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dc.contributor.authorAvcin, Tadej
dc.contributor.authorCimaz, Rolando
dc.contributor.authorSilverman, Earl D.
dc.contributor.authorCervera, Ricard
dc.contributor.authorGattorno, Marco
dc.contributor.authorGaray, Stella
dc.contributor.authorBerkun, Yackov
dc.contributor.authorSztajnbok, Flavio R.
dc.contributor.authorSilva, Clovis A.
dc.contributor.authorCampos, Lucia M.
dc.contributor.authorSaad-Magalhaes, Claudia [UNESP]
dc.contributor.authorRigante, Donato
dc.contributor.authorRavelli, Angelo
dc.contributor.authorMartini, Alberto
dc.contributor.authorRozman, Blaz
dc.contributor.authorMeroni, Pier Luigi
dc.identifier.citationPediatrics. Elk Grove Village: Amer Acad Pediatrics, v. 122, n. 5, p. E1100-E1107, 2008.
dc.description.abstractOBJECTIVES. 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. A European registry extended internationally of pediatric patients with antiphospholipid syndrome was established as a collaborative project of the European Antiphospholipid Antibodies Forum and Lupus Working Group of the Pediatric Rheumatology European Society. To be eligible for enrollment the patient must meet the preliminary criteria for the classification of pediatric antiphospholipid syndrome and the onset of antiphospholipid syndrome must have occurred before the patient's 18th birthday.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. Clinical and laboratory characterization of patients with pediatric antiphospholipid syndrome implies some important differences between antiphospholipid syndrome in pediatric and adult populations. Comparisons between children with primary antiphospholipid syndrome and antiphospholipid syndrome associated with autoimmune disease have revealed certain differences that suggest 2 distinct subgroups. Pediatrics 2008; 122: e1100-e1107en
dc.description.sponsorshipSlovenian Ministry of Higher Education, Science, and Technology
dc.publisherAmer Acad Pediatrics
dc.sourceWeb of Science
dc.subjectantiphospholipid syndromeen
dc.subjectantiphospholipid antibodiesen
dc.titlePediatric Antiphospholipid Syndrome: Clinical and Immunologic Features of 121 Patients in an International Registryen
dcterms.rightsHolderAmer Acad Pediatrics
dc.contributor.institutionUniv Childrens Hosp
dc.contributor.institutionUniv Med Ctr
dc.contributor.institutionUniv Florence
dc.contributor.institutionUniv Toronto
dc.contributor.institutionUniv Barcelona
dc.contributor.institutionIst Giannina Gaslini
dc.contributor.institutionUniv Genoa
dc.contributor.institutionHosp Sor Maria Ludovica
dc.contributor.institutionSafra Childrens Hosp
dc.contributor.institutionTel Aviv Univ
dc.contributor.institutionUniversidade do Estado do Rio de Janeiro (UERJ)
dc.contributor.institutionUniversidade de São Paulo (USP)
dc.contributor.institutionUniversidade Estadual Paulista (UNESP)
dc.contributor.institutionUniv Cattolica Sacro Cuore
dc.contributor.institutionUniv Milan
dc.contributor.institutionSan Luca Hosp
dc.description.affiliationUniv Childrens Hosp, Univ Med Ctr Ljubljana, Dept Allergol Rheumatol & Clin Immunol, SL-1525 Ljubljana, Slovenia
dc.description.affiliationUniv Med Ctr, Dept Rheumatol, Ljubljana, Slovenia
dc.description.affiliationUniv Florence, Dept Pediat, Meyer Hosp, Florence, Italy
dc.description.affiliationUniv Toronto, SickKids Hosp, Div Rheumatol, Toronto, on M5G 1X8, Canada
dc.description.affiliationUniv Barcelona, Hosp Clin, Dept Autoimmune Dis, Catalonia, Spain
dc.description.affiliationIst Giannina Gaslini, I-16148 Genoa, Italy
dc.description.affiliationUniv Genoa, Dept Pediat, Genoa, Italy
dc.description.affiliationHosp Sor Maria Ludovica, Rheumatol Unit, Buenos Aires, DF, Argentina
dc.description.affiliationSafra Childrens Hosp, Chaim Sheba Med Ctr, Tel Hashomer, Israel
dc.description.affiliationTel Aviv Univ, Sackler Fac Med, Dept Pediat, IL-69978 Tel Aviv, Israel
dc.description.affiliationUniversidade do Estado do Rio de Janeiro (UERJ), Rheumatol Sect, Adolescent Hlth Care Unit, BR-20550011 Rio de Janeiro, Brazil
dc.description.affiliationUniversidade do Estado do Rio de Janeiro (UERJ), Dept Pediat Rheumatol, BR-20550011 Rio de Janeiro, Brazil
dc.description.affiliationUniversidade do Estado do Rio de Janeiro (UERJ), Dept Pediat Rheumatol, Unigranrio, Brazil
dc.description.affiliationUniv São Paulo, Fac Med, Dept Pediat, Div Rheumatol, São Paulo, Brazil
dc.description.affiliationSão Paulo State Univ, Botucatu Med Sch, Dept Pediat, Botucatu, SP, Brazil
dc.description.affiliationUniv Cattolica Sacro Cuore, Dept Pediat Sci, Rome, Italy
dc.description.affiliationUniv Milan, Dept Internal Med, Milan, Italy
dc.description.affiliationSan Luca Hosp, Allergy Clin Immunol & Rheumatol Unit, IRCCS, Ist Auxol Italiano, Milan, Italy
dc.description.affiliationUnespSão Paulo State Univ, Botucatu Med Sch, Dept Pediat, Botucatu, SP, Brazil
dc.rights.accessRightsAcesso restrito
dc.description.sponsorshipIdSlovenian Ministry of Higher Education, Science, and Technology: L3-0624
dc.description.sponsorshipIdSlovenian Ministry of Higher Education, Science, and Technology: P3-0314
unesp.campusUniversidade Estadual Paulista (UNESP), Faculdade de Medicina, Botucatupt[16][11][13]
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