Each year, an estimated 300,000 infants are born with either of the two most common hemoglobinopathies: the sickle cell diseases or the thalassemias. These inherited diseases are the most prevalent monogenetic disorders worldwide. Sickle cell disease makes up 85% of the total infants, and thalassemias the remaining 15%. It is increasingly apparent that sickle cell disease and thalassemia have b...

INTRODUCTION Beta thalassemia is hereditary blood disorder which is due to a defect in beta globin gene with excess of free alpha globin chains which become abnormal components in maturing red blood cells (RBCs) leading to destruction of RBCs by the spleen with subsequent anemia. Patients with beta-thalassemia major require regular blood transfusions to survive. The primary long term complicati...

Iron overload in hemoglobinopathies is secondary to blood transfusions, chronic hemolysis, and increased iron absorption and leads to tissue injury requiring the early use of chelating agents. The available agents are parenteral deferoxamine and oral deferiprone and deferasirox. There are limited data on the safety and efficacy of deferiprone at a very young age. The aim of our study was the pr...

The aim of this study was to investigate the prevalence anemias in ancient Egyptian child mummies. Whole-body computed tomography (CT) examinations 21 mummies from European museums were evaluated for estimation sex and age at death. CT systematically assessed skeletal effects using a clinical radiological approach as well quantitative measurements thickness cranial vault diploe. Additionally, t...

BACKGROUND Advances in the management of thalassemia have resulted in increased life expectancy and new challenges. We conducted the first survey of education and employment status of people with thalassemia in North America. PROCEDURES A total of 633 patients (349 adults and 284 school age children) enrolled in the Thalassemia Clinical Research Network (TCRN) registry in Canada and the U.S. ...

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...

aim: the aim of this study is to assess the growth parameters, vitamin d, calcium, and phosphorous status in children with thalassemia major receiving packed red cells transfusion with chelation therapy. patients and methods: in a case control study, 100 patients with beta thalassemia major (aged from 4 to 15 years) were compared with 100 sex- and age-matched children serves as a control group....

Objective: β-Thalassemia is one of the familiar single gene disorders which passes from parents to offspring. The prevalence of β-thalassemia trait varies from 1-14% in different regions of India. Every year almost 9000 β-thalassemic major children are being born in the Indian sub-continent. In the present study, the prevalence of β-thalassemia trait was checked and some screening tests were pe...

Abstract Background Thalassemia constitutes a major health issue in Egypt. It is associated with significant mortality and morbidity that arise mainly from iron overload secondary to blood transfusions. Methods We conducted this cross-sectional study at Cairo University children hospitals aiming assessing the patients’ their caregivers’ knowledge about thalassemia, identify factors affecting pe...

Kolestasis dapat disebabkan oleh gangguan fungsional hepatosit dalam sekresi empedu dan/atau karena obstruksi pada setiap tingkat jalur ekskresi empedu. Pada pasien thalassemia terjadi hemolisis kronis yang berakibat bilirubin tak terkonjugasi akan mengkristal dan akhirnya membentuk batu. Selanjutnya penumpukan disaluran sehingga kemudian menyumbat pengeluaran direk kolestasis. Kebaruan penelit...