Nineteen children who presented with thromboses over a 7-year period w
ere found to have a lupus anticoagulant (LA), The initial thrombosis w
as proximal deep vein thrombosis (DVT) in six children, central nervou
s system (CNS) in five, primary pulmonary in four, distal DVT in two,
central venous in one, and proximal arterial in one. Five children wer
e diagnosed with systemic lupus erythematosus (SLE), including two chi
ldren for whom thrombosis was the presenting sign of SLE, The remainin
g 14 children were diagnosed with the antiphospholipid antibody (APA)
syndrome, The APA syndrome was manifest by venous or arterial thrombos
is in association with a positive LA; positive anticardiolipin antibod
ies and a fine, speckled antinuclear antibody (ANA) pattern were addit
ionally found in the majority of children, Approximately one-half of t
he children with SLE or the APA syndrome had a pulmonary embolus, and
one-half developed recurrent thrombosis. Oral anticoagulation with cou
madin to achieve an INR of >2.0 prevented thrombosis recurrence, The r
ecognition of a LA in children with thrombosis necessitates evaluation
for SLE, APA, and other autoantibodies. (C) 1995 Wiley-Liss, Inc.