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FIGURE 1 Pathophysiology of Sickle Cell Disease Sickle cell disease is caused by an abnormal HbS ( 2 S 2 ) in which glutamic acid at position 6 of the -globin chain of hemoglobin is changed to valine

15 Collectively, these events lead to a vicious cycle of persistent inflammation and chronic oxidative stress, which results in protease-anti-protease imbalance, defects in tissue repair mechanisms, accelerated apoptosis in lung cells and progression of COPD

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