title: Cerebral vasculopathy in children with sickle cell anemia.
abstract: Sickle cell anemia (SCA)-associated cerebral vasculopathy and moyamoya is a unique entity reflecting the abnormal interactions between sickled red blood cells (RBCs) and the cerebral arterial endothelium. Endothelial injury, coagulation activation, and the inflammatory response generated by sickled RBCs are implicated in the development of cerebral vasculopathy, but the pathophysiology remains incompletely understood. SCA-specific screening and treatment guidelines have successfully reduced the incidence of overt strokes in this high-risk population. However, despite aggressive hematological management, many children with cerebral vasculopathy due to SCA have progressive vasculopathy and recurrent strokes; therefore, more effective therapies, such as revascularization surgery and curative hematopoietic stem cell transplant, are urgently needed.
authors.affiliation: Center for Cancer and Blood Disorders, Children's National Health System, Washington, DC, USA; Department of Pediatrics, The George Washington University School of Medicine and Health Sciences, Washington, DC, USA.
authors.forename: Ross M
authors.initials: RM
authors.lastname: Fasano
authors.affiliation: Center for Cancer and Blood Disorders, Children's National Health System, Washington, DC, USA; Department of Pediatrics, The George Washington University School of Medicine and Health Sciences, Washington, DC, USA.
authors.forename: Emily R
authors.initials: ER
authors.lastname: Meier
authors.affiliation: Department of Pediatrics, Washington University School of Medicine, St. Louis, MO, USA. Electronic address: hulbert_m@kids.wustl.edu.
authors.forename: Monica L
authors.initials: ML
authors.lastname: Hulbert
mesh_terms: D000755:Anemia, Sickle Cell / Q000150:complications / Q000453:epidemiology / Q000378:metabolism / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D004913:Erythrocytes, Abnormal / Q000378:metabolism*; D005260:Female; D006801:Humans; D015994:Incidence; D007249:Inflammation / Q000453:epidemiology / Q000209:etiology / Q000378:metabolism / Q000628:therapy; D008297:Male; D020521:Stroke / Q000453:epidemiology / Q000209:etiology / Q000378:metabolism / Q000628:therapy; D020293:Vasculitis, Central Nervous System / Q000453:epidemiology / Q000209:etiology / Q000378:metabolism / Q000628:therapy
keywords: Cerebral vasculopathy; Moyamoya; Sickle cell anemia; Stroke