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Portal vein thrombosis with ruptured oesophageal varices as presenting manifestation of hereditary protein C deficiency.
  1. D Valla,
  2. M H Denninger,
  3. J M Delvigne,
  4. B Rueff,
  5. J P Benhamou
  1. Service d'Hépatologie, Hôpital Beaujon, Clichy, France.

    Abstract

    The protein C system is essential in limiting the activation of coagulation in vivo. We report the case of a 45 year old man with portal vein thrombosis complicated by ruptured oesophageal varices. Low concentration of plasma protein C was found in the patient and subsequently in one brother with a history of venous thromboembolism, and also in one son and one nephew who were asymptomatic. Hereditary protein C deficiency should be considered in patients with portal hypertension due to portal vein thrombosis.

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