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Antiphospholipid syndrome in pediatrics
Angelo Ravelli1, Alberto Martini
1Pediatria II, Istituto di Ricovero e Cura a Carattere Scientifico G. Gaslini, Largo G. Gaslini, 5, 16147, Genoa, Italy. angeloravelli@ospedale-gaslini.ge.it
Insights
Antiphospholipid syndrome (APS) in children shares many features with adults but differs in diagnostic and therapeutic approaches. A new registry aims to clarify the unique aspects of pediatric APS and antiphospholipid antibodies (aPL).
Area of Science:
- Pediatric Rheumatology
- Immunology
- Vascular Thrombosis
Background:
- Antiphospholipid syndrome (APS) is a significant cause of vascular thrombosis in children.
- Pediatric APS shares clinical features with adult APS, but lacks common adult prothrombotic factors.
- Children may have a higher prevalence of transient antiphospholipid antibodies (aPL) due to infections.
Purpose of the Study:
- To investigate the relationship between aPL and clinical manifestations in pediatric APS.
- To identify specific aPL associated with thrombosis in children.
- To understand the unique diagnostic and therapeutic challenges in childhood APS.
Main Methods:
- Establishment of an internet-based registry for pediatric patients with APS (Ped-APS Register).
- Data collection on aPL associations, clinical manifestations, treatment impact, and long-term outcomes.
- Multicenter, controlled studies are proposed for defining natural history and optimal management.
Main Results:
- Pediatric APS patients offer a unique cohort to study aPL-thrombosis relationships due to fewer confounding factors.
- The registry aims to gather crucial data on the specificities of aPL linked to pediatric thrombosis.
- Understanding transient aPL prevalence in children is key for accurate diagnosis.
Conclusions:
- The diagnostic and therapeutic approach for pediatric APS may differ from adults.
- Large, multicenter studies are essential due to the rarity of aPL-related thrombosis in children.
- The Ped-APS Register is a vital initiative for advancing knowledge and improving outcomes in childhood APS.
Abstract:
APS is recognized increasingly as a leading cause of vascular thrombosis in the pediatric population. With the obvious exception of pregnancy morbidity, most of the clinical features that may occur in adults with APS have been described also in children. Because the coincident prothrombotic factors that are common in adults have little or no impact in children, pediatric patients with APS constitute a suitable sample to investigate the relationship of aPL with the associated clinical manifestations, such as thrombocytopenia, hemolytic anemia, chorea, and livedo reticularis, and the specificities of aPL that are more linked to thrombosis. On the other hand, because of the high frequency of infectious processes in early life, children may have a greater prevalence of nonpathogenic and transient aPL. For these reasons, the diagnostic and therapeutic approach to APS in childhood may be different from that for adults. Because of the rarity of aPL-related thrombosis in children, the natural history and optimal management can be defined only through large, multicenter, controlled studies. A internet-based registry for pediatric patients with APS (Ped-APS Register) has been recently established as part of the activities of the Euro-aPL Forum and the Lupus Working Group of the Pediatric Rheumatology European Society. This registry is aimed to obtain information on APS in childhood, particularly regarding association of aPL with clinical manifestations, specificity of aPL, impact of treatment and long-term outcome (http://www.med.ub.es/MIMMUN/FORUM/PEDIATRIC.HTM).
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