Protein C and protein S activity in sickle cell disease and stroke.

Stroke is a significant complication of sickle cell anemia in the pediatric population. The pathophysiology of stroke in sickle cell anemia remains unclear. Protein C and protein S activities were measured in children with sickle cell anemia and stroke, and compared to those with sickle cell anemia who were neurologically normal. Results showed significantly decreased levels of both protein C and protein S activities in children with sickle cell anemia who have had a stroke. This pilot study suggests that a possible coagulopathic state in children with sickle cell anemia may be associated with an increased risk for cerebrovascular disease. Further research in this area is indicated.