Sickle Cell Disease

General Principles

  • SCD is a group of hereditary Hgb disorders in which Hgb undergoes a sickle shape transformation under conditions of deoxygenation.
  • The most common are homozygous sickle cell anemia (Hgb SS) or other double-heterozygous conditions (Hgb SC, Hgb S–β0, or Hgb S–β+ thalassemia).
  • Newborn screening programs for hemoglobinopathies are available throughout the US and identify most patients in infancy.
  • In the US, the incidence of SCD is approximately 1 in 625 births.
  • Sickle cell trait is present in 7%–8% of African Americans. It is generally considered to be a benign carrier state, but high-altitude hypoxia is associated with splenic infarction, whereas intense physical exertion has been associated with sudden death and rhabdomyolysis.
  • Sickle cell trait has been associated with an increased risk of pulmonary embolism, proteinuria, and CKD. Minimizing other risk factors for kidney disease is likely to benefit patients at risk.

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