Pediatric antiphospholipid syndrome (APS) is characterized by autoantibodies directed against protein complexes on cellular membranes and leads to a prothrombotic, proinflammatory state. A child with APS may present with venous, arterial, or small vessel thrombosis. Other manifestations of APS include nonthrombotic manifestations, such as hematologic and neurologic symptoms. APS may be a primary condition or related to other autoimmune diseases. If APS-related thrombosis is unrecognized, the child may suffer recurrent thrombotic events after the withdrawal of anticoagulation. Thus, it is important to consider APS as a cause of thrombosis in children. Appropriate testing confirms the diagnosis and directs further care.
Key points
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Antiphospholipid syndrome is rare in childhood but can be life threatening and needs to be identified quickly.
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Children with thrombosis should be screened for antiphospholipid antibodies. If positive, persistence should be confirmed.
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Treatment of children with antiphospholipid syndrome is complex and often requires the use of anticoagulants and immunosuppression.
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The use of estrogen contraceptives is contraindicated in the antiphospholipid syndrome and can lead to increased risk of thrombosis, limiting the choices for contraceptive use.
Introduction
The antiphospholipid syndrome (APS) is a systemic immune-mediated disease process characterized by thrombotic or obstetric complications in the presence of pathogenic antiphospholipid antibodies (aPL) [
, ]. These autoantibodies target anionic phospholipids and protein-phospholipid complexes and result in a proinflammatory and prothrombotic state [
]. Pediatric APS is defined as APS that occurs in someone less than 18 years of age.
APS is the most common cause of acquired thrombophilia [
]. Unfortunately, it can often lead to significant morbidity and mortality depending on the type and extent of thrombotic event. In addition, patients with APS often require long-term anticoagulation, which can be challenging to manage in an active child.
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