Dear Editor, The problem of congenital hemoglobin disorder is common in tropical Asia. In tropical Southeast Asian countries, very high prevalence of thalassemia disorder especially for beta thalassemia is observed. This tropical hematological problem affects millions of population and cause several health disorders. Of interest, the issue of reproductive health impairment of the population wit...

Deferitrin (GT-56-252) is the first drug in a class of desferrithiocin-derived hexadentate iron chelators. Genzyme Corp is developing this compound as an oral drug for the treatment of severe iron overload in people who require repeated erythrocyte transfusion for management of chronic anemia such as beta-thalassemia major. In phase I trials in adults with beta-thalassemia, deferitrin promoted ...

Structural hemoglobin (Hb) variants typically are based on a point mutation in a globin gene that produce a single amino acid substitution in a globin chain. Although most are of limited clinical significance, a few important subtypes have been identified with some frequency. Homozygous Hb C and Hb S (sickle cell disease) produce significant clinical manifestations, whereas Hb E and Hb D homozy...

Abstract Background Beta-thalassemia major patients are at increased risk of complications including endocrinopathies and bone disease due to iron overload. So, this study aimed assess the growth parameters, serum levels 25-OH-vitamin D, calcium, phosphorous in children with beta-thalassemia major. This was a case-control that included 55 compared 30 sex- age-matched healthy served as control g...

Background: One of the most common endocrine problems in major beta-thalassemia is hypothyroidism (HT). The aim of this study was to evaluate thyroid function status in major beta-thalassemia patients older than 10 years old. Methods: This cross sectional study was carried out on thalassemia major patients registered on Thalassemia Center of Amirkola Children Hospital in Babol. A questionnaire ...

Due to improvements in transfusion therapy in beta-thalassemia major patients, transfusional hemosiderosis has now become the major cause of late morbidity and mortality in them. In India and other developing countries, iron chelation therapy is still not strictly adhered to in these children, mostly due to financial constraints. An orally effective and cheap iron chelator is the need of the ho...

The protein composition of ghosts, inside-out vesicles (IOV), and membrane skeletons (MS) of erythrocytes (RBC) from splenectomized (spx) and nonsplenectomized (non-spx) patients with beta-thalassemia major and beta-thalassemia intermedia was determined. Ghosts from spx thalassemia intermedia patients had a significant increase in their globin content (which was mostly heme reactive) and contai...