Nese Ozkayin

The elevated markers of hypercoagulability in children with Henoch-Schönlein purpura

Twenty-eight children with HSP and 79 healthy children were entered into study. Activities of protein C, free- protein S and antithrombin, activated protein C resistance, levels of fibrinogen, D-dimer, thrombin-antithrombin complex (TAT), prothrombin fragments 1+2 (PF1 + 2) and von Willebrand factor antigen (vWAg) and its activity (RiCof), were investigated in acute and recovery phases of HSP and controls. Fibrinogen, D-dimer, TAT, PF1 + 2, vWAg and RiCof levels in patients with HSP during the acute phase were significantly higher than those of recovery phase and of the controls. A significant correlation was detected between severity of disease and TAT, PF1+2, vWAg, D-dimer levels.

Hypercoagulability risk factors in children with minimal change disease and the protective role of protein-C activity

It is believed that thrombotic activity in nephrotic syndrome is due to an imbalance between procoagulant/thrombotic and anticoagulant/antithrombotic factors in plasma. The aim of this study was to investigate the hypercoagulability risk in childhood minimal change disease and to find possible protective mechanisms with respect to hemostasis. Twenty-six children with minimal change disease were enrolled in this study. All patients were evaluated during an attack and on remission. The control group consisted of 33 healthy children. During the attack period, prothrombosis parameters, total lipid, cholesterol, fibrinogen levels and platelet count increased significantly compared to levels in the remission period. This denotes that hyperviscosity increases thrombosis tendency. In the attack period, the significant increase of prothrombin fragments 1+2 which shows thrombin formation and thrombin-antithrombin complex which causes prothrombin activation, are an indication of increased thrombosis risk. Five patients with lupus anticoagulant present and 7 patients with activated protein-C resistance ratios carried an increased thrombosis risk. D-dimer level of fibrinolytic factors significantly increased during the attack period. These findings emphasize the existence of thrombotic activity causing the activation of the fibrinolytic system. The significant increase in protein-C activity in these patients represents one of the protective mechanisms against thrombosis. The decrease in tissue plasminogen activator and antiplasmin indicates the protective role of fibrinolytic activity. Consequently, an increase in the protein-C activity is one of the protective mechanisms. The fibrinolytic system also plays an important role in preventing thrombotic activity in these patients.

Platelet-Activating Factor Levels in Term and Preterm Human Milk

It is well known that necrotizing enterocolitis (NEC) is less frequent in newborns being fed human breast milk. Since recent studies indicated that platelet-activating factor (PAF) plays an important role in pathogenesis of NEC, this study was conducted to investigate the PAF levels in human milk. Colostrum and mature human milk (samples obtained in the third week) of three groups of mothers were investigated. The first group had given birth within less than 32 weeks, the second between 33–37 weeks and the third group after 38 weeks of gestation. The PAF levels in colostrum of all three groups were similar (0.95 ± 0.57, 1.05 ± 0.52 and 1.19 ± 0.64 ng/ml, respectively). Mature human milk in groups I and II had similar PAF levels (1.16 ± 0.54 and 1.21 ± 0.60 ng/ml, respectively), however, mature human milk in group III had a significantly higher PAF concentration (2.04 ± 0.59 ng/ml) than both groups’ levels. However, this phenomenon by itself does not explain the protective effect of human milk against NEC.

Association Between Toll-like Receptors 4 and 2 Gene Polymorphisms With Chronic Allograft Nephropathy in Turkish Children

Toll-like receptor (TLR) gene polymorphism is known to impair intracellular signaling pathways following adaptive immune responses. Our aim was to investigate the distribution of TLR4 and TLR2 gene polymorphisms among pediatric renal transplantation patients in relation to chronic allograft nephropathy (CAN). In addition to 115 healthy controls, we included 69 renal recipients, 19 of whom were identified as CAN by biopsy scored according to the Banff criteria. Polymorphisms at TLR4 Asp299Gly and/or Thr399Ile were present in 11.6% of renal transplant recipients. None of these subjects was identified in cosegregation with the Thr399Ile allele, whereas three had an isolated Asp299Gly and five had an isolated Thr399Ile. Neither renal recipients nor healthy controls were homozygous for both Asp299Gly and Thr399Ile polymorphisms. However, TLR4 Thr399Ile polymorphism and Ile allele was greater among CAN (-) versus CAN (+) recipients (P > .05). The frequency of TLR2 mutant type Gln allele was significantly higher in recipients than among healthy controls (P < .0001). However, the Gln allele frequency was similar between CAN (+) and CAN (-) patients. The results of present study may be speculated to show TLR4 and TLR2 gene polymorphisms as protective factors from CAN development due to impaired immune responses.

The Role of Fcγ Receptor Gene Polymorphism in Pediatric Renal Transplant Rejections

The aim of the study reported herein was to determine whether panel-reactive antibody (PRA) and FcgammaR gene polymorphism act in the same way on acute rejection (AR) and chronic rejection (CR) in children who have undergone renal transplantation. The study evaluated 56 children who underwent transplantation and 115 healthy subjects. AR was observed in 13 cases; CR was observed in 7 patients. The assessment for FcgammaR of the groups in which AR was present showed statistical significance only for the FcgammaIIA genotype. There was no statistical significance for either the FcgammaIIIA or FcgammaIIIB genotypes. Assessment of the FcgammaIIA, IIIA, and IIIB genotypes of the groups in whom CR was present did not show statistical significance. As a result, the prediction of graft survival among transplant recipients is possible using molecular biology. The results of our study showed that individuals of the FcgammaRIIA genotype seemed to have a poorer prognosis similar to some autoimmune diseases. These individuals constitute a risk group for AR. If other studies are conducted with more patients to demonstrate the relationship of other FcgammaRs to rejection, the resultant predictive knowledge about the value of genotypes may lead to improved outcomes following renal transplantation.

The role of keto acids in the supportive treatment of children with chronic renal failure

According to the hyperfiltration theory of renal diseases characterized by a decrease in the number of functional nephrons, increased arterial blood pressure, excessive protein intake in the diet, high levels of calcium (Ca) and phosphorus (P), secondary hyperparathyroidism, hypertriglyceridemia and/or hypercholesterolemia, proteinuria and metabolic acidosis are some factors that impair the prognosis of the disease. The amount of protein in the diet is the most important of these factors. A protein-restricted diet administered to patients with chronic renal failure results in the risk of inadequate amino acid intake. To overcome this problem, the use of dysaminated α-keto analogues has been considered to reduce the risk of nitrogenemia resulting from the continuous intake of essential amino acids. Currently, the necessity of essential amino acids even in adult patients with chronic renal failure is controversial; besides, trials on the use of these amino acids in pediatric patients are scarce. The aim of this study is to investigate the efficacy and applicability of conservative therapy with a protein-restricted diet supplemented with keto acids in the management of chronic renal insufficiency or failure.

Complicated xanthogranulomatous pyelonephritis in a child

Xanthogranulomatous pyelonephritis (XPN) is a severe and atypical form of chronic renal parenchymal infection. Its clinical manifestations mimic neoplastic and other inflammatory diseases. XPN is seen more frequently in women. It is rare during pediatric ages and peaks in the sixth and seventh decades. We report an unusual case of complicated XPN in a boy and discuss its clinical features, histopathological findings and surgical treatment.

Evaluation of growth hormone secretion after completion of therapy.

Abstract Background : Growth hormone (GH) reserve in young adults previously diagnosed as having GH insufficiency, who were treated with human (h)GH replacement in childhood needs confirmation in adulthood.