Sickle cell disease (SCD) and ß-thalassemia represent the most common hemoglobinopathies caused, respectively, by the alteration of structural features or deficient production of the ß-chain of the Hb molecule. Other hemoglobinopathies are characterized by different mutations in the α- or ß-globin genes and are associated with anemia and might require periodic or chronic blood transfusions. The...

Background: Male hypogonadism is a clinical disorder of low testosterone and spermatozoa due to impaired production that can occur at one or more levels in the hypothalamic-pituitary-gonadal (HPG) axis. Apart from iron accumulation, also other mechanisms thalassemia patients, such as influence adipose tissue leptin. The study aimed assess relationship between leptin with free BMI transfusion-de...

BACKGROUND β-Thalassemia is an inherited hemoglobin disorder caused by defective synthesis of ß-globin chains. Hemoglobin (Hb) F induction is a possible therapeutic approach which can partially compensate for α and non-α globin chains imbalance. OBJECTIVES We aimed to investigate the efficacy and safety of Hydroxyurea (HU) in diminishing transfusion requirements of patients with β-thalassemia...

Background Thalassemia is a group of congenital disorders which is characterized by a deficient synthesis of alfa or beta globulin chains in hemoglobin molecules. Due to the problems resulting from changes in face, especially in dento-maxillary regions, such patients require special attention regarding oral health. Thus in this study, we aimed to assess the dento-maxillofacial changes in Beta-t...

Background: Thalassemia patients are more susceptible to hepatitis than the normal population due to the frequent blood transfusions. Objective: To determine the immune response of children with major ß-thalassemia, by measuring anti-hepatitis B surface antibody (anti-HBs Ab) following the last HBV vaccine injection. Methods: This study was carried out on 215 thalassemic children who received t...

Abstract Introduction Major ß Thalassemia represents a group of recessively inherited hemoglobin disorder, which is characterized by reduced synthesis of globins chains. Frequent blood transfusions can lead to iron overload, which may result in several endocrine complication especially in the absence of adequate chelating therapy. The objective of this study were to determine the prevalence of...

conclusions: hydroxyurea can be safely used in some transfusion-dependent β-thalassemia patients to decrease their transfusion needs. background: β-thalassemia is an inherited hemoglobin disorder caused by defective synthesis of ß-globin chains. hemoglobin (hb) f induction is a possible therapeutic approach which can partially compensate for α and non-α globin chains imbalance. objectives: we a...

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...