Sickle Cell Disease and Sickle Cell Anaemia
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چکیده
Sickle cell haemoglobin (HbS) results from an autosomal recessively inherited mutation in which the 17th nucleotide of the beta globin gene is changed from thymine to adenine and the amino acid glutamic acid is replaced by valine at position 6 in the beta globin chain. [1, 2]Sickle cells have a reduced deformability and are easily destroyed, causing occlusion of the microcirculation and a chronic haemolytic anaemia with a median haemoglobin concentration level of about 9 g/dL. [3] Sickling disorders include heterozygous (AS) sickle cell trait, homozygous (SS) sickle cell disease, compound heterozygous states for HbS with haemoglobins C, D, E, or other structural variants and the combination of the sickle cell gene with different forms of thalassaemia.
منابع مشابه
Antioxidant Enzymes and Acute Phase Proteins Correlate with Marker of Lipid Peroxide in Adult Nigerian Sickle Cell Disease Patients
Objective(s) Sickle cell disease is a genetic disorder characterized by chronic haemolytic anaemia. Haemoglobin S containing red blood cells may be susceptible to oxidative stress due to imbalance between production of reactive oxygen species and the countering effect of the various antioxidants present in the body. Materials and Methods We evaluated some antioxidant enzymes which include gl...
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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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