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    Congenital adrenal hyperplasia: A rare cause of renal failure and a successful renal transplantation
    (2012) Gungor O.; Kircelli F.; Carrero J.J.; Hur E.; Dheir H.; Simsir A.; Okmen F.; Toz H.; Hoscoskun C.
    Congenital adrenal hyperplasia belongs to a group of autosomal recessive disorders affecting steroid biosynthesis; a rare disease with a prevalence of 1 case per 16,000 population. A 30-year-old phenotypically male patient had been diagnosed with 11-ß hydroxylase deficiency at the age of 16; presenting with ambiguous genitalia, growth retardation, presence of menstrual cycles, severe hypertension, hypokalemia and renal dysfunction. He developed end-stage renal disease due to hypertension and was treated with hemodialysis for 3 years. After careful evaluation, he was approved to undergo renal transplantation. The patient has now finished 6th month after transplantation and is currently under follow-up at our out-patient clinic, having no problems related to the transplant. While early treatment to prevent hypertension is mandatory in patients with congenital adrenal hyperplasia, once renal failure occurs, renal transplantation may the best choice of treatment. In this study, we describe the first report of a successful renal transplantation in an adrenal hyperplasia. © 2012 Dustri-Verlag Dr. K. Feistle.
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    Endogenous testosterone and mortality in male hemodialysis patients: Is it the result of aging?
    (2010) Gungor O.; Kircelli F.; Carrero J.J.; Asci G.; Toz H.; Tatar E.; Hur E.; Sever M.S.; Arinsoy T.; Ok E.
    Background and objectives: Low serum testosterone levels in hemodialysis (HD) patients have recently been associated with cardiovascular risk factors and increased mortality. To confirm this observation, we investigated the predictive role of serum total testosterone levels on mortality in a large group of male HD patients from Turkey. Design, settings, participants, & measurements: A total of 420 prevalent male HD patients were sampled in March 2005 and followed up for all-cause mortality. Serum total testosterone levels were measured by ELISA at baseline and studied in relation to mortality and cardiovascular risk profile. Results: Mean testosterone level was 8.69 ± 4.10 (0.17 to 27.40) nmol/L. A large proportion of patients (66%) had testosterone deficiency (<10 nmol/L). In univariate analysis, serum testosterone levels were positively correlated with creatinine and inversely correlated with age, body mass index, and lipid parameters. During an average follow-up of 32 months, 104 (24.8%) patients died. The overall survival rate was significantly lower in patients within the low testosterone tertile (<6.8 nmol/L) compared with those within the high tertile (>10.1 nmol/L; 64 versus 81%; P = 0.004). A 1-nmol/L increase in serum testosterone level was associated with a 7% decrease in overall mortality (hazard ratio 0.93; 95% confidence interval 0.89 to 0.98; P = 0.01); however, this association was dependent on age and other risk factors in adjusted Cox regression analyses. Conclusions: Testosterone deficiency is common in male HD patients. Although testosterone levels, per se, predicted mortality in this population, this association was largely dependent on age. Copyright © 2010 by the American Society of Nephrology.
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    Soluble TWEAK level: Is it a marker for cardiovascular disease in long-term hemodialysis patients?
    (2013) Gungor O.; Kircelli F.; Asci G.; Carrero J.J.; Tatar E.; Demirci M.S.; Özbek S.S.; Ceylan N.; Toz H.; Ozkahya M.; Ok E.
    Background: Reduced soluble tumor necrosis factor- like weak inducer of apoptosis (sTWEAK) levels follow declining renal function, are strongly associated with endothelial dysfunction and predict cardiovascular events in nondialyzed chronic kidney disease patients. In contrast, elevated levels of sTWEAK predict poor survival in hemodialysis (HD) patients. Recent evidence suggests a role for sTWEAK in the pathophysiology of vascular calcification. The aim of the study was to investigate plausible links between sTWEAK, atherosclerosis, arterial stiffness and vascular calcification in HD patients. Methods: Coronary artery calcification score (CACs) determined by multislice computed tomography, arterial stiffness by pulse wave velocity (PWV) and carotid artery intima-media thickness (CA-IMT) by carotid Doppler ultrasonography were assessed in 131 longterm prevalent HD patients. sTWEAK levels were measured by ELISA (Bender MedSystems, Vienna, Austria). Results: Mean serum sTWEAK level was 237.0 ± 147.5 pg/mL (range 78-937). sTWEAK level was inversely correlated with CA-IMT at a borderline significance (r=-0.168, p=0.05). Neither carotid-radial PWV nor carotid-femoral PWV values correlated with sTWEAK. sTWEAK level was higher in patients with severe vascular calcification (CACs ?400) compared to patients with CACs <400 (264.5 ± 146.8 pg/mL vs. 205.04 ± 122.4 pg/mL, p=0.02). The association between sTWEAK and vascular calcification persisted after multivariate adjustment. Conclusions: There exists a weak inverse correlation between sTWEAK and carotid atherosclerosis and a positive correlation with coronary artery calcification in long-term HD patients. Our data give support for a role for sTWEAK in the pathogenesis of vascular injury in HD patients. © 2012 Società Italiana di Nefrologia - ISSN 1121-8428.