Fracture prevalence and relationship to endocrinopathy in iron overloaded patients with sickle cell disease and thalassemia

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Pulmonary Spirometry Parameters in Patients with Sickle Thalassemia and Sickle Cell Disease at Shafa Hospital in Khuzestan Province-Iran

Abstract Background Prevalence of hereditary blood diseases such as sickle cell anemia, sickle thalassemia and thalassemia major are high in Khuzestan province. Sickle cell anemia and beta-thalassemia are predominantly common in Iranian Arabs. Pulmonary complications account for a large proportion of morbidity and mortality in patients with and sickle cell disease. Periodic lung function asse...

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Side effects of hydroxyurea in patients with Thalassemia major and thalassemia intermedia and sickle cell anemia

Background Sickle hemoglobin is the most common abnormal hemoglobin in the United States. Hemoglobin S arises as a result of a single amino acid substitution (glutamic acid to valin at position 6 of the β-globine chain). The presence of fetal hemoglobin (HbF) plays a relatively protective role since a significant amount of HbF interferes with HbS polymerization, the pathogenesis mechanism of ...

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Iron Metabolism in Thalassemia and Sickle Cell Disease.

THERE ARE TWO MAIN MECHANISMS BY WHICH IRON OVERLOAD DEVELOPS IN THALASSEMIAS: increased iron absorption due to ineffective erythropoiesis and blood transfusions. In nontransfused patients with severe thalassemia, abnormal dietary iron absorption increases body iron burden between 2 and 5 g per year. If regular transfusions are required, this doubles the rate of iron accumulation leading to ear...

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The Effect of L-Glutamine on Pain Crisis Reduction in Patients with Sickle Cell Anemia and Sickle β°-Thalassemia

Background: A low level of L-glutamine, a precursor of nicotinamide adenine dinucleotide (NAD) in red blood cells (RBCs), is identified as an underlying mechanism for the potential decrement of the NAD redox and the incidence of pain crisis in sickle cell anemia (SCA). The aim of this study is to assess the impact of oral L-glutamine therapy on pain crisis reduction in patients with SCA and sic...

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ژورنال

عنوان ژورنال: Bone

سال: 2008

ISSN: 8756-3282

DOI: 10.1016/j.bone.2008.03.003