BACKGROUND Using two logistic regression models, we determined the associates of poor physical and mental health related quality of life (HRQoL) among beta thalassemia patients. METHODS In this cross-sectional study which was conducted during 2006 and 2007 in outpatient adult thalassemia clinic, Blood Transfusion Organization, Tehran, Iran, Short Form 36 (SF-36) was used for measuring HRQoL i...

BACKGROUND Cardiac complications are among the most serious problems of thalassemia intermedia patients. The current study was initiated to address the latter issue through the study of the echocardiographic findings and correlate it with clinical characteristics of thalassemia intermedia patients in Duhok, Kurdistan region, Iraq. METHODS An echocardiographic assessment of 61 beta-thalassemia...

BACKGROUND Beta thalassemia is one of the most common genetic disorders in the world. The aim of this study was to determine the frequency, characteristics, and pattern of malignancies in patients with beta thalassemia major (BTM) and beta thalassemia intermedia (BTI) in Iran. METHODS We conducted a multicenter study via a retrospective chart review of patients with BTM and BTI between 2002 a...

beta-thalassemias are the most common single gene disorders and are potentially amenable to gene therapy. However, retroviral vectors carrying the human beta-globin cassette have been notoriously unstable. Recently, considerable progress has been made using lentiviral vectors, which stably transmit the beta-globin expression cassette. Thus far, mouse studies have shown correction of the beta-th...

The hereditary persistence of fetal hemoglobin (HPFH) and delta beta thalassemia are heterogeneous disorders characterised by increased levels of fetal hemoglobin and high level of this Hb continues in adulthood. The distinction between these two conditions is not always possible with routine hematologic analysis and molecular characterisation of the defect is required. We encountered such a ra...

BACKGROUND Beta-thalassemia is one of the most common genetic disorders in Thailand. Clinical phenotype ranges from silent carrier to clinically manifested conditions including severe beta-thalassemia major and mild beta-thalassemia intermedia. OBJECTIVE This study aimed to characterize the spectrum of beta-globin gene mutations in pediatric patients who were followed-up in Phramongkutklao Ho...

3. Taher A, Isma'eel H, Cappellini MD. Thalassemia intermedia: revisited. Blood Cells Mol Dis 2006;37:12-20. 4. Huehns ER, Dance N, Beaven GH, Heclht F, Motulsky AG. Human embryonic hemoglobins. Cold Spring Harb Symp Quant Biol 1964;29:327-31. 5. Villegas A, Ropero P, González FA, Anguita E, Espinós D. The thalassemia syndromes: molecular characterization in the Spanish population. Hemoglobin 2...

Thalassemia is a congenital hemolytic disorder caused by a partial or complete deficiency of alpha- or beta-globin chain synthesis. Homozygous carriers of beta-globin gene defects suffer from severe anemia and other serious complications from early childhood. The disease is treated by chronic blood transfusion. However, this can cause severe iron overload resulting in progressive organ failure....

BACKGROUND Thalassemia intermedia patients usually do not require blood transfusions; however, all show variable degrees of erythropoietic marrow expansion to compensate for more or less marked anemia, and this represents the major cause of complications in untransfused individuals. MATERIALS AND METHODS To assess the degree of erythropoietic expansion in thalassemia intermedia, serum erythro...

Background: Expansion of bone marrow cavity and decreased cortical and trabecular bone tissues and osteoporosis are resulted from beta-thalassemia. The aim of this study was to assess bone mineral density (BMD) in patients with β thalassemia major and intermedia, and to determine their biochemical and hormonal profiles that may affect BMD. Materials and Methods: In a cross sectional study from ...