molecular aspects of bone resorption in β-thalassemia major
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منابع مشابه
Molecular Aspects of Bone Resorption in β-Thalassemia Major
β-thalassemia is the most common single gene disorder worldwide, in which hemoglobin β-chain production is decreased. Today, the life expectancy of thalassemic patients is increased because of a variety of treatment methods; however treatment related complications have also increased. The most common side effect is osteoporosis, which usually occurs in early adulthood as a consequence of increa...
متن کاملBone Mineral Density in β Thalassemia Major and Intermedia, Correlation with Biochemical and Hormonal Profiles
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 ...
متن کاملHLA-matched sibling bone marrow transplantation for β-thalassemia major.
We describe outcomes after human leukocyte antigen-matched sibling bone marrow transplantation (BMT) for 179 patients with β-thalassemia major. The median age at transplantation was 7 years and the median follow-up was 6 years. The distribution of Pesaro risk class I, II, and III categories was 2%, 42%, and 36%, respectively. The day 30 cumulative incidence of neutrophil recovery and day 100 pl...
متن کاملPortal Vein Thrombosis Following Splenectomy in β-thalassemia Major
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متن کاملMolecular Diagnostics of β-Thalassemia
A high-quality hemoglobinopathy diagnosis is based on the results of a number of tests including assays for molecular identification of causative mutations. We describe the current diagnostic strategy for the identification of β-thalassemias and hemoglobin (Hb) variants at the International Reference Laboratory for Haemoglobinopathies, Research Centre for Genetic Engineering and Biotechnology (...
متن کاملPhysiopathology of Bone Modifications in β-Thalassemia
β-thalassemia major (βTM) or Cooley anemia is characterized by significantly reduced or absent synthesis of β-globin chains, which induces important pathologic consequences including hemolytic anemia, altered erythropoiesis, and bone marrow overstimulation. The pathogenesis of bone changes in patients with βTM is not yet completely understood. However, an unbalance in bone mineral turnover resu...
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عنوان ژورنال:
cell journalجلد ۱۷، شماره ۲، صفحات ۱۹۳-۲۰۰
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