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Sickle Cell Anemia and Stroke in Children

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Sickle cell anemia is an inherited, chronic disease in which the red blood cells, normally disc-shaped, become crescent shaped. As a result, they function abnormally and break down, causing recurrent painful episodes.

Sickle cell anemia is caused by an abnormal type of hemoglobin (oxygen carrying pigment) called hemoglobin S. It is inherited as an autosomal recessive trait; that is, it occurs in someone who has inherited hemoglobin S from both parents.

From the medical college of Georgia (2003): 

"Children with SCD are 200 to 400 times as likely to suffer a stroke, compared to children without SCD or congenital heart disease.  The Cooperative Study of Sickle Cell Disease (CSSCD), a large prospective study, confirmed that stroke is a constant threat after age 2, but the incidence is highest in the middle of a child's first 10 years of life. About 11% of all children with homozygous SCD will develop stroke by 20 years of age."

Of the 2000 children with Sickle Cell Disease born in the United States each year, about 10 percent will develop stroke by adulthood.

Sickle Cell Disease Association of America

Comprehensive Sickle Cell Centers

Journal Articles

Stroke Research in Sickle Cell Disease

Stroke in children and sickle-cell disease Baltimore-Washington Cooperative Young Stroke Study

Genetic dissection and prognostic modeling of overt stroke in sickle cell anemia.  April 2005, Boston.

Sickle cell disease: primary stroke prevention. June 2004. Georgia.

Is Chlamydia pneumoniae infection associated with stroke in children with sickle cell disease? April 2004. New York.

Brain imaging findings in pediatric patients with sickle cell disease. July 2003. Tennessee.

Stroke risk in siblings with sickle cell anemia, March 2003, multicenter study.

Moyamoya syndrome in childhood sickle cell disease: a predictive factor for recurrent cerebrovascular events May 2002

Sickle cell disease: The neurological complications May 2002

Silent infarction as a risk factor for overt stroke in children with sickle cell anemia: a report from the Cooperative Study of Sickle Cell Disease  2001

Nocturnal hypoxaemia and central-nervous-system events in sickle-cell disease May 2001

Sickle Cell Disease in Childhood: Part I. Laboratory Diagnosis, Pathophysiology and Health Maintenance Doris Wethers, M.D., St. Luke's-Roosevelt Hospital Center, NY, NY

Sickle Cell Disease in Childhood: Part II. Diagnosis and Treatment of Major Complications and Recent Advances in Treatment Doris Wethers, M.D., St. Luke's-Roosevelt Hospital Center, NY, NY

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