الفهرس يوجد فقط 14 صفحة متاحة للعرض العام
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المستخلص
Beta thalassemia syndromes are a group of hereditary disorders characterized by a genetic deficiency in the synthesis of beta-globin chains.
In the homozygous state, beta thalassemia (thalassemia major)causes severe transfusion-dependent anemia.
Cure of thalassemia major is available with bone marrow transplantation.
However because of lack of awareness and affordability,regular blood transfusion remains the mainstay of treatment for thalassemic children.
Most of them are regularly transfused without iron chelation, leading to iron overload. Because of cost and duration of iron chelation therapy, very few patients can afford it. Thus, in thalassemics iron overload becomes a major problem which leads to multiorgan dysfunction.
Growth retardation has been reported to occur in most patients with thalassemia major.
This is believed to be due to a direct effect of iron overload on the endocrine gland.
Growth Hormone (GH) impairment at different levels (hypothalamic or pituitary) and/or reduced insulin like growth factor 1 (IGF-1) synthesis have been suggested to be the main causes of stunned growth in these patients.
The present study was carried out to evaluate the hormonal status and the effect of iron overload on growth status, liver and kidney functions of children with thalassemia major and to determine the value of chelating therapy on these children.
The present study was carried out on fifty five (55) children: forty(40) children with beta thalassemia major (patient group) and fifteen (15)healthy children (control group).
The thalassemic patients were attending pediatric hematology clinic and in-patient pediatric department in Menoufiya University Hospital. Their ages ranged from 5-12 years.