الفهرس 
Aim of The WorkOnly 14 pages are availabe for public view
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Abstract
Beta-thalassemia is one of the most common autosomal recessive disorders worldwide with high prevalence in the Mediterranean, Middle-East and Central Asia.Beta-thalassemia is caused by the reduced or absent beta globin chain synthesis of hemoglobin tetramer, which is made up of two alpha globin and two beta globin chains (alpha2beta2). The clinical severity of beta-thalassemia is related to the imbalance between the alpha globin and non-alpha globin chains. Children with transfusion-dependent thalassemia typically should undergo blood transfusions once or twice a month depending on the severity of the illness.
Sickle cell anemia is one of the commonest single gene disorders in man with variable distribution in different parts of the world and variable clinical manifestations. Sickle cell anemia commonly affects growth, leading to low mean weight, low mean height and decreased height velocity. In addition, a higher concentration of Hb F implies lower concentrations of Hb S within the cell and hence lower likelihood of untoward manifestations and complications.
Vascular endothelial growth factor (VEGF) is a mitogen for vascular endothelial cells derived from arteries, veins, and lymphatics. Previously it had been demonstrated that the vascular pathobiology of sickle cell anemia comprises a state of abnormally enhanced anti-apoptotic tone for endothelial cells and VEGF was responsible for the enhanced tone.
Our study aimed to assess serum VEGF level as a marker of angiogenesis in children with beta thalassemia major, intermedia and sickle cell anemia.
Our study was conducted on 90 Egyptian children divided in to 3 groups patient group and control group. (30) beta-thalassemia major, (15) intermedia and (15) sickle cell diseases cases considered as a patient group and (30) controls children were age and sex matched to the patient group. All patients were diagnosed as β-TM, thalassemia intermedia and sickle cell anemia based on clinical and hematological characteristics and were subjected to the following (Full history, full clinical examination, anthropometric measurements, Serum ferritin, Serum VEGF and Complete blood picture).
Our study relieved that:
• There is no statistically significant difference between studied groups regarding their age, sex and residence (p>0.05). Weight, height percentiles and consanguinity were statistically significant difference between studied groups, the highest frequency of under-weight and low height for age (< 5th percentile) among studied groups was detected among Thalassemia major followed by intermedia then sickle cell and least frequency was for control group. Consanguinity was significantly higher in Thalassemia major group than other groups.
• Median frequency of blood transfusion is significantly higher among cases with thalassemia major than intermedia and sickle cell (100% of cases with thalassemia major have frequency from 2wks-1month). Chelation therapy was significantly higher frequency among thalassemia major cases followed by thalassemia intermedia and sickle cell disease.
• There is statistically significant difference between studied groups regarding their Hb, HCT, WBCS & Platelet count. Mean hemoglobin and HCT are significantly higher among control group than cases with the highest level detected for sickle cell then Thalassemia. Median WBCS and Platelet count are significantly higher among
SUMMARY
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thalassemia than sickle cell and least was for control group. Mean serum ferritin was significantly higher among thalassemia major and least was for control group.
• There is statistically significant difference between studied groups regarding SGOT & SGPT median levels and HCV. Median SGOT, SGPT, and HCV are significantly higher among cases with thalassemia major, and then intermedia and the lowest value was detected for control group.
• Mean VEGF is significantly higher among Sickle cell disease than thalassemia major cases than thalassemia intermedia than control group.
• There is no statistically significant correlation between VEGF and any of the studied variables among thalassemia major case (p>0.05) except age of studied cases and age of disease onset are significantly negative correlation with VEGF
• VEGF and blood transfusion frequency are in significantly negative correlation in thalassemia intermedia cases, respectively. Serum transferrin, age and age of onset illustrate a significantly negative correlation with VEGF level.
• VEGF is in significantly negative correlation with transfusion frequency (r=-0.787, p=0.005).
• VEGF was significant predictor of cases with hemoglobinopathies complications with every increase one Picogram VEGF increase risk of disease complications by 1.39(OR: 1.39, 95% CI: 1. 16-1.68) with the overall percent predicted was 91.1%, ie increase VEGF increase prediction of the presence of new vessels formation in cases of hemoglobinopathies.