title: [Budd-Chiari syndrome].
abstract: The management of the Budd-Chiari syndrome improved dramatically during the last 10 years and includes less invasive diagnostic modalities using modern imaging, identification of a myeloproliferative disorder in 20 to 50% of the patients using the V617F JAK2 mutation, and a graduate therapeutic strategy. The common association of Budd-Chiari syndrome with a thrombotic disorder is a reason for a thorough work-up (myeloproliferative disorder, defect in C or S protein, factor V Leiden, factor II mutation, antiphosholipid syndrome, and other less common disorders). Ultrasonography should to be performed by an experimented examiner, informed of the diagnostic suspicion. The 5-year survival rate of patients with Budd-Chiari syndrome, treated with this contemporary approach (anticoagulation, treatment of the underlying cause, recanalization, transjugular intrahepatic portosystemic shunting, and liver transplantation) is above 80%.
authors.affiliation: Centre de référence des maladies vasculaires du foie, service d'hépatologie, hôpital Beaujon, 100, boulevard du Général-Leclerc, 92110 Clichy, France. Electronic address: aurelie.plessier@bjn.aphp.fr.
authors.forename: A
authors.initials: A
authors.lastname: Plessier
mesh_terms: D000925:Anticoagulants / Q000627:therapeutic use; D006502:Budd-Chiari Syndrome / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy*; D018450:Disease Progression; D006801:Humans; D016031:Liver Transplantation; D019168:Portasystemic Shunt, Transjugular Intrahepatic; D011379:Prognosis; D012307:Risk Factors
chemical_list: D000925:Anticoagulants
keywords: Budd-Chiari syndrome; Myeloproliferative disorders; Syndrome de Budd-Chiari; Syndrome myéloprolifératif; Thrombophilie; Thrombotic disorders