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...

The frequency of alpha-thalassemia was 3.6% among Turkish newborns in a study that employed globin gene mapping analysis of DnA [4]. In our study, in 13 out of 205 cord blood samples alpha-thalassemia was found 6.3%. There was mistake at incidence so hat we improved with erratum [5]. The incidence of β-thalassemia trait was very high level, the incidence of a-thalassemia trait was also found hi...

Heterozygous beta-thalassemia (β-thal) carriers are characterized by microcytosis, hypochromia, and elevated HbA2 levels (≥3.5%) [1]. Although an elevated HbA2 level is a diagnostic parameter for β-thal, the interaction between d-globin gene mutation and β-thal can result in a normal HbA2 level, leading to misdiagnosis [2]. As δ-thalassemia (δ-thal) has no clinical significance, a reduced HbA2 ...

β-thalassemia is a hereditary hemolytic anemia associated with a decrease or complete lack of synthesis of β-globin chains as a result of inheriting the mutant gene. Currently there are more than 100 varieties of hemoglobin gene mutations, which can lead to β-thalassemia. In the world there are 60-70 thousand patients with major form of this genetic anomaly. The basic treatment for β-thalassemi...

Introduction: The thalassamias refer to a diverse group of hemoglobin disorders characterized by a reduced synthesis of  one or more of globin chains (α,β, γ, δβ,γδβ, δ and εγδβ).The main cure available today for thalassamia is bone marrow transplantation (BMT)  from compatible donor.On December 3, 1981 a 14-month-old child with β-thalassemia major recieved BMT from his HLA-identical sister in...

Background: b-thalassemia major is a common hereditary blood disease that can affect patients’ oral health and quality of life. The present study aims to determine the relationship between health of deciduous teeth and the Oral Health-Related Quality of Life (OHRQoL) in children diagnosed with β-thalassemia major compared with healthy children. Materials and Methods This cross-sectional study w...

Among the reported single gene disorders/syndrome, hemoglobin infection is the most common and widespread genetic disorder. Thalassemia arises from defect in red blood cell proteinaceous component (hemoglobin), which carries oxygen and may lead to anemia. Present study aimed to detect the common molecular abnormalities of β-thalassemia syndrome in district Nowshehra, Khyber Pakhtunkhwa, Pakista...

Background: β-thalassemia is an autosomal hemoglobinopathy with inconsistent universal distribution. Among patients with thalassemia diverse non-siderotic complications distinctly influence the attribute of life, including zinc deficiency due to varied etiologies. The objective of the present study was to determine zinc levels in patients with β-thalassemia major and its correlation with mat...

As the life expectancy of β-thalassemia patients has markedly improved over the last decade, several new complications are being recognized. The presence of a high incidence of thromboembolic events, mainly in thalassemia intermedia patients, has led to the identification of a hypercoagulable state in thalassemia. In this review, the molecular and cellular mechanisms leading to hypercoagulabili...

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....