الفهرس 
Thalassemia
Endocrinal complications:Only 14 pages are availabe for public view
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Abstract
β-thalassemia is a hereditary disease resulting from defective hemoglobin production that causes chronic and severe hemolytic anemia. The underlying mechanism is defective production of hemoglobin beta-chains, resulting in excess of alpha-chains. This excessive intracellular deposition of alpha-chain material is responsible for accelerated apoptosis of the erythroid precursors and for peripheral haemolysis of the erythrocytes. By the age of 3 months, severe anaemia develops leading to
increased intestinal iron absorption. To maintain haemoglobin at a level of 10-12g/dl, patients suffering from b-thalassaemia major need to be given repeated blood transfusions. A major drawback of this treatment is transfusion siderosis, which, in association with the increased intestinal iron absorption, apoptosis of the erythroid precursors and peripheral haemolysis, leads to iron overload which leads to cell death. The aim of the study was to evaluate the cardiac and liver iron deposited using MRI T2* in children patients with beta-thalassemia major. The current study was conducted on 50 consecutive multi-transfused b thalassaemic children Patients with age ranging from 3 to 18 years old. These thalassemic patients were selected from those attending for a medical check-up at the Hematology Units of pediatric department of Tanta University Hospitals and referred to MRI Units of Radiodiagnosis Department, Tanta University Hospitals.