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Background An increase in the number of circulating endothelial cells (CEC) indicates endothelial damage and the risk of cardiovascular disease. The aim of our study was to investigate the association of CEC with various clinical parameters in pediatric renal transplant recipients. Methods CEC, defined as [CD45.sup.-] [CD146.sup.+], were enumerated by flow cytometry from the peripheral blood of 50 pediatric renal transplant recipients and 20 healthy controls. Clinical parameters, including renal function tests, fasting blood glucose, serum cholesterol and triglyceride, cyclosporine A (CsA) (trough and 2nd-hour) and tacrolimus (tac) trough blood levels and their association with CEC numbers were analyzed. Results CEC numbers of patients were higher than those of controls (respectively, 128±89 cells/ml (42-468 cells/ml), 82±33 cells/ml (32-137 cells/ml), p = 0.024). There was a statistically significant negative correlation between CEC numbers and glomerular filtration rate (GFR) (r = -0.300, p = 0.012). There was also a statistically positive association between CEC numbers and transplant duration as well as cyclosporine trough level (respectively, r = 0.397, p = 0.004, r=0.714,p =0.004). CEC numbers in patients on tac and CsA were similar (p =0.716). Conclusions Our results demonstrate that renal transplant recipients with high CsA trough blood level, longer transplant duration, and lower GFR, are at greater risk of developing endothelial damage. Keywords CD146 * Chronic kidney disease (CKD) * Cardiovascular disease (CVD) * Pediatric kidney transplantation * Endothelial damage * Cyclosporine, Introduction Life expectancy of renal transplant recipients is shorter than the general population due to cardiovascular diseases (CVD) [1]. The risk factors for CVD in patients with chronic kidney disease [...]

Limb Girdle Muscular Disease (LGMD) comprise a group of inherited muscular dystrophy with chronic progressive weakness of hip and shoulder girdles. The inheritance pattern is either autosomal dominant (LGMD1) or autosomal recessive (LGMD2). LGMD 2A is known as calpainopathy in which there was a defect of gene encoding the protein named as calpain. There are three calpainopathy phenotypes according to distribution of muscle weakness and age at onset. In this report, we presented an asymptomatic child with persistant hyper CKemia diagnosed with muscle biopsy and genetic testing. Genetical examination results of the patient showed homozygote mutation of CAPN3 gene(c.2092C>A) and parents revealed that they were heterozygous unaffected carriers. [ABSTRACT FROM AUTHOR]

Objectives: We sought to evaluate the prevalence and confounding clinical variables of hyperuricemia in pediatric kidney transplant patients., Materials and Methods: We retrospectively evaluated the medical records of 151 pediatric renal transplant recipients who received their grafts at Akdeniz University Medical Faculty in Antalya, Turkey, with a follow-up longer than 6 months. This retrospective, single-center study included 117 pediatric renal transplant recipients, after we had excluded the patients with changes in immunosuppressive treatment and graft loss, who were receiving therapy with allopurinol and furosemide. Patient information and laboratory data were obtained from patient charts and an electronic hospital database., Results: Mean uric acid levels of patients were 311 ± 74 μmol/L, and 24 of all of the patients (20%) had high uric acid levels. Fifteen patients taking tacrolimus (16%), and 9 of patients taking cyclosporine (39%) had hyperuricemia. The hyperuricemia rate of patients taking cyclosporine was significantly higher than it was for those patients taking tacrolimus (P = .014). Mean levels of uric acid in patients taking cyclosporine were higher than those of patients taking tacrolimus (344 ± 62 μmol/L and 303 ± 75 μmol/L; P = .006). There was a significant positive correlation between mean uric acid concentrations, and both serum creatinine (P = .000; r=0.487) and cystatin C (P = .000; r=0.433). There was negative correlation between mean uric acid concentration and estimated glomerular filtration rate (P = .000; r=-0.417). Mean uric acid levels of patients with intact graft function (estimated glomerular filtration rate ≥ 60 mL/min/1.73 m²) was lower than the patients with a low estimated glomerular filtration rate (291 ± 67 μmol/L and 353 ± 71 μmol/L; P = .000). Mean uric acid level of patients with normal body mass index was significantly lower than that of patients who were obese-overweight (301 ± 64 μmol/L vs 343 ± 94 μmol/L; P = .045)., Conclusions: We found 20% of our patient group had high uric acid levels. We also found that lower glomerular filtration rate, higher serum creatinine, cystatin c, obesity, and being overweight were risk factors for hyperuricemia in pediatric renal transplant recipients.