title: Antiphospholipid syndrome and recurrent thrombosis in children.
abstract: OBJECTIVE
  Few studies have addressed antiphospholipid syndrome (APS) among children. Our aims were to analyze the clinical and laboratory manifestations in a pediatric APS cohort and to assess the influence of inherited thrombophilia factors on the outcome of children with APS.
  
  
  METHODS
  This was a multicenter study of children with APS who had no previous systemic autoimmune disease. We retrospectively reviewed their clinical and laboratory data, including hereditary thrombophilic deficits and outcomes.
  
  
  RESULTS
  The cohort comprised 28 patients (17 females, mean +/- SD age at onset 10.6 +/- 6.1 years). The most common initial manifestations of APS were venous thrombosis, stroke, and thrombocytopenia. Lupus anticoagulant was detected in 96% of those tested. After a mean +/- SD followup of 5.7 +/- 4.8 years, 16 children (57.1%) had central nervous system disease, 9 exhibited hematologic involvement, and 5 (all females) had systemic lupus erythematosus (SLE). None had renal, heart, or new skin disease. Seven of 24 patients exhibiting vascular thrombotic events had recurrences. Infants with perinatal stroke had monophasic disease, and other manifestations of APS did not develop later. Hereditary thrombophilia was more common in children who experienced a single episode of APS (8 [53.3%] of 15 patients) than in those who experienced recurrences (2 [28.6%] of 7 patients). However, only 2 patients in the latter group (28.6%) received anticoagulants after the first manifestation, compared with 12 (70.6%) of the 17 patients without recurrences.
  
  
  CONCLUSIONS
  APS in children has unique features. SLE may develop in a significant percentage of girls presenting with APS. Hereditary thrombophilia did not predict recurrent thrombosis, whereas the preventive impact of anticoagulant treatment following the first thrombotic event was noteworthy.
authors.affiliation: Safra Children's Hospital, Sheba Medical Center, Tel-Hashomer, Israel.
authors.forename: Y
authors.initials: Y
authors.lastname: Berkun
authors.forename: S
authors.initials: S
authors.lastname: Padeh
authors.forename: J
authors.initials: J
authors.lastname: Barash
authors.forename: Y
authors.initials: Y
authors.lastname: Uziel
authors.forename: L
authors.initials: L
authors.lastname: Harel
authors.forename: M
authors.initials: M
authors.lastname: Mukamel
authors.forename: S
authors.initials: S
authors.lastname: Revel-Vilk
authors.forename: G
authors.initials: G
authors.lastname: Kenet
mesh_terms: D000293:Adolescent; D016736:Antiphospholipid Syndrome / Q000150:complications* / Q000235:genetics / Q000276:immunology; D001323:Autoantibodies / Q000097:blood; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000235:genetics / Q000276:immunology; D008297:Male; D011237:Predictive Value of Tests; D012008:Recurrence; D012189:Retrospective Studies; D020521:Stroke / Q000209:etiology / Q000235:genetics / Q000276:immunology; D013921:Thrombocytopenia / Q000209:etiology / Q000235:genetics / Q000276:immunology; D019851:Thrombophilia / Q000150:complications* / Q000235:genetics / Q000276:immunology; D020246:Venous Thrombosis / Q000209:etiology* / Q000235:genetics / Q000276:immunology
chemical_list: D001323:Autoantibodies