conclusion following splenectomy, thrombocytosis can predispose the patients to an increase in cerebral blood velocities more than respected with anemia. but by transcranial doppler ultrasonography no evidence of significant stenosis were found in intracerebral arteries to conclude that the beta-thalassemia major patients were more prone to the development of stroke secondary to this abnormalit...

Background: Hemoglobinopathies and betathalassemia is one of the most common autosomal disorders worldwide different molecular mechanisms, most of which are base substitution or small deletions or insertions of one or two nucleotides in the globin genes. It has been found that hemoglobinopathies and β-thalassemia mutations are relatively populations specific; each ethnic group has its own set o...

Background: Thalassemia is the most common blood disorder in the world. This hereditary anemia, Which develops due to a constitutional imbalance in alpha and beta chains of adult haemoglobin (hbA), has serious and life- threatening complications (accompanied by haemolysis and bone deformities) in its major form. We may encounter a diverse collection of articles in world research centers about t...

BACKGROUND Beta-thalassemia is the most common hematology disease in human and leptin is one of the hormone that produce by adiposities cells. The purpose of this study was to investigate the relationship between serum leptin level and thyroid hormones in children with major beta-thalassemia. MATERIALS AND METHODS This descriptive-cross sectional study was performed on 90 children aged 6-16 y...

Beta-thalassemia is due to a defect in the synthesis of the beta-globin chains, leading to alpha/beta imbalance, ineffective erythropoiesis, and chronic anemia. The spectrum of thalassemias is wide, with one end comprising thalassemia minor, which consists of a mild hypochromic microcytic anemia with no obvious clinical manifestations, while on the other end is thalassemia major, characterized ...

Heart failure secondary to iron overload is the main cause of death in patients with beta-thalassemia major. Combination therapy with deferoxamine and deferiprone has been shown to be more effective than either drug used alone in patients with beta-thalassemia major and symptomatic cardiomyopathy. Although monitoring the response to chelation therapy is usually carried out by indirect measureme...

We investigated the molecular basis for a mild phenotype in a group of patients with beta(+) thalassemia originating from Northern Sardinia by definition of the beta-thalassemia mutation, alpha-globin mapping and beta-globin haplotype determination. In nine patients, we detected the compound heterozygous state for the -87 promoter mutation and the codon 39 nonsense mutation; in one patient, we ...

Objectives: Hemoglobin E disease, c.26G>A variant of beta-globin gene, is the most common hemoglobinopathy in Asia. Compound heterozygotes inheriting Hb disease and beta-thalassemia generate beta-thalassemia-Hb with severe anemia. This study aimed to develop a pre-implantation genetic testing for monogenic disorders (PGT-M) protocol beta–thalassemia (c.17A>T mutation)-Hb (c.26G>A mutat...

Malaventura C, et al. Deferasirox, deferiprone and desferrioxamine treatment in thalassemia major patients: cardiac iron and function comparison determined by quantitative magnetic resonance imaging. Comparison of effects of oral deferiprone and subcutaneous desfer-rioxamine on myocardial iron concentrations and ventricular function in beta-thalassaemia. Effects of chelation therapy on cardiac ...

background: beta-thalassemia major is one of the major health problems in our country. many studies have confirmed the fact that, these patients have an increased susceptibility to bacterial infections. objective: in this study, we have assessed the humoral immune system in 68 thalassemic patients by measuring their serum concentration of immunoglobulin g (igg), igm, iga, c3 and c4 in order to ...