Abstract
Background: Beta thalassemia is an inherited hemoglobin disorder resulting in chronic hemolytic anemia requiring life-long blood transfusion that cause iron overload. Silymarin plays a role as an iron chelator in iron overloaded patients. The aim of this work was to compare the iron chelating efficacy of combination therapy of oral Deferiprone and silymarin with oral Deferiprone and placebo. Patients and methods: This study was conducted on 40 children with beta thalassemia major under follow up at Hematology Unit, Pediatric Department, Tanta University Hospital in the period between October 2012 and October 2013 with their serum ferritin levels more than 1000 ng/ml and they was divided in two groups. Group I: Received oral Deferiprone and silymarin for 6 months. Group II: Received oral Deferiprone and placebo for 6 months. Results: In the current study, there were no significant differences in the initial serum ferritin, serum iron and TIBC levels between group I and group II but after regular chelation therapy, serum ferritin and serum iron were significantly lower and TIBC was significantly higher in group I than group II. No statistically significant difference in serum creatinine, blood urea, ALT, AST and serum bilirubin levels between Group I and Group II before and after chelation therapy. Conclusion: From this study we concluded that, Deferiprone in combination with silymarin are better iron chelators in iron-loaded thalassemic patients than Deferiprone and placebo.
Highlights
Thalassemias are a heterogeneous group of inherited anemias that collectively represents the most common monogenic disorders [1]. β- thalassemias are characterized by absent or reduced synthesis of β- globin chains of hemoglobin, caused by mutations of β-globin gene cluster [2] resulting in reduced hemoglobin in Red Blood Cells (RBCs), decreased RBCs production and anemia [3]
Thalassemia’s are one of the most common genetic disorders worldwide. It is the commonest cause of chronic hemolytic anemia in Middle East [27]
Silymarin acts as an iron chelator by binding Fe (III)
Summary
Thalassemias are a heterogeneous group of inherited anemias that collectively represents the most common monogenic disorders [1]. β- thalassemias are characterized by absent or reduced synthesis of β- globin chains of hemoglobin, caused by mutations of β-globin gene cluster [2] resulting in reduced hemoglobin in Red Blood Cells (RBCs), decreased RBCs production and anemia [3]. In Egypt, β-thalassemia is the commonest cause of chronic hemolytic anemia (85%) and it represents a major public health problem It is common in populations of Upper Egypt and peoples of Delta and Red Sea Hill Region [4,5]. As each unit of packed cells contains approximately 200 mg of iron so a patient who receives 25 units per year accumulates 5 gram of iron per year in the absence of chelation. Add to this the increased intestinal iron absorption. Beta thalassemia is an inherited hemoglobin disorder resulting in chronic hemolytic anemia requiring life-long blood transfusion that cause iron overload. The aim of this work was to compare the iron chelating efficacy of combination therapy of oral Deferiprone and silymarin with oral Deferiprone and placebo
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