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Sickle cell disease affects approximately one in 400 to 500 blacks, and the carrier state, sickle cell trait, occurs in about 8% to 10% of the black population in the western hemisphere, and in up to 25% to 30% of black people of western Africa. Our knowledge of the molecular pathology, genetics, clinical manifestations, and natural history of sickle cell disease has expanded dramatically since J. B. Herrick's original observation in 1910 of "peculiar elongated and sickle shaped red blood corpuscles in a case of severe anemia." Until recently, a fashionable viewpoint suggested that sickle cell anemia has been a neglected disease and that we know little about its clinical manifestations or natural history. Yet, the classical breakthrough achieved by Linus Pauling and Vernon Ingram in unraveling the molecular structure of sickle cell hemoglobin led to a massive body of information on the structure and function of normal and other
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