Abstract
Antiphospholipid antibodies (aPL) play the central pathogenic role in antiphospholipid syndrome (APS) characterized by arterial and venous thrombosis, recurrent fetal loss and persistent circulating aPL. The diagnostic criteria of definite APS are not entirely applicable in pediatric population. An international multicentric project named Ped-APS registry included 121 patients with APS onset before 18th birthday. Almost half of them (49.5%) had an associated autoimmune disease. In this study a large percentage of children with aPL-related thrombotic event had at the time of the initial thrombotic event associated nonthrombotic clinical manifestations including: hematological manifestations (38%), dermatological manifestations (18%) and nonthrombotic neurological manifestations (16%).
Highlights
Antiphospholipid antibodies play the central pathogenic role in antiphospholipid syndrome (APS) characterized by arterial and venous thrombosis, recurrent fetal loss and persistent circulating aPL
In this study a large percentage of children with aPL-related thrombotic event had at the time of the initial thrombotic event associated nonthrombotic clinical manifestations including: hematological manifestations (38%), dermatological manifestations (18%) and nonthrombotic neurological manifestations (16%)
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Summary
Antiphospholipid antibodies (aPL) play the central pathogenic role in antiphospholipid syndrome (APS) characterized by arterial and venous thrombosis, recurrent fetal loss and persistent circulating aPL. The diagnostic criteria of definite APS are not entirely applicable in pediatric population. An international multicentric project named Ped-APS registry included 121 patients with APS onset before 18th birthday. Almost half of them (49.5%) had an associated autoimmune disease. In this study a large percentage of children with aPL-related thrombotic event had at the time of the initial thrombotic event associated nonthrombotic clinical manifestations including: hematological manifestations (38%), dermatological manifestations (18%) and nonthrombotic neurological manifestations (16%)
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