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Prethrombotic disorders in children with arterial ischemic stroke and sinovenous thrombosis
M Bonduel1, G Sciuccati, M Hepner
1Hematology-Oncology Department, Hospital de Pediatria "Prof. Dr. Juan P. Garrahan," Buenos Aires, Argentina.
Insights
Prethrombotic disorders are common in children with arterial ischemic stroke (AIS) and sinovenous thrombosis (SVT). A full hemostatic evaluation is recommended for all pediatric cases of AIS and SVT.
Area of Science:
- Pediatric Neurology
- Hematology
- Thrombosis Research
Background:
- Arterial ischemic stroke (AIS) and sinovenous thrombosis (SVT) are uncommon pediatric conditions.
- The role of prethrombotic disorders in pediatric AIS and SVT requires further elucidation.
Purpose of the Study:
- To determine the frequency of inherited and acquired prethrombotic disorders in children diagnosed with AIS or SVT.
- To characterize the clinical and radiological features associated with these conditions.
Main Methods:
- A cohort of 30 children with AIS and 10 with SVT underwent comprehensive hemostatic evaluation.
- Tests included assays for protein C, protein S, antithrombin, plasminogen, activated protein C resistance, Factor V Leiden mutation, and antiphospholipid antibodies.
- Demographic data, risk factors, clinical presentation, family history, and radiological findings were collected.
Main Results:
- Prethrombotic disorders were identified in 30% of children with AIS and 50% of children with SVT.
- Commonly found disorders included antiphospholipid antibodies, protein S deficiency, protein C deficiency, and antithrombin deficiency.
- Multiple risk factors and prethrombotic conditions were frequently observed concurrently.
Conclusions:
- A significant proportion of pediatric AIS and SVT cases are associated with prethrombotic disorders.
- Complete hemostatic evaluation is crucial for all children with AIS and SVT, irrespective of clinical risk factors or family history.
- These findings underscore the importance of investigating thrombophilia in pediatric stroke and thrombosis.
Background:
Arterial ischemic stroke (AIS) and sinovenous thrombosis (SVT) are relatively rare events in children. The contribution of prethrombotic disorders to the etiology of these entities has not been completely elucidated.
Objectives:
To determine the frequency of inherited and acquired prethrombotic disorders in a pediatric population with AIS and SVT and to report clinical and radiological features.
Methods:
From May 1992 to April 1997, 30 consecutive children with AIS and 10 children with SVT were assisted at a single institution. Hemostatic evaluation was performed for all the children. Evaluation included the following assays: protein C, protein S, antithrombin, plasminogen, activated protein C resistance, factor V Leiden mutation, and the detection of antiphospholipid antibodies. Data concerning baseline demographics, risk factors, presenting features, family history of thrombosis, and radiological findings were also recorded.
Results:
One or more prethrombotic disorders were present in 9 children (30%) with AIS (inherited protein S deficiency, 2 patients; inherited protein C deficiency, 1 patient; acquired antithrombin deficiency, 2 patients; antiphospholipid antibodies, 3 patients; and antiphospholipid antibodies and plaminogen deficiency, 1 patient) and in 5 children (50%) with SVT (inherited protein S deficiency, 1 patient; acquired antithrombin deficiency, 3 patients; and antiphospholipid antibodies, 1 patient).
Conclusions:
Most children studied presented both a variety of risk factors for thrombosis and concomitant prethrombotic disorders. Therefore, a complete hemostatic evaluation for all children with AIS and SVT should be performed, despite the presence of obvious clinical risk factors or lack of family history of thrombosis.