Adherence to thalassemia treatment including chelation drugs is influenced by numerous factors. This study aims to explore beliefs about iron chelation therapy and adherence to this medication in Jordanian children with thalassemia major. In this descriptive cross-sectional study, seventy three patients were selected conveniently to complete the study instruments. Participants reported high adh...

Normal red blood cells, when transfused to patients with thalassemia, have been shown to have a normal life span (1-4). It has also been demonstrated that transfused erythrocytes from donors with thalassemia major rapidly disappear from the circulation of normal recipients (2, 4). This evidence supports the contention that the hemolytic component of the anemia in thalassemia is due to an intrac...

OBJECTIVE Hypothyroidism usually appears in the second decade of life and is thought to be associated with iron overload in patients with thalassemia major. This study aimed to evaluate thyroid dysfunctions in patients with beta-thalassemia major and to see if they appear in the earlier period of life. METHODS Thyroid function and iron load status were evaluated in 90 children with a mean age...

Allogeneic bone marrow transplantation (BMT) from HLA-identical siblings is an accepted treatment for both thalassemia and sickle cell disease (SCD). However, it is associated with decided risk of both transplant-related mortality (TRM) and chronic graft-versus-host disease (GVHD). We analyzed 44 patients (median age, 5 years; range, 1-20 years) given an allogeneic related cord blood transplant...

The presence of increased Hb Bart's (gamma 4) in cord blood is believed to be an indication of alpha-thalassemia. We have used restriction endonuclease nalyses of DNA to compare the number of alpha-genes with the percentage of Hb Bart's in 6 older children who had Hb Bart's at birth and 17 newborns. Four children with > 2% Hb Bart's had Eco R1 alpha-gene patterns and hematologic data consistent...

Abstract A group of inherited blood defects is known as Thalassemia among the world’s most prevalent hemoglobinopathies. Thalassemias are two types such Alpha and Beta Thalassemia. The cause these gene mutations leading to low levels and/or malfunctioning ? ? globin proteins, respectively. In some cases, one proteins may be completely absent. chains form a fold or pocket for heme (Fe++) attachm...

Hemoglobinopathies are the most common recessive diseases worldwide but their prevalence in Uruguay has not been investigated. In this study, 397 unrelated outpatient children from the Pereira Rosell Hospital Center (CHPR), as well as 31 selected patients with microcytic anemia and 28 β-thalassemia carriers were analyzed for hemoglobinopathies by using biochemical and molecular biology methods....

background: thalassemia is the most common monogenic disease in south-east of iran. despite the 70% reduction in iranian thalassemia cases after thalassemia control comprehensive program, 601 affected babies were born in sistan and balouchistan province, iran from 2002 to 2010, so this study aims at investigating the causes of new thalassemia cases. methods: data from this retrospective cross-s...

Background To study the seroprevalence of hepatitis C virus in multi-transfused children with b-thalassemia and compared with non transfused children and healthy controls. b-thalassemic children fail to thrive, with growth and developmental retardation and suffer microcytic hypochromic anemia. Since regular blood transfusions are given to maintain haemoglobin at a safe level, these children are...