الفهرس 
Incidence of Thalassemia:
Biochemical Findings:Only 14 pages are availabe for public view
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Abstract
Beta-Thalassemias are heterogeneous autosomal recessive hereditary anemias characterized by reduced (β+) or absent (β°) synthesis of β-globin chains resulting in ineffective erythropoiesis, shortened red cell survival with subsequent anemia, elevated levels of erythropoietin (EPO), and secondary iron overload. The latter sequel is often worsened by the imperative repeated transfusions (Origa, 2017). Similar to other Mediterranean countries, β-thalassemia is a major public health problem in Egypt with particularly high incidence due to strong cultural preference for consanguineous marriages (Adly and Ebeid, 2015). It has been estimated that 1000 children out of 1.5 million live births are born annually with thalassemia major, in multicenter studies, the carrier rate in Egypt was reported to range from 5.3 to ≥ 9% and a gene frequency of 0.03 (Sherief et al., 2014). The principal source of morbidity and mortality in transfusiondependent β-thalassemia patients is iron overload. Since the body has no mechanism for excreting excess iron, untreated iron accumulation will be deposited in organs, mainly the liver, heart, and endocrine system with eventual organ impairment (Hoffbrand et al., 2012). Erythroferrone hormone (ERFE), a member of TNF-α superfamily produced from erythroblasts during erythropoiesis. ERFE production is stimulated by endogenous or exogenous erythropoietin (EPO) (Kautz et al., 2015), In such conditions, ERFE represses hepcidin production, with a consequent regain of function of ferroportin which is responsible for increasing intestinal iron absorption and mobilization of iron from stores. (Pasricha et al., 2016). A further understanding of the ERFE/hepcidin pathway may serve to define more precise targets for medical intervention to correct iron overload in β-thalassemias, other anemias associated with ineffective erythropoiesis (e.g., myelodysplastic syndromes and congenital anemia), and primary iron overload disorders (Moura and Hermine., 2015).