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[Hereditary thrombophilia as a cause of recurrent transplant thromboses]
B H Koester1, G B Köveker, B Pötsch
1Allgemeinchirurgische Klinik und Poliklinik, Universität Tübingen.
We report a case of hereditary protein S and protein C deficiency which are a rare defects of the anti-coagulation-system. Protein S is a vitamin K dependent glycoprotein that functions as a cofactor to activated Protein C in the inactivation of coagulation factors V and VIIIa. A deficiency of these proteins caused by a genetic defect increase the risk of recurrent thrombosis at a younger age. Acquired decreases in protein S and C concentration have been reported in connection with age, sex, pregnancy and with oral anticonception. The higher risk for thrombotic diseases of patients with thrombophilia requires a sufficient treatment and prophylaxis, e.g. with fresh frozen plasma or a protein C concentrate.
We report a case of hereditary protein S and protein C deficiency which are a rare defects of the anti-coagulation-system. Protein S is a vitamin K dependent glycoprotein that functions as a cofactor to activated Protein C in the inactivation of coagulation factors V and VIIIa. A deficiency of these proteins caused by a genetic defect increase the risk of recurrent thrombosis at a younger age. Acquired decreases in protein S and C concentration have been reported in connection with age, sex, pregnancy and with oral anticonception. The higher risk for thrombotic diseases of patients with thrombophilia requires a sufficient treatment and prophylaxis, e.g. with fresh frozen plasma or a protein C concentrate.