Factor V Leiden related Budd-Chiari syndrome.

Détails

ID Serval
serval:BIB_3D13B8E0E83C
Type
Article: article d'un périodique ou d'un magazine.
Sous-type
Etude de cas (case report): rapporte une observation et la commente brièvement.
Collection
Publications
Titre
Factor V Leiden related Budd-Chiari syndrome.
Périodique
Gut
Auteur⸱e⸱s
Deltenre P., Denninger M.H., Hillaire S., Guillin M.C., Casadevall N., Brière J., Erlinger S., Valla D.C.
ISSN
0017-5749 (Print)
ISSN-L
0017-5749
Statut éditorial
Publié
Date de publication
2001
Volume
48
Numéro
2
Pages
264-268
Langue
anglais
Notes
Publication types: Journal ArticlePublication Status: ppublish
Résumé
BACKGROUND: The role of factor V Leiden as a cause of Budd-Chiari syndrome has only recently been described.
AIMS: To assess the specific features of factor V Leiden related Budd-Chiari syndrome.
PATIENTS: Sixty three consecutive patients with hepatic vein or terminal inferior vena cava thrombosis.
METHODS: Standardised chart review.
RESULTS: Factor V Leiden was found in 20 patients (31% (95% CI 20-43)). In the subgroup of patients with, compared with the subgroup without, factor V Leiden, a combination of prothrombotic states was more common (70% (95% CI 50-90) v 14% (95% CI 3-24)); inferior vena cava thrombosis was more frequent (40% (95% CI 19-61) v 7% (95% CI 0-14)); and distribution of initial alanine aminotransferase values was bimodal (almost normal or extremely increased) versus unimodal (p=0.003). Factor V Leiden accounted for four of five cases of massive ischaemic necrosis (transaminases >50-fold the upper limit of normal values) (p=0.014), and also for all three cases developing during pregnancy. Patients with and without factor V Leiden did not differ with regard to mortality, portosytemic shunting, or listing for liver transplantation. Hepatocellular carcinoma developed in two patients; both had factor V Leiden and indolent obstruction of the inferior vena cava.
CONCLUSIONS: In patients with Budd-Chiari syndrome, factor V Leiden (a) is common; (b) precipitates thrombosis mostly when combined with another risk factor; (c) is associated with one of two contrasting clinical pictures: indolent thrombosis-particularly of the inferior vena cava-or massive ischaemic necrosis; and (d) is a major cofactor of Budd-Chiari syndrome developing during pregnancy.
Mots-clé
Adult, Alanine Transaminase/blood, Budd-Chiari Syndrome/diagnosis, Budd-Chiari Syndrome/genetics, Factor V/genetics, Female, Humans, Male, Middle Aged, Mutation, Pregnancy, Pregnancy Complications, Hematologic/etiology, Protein C/metabolism, Risk Factors, Thrombophilia/diagnosis, Thrombophilia/genetics
Pubmed
Web of science
Création de la notice
06/12/2013 10:30
Dernière modification de la notice
20/08/2019 13:33
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