Abstract
Sickle cell disease is define as a group of genetic disorders characterized by the presence of hemoglobin S, anemia, and acute and chronic tissue damage. Sickle cell anemia is the most common type of sickle cell disease, and is caused by the presence of the βs- globin gene in the homozygous state. At present there is no cure for sickle cell anemia except for bone marrow transplantantion, which is available only to a limited number of individuals. The lack of drugs or treatments to cure sickle cell disease is due in part to the absence of good experimental animal models. The recently available sickle cell transgenic mice models should promote the experimentation of new treatments for sickle cell disease. Until a cure is found, the treatment would continue to alleviate the clinical complications associated with the disease. Un update on immunizations, prophylactic penicillin, folic acid, trental, chelation and iron therapy, experimental and other drugs for treatment of sickle cell anemia is presented in this article.
Highlights
Sickle cell disease is define as a group of genetic disorders characterized by the presence
At present there is no cure for sickle cell anemia
treatments to cure sickle cell disease is due in part to the absence
Summary
Sickle cell disease is define as a group of genetic disorders characterized by the presence of hemoglobin S, anemia, and acute and chronic tissue damage. La enfermedad de células falciformes (ECF) o sickle cell disease se define como un grupo de desórdenes genéticos caracterizados por la presencia de hemoglobina S (Hb S), anemia y daño tisular agudo y crónico. La anemia falciforme es el tipo más común de enfermedad de células falciformes, y es causada por la presencia del gen de globina βs en el estado hemocigoto. Lamentablemente, a excepción del TMO no existe otro tratamiento curativo para la anemia drepanocítica, lo que si es factible de lograr para las complicaciones asociadas con estas enfermedades. Los enfoques terapéuticos recientes para el manejo de enfermedades de células falciformes son descritas en esta publicación. Diferentes clases de antibióticos, que son apropiados y efectivos para tratamiento en los individuos normales, pueden ser usados para las mismas indicaciones en los pacientes con ECF. Se debe tener cuidado con el trimetoprim sulfametoxazole por sus efectos colaterales en los pacientes que podrían tener deficiencia de Glucosa – 6 – Fosfato – Deshidrogenasa (G6PD) por lo potencial de causar una crisis hiperhemolítica
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