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BACKGROUND: Hepcidin, a small peptide hormone, is established as the main regulator of iron homeostasis. AIM: To estimate serum hepcidin, ferritin, and hepcidin: ferritin ratio in ß-thalassemia patients and to determine the effect of splenectomy and hydroxyurea on serum hepcidin. METHODS: A study was conducted on 30 thalassemia major (ßTM), 29 thalassemia intermedia (ßTI) and 29 healthy children's controls. Data were collected by patient interviewing where detailed history-taking and thorough clinical examinations were carried out. Serum ferritin and hepcidin were measured by ELISA assay (Bioneovan Co. Ltd Beijing, China). RESULTS: Βeta-thalassemia patients had higher serum ferritin, serum hepcidin and lower Hb and hepcidin: ferritin ratio compared to the controls (p < 0.001, 0.010, 0.001, 0.001) respectively. Β-TM patients had higher mean serum hepcidin and serum ferritin compared to ß-TI, with statistically significant difference (P = 0.042, P < 0.001, respectively). Twenty-one patients out of 29 ßTI was on hydroxyurea therapy; these patients had significantly lower levels of serum ferritin (P < 0.004) and significantly higher levels of Hb (P < 0.004). Serum ferritin was statistically significantly higher in splenectomized patients P < 0.009. Serum hepcidin level was insignificantly higher in splenectomized patients than non-splenectomized patients (21.6 ± 14.75, 17.76 ± 10.01 ng/mL). Hepcidin showed a significantly positive correlation with hepcidin: ferritin ratio in all studied groups. CONCLUSION: Serum hepcidin was elevated in ß-thalassemia children with more evident elevation in ßTM patients. Splenectomy played no major role in hepcidin regulation. Knowing that hepcidin in serum has a dynamic and multi-factorial regulation, individual evaluation of serum hepcidin and follow up, e.g. every 6 months could be valuable, and future therapeutic hepcidin agonists could be helpful in management of iron burden in such patient.

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AIM: To evaluate the relationship of apelin and nitric oxide (NO) to endothelial dysfunction in type 1 diabetics. PATIENTS AND METHODS: Sixty two type 1 diabetics and 30 healthy age and sex matched controls were included. Blood samples for apelin, NO, glycosylated hemoglobin (HbA1c), and lipid profile were collected. Albumin/creatinine ratio was assessed in urine. Flow mediated dilatation (FMD) via ultrasound was done. RESULTS: The mean age of diabetics were 16.3 ± 1.5 yrs (14.0 - 19.0 yrs), and duration of disease, were 9.4 ± 2.9 yrs (5.0 - 16.5 yrs). FMD and FMD/nitrate mediated dilatation (NMD) ratio were lower in diabetics. NO was decreased, while apelin and albumin/creatinine ratio were increased significantly in diabetics. There was a positive correlation between apelin and HbA1c. On the contrary, NO had a negative correlation with HbA1c, albumin/creatinine ratio, LDL-c and OxLDL. CONCLUSION: Diabetic patients had endothelial dysfunction and high apelin level, with no related to each other. High level of apelin is associated with bad glycemic control. Obesity had no role to increase in apelin level. NO is related to diabetic nephropathy and atherosclerosis. We recommend a further large study to evaluate the relationship of apelin with endothelial dysfunction.

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Systemic lupus erythematosus (SLE) is a life-long, life-limiting and multi-systemic autoimmune disease. Glomerulonephritis is one of the most serious manifestations of SLE. Younger children have an increased incidence, severity and morbidity of lupus nephritis (LN) compared with adult-onset disease. Monocyte chemoattractant protein-1 (MCP-1) enhances leukocyte adhesiveness and endothelial permeability in the kidneys of murine and human LN models. Our study aimed to assess the role of urinary MCP-1 in the early diagnosis of LN activity. Sixty children, of whom 45 children aged from six to 12 years old and of both sexes (15 SLE patients without nephritis, 15 active LN and 15 inactive LN) fulfilling the American College of Rheumatology Classification Criteria for SLE were studied in comparison with 15 healthy subjects. We investigated the serum and urinary MCP-1 in all groups using the enzyme-linked immunosorbent assay test. Urinary MCP-1 was significantly higher in active LN in comparison with inactive LN and controls, and also significantly higher in inactive LN in comparison with SLE without nephritis and controls. There was also a significant difference between SLE without nephritis and controls. Serum MCP-1 was significantly higher in the group with active LN in comparison with the inactive group and SLE without nephritis and controls, but there was no significant difference between SLE and controls. The urinary MCP-1 level correlated well with SLE disease activity as measured by the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). Urinary MCP-1 correlates positively with proteinuria, blood urea nitrogen level and creatinine and negatively with hemoglobin and creatinine clearance. We concluded that measurement of MCP-1 in urine may be useful for monitoring the severity of renal involvement in SLE. We recommend measuring urinary MCP-1 in pediatric SLE for the early diagnosis of LN and for the evaluation of the severity of renal involvement.

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BACKGROUND: There is no way to predict the size that proliferative infantile hemangiomas (IHs) can reach and to expect the occurrence of complications. Moreover, there are no well-known characteristics that can affect the rate of involution of IHs and to predict its completion. Accordingly, intervention is frequently indicated. Different modalities have been reported for treatment of IHs. The possible mechanisms of action of propranolol on IHs are complex. METHODS: Fifty infants presented with 80 IHs treated by oral propranolol at a dose of 2 mg/kg body weight per day. Treatment outcomes were clinically and radiologically evaluated. RESULTS: The first noticeable effects on propranolol treatment were the changes in color and softening of IHs, followed by regression of their sizes. The clinically elicited color changes of superficial IHs and superficial components of compound IHs have been objectively proven by statistically significant color clearance (P ≤ .001) and resisting index (P ≤ .01) (∼50% increase) as a good indicator of lower vascular activity within IHs. Moreover, the softening of lesions followed by the clinically elicited regression of sizes of deep IHs and deep components of compound IHs has been objectively proven by statistically significant changes at lesions' thickness (P ≤ .01) (∼50% regression) and resisting index (P ≤ .01) (∼50% increase). CONCLUSIONS: Collectively, high efficacy and tolerance of propranolol treatment have been elicited. However, propranolol treatment of IHs is still an issue suitable for more studies to confirm the safety and efficacy of the drug and to investigate whether there are some hemangiomas that are, perhaps, nonresponsive to propranolol treatment.

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INTRODUCTION: The production of tumor necrosis factor (TNF)-alpha has been deeply deregulated in systemic lupus erythematosus. We evaluated the association of -863C>A and -1031T>C polymorphisms of the TNF gene with susceptibility to and clinical manifestations of juvenile systemic lupus erythematosus. MATERIALS AND METHODS: This study was performed on 70 juvenile patients with systemic lupus erythematosus (mean age, 13.0 ± 4.2 years). Ninety age- and sex-matched children served as a controls. All participants were genotyped for the TNF -863C>A and -1031T>C polymorphisms by polymerase chain reaction-restriction fragment length polymorphism method. Analysis of serum TNF-alpha was done by solid-phase sandwich enzyme immunoassay. RESULTS: The mean serum TNF-alpha was significantly higher in the SLE patients compared to controls (P < .001). Regarding all participants, the mean serum TNF-alpha was significantly higher in children with -863AA genotype compared to carriers of -863C allele (P < .001). The TNF -863AA genotype frequencies were significantly higher in the patients group compared with the controls (P = .005) and were associated with increased risk for SLE development (odds ratio, 4.05; 95% confidence interval, 1.38 to 13.13; P = .005). The -863AA variant was associated with nephritis (P < .001) and Raynaud phenomenon (P = .001). CONCLUSIONS: The -863A allele of the TNF gene can be used as a genetic marker for SLE susceptibility and was associated with high TNF-alpha production, Raynaud phenomenon, and nephritis in juvenile SLE Egyptian patients.

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