الفهرس 
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Abstract
Thalassemias are heterogeneous group of genetic disorder of hemoglobin (Hb) synthesis result from reduced rate of production of one or more of globin chains of hemoglobin, they are characterized by varying degrees of ineffective hematopoiesis and increased hemolysis. It is one of the most common single-gene disorders with >400,000 new-born affected per year worldwide. There is a wide array of genetic defects and a corresponding diversity of clinical syndromes. Their clinical severity varies widely, ranging from asymptomatic forms to sever or even fatal entities.
This study was carried on one hundred children, fifty of them suffering from thalassemia as cases, and fifty as control, at the hematology clinic of Alexandria University Children’s hospital at El-Shatby. The aim was to evaluate cognitive functions, behavior and quality of life in children with beta thalassemia.
All patients were subjected to full history taking and clinical assessment. Cognitive functions were assessed using Vineland social maturity scale and Measurement of intelligences quotients by Standford-Binet scale. Assessment of child behavior was done through Child behavior check list, and assessment of quality of life was through Pediatric quality of life inventory (PedsQLTM) 4.0 generic core scale.
In this work, the mean age in patients was 11.33±3.05 and in control group was 10.3±2.85, there was no significant difference between the two studied groups regarding age and sex. Comparison between the two studied groups regarding the anthropometric measurements also showed no significant difference. The level of Hb in patients range from 3.5-10 gm/dl with a mean 7.79±1.69 and in control was 10.5-14.3 with a mean 12.46±1.39, there was a significant decrease in Hb level in patients than the control. WBCs show also a significant decrease in patients than control (P <0.05).