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Neurological complications in children with protein C deficiency
M A van Kuijck1, J J Rotteveel, C G van Oostrom
1Department of Pediatrics, University Hospital Nijmegen, The Netherlands.
Insights
Protein C deficiency, a rare condition, is linked to arterial clots in children, not just venous ones. This finding is crucial for diagnosing pediatric cerebrovascular accidents.
Area of Science:
- Neurology
- Pediatrics
- Hematology
Background:
- Protein C deficiency is a known risk factor for venous thrombosis.
- Cerebrovascular accidents (CVAs) in children are uncommon but serious events.
Observation:
- Four pediatric patients presented with cerebrovascular occlusive accidents.
- Two experienced acute hemiplegia; others had transient ischemic attacks and hydrocephalus due to sinus thrombosis.
- All patients were diagnosed with Type 1 protein C deficiency.
Findings:
- This study highlights a connection between protein C deficiency and arterial thrombosis in children.
- Cerebrovascular accidents in these patients were attributed to protein C deficiency after excluding other causes.
Implications:
- Pediatric cerebrovascular accidents warrant consideration of protein C deficiency.
- Early diagnosis and management of protein C deficiency can prevent severe neurological outcomes.
Abstract:
Four children with a cerebrovascular occlusive accident and protein C deficiency are described. Two patients presented with an acute hemiplegia, the others suffered from a transient ischemic attack and a progressive hydrocephalus as the result of sinus thrombosis. In all cases protein C deficiency, Type 1, was diagnosed. Other causes of cerebrovascular disease were excluded. Although venous thrombosis has been extensively reported in protein C deficiency, these cases indicate that protein C deficiency is also related to arterial thrombosis. In evaluating children with cerebrovascular accidents, protein C deficiency should also be considered.
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