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: Anemia is most common public health problems in developing countries. It important to know the cause of anemia treat patients. The Immature reticulocyte fraction (IRF) value an early marker for evaluating regeneration erythropoiesis. In addition conventional measurement, fluorescence method allows classification reticulocytes into three maturation stages – LFR, MFR and HFR. Aims: To...

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...

Previous studies of beta-thalassemic reticulocytes have implied a decreased amount of functional beta-mRNA but unimpaired translation of the beta-mRNA present. However, the beta/alpha synthetic ratios in beta-thalassemic marrow are higher than those observed in reticulocytes of the same patients. This could imply that marrow cells contain an abnormally functioning beta-mRNA no longer active in ...

introduction: leptin is an adipocyte-derived hormone. exogenous leptin allows the recovery of the reproductive function. in humans, leptin correlates positively with the body mass index (bmi). the aim of this study was to investigate the association of leptin with the toxic effects of iron overload. methods: 219 major beta thalassemic patients (119 men, 100 women) and 137 non thalassemic indivi...