Activated protein C resistance and the factor V Leiden mutation in children with thrombosis

Sifontes, Maria T.; Nuss, Rachelle; Hunger, Stephen P.; Waters, Jill; Jacobson, Linda; Manco‐Johnson, Marilyn J.

doi:10.1002/(sici)1096-8652(199801)57:1<29::aid-ajh5>3.0.co;2-3

Cited by 26 publications

(10 citation statements)

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“…The death rate of thrombosis was 24% in our registry. The all cause of mortality in children with thrombosis was 16-20% in literature [14,29]. In our patient population, only 3 children (2.1%) died as a direct consequence of their thromboembolic disease, which was very similar to the results reported by Andrew et al [1].…”

Section: Discussionsupporting

confidence: 90%

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Analysis of Pediatric Thrombotic Patients in Turkey

Ören

Devecioğlu

Kemahlı

et al. 2004

Pediatric Hematology and Oncology

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This study analyzes the data of thrombotic children who were followed up in different pediatric referral centers of Turkey, to obtain more general data on the diagnosis, risk factors, management, and outcome of thrombosis in Turkish children. A simple two-page questionnaire was distributed among contact people from each center to standardize data collection. Thirteen pediatric referral centers responded to the invitation and the total number of cases was 271. All children were diagnosed with thromboembolic disease between January 1995 and October 2001. Median age at time of first thrombotic event was 7.0 years. Of the children 4% of the cases were neonates, 12% were infants less than 1 year old, and 17% were adolescents. Thromboembolic event was mostly located in the cerebral vascular system (32%), deep venous system of the limbs, femoral and iliac veins (24%), portal veins (10%), and intracardiac region (9%). Acquired risk factors were present in 86% of the children. Infection was the most common underlying risk factor. Inherited risk factors were present in 30% of the children. FVL was the most common inherited risk factor. Acquired and inherited risk factors were present simultaneously in 19% of the patients. Eleven children had a history of familial thrombosis. Due to the local treatment preferences, the treatment of the children varied greatly. Outcome of the 142 patients (52%) was reported: 88 (62%) patients had complete resolution, 47 (33%) had complications, 12 (9%) had recurrent thrombosis, and 34 (24%) died. Three children (2.1%) died as a direct consequence of their thromboembolic disease. The significant morbidity and mortality found in this study supports the need for multicentric prospective clinical trials to obtain more generalizable data on management and outcome of thrombosis in Turkish children.

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Section: Discussionsupporting

confidence: 90%

“…FVL was the most common inherited risk factor as reported in previous studies [6,22,[27][28][29][30]. Acquired and inherited risk factors were present simultaneously in 19% of the patients.…”

Section: Discussionsupporting

confidence: 55%

Analysis of Pediatric Thrombotic Patients in Turkey

Ören

Devecioğlu

Kemahlı

et al. 2004

Pediatric Hematology and Oncology

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“…Inherited thrombophilias (i.e. antithrombin-, protein C-, and protein S-deficiency) and the mutations of coagulation factor (F) V G1691A and FII G20210A have been established as risk factors for VTE in adults [13][14][15][16][17], and have been described as additional risk factors in children [7,[18][19][20][21][22][23][24][25][26][27][28][29][30][31][32][33][34][35][36].…”

Section: Introductionmentioning

confidence: 99%

Influence of the factor II G20210A variant or the factor V G1691A mutation on symptomatic recurrent venous thromboembolism in children: an international multicenter cohort study

Becker

Düring

Friedrichs

et al. 2009

Journal of Thrombosis and Haemostasis

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of the factor II G20210A variant or the factor V G1691A mutation on symptomatic recurrent venous thromboembolism in children: an international multicenter cohort study. J Thromb Haemost 2009; 7: 72-9.Summary. Objective: To determine the relative importance of the factor (F) II G20210A or FV G1691A mutations as risk factors or predictors for fatal/non-fatal recurrent venous thromboembolism (VTE) in children. Methods: In the present cohort, the rate of VTE recurrence and the time to recurrence in relation to FII, FV, age, and sex was determined in consecutively enrolled patients with VTE aged newborn to £18 years carrying the FII (n = 64) or FV (n = 194) mutation. 158 children with VTE without thrombophilia served as controls. Patients were followed for a median of 58 months. Data were pooled across participating sites to increase power and to enhance the generalizability of the data. Incidence rates were given as events per 1000 person-years. Results: Of the 416 children enrolled, 44 had recurrent VTE at a median of 12 months following VTE onset. The overall incidence rate of recurrence was 19.8, 57.9 in patients with the FII variant, 17.9 for FV carriers, and 11.8 in the control cohort. When comparing FII patients, FV children and the control cohort multivariate analysis (Cox regression) adjusted for age and sex showed that the FII variant (hazard ratio 2.6; 95% confidence interval 1.1-5.9) influenced the hazard for recurrent VTE.Conclusions: Based on multivariate analysis, the presence of the FII variant was associated with an increased risk of VTE recurrence.

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“…19,20 However, clinical presentation in individuals or within affected kinships may vary over a broad range that includes cerebral sinus thrombosis, venous stasis ulcerations, and hepatic vein thrombosis. Additionally, early onset of thrombotic events 16,[19][20][21][22][23][24][25][26][27][28][29][30] is more frequently associated with genetic thrombophilic disorders.…”

Section: Discussionmentioning

confidence: 99%