Genetic mutations that block aor b-globin gene expression in humans can result in severe and frequently lethal thalassemic phenotypes. Homozygous inactivation of the endogenous aor b-globin genes in mice results in corresponding thalassemic syndromes that are uniformly fatal in utero. In the current study, we show that the viability of these mice can be rescued by expression of human embryonic ...

β-Thalassemias are a group of hereditary blood disorders characterized by abnormalities in the synthesis of the β hemoglobin (Hb) chains. This disease causes excessive storage of iron in all organs and endocrine glands. Treatment of β-thalassemia major (β-TM) consists of regular blood transfusions, iron chelation and management of secondary complications of iron overload. Endocrine abnormalitie...

background: hepatitis c virus (hcv) infection is the most common transfusion transmitted disease in poly-transfused patients worldwide. in this study we aimed to evaluate the effects of pegylated interferon alfa-2a (peg-ifn a-2a) in reducing serum alt and eradicating serum hepatitis c virus (hcv) rna in hcv infected polytransfused thalassemic patients. materials and methods: a cohort of 51 hcv-...

BACKGROUND Increased hepatic iron is assumed to potentiate progression towards liver fibrosis in chronic hepatitis C virus (HCV) infection. In this study we have evaluated the potentiating effect of marked hepatic iron overload and chronic HCV infection on hepatic fibrosis in thalassemic patients. METHODS Liver biopsies of one group of patients with beta thalassemia major and chronic HCV infe...

Hemoglobinopathies have a protective role in malaria that appears to be related to alterations in red blood cell (RBC) properties. Thalassemic RBCs infected with Plasmodium falciparum showed greatly reduced cytoadherence and rosetting properties as well as impaired growth and multiplication. A significant decrease in the levels of falciparum antigens associated with the membrane of infected 8-t...

Despite recent advances in iron chelation therapy, excess iron deposition in pituitary gonadotropic cells remains one of the major problems in thalassemic patients. Hypogonadism, mostly hypogonadotropic hypogonadism, is usually detected during puberty. Early diagnosis and treatment are crucial for normal pubertal development and to reduce the complications of hypogonadism. The risks and benefit...

Thalassemia is a chronic hereditary disease in which patients with severe disease present with anemia during their first year of life. In Thailand, stem cell transplantation is not an option for most patients. Supportive treatments, such as blood transfusions and iron chelation are used. Little data exists regarding the Health Related Quality of Life (HRQoL) of these patients. We conducted a st...

Beta-thalassemia major is characterized by ineffective erythropoiesis leading to severe anemia and extensive erythroid expansion. The ineffective erythropoiesis is in part due to accelerated apoptosis of the thalassemic erythroid precursors; however, the extent of apoptosis is surprisingly variable. To understand this variability as well as the fact that some patients undergoing allogeneic marr...

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...