Sickle cell disease (SCD) describes a family of molecular disorders in which patients inherit the hemoglobin S (HbS) mutation in one beta-globin gene and a second beta-globin mutation in the other beta-globin gene that impairs normal hemoglobin production and causes clinical sickling of red blood cells. People with SCD have a dramatically increased risk of pain, lung disease, renal failure, bone necrosis, sepsis, stroke, and early death. Preventative care and early recognition of sickle cell-related complications are critical to improve the quality of life for patients living with this disease.

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Sickle Cell Disease: Inpatient Management Issues

  • Beth Apsel Winger,
  • Elliott Vichinsky

摘要

Sickle cell disease (SCD) describes a family of molecular disorders in which patients inherit the hemoglobin S (HbS) mutation in one beta-globin gene and a second beta-globin mutation in the other beta-globin gene that impairs normal hemoglobin production and causes clinical sickling of red blood cells. People with SCD have a dramatically increased risk of pain, lung disease, renal failure, bone necrosis, sepsis, stroke, and early death. Preventative care and early recognition of sickle cell-related complications are critical to improve the quality of life for patients living with this disease.