Abstract

AYALA VILORIA, Alfonso J; GONZALEZ TORRES, Henry J  and  DAVID TARUD, Gabriel J. Sickle Cell Anemia: A review. Salud, Barranquilla [online]. 2016, vol.32, n.3, pp.513-527. ISSN 0120-5552.

Abstract The most common hemolytic anemia in the world population is sickle cell anemia, with an incidence of 1/600 newborns in the United States and Spain some regions 1/5000 incidence of infants; in Colombia there are no records regarding the incidence and prevalence. ACF transmission is autosomal dominant. Homozygotes (SS) do not synthesize Hb A and possess erythrocytes with 90 % Hb S. The carrier or heterozygous (AS) is greater Hb RBCs with 50 % A and Hb S of 20 - 40 % and are usually asymptomatic. Hb S is due to a mutation in the gene for beta globin chain, leading to polymerization of Hb in low oxygenation, resulting in a change in morphology sickle erythrocyte acquiring form. The symptoms are secondary to chronic hemolytic anemia, vaso-occlusion in the different organs and functional asplenia which predisposes to infection. Other associated manifestations are splenic sequestration, erythroid aplasia complications and organ - specific, which decrease the quality of life and predispose to increased mortality. Its management must be performed in reference centers where there is a comprehensive management including human and physical resources, as improper handling and its complications decreased survival which is not more than 45 years according to reports.

Keywords : hemolytic anemia; sickle cell syndrome; death in childhood.

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