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Sickle cell anemia (SCA) is the most prevalent monogenic disease in Brazil, characterized by the presence of hemoglobin S (HbS) in homozygosity. In the deoxygenated state, the HbS polymerizes by modifying the morphology and compromising the function of the red blood cells. Intravascular hemolysis promotes the release of free hemoglobin, the formation of free radicals that degrade nitric oxide (NO), and the rele...
Background. Sickle cell anaemia (SCA) is the most prevalentmonogenic disease in Brazil. In SCA, haemoglobin S (HbS) is formed, whichmodifies red blood cell morphology. Intravascular haemolysis occurs, in which free Hb and free radicals degrade nitric oxide (NO) and release arginase, which reduces arginine levels. Because arginine is a substrate for NO formation, this decrease leads to reduced NO (vasodilator) s...