Incidence of VTE in asymptomatic children with deficiencies of antithrombin, protein C, and protein S: a prospective cohort study.


Journal

Blood advances
ISSN: 2473-9537
Titre abrégé: Blood Adv
Pays: United States
ID NLM: 101698425

Informations de publication

Date de publication:
10 11 2020
Historique:
received: 25 06 2020
accepted: 16 09 2020
entrez: 6 11 2020
pubmed: 7 11 2020
medline: 15 5 2021
Statut: ppublish

Résumé

Although antithrombin, protein C, and protein S defects are well-recognized inherited risk factors for venous thromboembolism (VTE) in adults, whether they predispose children to these vascular disorders as well is undefined. In a prospective cohort study, we assessed the incidence of spontaneous and risk period-related VTE in children who were family members of adults who, after an episode of symptomatic VTE, had then been identified as carriers of these abnormalities. A total of 134 children from 87 families were enrolled. Seventy (51.5%) of these children were carriers of an inherited defect, and the remaining 64 were not; the mean observation period was 4 years (range, 1-16 years) and 3.9 years (range, 1-13), respectively. Sixteen risk periods were experienced by carriers, and 9 by noncarriers. Six VTE occurred in the 70 carriers during 287 observation-years, accounting for an annual incidence of 2.09% patient-years (95% confidence interval, 0.8-4.5), compared with none in the 64 noncarriers during 248 observation-years. Of the 14 children with thrombophilia who experienced a risk period for thrombosis, 4 (28.6%) developed a VTE episode. The overall incidence of risk-related VTE was 25% per risk period (95% confidence interval, 6.8-64). In conclusion, the thrombotic risk in otherwise healthy children with severe inherited thrombophilia does not seem to differ from that reported for adults with the same defects. Screening for thrombophilia in children who belong to families with these defects seems justified to identify those who may benefit from thromboprophylaxis during risk periods for thrombosis.

Identifiants

pubmed: 33156924
pii: S2473-9529(20)31940-6
doi: 10.1182/bloodadvances.2020002781
pmc: PMC7656938
doi:

Substances chimiques

Anticoagulants 0
Antithrombins 0
Protein C 0
Protein S 0

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

5442-5448

Informations de copyright

© 2020 by The American Society of Hematology.

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Auteurs

Daniela Tormene (D)

General Internal Medicine and Thrombotic and Haemorrhagic Diseases Unit, Department of Medicine, University of Padua Medical School, Padua, Italy; and.

Elena Campello (E)

General Internal Medicine and Thrombotic and Haemorrhagic Diseases Unit, Department of Medicine, University of Padua Medical School, Padua, Italy; and.

Chiara Simion (C)

General Internal Medicine and Thrombotic and Haemorrhagic Diseases Unit, Department of Medicine, University of Padua Medical School, Padua, Italy; and.

Giacomo Turatti (G)

General Internal Medicine and Thrombotic and Haemorrhagic Diseases Unit, Department of Medicine, University of Padua Medical School, Padua, Italy; and.

Michelangelo Marobin (M)

General Internal Medicine and Thrombotic and Haemorrhagic Diseases Unit, Department of Medicine, University of Padua Medical School, Padua, Italy; and.

Claudia Maria Radu (CM)

General Internal Medicine and Thrombotic and Haemorrhagic Diseases Unit, Department of Medicine, University of Padua Medical School, Padua, Italy; and.

Anna Pontarin (A)

General Internal Medicine and Thrombotic and Haemorrhagic Diseases Unit, Department of Medicine, University of Padua Medical School, Padua, Italy; and.

Paolo Prandoni (P)

Arianna Foundation, Bologna, Italy.

Paolo Simioni (P)

General Internal Medicine and Thrombotic and Haemorrhagic Diseases Unit, Department of Medicine, University of Padua Medical School, Padua, Italy; and.

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Classifications MeSH