الفهرس 
Sickle cell anemiaOnly 14 pages are availabe for public view
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Abstract
Sickle cell disease is a genetic abnormality involving the hemoglobin. Patients present with a wide spectrum of disorders because of a single-point mutation in which thymine substitute for adenine, thereby encoding valine instead of glutamine in the sixth position of the beta-chain. Hemoglobin S resulting from the substitution causes polymerization of hemoglobin and red cell sickling on exposure to low oxygen tension and unsickle on oxygenation. Sickle cell disease (SCD) is characterized by an increased susceptibility to infections and vaso-occlusive complications. Patients with SCD can develop specific and sometimes life-threatening complications, as well as extensive organ damage reducing both their quality of life and their life expectancy. Over many years, different methods for managing this disease have been proposed and studied including transfusion therapy, hydroxyurea, stem cell transplantation and gene therapy. However, frequent blood transfusions have been associated with iron overload, which may result in many complications. Endocrine glands affected by iron deposition leading to many complications including hypogonadism, hypothyroidism diabetes mellitus, hypoparathyroidism and other endocrine abnormalities.