The annual incidence of cerebrovascular disease in children is 2.5/100,000 and cerebral infarction is being increasingly recognised in neonates.1 Deficiency of proteins C and S and their roles in thrombosis have only recently been recognised.2 Immunologic and functional assays of these proteins now make it possible to determine whether deficiency of them is associated in any particular case of childhood cerebrovascular accident (CVA).3
We describe two patients, both presenting with stroke in childhood, who were found to be deficient, one in protein C and one in protein S.
Get full access to this article
View all access options for this article.
References
1.
SchoenbergBSMellingerJFSchoenbergDG. Cerebrovascular disease in infants and children: a study of incidence, clinical features and survival. Neurol1978; 28: 763.
2.
GriffinJHEvattBZimmermanTSKleissAJWidemanC.Deficiency of protein C in congenital thrombotic disease. J Clin Invest1981; 68: 1370–1373.
3.
UysalSAnlarBAltayCKirazliS.Role of protein C in childhood cerebrovascular accidents. Eur J Pediatr1989; 149: 216–218.
4.
ClauseHLCompPC. The regulation of haemostasis: the protein C system. New Engl J Med1986; 314: 1298–1304.
5.
IsraelsSJSeshiaSS. Childhood stroke associated with protein C or S deficiency. J Pediatr1987; 111: 562–564.
6.
MarsiniakEWilsonHDMarlarRA. Neonatal purpura fulminans: a genetic disorder related to the absence of protein C in blood. Blood1985; 65: 15–20.
7.
GladsonCLGriffinJHHachVBeckKHScharrerI.The incidence of protein C and protein S deficiency in young thrombotic patients. Blood1985; 66 (suppl 1): 350a.
