

The aim of the present study was to evaluate the variations of some major bone metabolism markers with reference to klotho gene polymorphism (KGP) and bone mineral density (BMD) values in patients on chronic peritoneal dialysis (CPD).In 51 CPD patients and 40 healthy persons, assays for intact parathormone (iPTH), fibroblast growth factor 23 (FGF-23), osteoprotegerin (OPG), osteocalcin (OC), procollagen type-1 N terminal propeptide (PINP), beta- crosslaps (beta CTx), tartrate resistant acid phosphatase (TRAP5b), bone alkaline phosphatase (BAP), 1,25(OH)D3, and 25(OH)D3 and α-klotho gene mutations were performed.In CPD patients, 1,25(OH)D3 and 25(OH)D3 deficiency rates were 96% and 94% respectively. iPTH (249 pg/mL vs 39 pg/mL) and FGF-23 (1089 RU/mL vs 153 RU/mL), OPG, OC, PINP, beta CTx, TRAP5b levels were significantly higher in patients. iPTH levels and whole-body BMD values were negatively correlated in patients. The rate of KGP was similar in all groups.In CPD patients, besides vitamin D deficiency, high levels of OPG, OC, PINP, beta CTx, TRAP5b were evident. Positive correlation between iPTH levels and BAP and PINP levels suggested a diagnostic value for those markers during the management of CKD MBD. On the other hand, high serum TRAP5b concentrations did not seem to be affected by neither calcitriol treatment nor the severity of hyperparathyroidism. iPTH and FGF-23 levels and whole-body BMD values showed a significant negative correlation. We were unable to show any correlation between KGP and any of the CKD-MBD parameters measured in this study.Çalışmamızda kronik periton diyalizi (KPD) hastalarında klotho gen polimorfizmi (KGP) ve kemik mineral yoğunluğu (KMY) ile bazı major kemik metabolizma belirteçleri arasındaki ilişkinin araştırılması amaçlanmıştır.Çalışmaya dahil edilen 51 KPD hastası ve 40 sağlıklı kontrol grubunda intakt parathormon (iPTH), fibroblast büyüme faktör (FGF-23), osteoprotogerin (OPG), osteokalsin (OK), prokollajen tip-1 N terminal propeptid (PINP), beta- crosslaps (beta KTx), tartarate resistan asid fosfataz (TRAF5b), kemik alkalen fosfataz (KAF), 1,25(OH)D3, 25(OH)D3 ve α-klotho gen mutasyonları ölçüldü.51 KPD hastasında 1,25(OH)D3 ve 25(OH)D3 eksikliği oranı sırasıyla %96 ve %94 olarak tespit edildi. iPTH (249 pg/mL ve 39 pg/mL) ve FGF-23 (1089 RU/mL ve 153 RU/mL), OPG, OK, PINP, beta CTx, TRAP5b seviyeleri hastalarda istatistiksel olarak anlamlı derecede daha yüksek bulundu. Hastalarda iPTH seviyeleri ve tüm vücut KMY arasında negatif korelasyon tespit edildi. KGP oranı gruplar arasında benzer bulundu.Kronik periton diyalizi hastalarında D vitamin eksikliğine ek olarak, OPG, OK, PINP, beta CTx, TRAP5b düzeyleri yüksek saptandı. iPTH seviyeleri ile KAF ve PINP arasındaki pozitif korelasyon bu belirteçlerin kronik böbrek hastalarındaki (KBH) kemik mineral hastalıklarının (KMH) tedavisinde tanısal değerinin olduğunu göstermiştir. Diğer taraftan yüksek serum TRAP5b konsantrasyonunun kalsitriol tedavisi ve hiperparatiroidinin şiddetinden etkilenmediği de gösterilmiştir. iPTH ve FGF-23 düzeyleri ile tüm vücut KMY arasında anlamlı negatif korelasyon tespit edilmiştir. Çalışmamızda klotho gen polimorfizmi ile KBH-KMH’nın hiçbir belirteci arasında anlamlı korelasyon gösterilememiştir.

Background: Bone disorders are next to cardiovascular problems in frequency in renal transplant (RT) recipients. Reduction in 1,25-dihydroxycholecalciferol (1,25D) levels is among the reasons causing bone loss in these patients. Klotho (KL) serves as a co-receptor for fibroblast growth factor 23 (FGF23), and functions in vitamin D metabolism. KL polymorphisms have been identified in several studies, and phenylalanine to valine substitution at amino acid position 352 seemed to be important to KL function. We investigated KL F352V polymorphism and its relation with 1,25D levels in RT recipients. Methods: The study included 25 RT recipients (8 female, 17 male) and 26 (14 female, 12 male) healthy control subjects who were wild (FF) phenotypes in terms of KL F352V polymorphism. RT recipients with (FV, n = 11) and without (FF, n = 14) a heterozygote polymorphism were determined with high resolution DNA melting analysis of KL F352V polymorphism. Serum 1,25D levels were measured using the RIA method. Results: RT recipients with FV phenotype had significantly lower 1,25D levels (17.58 ± 18.38 pg/ml) compared to recipients with FF phenotype (44.91 ± 24.68 pg/ml) and control subjects (28.24 ± 12.13 pg/ml). 1,25D levels in RT recipients with FF phenotype were significantly higher than control subjects. Conclusions: KL F352V polymorphism may increase the expression of FGF23 co-receptor, KL protein and thus may decrease renal expression of 1α-hydroxylase, and/or stimulate 24-hydroxylase in RT recipients. The resultant decrease 1,25D levels may participate in bone loss in these patients.

New-onset diabetes after transplantation and impaired glucose tolerance are very common in renal transplant patients. New-onset diabetes after transplantation (NODAT) is associated with increased cardiovascular morbidity and mortality, reduced graft and patient survival. Several risk factors for NODAT have been identified: age, obesity, family history of diabetes mellitus and HCV infection. In addition, steroid and calcineurin inhibitors also contribute to the development of NODAT. Sirolimus causes immunosuppressive effects by inhibiting mammalian target of rapamycin (mTOR), and has well known side effects. The effects of sirolimus on glucose metabolism and contribution to NODAT development are not clearly known. In this report, we presented five RTX patients who developed NODAT under the treatment of sirolimus.

The present study was aimed to evaluate erythrocyte folate and the iron levels in diabetes and hypertension patients treated with/without hemodialysis. The effects of erythropoietin and iron treatment as well as vitamin supplementation on measured parameters were considered. The 67 controls consisted of healthy subjects (n = 22), hypertensive subjects (n = 22), and diabetic subjects (n = 23) without any renal disorder. According to primary renal disorders, the patients undergoing hemodialysis (n = 68) were classified into four groups as diabetic nephropathy, hypertensive nephropathy, reflux nephropathy or interstitial nephritis, and renal insufficiency depending on other causative factors. The mean value of erythrocyte folate levels of all patients undergoing hemodialysis was higher than the healthy control group (P 0.05). The only significance observed within the subgroups was between diabetic controls and diabetic nephropathy patients (P 0.05). The increase in erythrocyte folate status of patients with end stage renal diseases might be the result of sum or individual effects of causative factors such as renal pathology, compensation mechanism against renal anemia, or routine folate supplementation.

This study aims to determine fibroblast growth factor-23 and soluble α-Klotho levels in patients with autosomal dominant polycystic kidney disease. A total of 76 patients with autosomal dominant polycystic kidney disease and 32 healthy volunteers were included in the study. Serum fibroblast growth factor-23 and soluble α-Klotho levels were measured with ELISA kits. Parathyroid hormone, phosphate, calcium, creatinine, 25-hydroxyvitamin D3 levels, urinary protein to creatinine ratio and estimated glomerular filtration rate were also measured or calculated. Patients with autosomal dominant polycystic kidney disease had significantly higher serum parathyroid hormone (p0.05). Fibroblast growth factor-23 (r=−0.251, p=0.034) and soluble α-Klotho levels (r=−0.251, p=0.034) were found to be negatively correlated with estimated glomerular filtration rate. This study shows increased fibroblast growth factor-23 levels in patients with autosomal dominant polycystic kidney disease which is in harmony with the general trend in patients with chronic kidney disease of other aetiologies, but, unlike them, also a significant increase in serum soluble α-Klotho levels in patients with autosomal dominant polycystic kidney disease suggesting an aberrant production or a decreased clearance of α-Klotho molecule. Considering the unique increases in erythropoietin levels due to erythropoietin production in renal cysts, we assume, patients with autosomal dominant polycystic kidney disease may potentially have different soluble α-Klotho production/clearance characteristics than the patients with other parenchymal renal diseases.

RESultS: The mean age at the start of PD was 51.9 ± 17.6 years and the mean PD duration was 27.5 ± 27.1 months (median: 20, range: 3–166). Hypertension was the most common cause of ESRD (36.1%), followed by diabetes mellitus (31.3%). Among these 313 patients, 58 (18.5%) died. Patients’ survival rates were 93.3%, 85%, and 72.4%, at one, three, and five years, respectively. A higher educational level (p=0.045), lower age (p=0.002), higher baseline creatinine (p=0.045), and higher baseline albumin level (p=0.003) were found to affect patients’ survival. Technique survival rates were 89.1%, 84%, and 74.1% at one, three and five years, respectively. Technique failure was associated with peritonitis rate (p=0.03).

Lupus nephritis (LN) is a type of organ involvement of systemic lupus erythematosus (SLE) that leads to disease-related morbidity and mortality. Lack of good treatments for LN continues to be problematic. Many different treatment protocols are applied in treatment centers. Not every treatment protocol is successful. Moreover, patients who reached remission may present with exacerbations. In this study, we aimed to evaluate the treatment results of our patients and investigate their remission rates as well as factors that affect remissions.We retrospectively investigated the results of 41 patients who were diagnosed with lupus nephritis after kidney biopsy in the Nephrology and Immunology-Rheumatology departments of Atatürk University Medical Faculty Training Hospital between January 2000 and December 2008. Demographic information, clinical history and laboratory results were collected from each patient's records. The relationships among clinical, laboratory, demographic parameters and remissions were investigated. The patients were grouped in terms of urine protein levels; patients with urine protein330 mg/day were regarded as in remission and patients with urine protein ≥ 330 mg/day were were regarded as uncontrolled.At the end of a 12-month period of therapy, 24 (58.5 %) of the patients were in remission. There were no statistically significant relationships among age, sex, anti-ds-DNA, C3, C4, activity indexes, chronicity indexes, serum level of creatinine, urine protein levels and remission (p0.05). We compared class 3 LN patients at the 6th and 12th months according to treatment protocols. Azathioprin or mycophenolate mophetil were significantly better at placing urine protein levels in remission as compared to cyclophosphamide (p0.05).According to our study, no relationship was found between basal clinical and laboratory parameters and patient remission. Response rates of our LN patients were similar to those in the literature. However, complete remission is still a problem in LN. The results of the protocols used in the treatment of LN show similarities. Although there are some data suggesting that MMF used in recent years is effective, it should be supported by prospective multicenter studies. It is important to note that it is difficult to achieve complete remission in LN patients.Lupus nefriti (LN), Sistemik lupus eritematozisin mortalite ve morbiditesine neden olan organ tutulumlarındandır. LN tedavisi, önemli bir problem olmaya devam etmektedir. Merkezler farklı tedavi protokolleri uygulamaktadır. Hiçbir protokol kesin tedavi sağlamadığı gibi, remisyondaki bir hastada daha sonra hastalık alevlenmesi gözlenebilmektedir. Bu çalışmada, merkezimizde takip ettiğimiz hastaların tedavi sonuçlarını değerlendirmeyi, remisyon oranlarımızı ve remisyona etki eden faktörleri araştırmayı amaçladık.Çalışmamızda, Ocak 2000-Aralık 2008 tarihleri arasında Atatürk Üniversitesi Tıp Fakültesi Nefroloji ve Romatoloji-İmmünoloji kliniklerinde böbrek biyopsisi sonucu LN tanısı konularak tedavi başlanan 41 hastanın tedavi sonuçları retrospektif olarak değerlendirildi. Hastalara ait bilgilere hasta dosyalarından ulaşıldı. Hastaların klinik, laboratuar ve demografik özelliklerinin remisyonla ilişkisi araştırıldı. Proteinürisi 330 mg/gün altında olan hastalar remisyona girmiş grup olarak, 330 mg/gün’ün üstünde olan hastalar da remisyona girmemiş grup olarak gruplandırıldı.Bir yıllık tedavi sonrasında hastaların 24’ü (%58.5) tam remisyona girdi. Yaş, cinsiyet, Anti ds-DNA, Kompleman 3 ve 4, aktivite ve kronisite indeksleri, serum kreatinin ve proteinüri düzeyleri ile remisyon arasında bir ilişki bulunamadı (p0.05). Klas 3 LN olan hastaların idame tedavisinde kullanılan azatioprin veya mikofenolat mofetil (AZA/MMF) ile siklofosfamid (CyP)’in 6. ve 12. aydaki proteinüri düzeyleri karşılaştırldığında, AZA/MMF alan grupta proteinüri düzeyleri CyP grubuna göre anlamlı düzeyde azalmaktaydı (p=0.04).Sonuç olarak, çalışmamızda hastaların bazal klinik ve laboratuar parametreleri ile remisyon arasında bir ilişki bulunamamıştır. Lupus nefritli hastalarda uygulamış olduğumuz tedavi rejimlerine alınan sonuçlar literatürdeki verilerle benzerlik göstermektedir. Bununla birlikte LN’de tam remisyon hala sorun olmaya devam etmektedir. LN’nin tedavisinde kullanılan protokollerin sonuçları birbirine benzerlik göstermektedir. Son yıllarda kullanılmakta olan MMF’in etkili olduğuna dair veriler olsa dahi, prospektif çok merkezli çalışmalarla desteklenmelidir. Daha etkili ve yan etkisi daha az olan yeni tedavi rejimlerine ihtiyaç vardır.

Familial Mediterranean fever (FMF) is an autosomal recessive hereditary disease. Clinical symptoms and findings (phenotypes) seen in this disease are generally fever, abdominal pain, and arthritis. Amyloidosis is also a significant complication. Phenotype-genotype correlations in FMF have not been conclusively resolved. The aims of this study were to find the most frequent mutation/genotype of FMF, as well as to investigate the role of genetic factors on the phenotype and on the development of amyloidosis in a population living in East Anatolia (Turkey). This study included 105 adult patients with FMF. DNA samples were obtained from peripheral blood lymphocytes of the patients. Mutations of the Mediterranean fever (MEFV) gene were analyzed with an FMF Strip Assay test kit (ViennaLab Labordiagnostika GmbH, Vienna, Austria). Patients were separated according to genotypes, and phenotypes were compared statistically by the chi-square test.The most frequent mutation was M694V (53%) and the most frequent genotype was M694V/M694V (26%). In total, 81% of the patients experienced abdominal pain, 76% had fever, and 22% had arthritis. Fever and arthritis were determined in similar ratios to other genotypes (76% and 19%, respectively) in the M694V/M694V genotype (74% and 29%, respectively) (p0.50 and p0.20, respectively). However, the patients without the M694V/M694V genotype (86%) had a higher abdominal pain ratio than did the patients with the M694V/M694V genotype (67%) (p0.05). Renal amyloidosis was determined in 33% of both M694V/M694V and M680I(G/C)/M680I(G/C) homozygous groups and in 12% of the heterozygous groups (p0.02 and p0.00002, respectively). In other words, homozygous groups had higher ratios of renal amyloidosis.The most frequent mutation in FMF was M694V and the most frequent genotype was M694V/M694V. Fever, abdominal pain, arthritis, and renal amyloidosis were determined not only in patients with M694V/M694V genotype but also in other genotypes. Therefore, genotypes may not predict phenotypes in FMF. Renal amyloidosis was seen more frequently in homozygous genotypes.

Background/aims This study evaluated the effect of telmisartan on the livers of diabetic rats and also aimed to determine the hepatic distribution and role of transforming growth factor β (TGF-β) in diabetes-related hepatic degeneration while taking into account the possible protective effects of telmisartan. Methods Fifteen adult male rats were used and divided into three groups: the non-diabetic healthy group, alloxan-induced diabetic control group, and the alloxan-induced diabetic telmisartan group. The non-diabetic healthy group and the diabetic control group were exposed to saline for 30 days, while the group treated with diabetic drugs was orally administered telmisartan for 30 days (10 mg/kg/day). At the end of the experiment, the rats were sacrificed and the livers were dissected and transferred into the fixation solution. The livers were then evaluated using stereological and histopathological methods. Results Our study of the numerical density of hepatocytes shows a significant difference between the diabetic control group and diabetic rats treated with telmisartan. Immunohistochemical staining for TGF-β in liver sections of the diabetic rats treated with telmisartan showed no immunoreactivity. The diabetic control group was determined to be strongly immunoreactive to TGF-β. Conclusion Results suggest that telmisartan may reduce type-I diabetes mellitus-induced hepatic injury by suppressing activated hepatic stellate cells through concomitant TGF-β1 down-regulation.

This study was carried out to determine lumbar and femoral bone mineral density (BMD) in patients with familial Mediterranean fever (FMF), an autosomal-recessive disease characterized by recurrent episodes of peritonitis, pleuritis, and arthritis, which are usually associated with fever. In patients with FMF and control subjects, erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were measured. BMD was determined at the lumbar spine (L1-4) and the femoral regions (neck and total) using dual energy X-ray absorptiometry. Twenty-eight FMF patients and 30 control subjects without a history of inflammatory disease participated in our study. The demographic variables, such as age, sex and body mass index were similar between patients and controls (P > 0.05). We found statistically significant difference in ESR and CRP between FMF patients and controls (P < 0.01, P < 0.05 respectively). There was statistically significant difference in lumbar spine, femoral neck, and total femur BMD between FMF patients and control groups (P < 0.001, P < 0.01, P < 0.01 respectively).Our study indicates that lumbar spine and femoral neck and total femur BMD in patients with FMF may be lower than in healthy subjects.

Aim: Von Willebrand Factor is a glycoprotein, which plays role in primary hemostasis, mainly acts getting platelets to adhere onto the subendothelial tissue and carrying Factor VIII. Although vWF deficient animals had been shown to be resistant to atherosclerosis and thrombotic events, it had not been proven in humans. In this study, we aimed to investigate whether a relationship exists between VWF activity and therapeutic success of thrombolysis. Methods: The study was carried out on 40 patients who were administered thrombolytic therapy due to myocardial infarction and 30 healthy controls. Von Willebrand Factor activity was measured in plasma samples which were taken before the procedure. The results were compared between therapy effective and ineffective patients and controls. Results: Average vWF activity was 131.0±64.0% in patients whereas 113.9±47.7% in controls (p>0.05). On the other hand, mean VWF activities of 24 patients who achieved therapeutic success and 16 therapy failed patients were 103.9±58.9% and 171.7±49.0% respectively (p

Purpose : To investigate the dual effect of angiotensin blockade by irbesartan and enalapril on proteinuria in diabetic patients with azotemia. Methods : Patients with diabetes of > 5 years duration, proteinuria at a nephrotic level and serum creatinine > 1.5 mg/dL were enrolled in the study. Forty-five enrolled patients were divided into three groups, those receiving enalapril , irbesartan, or enalapril plus irbesartan, respectively, over a period of 24 weeks. Urinary protein excretion and serum level of albumin, creatinine, potassium were measured before and after treatment Results : In patients receiving enalapril, irbesartan, and both drugs concomitantly, mean urinary protein excretion level decreased significantly at the end of 6 months from 6.46 ± 4.66 to 3.36 ± 1.60, 5.89 ± 5.34 to 3.22 ± 1.72 and 5.99 ± 3.77 to 2.10 ± 2.22 g/day, respectively (p = 0.001). Decrease in proteinuria in the group receiving the combined therapy was more significant than the other two groups (p = 0.025). During the period of therapy, serum albumin increased and mean arterial pressure decreased significantly (p = 0.02 and p = 0.002, respectively) but serum creatinine and potassium and creatinine clearance values showed insignificant increases (p = 0.28 and p = 0.57, respectively). Conclusion : The combined use of enalapril and irbesartan, in patients with diabetic nephropathy associated with azotemia, is more effective in decreasing proteinuria without causing any substantial increase in serum potassium levels. The combined use of these two drugs shows a more pronounced anti-proteinuric effect. Keywords : Angiotensin-converting enzyme inhibitor, Angiotensin receptor blocker, Diabetic nephropathy, Azotemia, Proteinuria, Aldosterone, Renin, Blood pressure

Background The aim of this study was to evaluate the long-term outcomes of renal transplantation from Hbs Ag-positive donors to Hbs Ag-negative recipients. Material and methods A total of 78 patients who underwent renal transplantation in our clinic between January 2006 and May 2014 were included in the study. Patients were divided into 2 groups: Group 1: Donor Hbs Ag (+) (n=26, Hbs Ab (-), Hbe Ag (-), Hbe Ab (+), Hbc Ig total (+) and HBV DNA (+), male/female (M/F): 16 (61.5%)/10 (38.5%), and Group 2: Donor Hbs Ag (-) (n=52, M/F: 41 (78.8%)/11 (21.2%). Hbs Ab levels were similar in recipients in both groups. Data were collected retrospectively. Analyses were performed by using SPSS 20.0 software, and patient and graft survival were measured by using Kaplan-Meier survival curve and compared by using the log-rank test. Results Demographic data were similar in the 2 groups. The rate of acute Hepatitis B infection was significantly higher in Group 1 than in Group 2 [n=3 (11.5%) vs. n=0 (0%), respectively, p=0.012]. Acute hepatitis B attacks were detected in vaccinated patients. Graft survival rates (groups 1 and 2, respectively; at 1st, 3rd, 5th and 8th years: 95% vs. 96%, 95% vs. 94%, 85% vs. 88%, 85% vs. 82%, p=0.970) and patient survival rates (p=0.098), acute rejection rates (p=0.725), delayed graft function, chronic allograft dysfunction, new-onset diabetes after transplantation (NODAT), cytomegalovirus infection, and the need for postoperative dialysis and plasmapheresis were similar between groups. Conclusions Our study revealed that the risk of developing acute hepatitis B was higher in patients renally transplanted from Hbs Ag (+) donors, but the other clinical outcomes were similar between groups.

The kidney is often affected in plasma cell dyscrasias, usually due to the effects of nephrotoxic monoclonal-free light chains. Renal failure due to a monoclonal gammopathy may be detected by the highly sensitive serum-free light-chain (sFLC) ratio yet missed by electrophoretic assays. The aim of this study was to assess sFLC levels in relation to markers of renal function.Five-hundred thirteen patients were included in this study. sFLC levels were measured by Freelite® (The Binding Site Group Ltd, Birmingham, UK) assay using the BNII nephelometer (Siemens Diagnostics, Germany). Kappa/lambda (κ/λ) sFLC ratio was calculated. Serum creatinine levels were analyzed by modified Jaffe method in Cobas 8000 analyser. GFR was estimated by the CKD-EPI (Chronic Kidney Disease Epidemiology Collaboration) equation. Patients were assigned to two groups depending on their eGFR values: ≤ 60 mL/min/1.73 m(2) (Group 1, n = 103) and60 mL/min/1.73 m(2) (Group 2, n = 410). Data were expressed as median and min-max. All the statistical analyses were done with SPSS version 20.0 and a significance level of 0.05 was considered.Serum κ-FLC median value was 36.4 (5.62-16,000) mg/L, serum λ-FLC was 21.7 (4.91-8770) mg/L, κ/λ sFLC ratio was 1.33 (0.01-3258) and serum creatinine was 1.56 (0.63-7.21) mg/dL in Group 1. Both λ sFLC and κ/λ sFLC ratios were correlated with eGFR (r = -0.318, r = 0.198, p0.05, respectively). We did not find any significant correlation between κ/λ sFLC ratio and eGFR in Group 2.We examined the association between sFLC concentrations and renal function. Our preliminary findings suggest that serum λ-FLC might be considered as a useful marker for predicting renal function. Prospective studies are needed to clarify the usefulness of these parameters for identifying renal failure due to a monoclonal gammopathy.

Objectives Renal transplantation (RT) is the most appropriate form of treatment for end-stage renal disease (ESRD). Pre-emptive RT decreases the rates of delayed graft function and acute rejection episodes, increasing patient and graft survival, while reducing costs and complications associated with dialysis. In this study, we investigated the relationship between a predialysis education program (PDEP) for patients and their relatives and pre-emptive RT. Methods We divided 88 live donor kidney transplant recipients into 2 groups: transplantation without education (non-PDEP group; n = 27), and enrollment in an education program before RT (PDEP group n = 61). Results Five patients in the non-PDEP group underwent pre-emptive transplantation, versus 26 of the PDEP group. The rate of pre-emptive transplantations was significantly higher among the educated (42.62%) versus the noneducated group (18.51%; P Conclusion PDEP increased the number of pre-emptive kidney transplantations among ESRD patients.

Summary The benefits of angiotensin-converting enzyme inhibitors and angiotensin II (ATII) receptor antagonist therapy of diabetic nephropathy (DNP) are thought to be largely the result of attenuation of ATII effects on proteinuria. The aim of the study was to ascertain whether there is the additive anti-proteinuric effect of enalapril plus losartan in DNP. Twenty-two patients with DNP were studied. Patients were randomly assigned to enalapril 10 mg/day (11 patients) or losartan 50 mg/day (11 patients) administered in a single oral dose in the morning for 12 weeks. and then, in 10 patients (five patients from enalapril group and five patients from losartan group), combination therapy (10 mg/day enalapril and 50 mg/day losartan) was started and continued for 12 weeks. In 12 patients, initial drugs dosages were doubled (six patients 20 mg/day enalapril and six patients 100 mg/day losartan), and monotherapy was continued for 12 weeks. Blood pressure and proteinuria were measured before and after therapy. Adverse effects were recorded at every visit. Proteinuria decreased by 33% with enalapril and losartan administered alone (p

Background: Familial Mediterranean fever (FMF) is a genetic multisystem disease, characterised by recurrent episodes of fever, peritonitis, pleuritis and arthritis. The most dangereous complication of the FMF is amyloidosis leading to end stage renal disease. The purpose of this study was to evaluate clinical and laboratory characteristics of patients with amyloidos due to FMF. Methods: This study was done in Ataturk University Hospital, situated in the east of Anatolia. A total of 51 patients with systemic AA amyloidosis due to FMF were evaluated retrospectively for clinical and laboratory parameters. Results: All patients were Turks. Of these, 25 (49.3%) had family history, 41 (80.3%) abdominal pain, 4 (7.8%) chest pain, 14 (27.4%) arthritis, 2 (3.9%) febrile myalgia, 1 (1.9%) erysipelas-like skin lesions and 34 (66.6) episodic fever. Hepatomegaly, splenomegaly and oedema were recorded in 22 (43.1%), 28 (54.9%), and 32 (62.7%) of patients respectively. Scrotal swelling was observed in 3 (5.8%) ...

OZET Son 6 aydir surekli afyon kullanan ve kas gucsuzlugu sikâyeti olan 60 yasindaki hipertansif ve diyabetik erkek hasta, rabdomiyoliz ve akut bobrek yetmezligi nedeniyle hastanemize gonderildi. Laboratuvar incelemesinde serum kreatinin kinaz, kreatinin ve laktat dehidrogenaz enzim yuksekligi saptandi. Intravenoz hidrasyon, bikarbonat ve mannitol tedavisi uygulandi. Takipler esnasinda serum kreatin kinaz duzeyleri ve bobrek fonksiyon testlerinde gerileme oldu. Opiyat iceren ilaclarin akut zehirlenmeleri rabdomiyolize neden olabilirken; surekli afyon kullanimi da rabdomiyoliz nedenlerinden biridir. Bu yazida afyon kullanimi sirasinda rabdomiyoliz ve akut bobrek yetmezligi gelisen bir olgu sunuluyor ve surekli afyon kullaniminin rabdomiyoliz ve onunla iliskili akut bobrek yetmezligine neden olabilecegi vurgulaniyor. Anah tar kelimeler: Akut bobrek hasari, opiyum, rabdomiyoliz

Adult coeliac disease commonly presents without classical symptoms as chronic diarrhea and weight loss. We describe the case of a 31-year-old woman with persistent life-threatening hypokalemia, acute renal failure, and acute quadriplegia due to diarrhea that had continued for one month. Although there are cases of coeliac disease diagnosed with hypokalemic rhabdomyolysis in the literature, none of the cases developed acute renal failure. This is the first case in the literature diagnosed with acute renal failure due to hypokalemic rhabdomyolysis as a presentation of coeliac disease. In acute renal failure cases that present with hypokalemic rhabdomyolysis due to severe diarrhea, coeliac disease should be considered as a differential diagnosis despite the negative antigliadin IgA antibody. Turkish Başlık: Çöliak hastalığı prezentasyonu olarak hipokalemik rabdomiyoliz ilişkili akut böbrek yetmezliği Anahtar Kelimeler: Çöliak hastalığı, akut böbrek yetmezliği, hipokalemik rabdomiyoliz Erişkin çöliak hastalığı sıklıkla kronik ishal ve kilo kaybı gibi klasik semptomlar olmaksızın görülür. Biz ishale bağlı hayatı tehdit edici hipokalemi, akut böbrek yetmezliği ve akut quadripleji gelişen 31 yaşında bir kadın çöliak hastası sunmaktayız. Literatürde hipokalemik rabdomiyolizli çöliak hastaları sunulmasına karşın akut böbrek yetmezliği gelişmiş hasta bildirilmemiştir. Bu vaka hipokalemik rabdomiyolize bağlı akut böbrek yetmezliği ile başvuran ilk çöliak hastasıdır. Şiddetli ishale bağlı hipokalemik rabdomiyoliz ile başvuran akut böbrek yetmezliği vakalarında anti gliadin IgA antikoru negatif olsa bile ayırıcı tanıda çöliak hastalığı da düşünülmelidir.

Objective -The aim of this study was to investigate the potential effects of haemodialysis on left atrial (LA) mechanical functions in patients with chronic renal failure. Methods- Thirty-two patients with chronic renal failure (mean age 42.8 ± 19.6 years) were included in this study. LA volumes were determined echocardiographically at the time of mitral valve opening (maximal,Vmax), at the onset of atrial systole (p wave at the electrocardiography = V P ) and at the mitral valve closure (minimal, V min ) according to the biplane area-length method in apical 4-chamber and 2-chamber view. All volumes were corrected to the body surface area, and the following left atrial emptying functions were calculated. LA passive emptying volume = V max - V P , LA passive emptying fraction = LA passive emptying volume/V max . Conduit volume = LV stroke volume-(V max - V min ), LA active emptying volume = V P -V min . LA active emptying fraction = LA active emptying volume /V P , LA total emptying volume = (V max - V min ), LA total emptying fraction = LA total emptying volume / V max . Results - Mean fluid removal was 1875 ± 812 milliliter. There was no difference between in the LA passive emptying volume before and after dialysis (10.83 ± 7.44 vs. I1.47 ± 7.73 cm 3 /m 2 , p >0.05). Conduit volume (from 15.30 ± 10.68 to 10.31 ± 6.83 cm 3 /m 2 , p < 0.05), LA active emptying volume (from 12.61 ± 6.39 to 9.25 ± 4.40 cm 3 /m 2 , p < 0.005), LA total emptying volume (from 23.44 ± 8.52 to 20.72 ± 8.58 cm 3 /m 2 , p < 0.05), LA maximal volume (from 39.44 ± 14.07 to 28.89 ± 11.80 cm 3 /m 2 , p < 0.001), LA minimal volume (from 15.99 ± 9.70 to 8.17 ± 4.52 cm 3 /m 2 , p < 0.001), and the volume at the onset of atrial systole (from 28.61 ± 10.36 to 17.42 ± 7.20 cm 3 /m 2 , p < 0.001) decreased significantly after the haemodialysis session, whereas LA passive emptying fraction (from 0.27 ± 0.14 to 0.38 ± 0.14%, p < 0.001), LA active emptying fraction (from 0.46 ± 0.18 to 0.53 ± 0.17%, p < 0.05), LA total emptying fraction (from 0.61 ± 0.14 to 0.72 ± 0.09%, p < 0.001) increased significantly after haemodialysis. Conclusion -The results of this study suggest that left atrial mechanical functions improve after haemodialysis in patients with chronic renal failure.

The thyroid functions of 42 subjects with bipolar affective disorder receiving regular lithium therapy were analysed and their thyroid glands were examined by ultrasonography. Following the receipt of lithium therapy (duration 4–156 months), three subjects displayed subclinical hypothyroidism (7.1%), three subclinical hyperthyroidism (7.1%) and one hyperthyroidism (2.4%). Moreover, goitre was detected in 16 (38.1%) subjects. An increase in the conversion of free thyroxine (T4) to free tri-iodothyrosine (T3), which is an indication of mild thyroid dysfunction, was identified in 20 (47.6%) subjects, and was mostly seen in male subjects under 40 years of age and in those having weight gain. In conclusion, some thyroid dysfunctions were observed in the patients treated with lithium.

Intercellular adhesion molecule-1 (ICAM-1) is a membrane-bound molecule primarily involved in cell-cell adhesive interactions of the immune system. It is a cytokine-induced glycoprotein involved in the recruitment of cells into tissues undergoing inflammatory responses. The levels of soluble ICAM-1 were measured in sera of patients with systemic lupus erythematosus (SLE) using sandwich enzyme-linked immunoassay. Serum levels (mean +/- SD) of soluble ICAM-1 (sICAM-1) were significantly higher in 24 patients with SLE than in 20 controls (372+/-42 ng/dL vs 231+/-29 ng/dL, P0.001). A statistically significant positive correlation was observed between sICAM-1 levels and SLE disease activity index (SLEDAI) score in SLE patients, and no correlation was found between sICAM-1 and CRP, SLEDAI and CRP, or SLEDAI and ESR in patient groups (P0.05). These findings suggest that sICAM-1 measurement may serve as an additional serologic marker of disease activity in patients with SLE.

Secondary amyloidosis (AA amyloidosis) is a well known cause of nephrotic syndrome and renal failure. Several studies in patients with nephrotic syndrome have suggested a beneficial effect of angiotensin-converting enzyme inhibitors (ACEI). Angiotensin II (ATII) receptor antagonists effect on the long term is not known. In this study, we intended to study the effect of losartan, as an ATII receptor antagonist, on proteinuria and renal functions in patients with normotensive secondary amyloidosis. In total 44 patients with biopsy proven AA amyloidosis associated with nephrotic proteinuria were included. The first group of patients (n=22) was treated with losartan 50 mg/day. The second group of patients (n=22) did not receive any specific antiproteinuric treatment. Urinary protein loss was effectively lowered by losartan from 4.38 +/- 1.0 to 2.8 +/- 0.61 g/day (p0.0001), whereas the control group showed a slight fall in proteinuria as 4.21 +/- 1.06 to 4.12 +/- 1.07 g/day (p = 0.176). Hypoalbuminemia improved significantly from 2.52 +/- 0.69 to 2.78 +/- 0.46 g/dl (p = 0.004), in the losartan group, whereas serum albumin had fallen in the control group from 2.44 +/- 0.57 to 2.27 +/- 0.41 (p = 0.041). Serum creatinine increased in the control group from 1.52 +/- 0.42 to 2.39 +/- 0.51 mg/dl (p0.0001), and in the losartan group from 1.59 +/- 0.50 to 1.84 +/- 0.6 mg/dl (p0.001), after 24 months of treatment. The ATII receptor blocker losartan is effective in protecting against the progression of nephropathy due to AA amyloidosis. Symptomatic treatment of proteinuria with losartan is therefore to be considered, especially with severe proteinuria even in normotensive patients.

HELLP syndrome, a syndrome of hemolysis, elevated liver enzymes and low platelets may occur in pregnancy with pre-eclampsia/eclampsia, and its a significant complication is acute renal failure (ARF). The aim of study was to determine frequency and outcome of HELLP syndrome complicated by ARF. Thirty-nine patients with pregnancy-related ARF were treated between Jan 1, 1989 and Jan 1, 1999. In these patients, the most frequent causes were HELLP syndrome (n = 14; 36%), postpartum hemorrhage (n = 10; 26%), pre-eclampsia/eclampsia (n = 6; 15%) and abruptio placenta (n = 4; 10%). Seven of the patients with HELLP syndrome had impairment of consciousness during hospitalization. Of these patients, coma in 5, stupor in 1, confusion in 1 were diagnosed. Twelve of the patients with HELLP syndrome and 14 of the other patients were treated by dialysis. Mann-Whitney U test and chi2 test(corrected by Yates and Fisher exact) were used for statistical analysis. Although serious clinical findings, with supportive treatment, 12 patients with HELLP syndrome and 21 other patients were fully recovered. One patient both with and without HELLP syndrome could not recovered due to diffuse cortical necrosis. Moreover, one patient with HELLP syndrome and 3 other patients were died. Mortality rate of the patients with HELLP syndrome was not found different from those of the other patients (p = 0.544). The causes of death were cerebral hemorrhage in patient with HELLP syndrome and disseminated intravascular coagulation (n = 1), cerebral emboli (n = 1), adult respiratory distress syndrome (n = 1). Fetal death occurred in 4 patients with HELLP syndrome (28.5%) and 7 other patients (28%), and rates were similar (p0.5). Finally, HELLP syndrome was the most frequent cause leading to ARF in pregnancy and their prognosis was not different from those of the other patients.