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Pediatric Antiphospholipid Syndrome: from Pathogenesis to Clinical Management.


ABSTRACT:

Purpose of review

Elucidating the pathogenic mechanisms mediated by antiphospholipid antibodies (aPL) might exert important clinical implications in pediatric antiphospholipid syndrome (APS).

Recent findings

aPL are traditionally regarded as the main pathogenic players in APS, inducing thrombosis via the interaction with fluid-phase and cellular components of coagulation. Recent APS research has focused on the role of ?2 glycoprotein I, which bridges innate immunity and coagulation. In pediatric populations, aPL should be screened in appropriate clinical settings, such as thrombosis, multiple-organ dysfunction, or concomitant systemic autoimmune diseases. Children positive for aPL tests often present non-thrombotic non-criteria manifestations or asymptomatic aPL positivity. In utero aPL exposure has been suggested to result in developmental disabilities, warranting long-term follow-up. The knowledge of the multifaceted nature of pediatric APS should be implemented to reduce the risk of underdiagnosing/undertreating this condition. Hopefully, recent pathogenic insights will open new windows of opportunity in the management of pediatric APS.

SUBMITTER: Rosina S 

PROVIDER: S-EPMC7843475 | biostudies-literature | 2021 Jan

REPOSITORIES: biostudies-literature

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Publications

Pediatric Antiphospholipid Syndrome: from Pathogenesis to Clinical Management.

Rosina Silvia S   Chighizola Cecilia Beatrice CB   Ravelli Angelo A   Cimaz Rolando R  

Current rheumatology reports 20210128 2


<h4>Purpose of review</h4>Elucidating the pathogenic mechanisms mediated by antiphospholipid antibodies (aPL) might exert important clinical implications in pediatric antiphospholipid syndrome (APS).<h4>Recent findings</h4>aPL are traditionally regarded as the main pathogenic players in APS, inducing thrombosis via the interaction with fluid-phase and cellular components of coagulation. Recent APS research has focused on the role of β2 glycoprotein I, which bridges innate immunity and coagulatio  ...[more]

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