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Created with Fabric.js 1.4.5 Sociedades Jurídicas STATISTICS 2.5 million Americans have the genetic trait for the disease, but only 70,000 people in the United States have sickle cell disease. Víctor Rangel Galera HISTORY In 1904, Chicago cardiologist and professor of medicine James B. Herrick, with the help of his intern Ernest Edward Irons found "peculiarelongated and sickle-shaped" cells in the blood of Walter Clement Noel. The disease was named "sickle-cellanaemia" by Vernon Mason in 1922. Linus Pauling and colleagues were the first, in 1949, to demonstrate that sickle-cell diseaseoccurs as a result of an abnormality in the hemoglobin molecule. Cause & Effect Complications include anemia, jaundice, and gallstones. Blood flow problems can cause lung damage, pain in the arms, legs, chest, and abdomen, and stroke. Organs can bedamaged by poor blood flow as well, and include the spleen, kidneys, and liver leavingyoung children highly vulnerable to bacterial infections. The discovery of Sickle Cell was the first time agenetic disease was linked to a mutation of aspecific protein, a milestone in the history ofmolecular biology. Sickle cell disease is caused by a genetic abnormality in the gene for hemoglobin, which results in the production of sickle hemoglobin. When oxygen is released fromsickle hemoglobin, it sticks together and forms long rods, which damage and changethe shape of the red blood cell. More than 200 patients with sickle cell diseasehave undergone stem cell transplants from amatched sibling donor. Stem cell transplanthas a 5%-10% risk of death, but patients withsuccessful transplants were completely curedof sickle cell disease, with no further episodesof pain. In 1973, the average life span was 14, but people with sickle cell disease nowlive into their 40s and 50s, and in somecases, beyond the age of 60.
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