[Budd-Chiari syndrome]

Rev Med Interne. 2013 Dec;34(12):741-5. doi: 10.1016/j.revmed.2013.02.038. Epub 2013 Nov 18.
[Article in French]

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%.

Keywords: Budd-Chiari syndrome; Myeloproliferative disorders; Syndrome de Budd-Chiari; Syndrome myéloprolifératif; Thrombophilie; Thrombotic disorders.

Publication types

  • English Abstract
  • Review

MeSH terms

  • Anticoagulants / therapeutic use
  • Budd-Chiari Syndrome / diagnosis*
  • Budd-Chiari Syndrome / etiology
  • Budd-Chiari Syndrome / therapy*
  • Disease Progression
  • Humans
  • Liver Transplantation
  • Portasystemic Shunt, Transjugular Intrahepatic
  • Prognosis
  • Risk Factors

Substances

  • Anticoagulants