1/2. Familial type II protein c deficiency associated with warfarin-induced skin necrosis and bilateral adrenal hemorrhage.A family is described in which venous thromboembolic disease is associated with reduced plasma protein C activity and normal levels of protein C antigen. Immunoelectrophoretic analysis of protein C antigen gave an abnormal pattern in all affected members, suggesting that the disorder is related to the presence of a structurally and functionally abnormal form of protein C. The propositus developed simultaneous warfarin-induced skin necrosis and bilateral adrenal hemorrhage. This is the first reported instance of warfarin-induced skin necrosis associated with a dysfunctional protein C molecule and the first reported instance of simultaneous warfarin-induced skin necrosis and bilateral adrenal hemorrhage.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/2. Treatment of hereditary protein c deficiency with stanozolol.Five type I protein C deficient male patients received 5 mg stanozolol b.i.d. during 4 weeks. After four weeks of treatment plasma protein C activity increased from 0.42 to 0.74 U/ml and protein C antigen from 0.49 to 0.75 U/ml. This approximately 1.6 fold increase in plasma protein C was accompanied by an increase in factor II antigen (1.5 fold), factor V activity (1.6 fold), factor x antigen (1.1 fold), antithrombin iii antigen (1.3 fold) and heparin cofactor ii antigen (1.5 fold), while the concentration of factor VII, factor viii, and factor ix activity, and of protein s antigen remained unchanged. prothrombin fragment F1 2, measured in two patients, increased 1.3 fold. In addition to its effect on procoagulant and anticoagulant factors stanozolol had profibrinolytic effects, reflected in an increase in tPA activity and in the concentration of plasminogen. These data indicate that in type I protein C deficient patients stanozolol increases the concentrations of both procoagulant and anticoagulant factors and favours fibrinolysis. The efficacy of stanozolol in preventing thrombotic disease in type I protein C deficient patients, however, remains to be established. During the four weeks of stanozolol treatment no thrombotic manifestations were observed in the protein C deficient patients.- - - - - - - - - - ranking = 3keywords = antigen (Clic here for more details about this article) |