Congenital thrombophilia and thrombosis: a study in a single centre
S E Lawson, D Butler, M S Enayat, M D Williams
Department of
Haematology, Birmingham Children's Hospital, Birmingham B4 6NH, UK
Correspondence to: Dr Lawson.
Accepted 26 April 1999
AIM
To identify the
incidence of congenital thrombophilia in a cohort of children
presenting with symptomatic thromboembolism.
METHODA review of
children with thromboembolism investigated for thrombophilia over a 12 month period.
SUBJECTSThirty
children with thromboembolic episodes and 16 of their family members.
MEASUREMENTS AND DATA
COLLECTIONData were collected on age at
diagnosis, underlying diagnosis, site of thrombosis, associated
precipitating factors, occurrence of other thromboembolic events, and
family history. Investigations included measurement of protein C
activity, total and free protein S antigen, antithrombin III activity,
screening for factor V Leiden and prothrombin 20210A, urinary
homocysteine estimation, and a screen for lupus anticoagulant.
RESULTSTwenty seven
of 30 patients had one or more risk factors present at the time of
thromboembolism. Eighty three per cent had acquired precipitating
factors present, and 43% had underlying congenital thrombophilia.
CONCLUSIONSThere was
a high incidence of congenital thrombophilia in this group of patients
with symptomatic thromboembolism. These findings emphasise the
importance of such defects in the pathogenesis of childhood thrombosis,
and suggest that full thrombophilia investigations should be performed
on all children presenting with thromboembolic disease.
Keywords: thrombosis; congenital thrombophilia
© 1999 by Archives of Disease in Childhood
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