الفهرس 
eta ThalassemiaOnly 14 pages are availabe for public view
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Abstract
Introduction: B-thalassemias are heterogeneous autosomal recessive hereditary anaemias characterized by reduced (B+) or absent (B°) synthesis of B-globin chains resulting in ineffective erythropoiesis, shortened red cell survival with subsequent anaemia, elevated levels of erythropoietin (EPO), and secondary iron overload. The latter sequel is often worsened by repeated transfusions (Mangaonkar et al.,2020). Hepcidin is a hormone, secreted by the liver, that regulates iron homeostasis. In contrast, as humans have little ability to excrete iron, iron overload in patients with transfusion dependent thalassemia (TDT) arises when frequent blood transfusion leads to iron deposition in the liver and heart (Badawy et al., 2016). The Aim of this study:To evaluate serum hepcidin and erythroferrone concentrations as new biomarkers that could predict iron status in B-thalassemia patients. Materials and methods: This was a case-control study conducted on a total number of 120 subjects classified as follow: • Disease group : •Seventy (70) cases of B-thalassemia major and intermedia who were admitted at Hematology clinic at Mansoura University Children Hospital within one year .Patient group was diagnosed on the basis of clinical findings and Hb electrophoresis. •Control groups. •30 healthy individuals of matched age and sex as normal control group. Healthy control group was taken from blood bank donors. •20 cases of sickle cell disease patients act as a disease control group. They were diagnosed on the basis of clinical findings, Hb electrophoresis and sickling test. A detailed history was taken from parents of both patients and control groups. •Serum hepcidin and erythroferrone levels were done by using ELISA Technique. Results: •A highly significant increase in patients’ iron indices (ferritin, TS%, serum iron) as compared to normal control group except for TIBC which is significantly higher in normal control group compared to patient groups. •In cases of B-thalassemia, there was a highly significant erythroferrone levels and significantly low hepcidin levels compared to normal control group. •A significant negative correlation between erythroferrone and hepcidin was found in B-thalassemia and normal groups but such correlation was absent in sickle cell group. Recommendations: •Due to the small number of patients included in the study , further studies are needed in larger scale to confirm results of our study. •Because of inter individual variations, we need more studies to confirm the effect of correlation between hepcidin and erythroferrone in prediction of iron overload in iron loading anemias. •A Further larger scale follow up studies are needed to confirm the effect of iron regulatory hormone hepcidin on iron status.