BACKGROUND The objective of this study is to evaluate the prevalence of malocclusion and treatment needs in transfusion dependent β-thalassemia major children. METHODS One hundred transfusion dependent β-thalassemia major children visiting the Department of Pediatrics were selected randomly and evaluated for malocclusion with Angle's classification and Dewey's modification. The orthodontic tr...

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 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...

A cross sectional study was carried out at various hospitals of Faisalabad city. The aim of current study was to investigate the prevalence of β-thalassemia disease in hospital population related to age, consanguinity and anti-HCV antibody positivity. For this purpose, 300 patients were interviewed for the different parameters including specific type of β-thalassemia disease, their family histo...

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...

To further define the nature of abnormal iron deposits on the membranes of pathologic red blood cells, we have used sickle cell anemia (HbSS), HbSC, and beta-thalassemic erythrocytes (RBCs) to prepare inside-out membranes (IOM) and insoluble membrane aggregates (AGGs) containing coclustered hemichrome and band 3. Study of IOM from HbSC and thalassemic patients showed that amounts of heme iron a...