Abstract | OBJECTIVES: The purpose of this study was to obtain data on the association of antiphospholipid antibodies with clinical manifestations in childhood and to enable future studies to determine the impact of treatment and long-term outcome of pediatric antiphospholipid syndrome. PATIENTS AND METHODS: RESULTS: As of December 1, 2007, there were 121 confirmed antiphospholipid syndrome cases registered from 14 countries. Fifty-six patients were male, and 65 were female, with a mean age at the onset of antiphospholipid syndrome of 10.7 years. Sixty (49.5%) patients had underlying autoimmune disease. Venous thrombosis occurred in 72 (60%), arterial thrombosis in 39 (32%), small-vessel thrombosis in 7 (6%), and mixed arterial and venous thrombosis in 3 (2%). Associated nonthrombotic clinical manifestations included hematologic manifestations (38%), skin disorders (18%), and nonthrombotic neurologic manifestations (16%). Laboratory investigations revealed positive anticardiolipin antibodies in 81% of the patients, anti-beta(2)-glycoprotein I antibodies in 67%, and lupus anticoagulant in 72%. Comparisons between different subgroups revealed that patients with primary antiphospholipid syndrome were younger and had a higher frequency of arterial thrombotic events, whereas patients with antiphospholipid syndrome associated with underlying autoimmune disease were older and had a higher frequency of venous thrombotic events associated with hematologic and skin manifestations. CONCLUSIONS:
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Authors | Tadej Avcin, Rolando Cimaz, Earl D Silverman, Ricard Cervera, Marco Gattorno, Stella Garay, Yackov Berkun, Flavio R Sztajnbok, Clovis A Silva, Lucia M Campos, Claudia Saad-Magalhaes, Donato Rigante, Angelo Ravelli, Alberto Martini, Blaz Rozman, Pier Luigi Meroni |
Journal | Pediatrics
(Pediatrics)
Vol. 122
Issue 5
Pg. e1100-7
(Nov 2008)
ISSN: 1098-4275 [Electronic] United States |
PMID | 18955411
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Topics |
- Adolescent
- Age Factors
- Antiphospholipid Syndrome
(diagnosis, epidemiology, genetics, immunology)
- Autoimmune Diseases
(epidemiology)
- Cerebrovascular Disorders
(epidemiology)
- Child
- Child, Preschool
- Comorbidity
- Female
- Humans
- Infant
- Ischemia
(epidemiology)
- Male
- Registries
- Thrombosis
(epidemiology)
- Venous Thrombosis
(epidemiology, genetics)
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