BACKGROUND beta-thalassemia occurs from the imbalanced globin chain synthesis due to the absence or inadequate beta-globin chain production. The excessive unbound alpha-globin chains precipitate in erythroid precursors and mature red blood cells leading to ineffective erythropoiesis and hemolysis. DESIGN AND METHODS In vitro globin chain synthesis in reticulocytes from different types of thal...

BACKGROUND β-Thalassemic children have oxidative stress and antioxidant deficiency even without iron overload status. In these patients, tissue damage due to oxidative stress may be occurred. Also, it seems that thalassemic patients have higher levels of ALT, AST therefore, the main aim of the present study was to determine the benefits of vitamin E as an antioxidant supplements in β-Thalassemi...

Objective: to study the impact of haptoglobin gene polymorphism on iron overload, oxidative stress and antimullerian hormone in BTM women in Egypt. Methods: case-control study. 47 BTM women, aged between 16-26 years and 47 age-matched regularly menstruating women as control. Haptoglobin Hp1/2 gene polymorphism by PCR, Hemoglobin electrophoresis, serum haptoglobin, ferritin, malondialdehyde MDA,...

In severe human beta-thalassemia, the pathophysiology relates to accumulation of excess alpha-globin chains at the membrane. One hypothesis is that membrane-associated alpha-globin by virtue of it's iron or hemichromes produces oxidation of adjacent membrane proteins. The availability of a mouse model of severe beta-thalassemia, as well as a transgenic (thalassemic-sickle) mouse that expresses ...

β-thalassemia is one of the most common hereditary diseases in Egypt. The leading causes renal dysfunction thalassemic patients include chronic anemia, iron overload from repeated blood transfusions, and kidney-induced damage use chelators.

OBJECTIVE In beta thalassemic patients, tissue damage occurs due to oxidative stress and it happens because of the accumulation of iron in the body. This study was conducted to determine the effect of zinc and vitamin E supplementation on antioxidant status in beta-thalassemic major patients. METHODS This double blind randomized clinical trial was carried out on 120 beta thalassemic patients ...

BACKGROUND Beta-thalassemia/Hemoglobin E (beta-thal/Hb E) is a congenital hemolytic anemia that is prevalent in Thailand Pulmonary arterial occlusion is the cause of morbidity and mortality in these patients. Abnormality of platelets has been implicated as pathogenesis of this condition. However the blood-borne factors that induce platelet activation are not identified Recently, oxidized low-de...

Beta-thalassemia is an inherited hematological disease caused by a decrease or absence of production of beta-globin that requires chronic therapeutic interventions. This condition leads to important arterial and venous thromboembolic events, transitory ischemic attacks, and microcirculatory obstructions, indicative of circulatory disturbances. To investigate the presence of microcirculatory dis...

BACKGROUND Zinc deficiency has been reported frequently in hepatitis C patients in the literature. Furthermore, a decrease in zinc level has been shown in beta thalassemia major as well. Iranians consume a large amount of phytate in their regimens which can bind with zinc and decrease its gastrointestinal absorption. OBJECTIVES This study was designed to determine plasma zinc level in an Iran...

The pathophysiology of thalassemia is, to a certain extent, associated with the generation of labile iron in the pathological red blood cell (RBC). The appearance of such forms of iron at the inner and outer cell surfaces exposes the cell to conditions whereby the labile metal promotes the formation of reactive oxygen species (ROS) leading to cumulative cell damage. Another source of iron accum...