background: the classical supportive therapy for î² -thalassemia major consists of regular blood transfusion, iron-chelation therapy and specific treatment of the related complications. a balance between the maintenance of the highest possible level of haemoglobin and lowest possible level of iron accumulation in heart and liver give the best chance of survival and the best quality of life. met...

Thalassemias are a group of inherited disorders that are characterized by decreased production of the alpha or beta globin chains. The amount of the produced normal hemoglobin within the red blood cells correlates with the severity of the symptoms. Fetuses with alpha-thalassemia major usually die. Children with beta-thalassemia major are usually dependent on repeated blood transfusions. Splenec...

Background Patients with thalassemia major and intermedia are susceptible to osteopenia and osteoporosis. The mechanism of osteoporosis in these patients is multifactorial. Transfusion related iron overload in endocrine organs leads to impaired growth hormone secretion, diabetes mellitus, hypothyroidism, hypoparathyroidism, lack of sex steroids and vitamin D deficiency that contribute t...

The haemoglobinopathies and thalassemias represent the most common inherited monogenic disorders in the world. Beta-thalassaemia major is an ongoing public health problem in Malaysia. Prior to 2004, the country had no national policy for screening and registry for thalassemia. In the absence of a national audit, the true figure of the extent of thalassemia in the Malaysian population was largel...

background patients with thalassemia major and intermedia are susceptible to osteopenia and osteoporosis. the mechanism of osteoporosis in these patients is multifactorial. transfusion related iron overload in endocrine organs leads to impaired growth hormone secretion, diabetes mellitus, hypothyroidism, hypoparathyroidism, lack of sex steroids and vitamin d deficiency that contribute to impair...

Background: In recent years, the success in management of thalassemic patients, has allowed for some previously unrecognized complications including renal abnormalities to emerge. This prospective study aimed to investigate kidney iron overload by means of MRI T2* and also renal function based on laboratory tests for early markers of glomerular and tubular dysfunction among adult Iranian transf...

background: many efforts have been undertaken until now to find an alternative approach to packed red cell tranfusion in major β-thalassemia. augmentation of fetal hemoglobin by hydroxyurea has proved to be less effective in this condition as compared to sickle cell anemia, probably due to molecular heterogeneity of the former disease. hydroxyurea efficacy and its relation to xmn1 polymorphism ...

conclusions there was insignificant correlation between serum hepcidin and ferritin levels in the two groups of patients with tm and ti. it seems that regulation of hepcidin in patients with thalassemia is more affected by erythropoeitic activity than iron stores. also, hepcidin levels were significantly higher in patients with tm than ti, possibly due to higher erythropoeitic activity in ti. i...

Abstract Background Beta-thalassemia is a common hereditary hemoglobinopathy, which is a reason of microcytic hypochromic anemia. Patients with major thalassemia require multiple blood transfusions. This study evaluated growth in thalassemic patient and relationship with ferritin level. Materials and Methods This is a cross sectional study on seventy patients (36 boys, 34girls) with transfu...