Protein C deficiency and portal thrombosis in liver transplantation in children.
Changes in plasma protein C and antithrombin concentrations after liver transplantation were monitored in fourteen children and a control group of fourteen adults. In the children, there was a persistent deficiency in the plasma concentration of protein C and a less pronounced deficiency in antithrombin during the early postoperative period, causing a hypercoaguable state. A concomitant rise in plasminogen activator inhibitor further increased the risk of thrombosis by inhibiting fibrinolysis. These changes coincided with the peak incidence of portal vessel thrombosis (4-10 days). Replacement of plasma antithrombin, together with heparin, did not prevent portal thrombosis in two of the children. It is concluded that successful prevention will require protein C replacement together with antithrombin supplements up to, but not exceeding, normal plasma activity.
D000328:Adult; D000991:Antithrombins / Q000172:deficiency / Q000627:therapeutic use; D001656:Biliary Atresia / Q000601:surgery; D001779:Blood Coagulation Factors / Q000032:analysis; D002648:Child; D002675:Child, Preschool; D006023:Glycoproteins / Q000032:analysis; D006493:Heparin / Q000627:therapeutic use; D006801:Humans; D007223:Infant; D008107:Liver Diseases / Q000601:surgery; D016031:Liver Transplantation; D008875:Middle Aged; D010960:Plasminogen Activators / Q000037:antagonists & inhibitors; D015849:Plasminogen Inactivators; D011169:Portal Vein; D020151:Protein C Deficiency; D013927:Thrombosis / Q000209:etiology* / Q000517:prevention & control
D000991:Antithrombins; D001779:Blood Coagulation Factors; D006023:Glycoproteins; D015849:Plasminogen Inactivators; D006493:Heparin; D010960:Plasminogen Activators