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Patients with end-stage renal disease (ESRD) display enhanced genomic damage. DNA repair gene polymorphisms may affect DNA repair capacity and modulate susceptibility to ESRD. In this study, we aimed to determine the frequency of polymorphisms in two DNA repair enzyme genes, Xeroderma pigmentosum complementation group D (XPD) and X-ray cross-complementing group 1 (XRCC1), in patients with ESRD and to evaluate their association with ESRD development. By using polymerase chain reaction (PCR) and restriction fragment length polymorphism (RFLP), we genotyped four single nucleotide polymorphisms (SNPs) in XPD codons 312 and 751 and XRCC1 codons 194 and 399 in 136 dialysis patients (71 patients undergoing hemodialysis and 65 subjected to peritoneal dialysis) and 147 healthy controls. Patients having XRCC1 399 Arg/Gln (OR:1.98; 95% CI: 1.21-3.25, P = 0.007) or XRCC1-399 Gln/Gln (OR: 3.95; 95% CI: 1.45-10.76, P = 0.005) genotype had a significantly higher risk of ESRD than those with XRCC1 399 Arg/Arg genotype. We also found a significantly higher frequency of the XRCC1 399Gln allele in patients with ESRD than in controls, with OR = 2.03 (95% CI = 1.08-3.81, P = 0.03). We further investigated the potential combined effect of these DNA repair variants on the risk of ESRD development. It was found that combination of the Arg/Gln or Gln/Gln genotypes of XRCC1 Arg399Gln polymorphism with the two possible genotypes of XPD-Asp312Asn or with the Lys/Gln or Gln/Gln genotypes of XPD Lys751Gln was significantly associated with the development of ESRD. This is the first report showing an association between DNA repair gene polymorphisms and ESRD development, and suggests that XRCC1 Arg399Gln polymorphism may confer increased risk for the development of the disease. Further larger studies should be conducted to confirm these results.

Background: Cardiovascular disease is the leading cause of mortality among renal transplant recipients. In the general population, coronary artery calcification (CAC) and progression of CAC are predictors of future cardiac risk. We conducted a study to determine the progression of CAC in renal transplant recipients; we also examined the factors associated with progression and the impact of the analytic methods used to determine CAC progression., Methods: We used multi-detector computed tomography to examine CAC in 150 prevalent renal transplant recipients, who did not have a documented cardiovascular disease. A baseline and a follow-up scan were performed and changes in CAC scores were evaluated in each patient individually, to calculate the incidence of CAC progression. Multivariate logistic regression analysis was used to evaluate the determinants of CAC progression., Results: Baseline CAC prevalence was 35.3% and the mean CAC score was 60.0 ± 174.8. At follow-up scan that was performed after an average of 2.8 ± 0.4 years, CAC prevalence increased to 64.6% and the mean CAC score to 94.9 ± 245.7. Progression of individual CAC score was found between 28.0 and 38.0%, depending on the method used to define progression. In patients with baseline CAC, median annualized rate of CAC progression was 11.1. Baseline CAC, high triglyceride and bisphosphonate use were the independent determinants of CAC progression., Conclusions: Renal transplantation does not stop or reverse CAC. Progression of CAC is the usual evolution pattern of CAC in renal transplant recipients. Beside baseline CAC, high triglyceride level and bisphosphonate use were associated with progression of CAC.

Background: Chronic kidney disease (CKD) is a growing health problem worldwide that leads to end-stage kidney failure and cardiovascular complications. We aimed to determine the prevalence of CKD in Turkey, and to evaluate relationships between CKD and cardiovascular risk factors in a population-based survey., Methods: Medical data were collected through home visits and interviews. Serum creatinine, blood glucose, total cholesterol, triglycerides, HDL, LDL and uric acid were determined from 12-h fasting blood samples, and spot urine tests were performed for subjects who gave consent to laboratory evaluation., Results: A total of 10 872 participants were included in the study. The final analysis was performed on 10 748 subjects (mean age 40.5 ± 16.3 years; 55.7% women) and excluded 124 pregnant women. A low glomerular filtration rate (GFR) (< 60 mL/min/1.73 m(2)) was present in 5.2% of the subjects who were evaluated for GFR, while microalbuminuria and macroalbuminuria were observed in 10.2% and 2% of the subjects, respectively. The presence of CKD was assessed in subjects who gave consent for urinary albumin excretion measurement (n = 8765). The overall prevalence of CKD was 15.7%; it was higher in women than men (18.4% vs. 12.8%, P < 0.001) and increased with increasing age of the subjects. The prevalence of hypertension (32.7% in the general population), diabetes (12.7%), dyslipidaemia (76.3%), obesity (20.1%) and metabolic syndrome (31.3%) was significantly higher in subjects with CKD than subjects without CKD (P < 0.001 for all)., Conclusions: The prevalence of CKD in Turkey is 15.7%. Cardiovascular risk factors were significantly more prevalent in CKD patients.

OBJECTIVE. We aimed to determine clinical and laboratory findings that were different between those patients who died and those who survived and to look for factors associated with the mortality in amatoxin-containing mushroom poisoning. METHODS. The mushroom poisoning patients who were admitted to our clinic between 1996 and 2009 were retrospectively evaluated. The diagnosis was based on a history of mushroom ingestion, clinical picture and the presence of serum alpha-amanitin. Patients were divided into two groups as the survival group and the fatality group. Clinical and laboratory findings were compared between the two groups. Relation between variables and clinical outcome was analyzed. RESULTS. A total of 144 amatoxin poisoning patients were included in this study. Patients who died were more likely to have demonstrated low mean arterial pressure, encephalopathy, mucosal hemorrhage, oliguria-anuria, hypoglycemia, and thrombocytopenia during the hospitalization. Low sodium values and high urea, AST, ALT, total bilirubin, LDH, prothrombin time, international normalized ratio, and activated partial thromboplastin time values were associated with increased likelihood of mortality. Nineteen patients developed acute renal failure. Fourteen patients developed acute hepatic failure. All the 14 patients who died developed acute hepatic failure. The mortality rate was 9.7%. CONCLUSIONS. The factors associated with mortality determined in this retrospective study may be helpful for clinical outcome assessment and monitoring of patients with amatoxin-containing mushroom poisoning.

Background: National renal registry studies providing data for incidence, prevalence, and characteristics of end-stage renal disease and renal replacement therapy (RRT) serve as a basis to determine national strategies for the prevention and treatment of these diseases and identify new areas for special studies., Study Design: Since 1990, the Turkish Society of Nephrology has been coordinating a national renal registry that collects data on patients receiving RRT. This report focuses on data collected from 1996-2008., Setting & Participants: Data were collected in dialysis centers for patients on RRT., Predictor: Year., Outcomes: Point prevalence and incidence of RRT, RRT modalities, demographic and clinical characteristics of patients on RRT., Results: From 1996 to 2008, the number of centers (199 and 760) and response rates to the registry (76% and 99.4%) increased. In 2008, the point prevalence of RRT was 756 per million population (pmp) and incidence was 188 pmp, including pediatric patients. In prevalent patients, the most common RRT modality was hemodialysis (77.0% of patients), followed by peritoneal dialysis (10.1%) and transplant (12.9%). The age of hemodialysis and transplant patients increased, with a predominance of male patients. Percentages of diabetes mellitus and hypertension as causes of ESRD increased, whereas those of chronic glomerulonephritis and urologic disease decreased. Infection and crude death rates decreased in all treatment modalities., Limitations: The main study limitations were registry design and low number of kidney transplants., Conclusion: With increasing numbers of dialysis centers and RRT patients during the last 12 years, the need for RRT in Turkey has been better met. The quality of RRT care has improved, especially regarding prevention and treatment of infections., (Copyright © 2011 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.)

Background: Cardiovascular disease is the leading cause of mortality among renal transplant recipients. Data on the relationship between coronary artery calcification (CAC) and coronary ischaemia in renal transplantation patients are scant. We conducted a study to determine the prevalence and determinants of CAC in these patients; we also examined the frequency of coronary ischaemia in patients with moderate and severe CAC., Methods: We used multi-detector spiral computed tomography to examine CAC in 178 consecutive renal transplant recipients. Angina pectoris was sought with the Rose questionnaire. The extent of calcification was measured by Agatston score. Myocardial perfusion scintigraphy was performed in patients with moderate and severe CAC. Multivariate logistic and linear regression analysis was used to evaluate the determinants of CAC presence and CAC score, respectively., Results: CAC was present in 72 patients (40.4%), mean CAC score was 113.7±275.5 (median: 0 and range: 0-1712). Age, time on transplantation and Rose angina pectoris were the independent determinants of both CAC presence and high CAC scores in all multivariate models. Coronary ischaemia was detected in 17.1% of the patients with moderate-to-severe CAC., Conclusions: CAC is highly prevalent in renal transplant recipients; it is associated with symptoms of coronary ischaemia. Time on transplantation is an independent determinant of CAC. Future studies to evaluate the prognostic significance of CAC in these patients are necessary.

Aim: Peritonitis is one of the major comorbidities of peritoneal dialysis (PD) patients. The aim of this study was to concentrate on potential risk factors, including more recently studied ones among the classical ones for peritonitis, in PD patients., Materials and Methods: We analysed 109 patients (F/M = 42/67) followed up at least for 3 months in a single centre, a tertiary referral hospital for 360.1 patient years. In the study which is designed as a retrospective cohort study, demographic characteristics, conditions for choosing PD, type of PD treatment, some chemical tests and peritonitis episodes were recorded from the files of the patients., Results: The rate of peritonitis was found to be 0.22 episode/patient year and 22 (20.18%) of the patients had more than one episode. Twenty seven (24.8%) of the patients were allocated to PD due to obligatory reasons. According to multiple regression analysis, the associated factors were found to be PD allocation type (obligatory versus voluntary) (p = 0.04; RR = 2.6), serum albumin level (p = 0.05; RR = 1.2), and anti-hepatitis C Virus Antibody positivity (p = 0.03; RR = 1.6). Frequency of female patients were significantly higher in the group who had multiple episodes (p = 0.01)., Conclusion: Obligatory referral which can be an indication of loss of motivation for peritoneal dialysis procedures, is thought to be a strong risk factor for peritonitis in PD patients and should be further studied. Patients with multiple episodes had a higher frequency of obligatory referral as expected and additionally, they were higher in number of females when compared to the ones with single episode.

Aim: Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease. It accounts for 5-10% of patients with end-stage renal disease (ESRD). The aim of this multicenter study was to investigate the demographic and clinical characteristics of patients with ADPKD., Methods: 1,139 patients with ADPKD who were followed up at 12 different centers were recruited for this study. The investigated demographic and clinical characteristics were gender, age, smoking history, educational status, the existence of hypertension, hematuria, urinary tract infection, urinary tract stones and renal replacement therapy. Patients were considered as hypertensive if they were taking antihypertensive medications or if they had blood pressure (BP) of 140/90 mm Hg or greater. If the patients were currently on antihypertensive drugs, the classes of these agents were noted., Results: 548 male and 591 female patients were included and the mean age at initial diagnosis was 37.1 ± 16.3 years. 20.3% were current smokers whereas 15% were ex-smokers. The mean systolic and diastolic BPs were 136.1 ± 29.8 and 84.9 ± 17.8 mm Hg, respectively. 63.7% used antihypertensive drugs and 73.1% of those used renin-angiotensin system blockers. 11.8% had ESRD, of which 75.8% were treated with hemodialysis., Conclusion: This study showed that hypertension is the most common (72.6%) clinical finding in ADPKD patients in Turkey and renin-angiotensin system blockers are widely used., (Copyright © 2010 S. Karger AG, Basel.)

Data on free water excretion capacity of renal transplant recipients are scant. The aim of this study was to evaluate the ability of electrolyte free water clearance (E-CH(2)O) by the allograft in renal transplant patients and the effects of various immunosuppressive drugs. Renal transplant recipients with good graft function (creatinine < 1.5 mg/dL) as well as controls were divided into five groups according to their immunosuppressive regimen: group I, azathioprine (n = 15); group II, cyclosporine (n = 28); group III, tacrolimus (n = 28); group IV healthy controls (n = 20); and group V renal transplant donors (n = 16). Following a 12-hour fast, we administered oral water loading (20 mL/kg) with urine collection for 3 hours. We calculated creatinine clearance for 3 hours and E-CH(2)O. No matter which immunosuppressive drug, the E-CH(2)O of recipients (groups I, II, and III) was lower than that of donors or healthy controls. The creatinine clearance of the cyclosporine arm was significantly lower than all of the other groups. Decreased E-CH(2)O in renal transplant patients might be due to diminished water input to the loop of Henle related to subclinical allograft insufficiency as a result of posttransplantation pathology and/or immunosuppressive drug therapy or the transport of water into the extrarenal interstitium as a result of vascular endothelial dysfunction due to the pretransplant uremic milleu.

Although less common than bacterial peritonitis, fungal peritonitis is associated with much higher morbidity and mortality. In this study, we aimed to determine the risk factors for fungal peritonitis in peritoneal dialysis patients. The records of 109 peritoneal dialysis patients were analyzed. A total of 86 episodes of peritonitis attacks were recorded. Nine (10.5%) of these attacks were fungal peritonitis attacks. The fungal peritonitis attack rate of the population was 1 attack per 480.1 patient months. In order to determine predisposing factors for fungal peritonitis patients, patients with bacterial peritonitis and with no peritonitis admitted immediately before and after those with fungal peritonitis were used as controls. There was no statistically significant difference between the bacterial and fungal peritonitis groups with respect to symptoms and signs. Obligatory peritoneal dialysis treatment due to access or other medical problems (p = 0.04) and serum albumin levels (p = 0.01) were found to be significantly related with fungal peritonitis (p = 0.04). The mortality rate was 11.1%. When compared with the mortality rate of bacterial peritonitis (1.8%) during the same period, it was found to be significantly higher (p = 0.0001). The catheter removal was performed within 2-7 days (mean = 5.2 days) of the fungal peritonitis attacks. In conclusion, we decided that because fungal peritonitis attacks end up with high morbidity and mortality, prompt diagnosis and removal of the catheter is mandatory. Diagnosis highly depends on culture results, as signs and symptoms usually do not differ from that of bacterial peritonitis. Low serum albumin levels and obligatory peritoneal dialysis treatment are found to be the risk factors for fungal peritonitis.

We sought to compare the treatment modalities of alendronate, alfacalcidol, and alendronate combined with alfacalcidol in renal transplant recipients with low bone mineral density. Sixty-four kidney graft recipients (22 women, 42 men) were recruited to this study. Of these 64 patients, 9 served as the control group with T scores more than -1. The remaining 55 patients randomly assigned to treatment had T scores less than -1 and were assigned to 3 groups: group 1 received alfacalcidol (0.5 microg/d); group 2, alendronate (10 mg/d); and group 3, alendronate (10 mg/d) + alfacalcidol (0.5 microg/d per os). Twenty-five patients were allocated to alfacalcidol, 13 patients to alendronate, and 17 patients to alendronate + alfacalcidol treatment. Bone mineral densities of the lumbar spine and femoral neck were measured before and 12 months after treatment. The groups were compared for risk factors of osteoporosis, biochemistry, and bone mineral density. Kruskal-Wallis, one-way ANOVA, and Student t tests were used. With the alendronate + alfacalcidol group, bone mineral density at the lumbar spine significantly increased by 7.9% (P = .006) with a significant improvement in T score (P = .003). Bone mineral density at the femoral neck significantly increased by 8% in the alendronate + alfacalcidol group (P = .01) with a significant improvement in T score (P = .02). The use of a combination of alendronate and alfacalcidol seemed to be safe and more effective than the separate use of the 2 agents to improve bone mass in renal transplant recipients.

We report the case of a young male patient with nephrotic syndrome and multiple venous thromboses. The patient presented various aggregated thrombophilic risk factors. He was found to be homozygous for factor V Leiden mutation and his anticardiolipin antibody and homocysteine levels were high. The association between nephrotic syndrome and venous thrombosis is well known. However the presence of disseminated thrombosis should prompt an intensive work-up for the detection of thrombotic risk factors and aggressive anticoagulant therapy.

Background: In the presence of decreased glomerular filtration rate (GFR), the risk of morbidity and mortality caused by cardiovascular disease (CVD) is increased markedly. Increased coronary artery calcification (CAC) is proposed as a pathogenetic link between CVD and chronic kidney disease. We examined the frequency and severity of CAC in living kidney donors to test the hypothesis that decreased GFR is associated with increased CAC., Methods: We used multidetector spiral computed tomography to examine CAC in 101 living kidney donors and 99 age- and sex-matched healthy control subjects without diabetes and a history of coronary artery disease. The extent of calcification was measured by means of the Agatston score. GFR was calculated by using the abbreviated Modification of Diet in Renal Disease formula. The frequency of risk factors for coronary artery disease was compared in kidney donors and controls, and the relation between kidney donors' clinical characteristics and the presence or absence of CAC was examined., Results: CAC frequency and mean calcification scores were similar between kidney donors (13.9%; 4.5 +/- 22.6) and controls (17.2%; 13.2 +/- 89.2). CAC was not associated with decreased GFR, and the correlation between CAC and GFR was not statistically significant. Kidney donors with calcification were more likely to be older (P = 0.003) and male (P = 0.001). Age- and sex-adjusted analysis showed an association between greater parathormone levels (odds ratio, 1.023; 95% confidence interval, 1.001 to 1.045; P = 0.037) and CAC in kidney donors., Conclusion: A mild decrease in GFR without the presence of diabetes does not seem to be associated with increased CAC. These findings need to be confirmed in different and larger study populations.

Background/aims: Ideal time needed for arteriovenous fistula (AVF) maturation is still controversial. In this study, we aimed to investigate the natural course of AVF maturation and also investigated the factors affecting AVF maturation., Methods: We studied 31 (21M/10F, mean age 55.8 +/- 16.2) chronic renal failure patients. We evaluated the patients with color Doppler ultrasound examination before the fistula operation, at the first day, and at the first, second, third, and sixth months. Radial artery (RA) diameter, flow velocity, flow, resistance index, fistula vein diameter, flow velocity, and flow were measured., Results: Patency rates at the first post-operative day and the sixth month were 87.1% and 67.1%, respectively. Cephalic vein flow was 451.2 +/- 248.6 mL/min at the first month and 528.6 +/- 316.5 mL/min at the sixth month. Baseline RA diameter was lower in failing fistulas than that of patent fistulas. Failing fistulas were more common in women., Conclusion: Blood flow was enough for hemodialysis at the end of the first month. However, fistula maturation had continued until the end of the study; women and patients with low RA diameter are particularly prone to fistula failure. Therefore, especially in these patients, AVF must be created at least three or four months before the predicted hemodialysis initiation time.

This paper describes the first case of common variable immunodeficiency (CVID) and AA amyloidosis. A recently treated tuberculosis, and chronic inflammation induced by frequent respiratory tract infections, were thought to be responsible for the amyloidosis. No other reason for this condition could be detected. Although T cell dysfunction in some CVID patients has been reported, pulmonary tuberculosis is quite rare with this condition. Bacterial or viral agents or evidence in favour of intestinal tuberculosis, which would explain this patient's recurrent diarrhea, were not found. In this case, the response of the attacks of diarrhea to metranidazole and the histologic observation of extensive intestinal amyloid deposition, which is known to decrease intestinal motility, made us conclude that the diarrhea was associated with bacterial overgrowth. In this report, we discuss the association of CVID and tuberculosis to secondary amyloidosis and recurrent diarrhea.

Background: Patients requiring dialysis due to acute or chronic renal failure frequently require temporary vascular access. Femoral vein catheterization is the easiest method for obtaining temporary vascular access in haemodialysis patients. The aim of this study was to utilize ultrasound imaging to describe femoral vein structures and to examine anatomical variations in uraemic patients., Methods: We evaluated 114 (70 males, 44 females) renal failure patients. Femoral arteries were localized manually inferior to the femoral ligament, and ultrasonographic examination was performed from this location. Images of the vessels and demographic data of patients were recorded and analysed. Femoral veins were classified according to their diameter, patency and palpation status of the neighbouring femoral artery., Results: Three patients had a history of prior femoral catheterization. In one of these, who had a history of bilateral catheterization, we detected bilateral femoral vein thrombosis. Overall, non-palpable femoral arteries or unsuitable femoral veins were found unilaterally in 16 patients (14.0%) and bilaterally in six patients (5.2%). The depth of femoral arteries (r = 0.54, P<0.001) and femoral veins (r = 0.59, P<0.001) was correlated with body mass index (BMI). Femoral arteries and femoral veins were located significantly deeper in overweight (BMI >25) patients compared with normal weight patients (20.7+/-6.5 vs 14.6+/-5.1 mm, P<0.001 and 26.1+/-6.7 vs 18.9+/-5.5 mm, P<0.001)., Conclusions: Bilateral anatomical variations of femoral veins were relatively rare. However, ultrasound surveys should be performed in obese patients or when the femoral artery is not palpable.

Background: Conjunctival and corneal calcification (CCC) is a well-known and easily detectable extraskeletal calcification, but its association with vascular calcification was not investigated previously. The aim of this study is to investigate the relationship of CCC with vascular calcification and bone metabolism parameters in dialysis patients., Methods: We evaluated 63 patients (30 men, 33 women; mean age, 43.5 +/- 13.4 years) who were on dialysis therapy for more than 6 months. Forty-four patients were on peritoneal dialysis and 19 patients were on hemodialysis therapy. The same observer evaluated the presence of CCC by using a slit-lamp microscope, and a total CCC score was recorded for each patient. Fifty-two age- and sex-matched healthy controls also were evaluated by using the same method. Biochemical data were collected from patient files. Bone mineral density (BMD) of the lumbar spine and femoral neck was measured, and the presence of vascular calcification was assessed by using x-ray examinations of the pelvis and hands., Results: Mean CCC score in patients was significantly higher than that in controls (6.2 +/- 5.1 versus 1.3 +/- 1.8; P = 0.001). CCC score correlated significantly with duration of renal replacement therapy ( r s = 0.392; P = 0.002), serum phosphorus level ( r s = 0.259; P = 0.042), and calcium x phosphorus product ( r s = 0.337; P = 0.007). However, we did not find a significant correlation with calcium, parathyroid hormone, alkaline phosphatase, albumin, or C-reactive protein level or BMD. The frequency of vascular calcification was significantly greater in patients with a high CCC score (CCC score > or = 10) compared with a low CCC score (< or =3; 56.3% versus 5.6%; P = 0.002)., Conclusion: Evaluation of CCC score is an easy, fast, and noninvasive method. It seems that CCC score can be used as an additional tool to assess the status of extraskeletal calcification in dialysis patients.

Background and Results: By the end 2000, 22224 patients were on renal replacement therapy (RRT) in Turkey. We investigated the cost of RRT in three medical faculties and one private dialysis centre. Yearly expenses were US dollars 22759 for haemodialysis (HD), US dollars 22350 for continuous ambulatory peritoneal dialysis (CAPD), and US dollars 23393 and US dollars 10028, respectively, for the first and second years of transplantation (Tx). In the first year, renal Tx was significantly more expensive than CAPD. However, after the first year of renal transplantation, Tx became significantly more economical than both CAPD and HD. The sum of all yearly RRT expenses for the country was US dollars 488958709, which corresponds to nearly 5.5% of Turkey's total health expenditure., Conclusion: Measures such as early construction of vascular access, promoting home dialysis and the reuse of the dialysers, strict control of the use of some expensive drugs like erythropoietin and active vitamin D, and also increasing the number of transplantations, especially if pre-emptive transplantation is possible, should be taken into account in order to reduce these expenses.

Background/aims: Familial Mediterranean fever (FMF) is an autosomal recessive disease characterised by recurrent fever attacks and polyserositis which may lead to the development of AA amyloidosis and end-stage renal disease (ESRD). In this study, we aimed to evaluate the efficacy of continuous ambulatory peritoneal dialysis (CAPD) in FMF-amyloidosis patients with ESRD., Methods: Forty age- and sex-matched patients undergoing CAPD at our centre between 1996 and 2002 were included in the study. Of these, 10 had FMF-amyloidosis, 10 had diabetes mellitus (DM), 10 had chronic glomerulonephritis (CGN) and 10 had chronic interstitial nephritis (CIN). Efficiency of CAPD, development of complications, presence of other diseases and survival were compared., Results: With the onset of ESRD, the frequency of FMF peritonitis attacks decreased, with less attacks occurring during CAPD in FMF-amyloidosis patients (p < 0.05). There was no significant difference between the FMF-amyloidosis group and other groups in terms of efficiency of CAPD, peritoneal function, complications and survival. DM patients had a shorter survival period compared with CGN and CIN patients (p < 0.05), but there was no survival difference between FMF-amyloidosis patients and other groups (p > 0.05)., Conclusions: We conclude that CAPD is an effective and safe renal replacement therapy for FMF-amyloidosis patients with ESRD., (Copyright 2004 S. Karger AG, Basel.)

Carnitine palmitoyltransferase II (CPT-II) deficiency is the most common long-chain fatty acid oxidation defect, resulting in rhabdomyolysis and acute renal failure (ARF). There are three forms of CPT-II deficiency: the neonatal, infantile and adult form. We report an adult form of CPT-II deficiency in a patient who presented with attacks of exercise-induced rhabdomyolysis and ARF.

In this study, we aimed to determine the frequencies of catheter exit-site infection (CESI), catheter-related bloodstream infection (CR-BSI) and catheter colonization (CC); causative microorganisms; and resistance patterns in patients with temporary hemodialysis catheters. From March 1999 to March 2000, 67 hemodialysis patients (38 males, 29 females; median age: 52, range: 17-84) were evaluated. The CDC criteria were used to diagnose CESI, CR-BSI and CC. The tips of catheters were cultured by Maki's method. At the same time, two different blood cultures, one from peripheral vein and the other through the catheter lumen were drawn. Swab cultures from the catheter exit sites were also performed. The isolation and identification of bacteria were performed by conventional methods and the susceptibility testing by the Kirby-Bauer method. CESI, CR-BSI and CC were found in, respectively, 20 (29.8%), 16 (23.8%) and 11 (16.4%) patients. The etiologic agents in CR-BSI were as follows: Staphylococcus aureus (5), coagulase-negative staphylococci (2), Enterococcus sp. (1), Escherichia coli (1), Acinetobacter sp. (1) and Proteus sp. (1). Methicillin-resistant coagulase-negative staphylococci and methicillin-resistant S. aureus were found in proportions of 45.5% and 63.6% in CESI and CR-BSI+CC. The only risk factor for the development of CR-BSI and CC was intravenous drug use. In our center, the majority of CESI, CR-BSI and CC were due to staphylococci and there was a high rate of methicillin resistance.

Patients with seminal vesicle and epididymal cysts are mostly asymptomatic. To date, only one patient presenting with bloody ejaculate and acute scrotum has been reported. Different extrarenal manifestations and the association of adult polycystic kidney disease (APKD) with some connective tissue diseases are known. We report on a 60-year-old male patient with bloody ejaculate and acute scrotum who had been diagnosed as having APKD 1 year earlier and whose past medical history revealed inflammatory low back pain, psoriasis, and the diagnosis of psoriatic arthritis. Cultures of urine and ejaculate were sterile, and the patient's renal functions were normal. Ultrasound showed epididymal and seminal vesicle cysts in addition to hepatic and renal cysts. Our case is the first in which psoriatic arthritis accompanied APKD, seminal vesicle cysts, and epididymal cysts. We also review other APKD cases that have accompanied seminal vesicle cysts.

In a 53-year-old male patient with metastatic adrenal carcinoma, treatment with mitotane was instituted but he was lost to follow-up. Two years later, he presented with oedema and nephrotic-range proteinuria. The rectal and renal biopsies revealed an accumulation of secondary amyloid material. The patient died of respiratory failure caused by the progressive pulmonary metastases. This is the first report of a patient with adrenal carcinoma who developed secondary amyloidosis.

Objective: In this case-control study, we sought to determine whether the incidence of gallbladder stones (GBS) was increased in chronic renal failure (CRF) patients on a hemodialysis (HD) program. We also evaluated factors, such as lipid profiles and gallbladder motility, that could affect the formation of GBS. In addition, we reviewed other available studies on this subject and compared the factors that might have some influence on the development of GBS., Methods: A total of 182 CRF patients (135 male, 47 female, mean age 32.1 yr) undergoing chronic HD and who were referred to our transplantation center in the last 10 yr and 194 healthy controls (137 male, 57 female, mean age 33.3 yr) were included in the study. Abdominal ultrasound was performed on all patients, and ALT, AST, and lipid profiles were determined. In addition, 19 patients with CRF (12 male, 7 female, mean age 33.5 yr) and 22 controls (14 male, 8 female, mean age 33.2 yr) who were age and sex matched were randomly chosen for gallbladder emptying, monitored by ultrasound at 30-min intervals for 2 h after a mixed meal. Fasting volume, minimal residual volume, and ejection fraction of the gallbladder were assessed. For statistical analysis, chi(2), t test, and logistic regression analysis were used., Results: GBS were detected in seven patients with CRF (3.85%, 5 male, 2 female) and three controls (1.55%, one male, two female) (p > 0.05). The mean follow-up time of CRF patients after diagnosis was 39.3 months (range: 2-168), the mean duration of HD was 21.8 months (range: 1-120). The analysis of seven stones in the CRF group revealed that five were cholesterol-rich stones, and two were mixed (cholesterol and bilirubin) stones. Cholesterol levels were higher in the control group, and triglycerides were higher in the CRF group, but these findings were nonsignificant (p > 0.05). Other biochemical values were not significantly different between the groups. CRF patients with and without GBS were similar in their duration of CRF and HD, age, and other biochemical parameters (p > 0.05). When gallbladder emptying was considered, there was no difference between the two groups in fasting volume, residual volume, and ejection fraction (CRF: 89.7%; controls: 92.3%) of the gallbladders (p > 0.05)., Conclusions: We detected similar incidences of GBS in CRF patients undergoing HD and healthy controls, and this was comparable to the results of most of the previous studies. Young male CRF patients had a nonsignificantly higher incidence of GBS than control males. Although cholesterol-rich GBS were predominant, we could not find any significant difference between the groups when factors that could affect GBS formation, such as lipid profiles and gallbladder motility, were taken into account.

Toxic shock syndrome (TSS) is an illness defined by the occurrence of fever, rash, hypotension, multiple organ system dysfunction, and desquamation. Nonmenstrual TSS is often associated with surgical or nonsurgical cutaneous infections, which are rarely purulent or inflamed (Reingold AL, et al. Nonmenstrual toxic shock syndrome: a review of 130 cases. Ann Intern Med 1982; 96:871-4). Toxic shock syndrome associated with peritoneal exit-site infection but without peritonitis is extremely unusual (Sherbotie JR, et al. Toxic shock syndrome with Staphylococcus aureus exit-site infection in a patient on peritoneal dialysis. Am J Kidney Dis 1990; 15:80-3). We describe 2 patients that met the Centers for Disease Control case definition of TSS secondary to a peritoneal dialysis catheter exit-site infection with signs of mild inflammation and growth of Staphylococcus aureus, but with no evidence of peritonitis.

A 26-year-old woman presented at our hospital with pretibial edema, swelling, and vaginal bleeding. She had nephrotic-level proteinuria and was diagnosed with choriocarcinoma after a histologic examination of her uterine curetting was performed. The renal biopsy specimen was compatible with membranous glomerulonephritis. The renal pathology in this case was thought to be secondary to choriocarcinoma because the nephrotic syndrome regressed after hysterectomy. We briefly present this first case of choriocarcinoma causing membranous glomerulonephritis.

This study was performed to determine risk factors associated with osteoporosis that develops after renal transplantation. Sixty-five kidney graft recipients were included in this study. They were divided into four groups according to the time since transplantation: Group 1 (< 1 year; n = 26), group 2 (1-3 years; n = 16), group 3 (3-5 years; n = 12) and group 4 (> 5 years; n = 11). These groups were matched according to probable risk factors for osteoporosis, findings of serum biochemistry, biochemical markers of bone turnover and measurements of bone mineral density. One way ANOVA test and Kruskal-Wallis test were used for statistical analysis. Osteoporosis was found in 22 recipients (33.8%). There were significant differences in recipient age, cumulative steroid dose, and episodes of acute rejection between the four groups. Increasing age, cumulative steroid dose and episodes of acute rejection were found to be risk factors for osteoporosis in our study.

Familial Mediterranean fever (FMF) is a hereditary disease, the most threatening complication of which is systemic amyloidosis. The thyroid gland may be asymptomatically involved in most patients with systemic amyloidosis secondary to FMF. However, clinically detectable thyroid goitre is quite rare, and until now only nine cases of thyroid goitre secondary to amyloid deposition in FMF have been reported. Of 1,100 FMF patients regularly followed up at our centre, thyroid goitre due to the accumulation of amyloid substance could be detected in only three (0.27%). In this report, we summarise the clinical and laboratory features of these patients. All three patients were euthyroid. Total thyroidectomy was performed for compressive symptoms in one patient and for aesthetic purposes in the other two. In countries with a high prevalence of FMF, such as Turkey, secondary amyloidosis of the thyroid gland should be borne in mind in long-standing FMF patients.

Colchicine has been in use for therapeutic purposes for many years. It can, however, cause subacute onset muscle and peripheral nerve toxicity in patients with chronic renal failure. In this report we describe 6 patients who developed neuromyopathy after the administration of colchicine. All patients presented with proximal muscle weakness, elevated serum creatine kinase (CK) levels, and neuropathy and/or myopathy on electromyography (EMG). The diagnosis of colchicine toxicity was confirmed in all cases by the normalization of CK levels and EMG after discontinuation of the drug. Toxicity developed in 4 renal failure patients on therapeutic doses of the drug, while one patient took a massive dose for suicidal reasons, and the other was on high-dose therapy. Patients using colchicine--especially those with renal failure--should be warned about the side effects of the drug and physicians should be careful in the administration of the drug.

Diabetic glomerulosclerosis might be seen in diabetics but its presence in patients with impaired glucose tolerance is quite rare. A 31-year-old woman who was admitted to our department was diagnosed with hypertension, nephrotic syndrome and impaired glucose tolerance. Her renal biopsy was compatible with diabetic glomerulosclerosis. She developed overt diabetes mellitus (DM) after one year of impaired glucose tolerance. Hypertension might have accelerated the progression of diabetic nephropathy.

In this study, we retrospectively evaluated all attacks of diarrhoea in our renal transplant recipients that came to our medical attention between 1985 and 2000. Also, the clinical features of patients with diarrhoea were compared with the features of recipients without diarrhoea. We diagnosed 41 attacks of diarrhoea in 39 (12.6%) of 308 renal transplant recipients during this time period. An aetiology was detected in 33 (80.5%) of all diarrhoeal episodes and in seven (17.1%) of those the specific agent was diagnosed with the help of stool microscopy. The most frequent causes of diarrhoeal attacks were infectious agents (41.5%) and drugs (34%). Six (14.6%) episodes of diarrhoea were chronic and six were nosocomial. About two-thirds of diarrhoea developed within the late post-transplant period (>6 months). When recipients with diarrhoea were compared with those without diarrhoea, it was seen that diarrhoeal patients had significantly higher creatinine and significantly lower albumin levels when compared with the latter group (p < 0.05). Also, the frequency of antibiotic usage was significantly higher in diarrhoeal patients than in the control group (p < 0.05). Four (10.2%) patients with diarrhoea died despite institution of the appropriate therapy. Two of these deaths were primarily related to diarrhoea and the aetiological agent was Clostridium difficile in both these cases. During the 15-yr study period, 3.6% of all deaths and 5.1% of infection-related deaths in transplant recipients were secondary to diarrhoea. As a result, we observed that infections and drugs were the most frequent causes for diarrhoea in our series of renal transplant recipients. Also, diarrhoea was an important cause of mortality in this patient population.

It is quite rare to diagnose secondary amyloidosis during the course of Castleman's disease (CD). A 51-year-old female who complained of fatigue, weight loss, and fever was diagnosed with CD -- plasma cell type -- in our hospital in 1993. One year after diagnosis, she developed nephrotic syndrome, the etiology of which was found to be secondary amyloidosis based on renal biopsy. As the patient rejected therapy, she was discharged after only symptomatic treatment. At her last follow-up in March 2001, she had no complaints; physical examination, blood chemistries, and urinalysis were normal. Abdominopelvic tomography revealed no lymphadenopathy in the abdomen, which had been previously present. We could identify 17 other cases of CD with secondary amyloidosis in the literature. Ours is the 18th such case and the 2nd case of multicentric CD leading to amyloidosis. This case also shows that CD might sometimes run a relatively benign course being cured with no therapy, whereas it might have a rapidly fatal downhill course -- even with therapy -- in others. Still, effective treatment strategies need to be developed.

Extramedullary hematopoiesis being an important feature of agnogenic myeloid metaplasia (AMM), a chronic myeloproliferative disease of clonal origin, may affect the kidneys, but this condition is usually asymptomatic. Until now, there is only one reported case of nephrotic syndrome associated with AMM. We present a patient with AMM who had nephrotic syndrome and whose renal biopsy revealed membranous glomerulonephritis together with renal extramedullary hematopoiesis.

Despite being recognised much more frequently than in the past, renal involvement has not previously been regarded as a feature of Behcet's disease (BD). In this study we aimed to assess the frequency of renal involvement in BD by performing urinalyses of 674 consecutive BD patients; we also retrospectively evaluated the charts of 4212 BD patients for the incidence of glomerulonephritis (GN). Urinary abnormalities (proteinuria and/or haematuria) were present in 10.8%; and during a period of 23 years GN was detected by renal biopsy in seven (0.16%) BD patients. Two patients with GN were lost to follow-up; end-stage renal failure developed in only one patient, and she underwent renal transplantation. We were unable to determine any pathognomonic feature that was predictive of renal involvement. Although males tend to have a more serious clinical course of BD the incidences of urinary abnormalities and GN were similar in both sexes in our series. According to our results, we can conclude that urinary abnormalities are more frequent in BD; however, serious renal lesions develop in only very few of these patients.

A 58-year-old female patient diagnosed as having sarcoidosis 23 years ago developed nephrotic syndrome. No pathology was found which could explain this, so it was attributed to her sarcoidosis. Renal biopsy showed global and segmental sclerosis. The occurrence of focal segmentary glomerulosclerosis in a case of sarcoidosis is rare. In systemic sarcoidosis it is thought that T-cell dysfunction may play a role in the pathogenesis of glomerulonephritis. When treatment is considered, corticosteroid therapy may be used according to the clinical status at diagnosis as well as on follow-up of the patient., (Copyright 2002 S. Karger AG, Basel)

Androgen blockage, with either orchiectomy or luteinizing hormone releasing hormone (LHRH) analogs combined with an antiandrogen drug, is the standard treatment for metastatic prostate cancer. Flutamide is a non-steroidal antiandrogen drug that is frequently used for total androgen blockage. We report on a 54-yr-old man with metastatic prostate cancer who developed nonoliguric acute renal failure (ARF) during treatment with flutamide. Following discontinuation of flutamide therapy, his renal functions returned to normal limits within 4 wk. After a rechallenge with flutamide, serum levels of BUN and creatinine increased again. His renal function recovered completely after the cessation of the drug for the second time. This observation confirm that ARF may be clearly attributed to flutamide therapy. Although very rare, flutamide-induced ARF should be considered.

In a retrospective evaluation, the incidence of systemic fungal infections (SFIs) in 296 kidney graft recipients admitted to our center between 1986 and 1999 was found to be 4%. Eighteen percent of 28 recipients transplanted in India and 8% of 12 recipients transplanted in Russia developed SFI. In contrast, SFI was encountered in only 2% of recipients transplanted at our center. The median time of diagnosis of SFI was 5 months after transplantation. The lungs and central nervous system were the most frequently affected sites. The most common etiologic agent was Aspergillus fumigatus (n = 7) but Candida spp. (n = 1), Rhizopus spp. (n = 1) and Cryptococcus neoformans (n = 1) were also encountered. In 2 patients, 2 different pathogens were isolated at the same time: A. fumigatus and Rhizopus spp. in 1 patient and Candida spp. and A. fumigatus in another. In order to determine predisposing factors for SFI, patients admitted immediately before and after those with SFI were used as controls: long-term hospitalization, long-term antibiotic use and post-transplant diabetes mellitus were found to be predisposing factors. Eight patients were treated with antifungal drugs and a good response to liposomal amphotericin B therapy was obtained in 3/5. Nine patients (75%) with SFI died. As SFIs are associated with a high mortality rate in renal transplant recipients, antifungal therapy, especially with liposomal amphotericin B, should be started whenever fungal infection is suspected, even before the results of microbiologic and/or histologic examinations are known.

Background and Aim: Although the majority of patients with end stage renal failure have anemia, some have relative erythrocytosis. Patients treated with continuous ambulatory peritoneal dialysis (CAPD) having relative erythrocytosis were studied in order to determine the factors that would be responsible., Methods: Nine out of 89 CAPD patients (10%) were identified as having relative erythrocytosis. Age-, sex- and duration of disease-matched eight patients undergoing CAPD were taken as control. Beside factors of etiologies of renal failure, smoking, renal cysts, viral hepatitides, residual renal function, the adequacy of CAPD, nutritional status, hypertension, serum levels of erythropoietin, IL-1, IL-6, TNF-, and IGF-1 levels were also investigated., Results: Relative erythrocytosis occurred most often in diabetic and amyloidosis patients. None of the parameters studied were found to be significantly different between groups. During 2-year follow-up, although statistically non-significant, patients having relative erythrocytosis seemed to have higher mortality rate due to vascular complications., Conclusion: No single factor seemed to explain erythrocytosis in patients undergoing CAPD. Being diabetic or with amyloidosis may increase the risk.

Although mild proteinuria is commonly observed during the course of brucellosis, biopsy-proven glomerulonephritis (GN) is quite rare. We present the first case of mesangiocapillary glomerulonephritis (MCGN) associated with brucellosis and summarize all cases of Brucella GN published to date. Our patient, who had a congenital bicuspid aortic valve, also had heart failure, fever, urinary abnormalities and proteinuria. Renal biopsy revealed MCGN. Although the clinical features raised the possibility of GN associated with endocarditis, transesophageal echocardiography did not show any vegetations.

We retrospectively analysed the medical records of patients who underwent renal biopsy in our nephrology department over the past 8 years. Our aim was to discover the frequency of hepatitis B (HBV) and hepatitis C virus (HCV) in patients with primary glomerulonephritis (GN) in Turkey, a moderately endemic country for both infections. The study included 144 male and 104 female patients aged between 12-70 years. The frequency of HBsAg and anti-HCV were found to be similar to healthy controls, respectively 6.5% and 1.6% (p>0.05). As is true for low endemic countries, HBV and HCV infections might be rarely associated with GN in Turkey.

Objective: To evaluate the clinical features and outcome of patients with Behçet's syndrome (BS) and amyloidosis and to assess the associated risk factors., Method: A chart review was done to determine the frequency of amyloidosis in BS among 4000 patients. Data from 14 BS patients with amyloidosis were compared with data obtained from 718 patients with BS without amyloidosis. Multiple stepwise logistic regression analysis was used to assess the risk factors., Results: All patients with amyloidosis presented with the nephrotic syndrome or significant proteinuria. The mean time to the onset of amyloidosis was 8.1 yr (range 3-15 yr). The mean duration of follow-up after amyloidosis was 3.4 yr (range 1-11 yr). Seven out of 14 patients were alive at the time of the evaluation. Peripheral and pulmonary arterial involvement and arthritis were associated with amyloidosis (P<0.05)., Conclusion: Amyloidosis in BS is rare and has a 50% mortality rate at 3.4 yr (range 1-11 yr). Peripheral and pulmonary arterial involvement and arthritis seem to be the strongest predictors of amyloidosis in BS.

Progressive systemic sclerosis (PSS) is a connective tissue disease that may affect many organs, including the kidneys. It is quite rare to see secondary amyloidosis due to PSS. We present a patient with a 9-year history of PSS who developed nephrotic syndrome, and whose renal biopsy was compatible with secondary amyloidosis. He died from massive upper gastrointestinal bleeding caused by oesophageal telangiectasia.

Twenty-nine uremic patients with fever of unknown origin (FUO) admitted to our clinic between 1994 and 1998 were evaluated prospectively. A group of 50 consecutive non-uremic patients with FUO followed up during the same period was used for comparison. The causes of FUO found in the uremic and non-uremic groups, respectively were as follows: infectious diseases, 69 vs. 44% (p = 0.03); collagen vascular diseases, 6.9 vs. 6%; neoplasms, 3.4 vs. 26%; miscellaneous causes, 3.4 vs. 16%; and undiagnosed, 17.2 vs. 8%. Tuberculosis was the most common cause of FUO in both groups. The spectrum of underlying conditions for FUO in our uremic patients differed from that in the non-uremic patients and the uremic patients had a very high propensity for infectious diseases, especially tuberculosis.

Giant cell (temporal) arteritis (GCA) may be a cause of fever of unknown origin (FUO) in elderly patients. The development of secondary (reactive) amyloidosis is an unusual complication of the disease. We describe a 65-year-old male patient who was hospitalized in our hospital with FUO and was diagnosed as having GCA 5 years later. At that time, he also had a nephrotic syndrome and secondary amyloidosis (AA-type). He died due to end-stage renal failure. The probable explanation for the development of this rare complication might be the late diagnosis of this chronic inflammatory disease, which was left untreated for a long period of time.