Sickle Cell Disease

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Thao Tran
Prof.Thomas
Hematology
09/08/2012
Sickle Cell Disease
Blood has been considered the essence of life for centuries, and our blood is a living tissue made up of liquid and solids. There are many diseases related to blood such as anemia which is the decrease in oxygen supply to tissue and causes hypoxia, thalassemia is the mutation in one or more globin gene of hemoglobin, etc. Therefore, one of blood disorders is the most common symptomatic hemoglobinopathy with the greatest prevalence in worldwide which is called sickle cell disease. Sickle cell disease is popular in tropical Africa, Mediterranean, the Middle East, India, Napel, and in geographic regions where there has been migrations from endemic area such as North, Central and South America. According to Center for Disease Control, sickle cell disease affects from 90,000 to 100,000 people in United States and causes death among Black or Africa-American children from 4 years of age by 42% from 1999 through 2002. (www.cdc.gov). It is interesting to note that the areas with the highest frequency of sickle cell disease also have infection with Plasmodium falciparum that causes malaria in human. In the United States, there is an estimated population of 270 millions about 1,000 babies are born with sickle cell disease each year. It means there are 1-2% of all babies having this disease in the total of 25% of people with sickle cell disease. Moreover, sickle cell disease had already spread from Africa to American by the time of slave trade. Sickle cell anemia is caused by an abnormal type of hemoglobin called hemoglobin S due to the replacement of a non-polar valine amino acid for a polar glutamic acid at the seventh position of beta chain. This substitution produces a net of decrease in negative charge; thus, it changes the molecule’s electrophoretic mobility. Hemoglobin S distorts the shape of red blood cells, especially when exposed to low oxygen levels. The distorted red blood cells are shaped like...
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