title: Neurocognitive sequelae of sickle cell disease: current issues and future directions.
abstract: Sickle cell disease is a genetic disorder with an autosomal recessive pattern of inheritance. This disease is most prevalent among those of African American and Mediterranean descent. Cerebral vascular accident (CVA) or stroke is one of the major complications of hemoglobin SS (Hgb SS) disease. CVA has implications for physical as well as neurocognitive function for children. Recent literature suggests that some children with sickle cell disease without overt signs of CVA may still have evidence of neurological deficit, both on magnetic resonance imaging and neurological examination. There is a growing body of knowledge that further aids in delineation of risk factors for CVA, silent infarct, and neurocognitive deficits in children with Hgb SS disease. More research is needed to continue to explore avenues for identification and intervention. The purpose of this article is to delineate areas of ongoing research in this important area.
authors.affiliation: Children's Mercy Hospital, 2401 Gillham Road, Kansas City, MO 64108, USA. jrouthieaux@cmh.edu
authors.forename: Julie
authors.initials: J
authors.lastname: Routhieaux
authors.forename: Susan
authors.initials: S
authors.lastname: Sarcone
authors.forename: Kristin
authors.initials: K
authors.lastname: Stegenga
mesh_terms: D000755:Anemia, Sickle Cell / Q000150:complications / Q000503:physiopathology / Q000523:psychology*; D002648:Child; D003071:Cognition; D003072:Cognition Disorders / Q000453:epidemiology*; D005260:Female; D006801:Humans; D008297:Male; D009203:Myocardial Infarction / Q000453:epidemiology; D009422:Nervous System Diseases / Q000453:epidemiology*; D020521:Stroke / Q000453:epidemiology*