FAMILIAL PROTEIN-S DEFICIENCY - MESENTERI C AND PORTAL-VEIN THROMBOSIS

Protein S, a vitamin-K dependent glycoprotein is a cofactor of protein -C system, which acts as an inhibitor of the plasmatic coagulation. Pr otein-S congenital deficiency results in recurrent venous thromboses, atypical locations in portal and mesenteric veins are possible. In our patient the partial thrombosis of the portal vein was diagnosed by co mputed tomography and angiography. Small bowel ischaemia due to mesent eric vein thrombosis required segmental resection. Postoperatively the patient was heparinized and later phenprocoumon was applied to a long -term therapy.