Beta-thalassemias are a group of hereditary blood disorders characterized by anomalies in the synthesis of the beta chains of hemoglobin resulting in variable phenotypes ranging from severe anemia to clinically asymptomatic individuals. The total annual incidence of symptomatic individuals is estimated at 1 in 100,000 throughout the world and 1 in 10,000 people in the European Union. Three main...

β-thalassemia major can be caused by homozygosity or compound heterozygosity for β-globin gene mutations (HBB gene). Most cases are inherited from parents who both have diseased alleles of the HBB gene. We report a patient with late-onset β-thalassemia major that evolved from β-thalassemia minor in which only one of her parents had the diseased HBB gene. To study the cause of β-thalassemia majo...

  Background There is evidence indicating impaired cardiomyocytic contractility, delayed electrical conduction and increased electrophysiological heterogeneities due to iron toxicity in beta-thalassemia major patients. In the present study, we compared the electrocardiographic and echocardiographic features of beta-thalassemia major patients with a healthy control group. Materials and Methods ...

BACKGROUND AND AIM: This study was conducted to evaluate salivary immunoglobulin A (IgA) level in thalassemic patients with periodontitis in comparison to thalassemic patients with healthy periodontium.METHODS: Seventy-five patients were included in this study and were divided into three groups, group A: 25 major thalassemic patients with mild to moderate periodontitis, group B: 25 thalassemic ...

Hyperleukocytosis is an oncological emergency but is extremely rare in non-malignant conditions. Nucleated RBCs give rise to spuriously high total leucocyte count and cause clinical dilemma. Thalassemia major patients are known to have leucocytosis even after correction for nucleated RBCs. We report a case of undiagnosed Thalassemia major in a 4 month old infant with total leucocyte count highe...

introduction: in  major b -thalassemia impaired biosynthesis of beta hemoglobin leads to accumulation of unpaired alpha hemoglobin chain. an iron overload generates oxygen-free radicals which ultimately leads to tissue injury. the aim of this investigation was to evaluate serum antioxidants in patients with major b -thalassemia and those with minor thalassemia in comparison with respective age ...

Transfusion-induced hemochromatosis remains a major therapeutic complication in the management of thalassemia major patients. Using available blood cell component separators, a system has been devised to selectively harvest young red cells from transfusion support of these subjects. Red cell units isolated by this method have an average estimated mean cell age of 30 days, compared to 60 days fo...

normal 0 false false false en-us x-none ar-sa microsoftinternetexplorer4 β-thalassemia (cooley anemia) initially was described by dr cooley. [1] it is recognized that various types of thalassemia are inherited anemia caused by mutations at the globin gene, affecting the production of é‘ - or β-globin protein. the anemia interferes with red cell maturation. [2] β-globin gene mutations give rise ...