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A 38-year-old African-American woman was admitted for arthroscopic knee surgery. A 38-year-old African-American woman was admitted for arthroscopic knee surgery.Pathophysiology of sickle cell disease essayA 38-year-old African-American woman was admitted for arthroscopic knee surgery. Her hematocrit was 25%, blood pressure was 140/94 mm Hg, and pulse was 112 beats/minute. She had a history of joint and bone pain, jaundice, and abdominal pain.Due to the presenting symptoms (joint and bone pain, abdominal pain, and jaundice) a diagnosis of Sickle Cell Disease was considered.1. Provide a brief discussion of the pathophysiology of sickle cell disease and discuss the clinical manifestations and the etiology associated with each manifestation of this disease. References must be scholarly articles not less than 5 years.( be between 2013-2018)Pathophysiology of sickle cell diseaseThe sickle cell disease is a hereditary condition that results from β-globin mutation leading to deformed hemoglobin. As a result of the mutation, the sixth amino acid changes to valine from glutamic acid. The resultant deformed sickle-shaped hemoglobin(referred to as HbS) polymerizes on deoxygenation or in events of low oxygen supply since it has unusual physiochemical properties (Vichinsky, 2014). The resultant effects are felt by the erythrocytes in the short and the long run.ORDER A PLAGIARISM-FREE PAPER HEREAs a consequence, the viscosity of the red cells’ cytosol increases, limiting their ability to flow normally and freely within the capillaries. The continued polymerization of the HbS leads to the deformation of the red blood cells which results in the sickle shape of the cells are characteristic of the condition.Manifestations and the etiology associated with each manifestation of this diseaseThe SCD shows its early signs in early life within the first six months. Since infants have higher levels of Hb F, they are protected from the condition.Vaso-occlusive crisis is the main way in which the condition clinically manifests itself. The vaso-occlusive crisis results when the sickle-shaped erythrocytes obstruct the normal micro-circulation of blood leading to ischemic injury to those organs resulting in pain (Nouraieet al. 2013). The most distinctive clinical manifestation is pain crises and in many cases, they are the causes of medical emergencies and hospitalization of the victims. The pain are abrupt and last from a few hours to several days and stop as suddenly as they started. The pains are not localized and affect virtually any organ in the body including the bones, abdomen, soft tissues and joints. It may present itself as swollen painful feet and hands in children. It could also manifest itself as acute avascular necrosis and/or joint necrosis. Life-threatening Infarctions become apparent due to the repetitive episodes which may happen in the spleen. Over time, liver failure may also be apparent due to due to the infarction. In the renal aspects, vaso-occlusion is manifested by the inability to concentrate urine (isothenuria) (Serjeant, 2013). A 38-year-old African-American woman was admitted for arthroscopic knee surgery.The pain in the bones is usually as a result of marrow infarction. Because the changes in bone marrow are age-related, it is easy to predict marrow infraction since the bones involved are those with high bone marrow activities. Both metacarpals and metatarsals could be involved in the first 18 months manifesting as the hand-foot syndrome or dactylitis.Although the areas discussed here are the most common, the condition can affect any part of the body where even the sensory nerves could be victims. ReferencesNouraie, M., Lee, J. S., Zhang, Y., Kanias, T., Zhao, X., Xiong, Z., …& Hildesheim, M. E. (2013). The relationship between the severity of hemolysis, clinical manifestations and risk of death in 415 patients with sickle cell anemia in the US and Europe. Haematologica, 98(3), 464-472.Serjeant, G. R. (2013).The natural history of sickle cell disease. Cold Spring Harbor perspectives in medicine, 3(10), a011783Vichinsky, E. (2014). Overview of the clinical manifestations of sickle cell disease. UptoDate online. Available from URL: http://www. uptodate. com/contents/overview-of-the-clinical-manifestations-of-sickle-cell-disease. A 38-year-old African-American woman was admitted for arthroscopic knee surgery.
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