Syndrome of Budd-Chiari

Definition

The Syndrome of Budd-Chiari is a rare Maladie Foie which results from the obstruction of at least one of the three know-hepatic veins by a Thrombose or a Tumeur.

Symptoms

  • Ascite
  • hepatic Pains (abdominal)
  • Sometimes a hepatic insufficiency
  • Hypertension portale
  • collateral Circulation
  • painful Hepatomegaly

Causes & Risk factors

  • Syndromes myéloprolifératifs
  • Change of the factor V Leiden
  • Anomaly of the Factor II
  • Deficits in Proteins C, Proteins S, Antithrombine III
  • lupic Disease of Behçet
  • Anticoagulant
  • Pregnancy
  • inflammatory State
  • Contraceptive oral

Diagnosis

  • Scanner with injection
  • IRM with injection
  • Echography

Incidence

There would be approximately a case on 100  000 people. In France, one estimates the number of touched people at less than one thousand.

Life expectancy

  • 90% to 10 years in 2005

  • 70% to 10 years in 2000
  • 10% in the Years 1970, period during which the diagnosis of the disease existed but one could not look after it yet.

Treatments

  • surgical Medical care or of the ascite
  • Anticoagulants with the long course

Surgery

  • the Transplantation is vital in the cases presenting a serious hepatic insufficiency.

Complications

External bonds

  • the syndrome of budd-chiari by the professor Dominique Charles Valla
  • Association of the Patients of the Vessels of the Liver

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