Thalassemia is a group of hereditary hemoglobin disorders characterized by insufficient production at least one globin chain, resulting in unbalanced chains. Homozygous mutations the β-globin gene, absence β-chain, are main cause β-thalassemia major. Because β-chain major not formed, there an accumulation free α-chains red blood cells, which can trigger apoptosis and hemolysis ineffective eryth...

This study investigated prenatal diagnosis of α-thalassemia and β-thalassemia in 3049 families 18 regions Hainan Province. Molecular was performed couples with thalassemia Genomic DNA extracted from peripheral blood the villus, amniotic fluid, or cord fetuses. DNA-based using polymerase chain reaction. The most commonly detected mutation for was− SEA/αα (31.53%), followed by − α4.2/αα (11.15%) ...

AIM to describe non-spesific and specific immune response profile in Indonesian thalassemia major with and without splenectomy. METHODS this study was held at Thalassaemia Centre, Cipto Mangunkusumo Hospital Jakarta on September 2013-February 2014. A comparative cross sectional study was conducted in healthy, thalassemia major aged more than 12 year and seronegative HIV. They were matched in ...

BACKGROUND Thalassemia major is one of the most common genetic disorders in Pakistan and over five thousand new patients are added in the pool annually. This familial disease has both medical and social implications, and therefore there is a need to assess the magnitude of beta-Thalassemia trait amongst family members of Thalassemia major patients. METHODS This cross-sectional descriptive stu...

Purpose To measure the retinal nerve fiber layer thickness (RNFLT) in children with β-thalassemia major and to compare with healthy controls. Methods A total of 47 patients with β-thalassemia major and 51 healthy controls were included. Each subject underwent a standard ophthalmological examination. RNFLT measurements were performed using optical coherence tomography. Results Mean age of th...

OBJECTIVE Growth retardation in children with b-thalassemia major is multifactorial. Some etiologies described for this condition are hemochromatosis, disturbed growth hormone (GH) / insulin growth factor-1 (IGF-1) axis, undernutrition and hypermetabolism. It has also been proven that growth retardation is present in b-thalassemia major children despite regular transfusion and chelation. Our ai...

Introduction: The thalassamias refer to a diverse group of hemoglobin disorders characterized by a reduced synthesis of  one or more of globin chains (α,β, γ, δβ,γδβ, δ and εγδβ).The main cure available today for thalassamia is bone marrow transplantation (BMT)  from compatible donor.On December 3, 1981 a 14-month-old child with β-thalassemia major recieved BMT from his HLA-identical sister in...

Thalassemia intermedia encompasses a wide clinical spectrum of beta-thalassemia phenotypes. Some thalassemia intermedia patients are asymptomatic until adult life, whereas others are symptomatic from as young as 2 years of age. A number of clinical complications commonly associated with thalassemia intermedia are rarely seen in thalassemia major, including extramedullary hematopoiesis, leg ulce...

Thalassemia is a hereditary condition that affects the formation of globin chains. Beta thalassemia characterized by either total loss manufacture (β-thalassemia major) or partial minor). This type inherited anemias found in Mediterranean and Southeast Asian communities. Objective: Assessment parents' knowledge regarding home health care management related to children with β-Thalassemia Major. ...