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Protein C and S deficiency in children with ischemic cerebrovascular accident
1Division of Neurology, Childrens Hospital Los Angeles, University of Southern California, 90027, USA.
Insights
Protein C or S deficiency can cause a prothrombotic state, increasing the risk of childhood ischemic cerebrovascular accident (ICVA). This study found these deficiencies were the sole identified risk factor in several pediatric ICVA cases.
Area of Science:
- Pediatric Neurology
- Hematology
- Vascular Medicine
Background:
- A prothrombotic state, characterized by an imbalance in the coagulation cascade favoring clot formation, is a known risk factor for ischemic cerebrovascular accident (ICVA).
- Deficiencies in natural anticoagulants like protein C and protein S are recognized causes of inherited thrombophilia, particularly in adults.
- The role of these deficiencies in pediatric ICVA remains less defined, necessitating further investigation into their prevalence and impact in younger populations.
Purpose of the Study:
- To investigate the association between protein C or protein S deficiency and the occurrence of ischemic cerebrovascular accident (ICVA) in children.
- To determine the frequency of protein C and protein S deficiencies as the sole identified risk factor in a cohort of pediatric ICVA patients.
Main Methods:
- A retrospective review of medical records was conducted for children diagnosed with ICVA between 1989 and 1995.
- Patients with ICVA unrelated to any known preexisting diagnosis were identified using neuroimaging.
- The prevalence of protein C and protein S deficiencies was assessed in this cohort.
Main Results:
- Out of 37 children diagnosed with ICVA, 2 patients (5.4%) had protein C deficiency.
- Additionally, 5 patients (13.5%) were found to have protein S deficiency.
- These deficiencies were the only identified risk factors for ICVA in these pediatric cases.
Conclusions:
- Protein C deficiency and protein S deficiency are significant risk factors for ischemic cerebrovascular accident (ICVA) in the pediatric population.
- The findings highlight the importance of screening for these thrombophilias in children presenting with unexplained ICVA.
- Early identification and management of protein C or S deficiency can potentially mitigate the risk of recurrent cerebrovascular events in children.
Abstract:
A prothrombotic state caused by a deficiency of protein C or protein S, each an inhibitor of coagulation cascade, increases the risk of ischemic cerebrovascular accident (ICVA) in young adults. This retrospective review studies the relationship of protein C or S deficiency with childhood ICVA. From 1989 to 1995, 37 children were diagnosed by neuroimaging with ICVA unrelated to any known preexisting diagnosis. Protein C deficiency or protein S deficiency was the only identified risk factor for 5.4% (2 patients) and 13.5% (5 patients), respectively, of these 37 children. The data suggest that protein C or protein S deficiency is an important risk factor of ICVA in children.