“This disease has impacted my life in so many different ways: good and bad.” These are the words from Natasha Thomas, a woman who was diagnosed with sickle cell disease at the age of seven. Sickle cell disease is a blood disorder that affects the red blood cells in the body. A healthy red blood cell is normally round and the sickle cells are half-moon shaped. This irregular shape does not carry enough oxygen throughout the body causing organ damage...
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Sickle cell anemia is severe, chronic and even fatal disease. It causes red blood cells to break down and and sickle (form a crescent shape). It occurs due to a point mutation in the Red blood cells which blocks blood flow therefore there is a chronic deprivation of oxygen which leads to complications such as damage to nerves, kidneys, liver, spleen and other organs in the body. Therefore it is important to find a cure, Gene therapy is one of...
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What is sickle-cell anaemia Sickle-cell anaemia is a hereditary, homozygous, genetic blood disorder that occurs within a person who has abnormal haemoglobin on their red blood cells. The Haemoglobin are sensitive to low amounts of oxygen in the body which makes them transform into sickle or crescent shapes. This causes the abnormal haemoglobin to become stiff and sticky blocking blood flow to important organs and tissues. Healthy red blood cells will survive within the body for 120 days however, sickle-cells...
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Introduction Haemoglobin is a word that was coined from two words “haemo” which means blood and “globin” meaning protein. Globin is a protein substance of four different polypeptide chains that have (141-146)amino acids. Haemoglobin is a conjugated globular protein. There are two important oxygenbinding proteins in vertebrates namely haemoglobin (Hb or Hgb) and myoglobin. Haemoglobin supplies oxygen (O2) to tissues. Haemoglobin’s function is to transport oxygen (O2) in the blood from the lungs to other tissues of the body and...
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Abstract Sickle cell anemia (SCA) is a globally prevalent, monogenic, life-threatening blood disorder with a complex pathology that remains obscure. A deepened understanding of the malady in the recent years has led to pharmaceutical advancements that target pathophysiology and ultimately ameliorate associated multivariate clinical manifestations. Abnormal cell to cell interactions, endothelial adhesion, induced oxidative stress, intracellular erythrocyte dehydration and concentration levels of fetal hemoglobin are a few factors know to play a key role in microvascular obstruction in individuals with...
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Sickle cell disease (SCD) is a group of genetically inherited diseases that affect the red blood cells. This disease is caused by a base substitution – which is a type of mutation- where the sixth amino acid valine is substituted for glutamic acid. The difference in the solubility of the two amino acids causes a change in the shape of the haemoglobin, where the round shape of the Hb becomes sickled. The sickled haemoglobin carries oxygen less efficiently due to...
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Oxidative Stress and Vascular Tone The aberrant biochemistry of SCA promotes premature intravascular hemolysis. This occurs when sickled red cells release free hemoglobin polymers into the blood plasma via the free hemoglobin scavenging mechanism. This consequentially impairs the synthesis and circulation of nitric oxide thus impedes on vascular constriction( Rees, Williams, & Gladwin, 2010). Normal levels of nitric oxide maintain vascular tone by stimulating the vascular endothelium to relax. Accumulation of hemoglobin in the plasma are reported to be avid...
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Sickle Cell Anaemia is an autosomal recessive disorder which is passed through generations; meaning it is a hereditary disease. To inherit Sickle Cell Anaemia an individual must receive a mutated gene from both their paternal and maternal side. Those who only receive one mutated gene are said to be carriers of the disease but are not affected. Sickle Cell Anaemia is most commonly found in Mediterranean countries which have African descent. According to (Hematology.org, 2019) Sickle Cell Anaemia affects 1...
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