THE INCIDENCE OF PROTEIN-C DEFICIENCY IN THROMBOSIS-RELATED PORTAL-HYPERTENSION

Objective: To know the incidence of protein C deficiency associated wi th noncirrhotic, thrombosis-related portal hypertension, Methods: Thir ty-six patients were studied who had thrombosis-related portal hyperte nsion diagnosed by means of hepatic venography or abdominal echocardio graphy or during abdominal surgery, Liver disease was excluded in 20 p atients based on normal liver function tests and normal histology on l iver biopsy, At the time of protein C assays, these patients were not receiving oral anticoagulation, and, in those recently diagnosed, the assays were performed more than 14 days after the last thrombotic even t, Antigenic and functional assays for protein C were performed by ELI SA and chromogenic assay, respectively, Results: We found 11 patients with protein C deficiency who had a median age of 28 yr (range 19-55 y r) at time of diagnosis, Five patients had a history of systemic throm boembolism, and upper GI bleeding was the most frequent symptom relate d to portal hypertension (six cases), Antigenic protein C levels were measured in nine of the 11 patients (mean 31.88%, range 10-49%), Funct ional protein C level was assayed for all 11 patients (mean 40.90%, ra nge 15-58%), After diagnosis, all patients received oral anticoagulant s (ideally International Normalized Ratio: 2-3), Conclusion: We sugges t that protein C screening should be performed in patients with thromb osis-related portal hypertension.