الفهرس | Only 14 pages are availabe for public view |
Abstract Thalassemia is among the most common genetic disorder worldwide. In ?-thalassemia major, apoptosis appears to be greatly enhanced in the early stage erythroid precursors in the bone marrow leading to ineffective erythropoiesis. Transferring receptors are present on almost all mammalian cells. They are used to evaluate the body’s iron status and erythropoietic activity and its level provides a sensitive quantitative measure of total endogenous erythropoiesis that is less invasive than bone marrow examination. L-carnitine facilitates the transfer of activated long chain fatty acids from the cytoplasm to the mitochondria, and so strongly reduce apoptosis in different diseases. The aim of this study is to evaluate the erythropoietic activity by estimating both the serum EPO and sTfR levels in somes ?-thalassemia cases and study the effect of L-carnitine on improving ineffective erythropoiesis among those patients. This study included 30 patients with ?-thalassemia, 25 were ?-thalassemia major and 5 were ?-thalassemia intermedia. They were divided into patients under regular blood transfusion group and another group which was under irregular blood transfusion. The 30 subjects were also, divided into a group with positive history of L-carnitine therapy and another group with negative history of L-carnitine therapy. And 15 healthy children were included as a control group. For each individual, the following was done:- full medical history, examination especially for hepatomegaly and Splenomegaly |