Objective: To evaluate the clinical features and outcome of antiphosph
olipid syndrome (APS) in children. Study design: Retrospective chart r
eview of patients seen at the Children's Hospital of Philadelphia and
Children's Seashore House Pediatric Rheumatology Center between 1988 a
nd 1993. Results: Nine patients with ages ranging from 8 months to 17
years are presented. Clinical features of five patients with primary A
PS, described in detail, were digital ischemia, stroke, chorea, Addiso
n disease, and pulmonary vaso-occlusive disease. The four children wit
h secondary APS had systemic lupus erythematosus. Clinical features of
these patients include livedo reticularis, deep venous thrombosis, an
d pulmonary hypertension. Antiphospholipid titers, results of coagulat
ion studies, and serologic findings did not predict outcome. Conclusio
n: APS in children has diverse clinical features similar to those in a
dults and should be considered in cases of unexplained vaso-occlusive
disease.