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Pediatric venous thromboembolic disease in one single center: congenital prothrombotic disorders and the clinical outcome
Author(s) -
Van Ommen C. H.,
Heijboer H.,
Van Den Dool E. J.,
Hutten B. A.,
Peters M.
Publication year - 2003
Publication title -
journal of thrombosis and haemostasis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.947
H-Index - 178
eISSN - 1538-7836
pISSN - 1538-7933
DOI - 10.1046/j.1538-7836.2003.00465.x
Subject(s) - medicine , pediatrics , incidence (geometry) , protein c deficiency , family history , post thrombotic syndrome , single center , protein s deficiency , disease , venous thromboembolism , thrombosis , surgery , venous thrombosis , physics , optics
Summary. To learn more about the frequencies of congenital prothrombotic disorders in pediatric venous thromboembolism (VTE) and the outcome of this disease, we evaluated consecutive patients from 0 to 18 years with objectively diagnosed VTE at a single tertiary center over a 12‐year period. We included 100 patients, with a median age at diagnosis of 1.0 year (range 2 days to 17 years). At least one underlying clinical condition was present in 96% of the patients. Factor (F)V G1691A mutation was present in 13%, FII G20210A mutation in 3%, antithrombin deficiency in 1%, protein C deficiency in 1% and protein S deficiency in 1% of the tested patients. Combined defects were present in 2.6% of the 77 patients with a complete work‐up. Positive family history appeared to be the only predictor for positive testing for congenital disorders (OR 14.9, 95% CI 1.9–113). The overall mortality rate was 20%. The cumulative recurrence‐free survival was 92% after 1 year of follow‐up, and 82% after 7 years. The incidence and severity of the post‐thrombotic syndrome was analyzed in a subgroup of 33 patients with VTE of the lower extremities. Twenty‐three (70%) patients developed PTS: moderate in three and mild in 20 patients. In conclusion, congenital prothrombotic disorders seem to play a role in the development of pediatric VTE, and the risk of complications of this disease is high.