Sickle cell disease (CSD) is an inherited blood disorder, characterized primarily by chronic anemia and periodic episodes of pain. The underlying problem involves hemoglobin, a component of the red cells in the blood. The hemoglobin molecules in each red blood cell carry oxygen from the lungs to the body organs and tissues and bring back carbon dioxide to the lungs.
By occluding blood vessels sickle cells cause vascular injury. This vaso-occlusion, which is responsible for most of the severe complications of sickle cell disease, can occur wherever blood flows. This process produces the periodic episodes of pain and ultimately can damage the tissues and vital organs and lead to other serious medical problems.
One of every 600 African Americans in the United States has sickle cell anemia. Fortunately, because of significant advances in treatment and preventive care, persons with sickle cell disease now survive into their fifth and sixth decades.
Health maintenance is extremely important in SCD and must include measures to prevent specific disease complications or at least to facilitate their diagnosis and minimize their impact.
Management of a crisis involves control of pain, maintenance of adequate hydration, and treatment of any underlying precipitating factors.
This MediFocus Guide contains an extensive listing of citations and abstracts of recent journal articles that have been published about this condition in trustworthy medical journals. This is the same type of information that is available to physicians and other health care professionals. A partial selection of journal articles that are abstracted in this MediFocus Guide includes:
Sickle cell disease in childhood: Part I. Laboratory diagnosis, pathophysiology and health maintenance. American Family Physician. 2000
Reactive species in sickle cell disease. Annals of the New York Academy of Sciences. 2000
Blockade of adhesion of sickle cells to endothelium by monoclonal antibodies. New England Journal of Medicine. 2000
Screening for sickle cell disease and thalassaemia: a systematic review with supplementary research. Health Technology Assessment (South Hampton, NY). 2000
The management of pain in sickle cell disease. Pediatric Clinics of North America. 2000
Lessons from the Stroke Prevention Trial in Sickle Cell Anemia (STOP) study. Journal of Child Neurology. 2000
Laparoscopic splenectomy for splenic sequestration crisis. AORN Journal. 2000
Neonatal screening for sickle cell disease. Cochrane Database of Systematic Reviews [computer file]. 2000
Prophylactic versus selective blood transfusion for sickle cell anaemia during pregnancy. Cochrane Database of Systematic Reviews [computer file]. 2000
Sickle hemoglobin (HbS) allele and sickle cell disease: a HuGE review. American Journal of Epidemiology. 2000
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