The protein C system is essential in limiting the activation of coagulation in vivo. We report on a 29 year old woman with Budd-Chiari syndrome and occlusion of the inferior vena cava who presented with acute liver failure. She was successfully treated with an emergency mesoatrial shunt. Eight months after surgery, she has no ascites and normal liver function. She had a low concentration of plasma protein C on admission to hospital and during the follow up. Protein C deficiency subsequently was found in her father and two sisters, who were asymptomatic. Hereditary protein C deficiency should be considered in patients with Budd-Chiari syndrome.
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