الفهرس 
THALASSEMIAOnly 14 pages are availabe for public view
Abstract
T
halassemia is a heterogeneous inherited disorder of hemoglobin synthesis due to mutations of the globin gene, leading to various degrees of quantitative defect in globin production and reduced synthesis or complete absence of one or more of the globin chains, resulting in ineffective erythropoiesis and anemia.
Beta-thalassemias are a group of hereditary blood disorders characterized by anomalies in the synthesis of the beta chains of hemoglobin resulting in variable phenotypes ranging from severe anemia to clinically asymptomatic individuals. Three main forms have been described: thalassemia major, thalassemia intermedia and thalassemia minor and is usually presented at 4 - 6 months of life, due to the protective effect of high hemoglobin F concentration at birth that slowly declines through the first year of life.
This study is a retrospective cross-sectional study included 250 children aged (5-15 years) divided into 200 child with thalathaemia and 50 normal control children, attending the hematology clinic of pediatric hospital of Ain Shams University, Cairo, Egypt.
The objective of the current study was to assess growth and body composition in patients with thalassemia using body composition of fat, lean body mass, which was assessed using (TANITA SC330), measurement of serum lipid profile (high density lipoprotein, low density lipoprotein, cholesterol and triglyceride) and serum albumin and anthropometric data to every patient (weight, height, mid arm circumference, skin fold thickness and body mass index). Particular emphasis was placed on the relationship between body composition, growth, lipid profile and insulin profile.
The specific hypothesis for this study were as follows: (a) patients with thalassemia have lower body mass index (BMI) in comparison with age and sex-matched published normative values, (b) calcium intake and physical activity are related to body composition, and finally (c) insufficient body fat is associated with poor growth, pubertal development and bone health,(d) there is correlation between body composition, lipid profile and glucose profile.
The results of the current study can be summarized into the following:
Significant increase in male % in both patients group and control group was noticed. Moreover, significant increase in consanguinity in both patients and control group was recorded. However, thalassemia % decreased with family history in the patient group.
Concerning results of laboratory hematological parameters in our study, data revealed significant decrease in hemoglobin concentration in thalassemic patients in comparison to control group. On the other hand, total leukocytes count, platelets count and serum ferritin exhibited marked elevation in thalassemic group compared to the control group.
Regarding laboratory metabolic assessment, there was significant decrease in Serum Insulin recorded in patient group compared to the control group.
As regards the components of food analysis, there was a significant decrease in the amount of all components of diet including Kilocalories, Protein, Carbohydrate, Calcium, Potassium and phosphorus in patient group as compared to the control group.
Significant correlation was recorded between total leucocytic count, platelet count and high density lipoproteins level in patient group and all parameters of body composition
Concerning the relation between sex and body composition in patient group, there was significant increase in body fat % and body fat value in female patients compared to male patients, while there was significant increase in Muscle mass % and Body water % in male patients as compared to female patients.