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Sickle Cell Anemia

What is Sickle Cell anemia







What is sickle cell anemia

Sickle cell diss are a group of hereditary diss that occurs due to the transmission of a faulty gene that expresses an altered beta-globulin chain in the hemoglobin (Mackie , 1996 , pp . 792 . The sixth amino acid present in the beta-globulin is replaced from glutamic acid to valine (amino acids . The individual has higher amount of defective hemoglobin present in the red blood cells

known as `hemoglobin sickle cell ' or `Hb-S . Several defects develop in the red blood cells and in the low-oxygen conditions , the hemoglobin molecules polymerize resulting in formation of crystal-like structures , the membrane of the red blood cells (RBC 's ) become thick and sticky , and the cell gets dehydrated resulting in the formation of `sickle-shaped cells (Benz 2000 , ch . 106-CD-ROM . The affected individual develops certain characteristic features such involvement of several organs and tissues in the boy , anemia resulting from the destruction of red blood cells (hemolytic anemia ) and episodes of severe pain . The RBC 's in low oxygen conditions , get sickled and begin to clog the blood vessels (Saunthararajah , 2005 , ch . 37-Online

There are two major conditions related to sickle-cell disease , namely sickle cell anemia and sickle cell trait . These conditions are inherited in an autosomal co-dominant pattern due to a defect in the gene that expresses or codes for beta-globulin protein of the hemoglobin . In sickle cell anemia , the individual has two copies of the defective gene that codes for hemoglobin inherited from both the parents (homozygous condition . The individual develops symptoms of sickling as the level of hemoglobin -S is high in the blood . On the other hand sickle cell trait is a condition in which the individual inherits only one copy of the defective gene that codes for the defective hemoglobin inherited from either of the parents (heterozygous condition . The blood counts and the general health of the individual may be usually normal (symptoms of anemia and recurrent pain are usually not present (Saunthararajah , 2005 , ch . 37-Online

The highest incidences of sickle-cell anemia is seen the African continent , especially in the malarial belt . The prevalence of Sickle cell trait in the African-Americans is as high as 8 to 10 in babies Sickle cell anemia occurs in one individual out of every 400 African-Americans on an average . In parts of Africa , the incidences of sickle cell trait are as high as 25 to 30 (Forget , 1997 , pp . 888 . In Africa , more than a hundred thousand babies are born each year with the sickle-cell disease . Since a long time , several of the members of African tribes have experienced episodes of severe recurrent pain Herrick began to study sickle-cell anemia during the beginning of the 20th century in Chicago . Hahn and Gillespie in the year 1927 demonstrated that de-oxygenation was associated with sickling . They also showed that the condition was reversible following oxygenation Pauling et al in 1927 demonstrated that the defective nature of the...

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