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Noé D. Romo, MD, FAAP, "Sickle Cell Disease", Caring for the Hospitalized Child: A Handbook of Inpatient Pediatrics, American Academy of Pediatrics Section on Hospital Medicine, Jeffrey C. Gershel, MD, FAAP, Daniel A. Rauch, MD, FAAP, SFHM
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Sickle cell disease (SCD) is an inherited hematologic disorder characterized by having genes for 2 abnormal hemoglobins that result in the sickling of red cells. The clinical features of the disease result from chronic hemolysis and end-organ insult secondary to vasoocclusion and vascular injury. Homozygous hemoglobin SS is the most common and severe form of SCD, but similar manifestations may occur when heterozygous hemoglobin S is combined with an alternative hemoglobin abnormality, such as in the conditions of hemoglobin SC (also known as sickle hemoglobin C disease) and hemoglobin S β-thalassemia (which can be as severe as homozygous SS disease)....