الفهرس 
Thalassemia syndromesOnly 14 pages are availabe for public view
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Abstract
Beta thalassemia is an inherited disorder of ~-globin synthesis due to mutations that result in decreased levels of a structurally normal ~-globin protein.In ~-thalassemia,excess unpaired a-globin chains resulting from the ~-globin synthesis defect are toxic to erythroid precursors,causing their premature death in the bone marrow. Such abortive erythropoiesis is termed ineffective erythropoiesis and is a particulary potent inducer of the erythroid regulator of iron homeostasis. The peptide hormone hepcidin is the key regulator of iron absorption and recycling.Hepcidin binds to and induces the internalization and degradation of ferroportin,an iron export protein present on the cell surface of macrophages and duodenal enterocytes.Hepcidin limits iron recycling and absorption,thereby controlling body iron stores.Hepcidin expression is inversely correlated with body iron stores.So in beta thalassemic patients the level of hepcidin is markedly decreased.