Circulating dendritic cells in pediatric patients with nephrotic syndrome

Background: Dendritic cells (DCs) represent one of the most extensively studied topics in immunology, because of their central role in the induction and regulation of adaptive immunity, and because of their therapeutic potential for manipulating immune responses. Objectives: To evaluate circulating DC levels in pediatric patients with idiopathic nephrotic syndrome (NS) and its relation to disease activity in these patients. Methods: Fifteen nephrotic patients in relapse (proteinuria>40mg/m2/hour, hypoalbuminemia, and edema) before initiating steroid therapy (Group I), and another15 nephrotic patients in remission after withdrawal of steroid therapy (Group II) were compared to 15 age- and sex- matched healthy children. Besides clinical evaluation and routine laboratory investigations of nephrotic syndrome, circulating DCs were measured by flowcytometry. Results: Circulating DC count was lower in nephrotic patients in both proteinuria and remission groups [(48.89±13.52) and (64.64±7.69) X106/liter respectively] than in the control group (78.54±9.8) X106/liter with highly significant statistical difference (p < 0.001), and lower in proteinuria group than the remission group with highly significant statistical difference (p < 0.001). There was a positive correlation between DC count and serum albumin (moderate association) (p=0.002) and a negative correlation between DC count and urine protein /creatinine ratio (strong association) (p=0.001). Conclusion: Nephrotic syndrome was associated with decreased number of circulating DCs and the decrease was more apparent in patients with active disease. The positive correlation between DC counts and total protein, and serum albumin, and the negative correlation between DC count and urine protein/creatinine ratio point to the link between the decrease in DC count and the severity of the disease process.Keywords: Denderitic cells, nephrotic syndrome, immune deficiencyEgypt J Pediatr Allergy Immunol 2011;9(1):41-4

Serum transforming growth factor-beta1 in asthmatic children

Background: Transforming growth factor-beta1 is a multifunctional cytokine which has been linked to the pathogenesis of subepithelial fibrosis and airway wall remodeling in bronchial asthma. Objective: To outline the changes in serum TGF-beta1 in children with bronchial asthma in relation to severity of asthma and different treatment modalities. Methods: Twenty-three children with bronchial asthma recruited from the Pediatric Allergy and Immunology Clinic of Ain Shams University Children’s Hospital were enrolled in the study as well as 29 healthy controls. Asthmatic children were classified according to severity into two groups; the mild asthma group which included 12 children, 4 with mild intermittent and 8 with mild persistent asthma (none received steroid therapy), and the severe persistent asthma group which included 11 children (all were on steroid therapy). All patients were subjected to clinical evaluation and laboratory investigations including absolute eosinophilic count (AEC), total serum IgE% and biologically active serum TGF-beta1 by ELISA technique. All patients were studied during acute asthma exacerbations. Reevaluation during steady state asthma was carried out for 8 patients with mild persistent asthma and 9 with severe persistent asthma. Results: During acute asthma exacerbations, the mean serum TGF-beta1 was significantly elevated in mild asthma (77.04 ± 57.04 ng/ml) compared to controls (21.81 ± 22.09 ng/ml). However for severe persistent asthma , the mean serum TGF-beta1 was significantly lower (4.23 ± 0.85 ng/ml) than in controls. Comparison of paired observations of serum TGF-beta1 revealed a significant drop, during steady state, in patients with mild asthma, whereas in severe asthma, a significant rise was observed. The levels of both asthma groups during steady state were comparable to the control values. A positive correlation, of borderline significance, between serum TGF-beta1 and total serum IgE% was observed among mild asthamtics during acute exacerbations (r = 0.55). Conclusion: The behavior of serum TGF-beta1 in acute asthma exacerbations depends on asthma severity and is perhaps related to steroid inhalation therapy. The tendency towards normality of serum TGF-beta1 in steady state asthma is possibly a good prognostic sign.Keywords: TGF-beta1, bronchial asthma, children, remodelingEgypt J Pediatr Allergy Immunol 2004; 2(1): 46-5

Interferon gamma: is it a co-player in the pathogenesis of idiopathic nephrotic syndrome

Introduction: Idiopathic nephrotic syndrome (INS), the most common form of NS in childhood, was considered 4 decades ago as a systemic disorder of T cells, mediated through its released cytokines. To date, the exact incriminated cytokine or immunological mediator is not properly defined. Interferon gamma (IFN-γ), a pro-inflammatory cytokine, is thought to have a role in the provocation of the T cell mediated INS relapse, through promotion of T helper1 (Th1) differentiation and suppression of regulatory T cells (Treg). Aim of the study: to evaluate the immunopathogenic role of IFN-γ in children with steroid sensitive idiopathic nephrotic syndrome (SSNS) through monitoring the changes in its levels with disease course. Methods: This study included twenty-five newly diagnosed children with SSINS. They were all given full dose prednisolone, evaluated at initial diagnosis and at full remission as regards the serum level of IFN-γ. Results: Serum levels of IFN-γ were lowermost at time of diagnosis and increased with remission on corticosteroids. Conclusions: this study points to a role for the lower serum IFN-γ at diagnosis, in the immunopathogenesis of INS than at remission and the rise in its serum level might be a marker of remission induction, however this awaits confirmation in larger scale studies. Studies on renal biopsy specimens are needed to determine the exact renal in situ levels and effects of IFN-