Clinical relevance of protein C.
Protein C is, after activation by thrombin, a potent inhibitor of blood coagulation. An isolated deficiency of protein C increases the risk of thrombosis. The two forms of protein C deficiency, the heterozygous and the homozygous deficiency state, have different clinical features. Patients with heterozygous protein C deficiency are at a high risk to develop venous thrombosis and pulmonary embolism. In newborns with homozygous protein C deficiency with very low protein C levels (1%) a purpura fulminans like syndrome was observed. Heparin and coumarin derivatives are effective drugs in heterozygous protein C deficiency, homozygous patients may be treated either by replacement of protein C or coumarin derivatives. Decreased protein C levels were observed in various other diseases: Chronic and acute liver disease, disseminated intravascular coagulation, malignancy, postoperatively and during treatment with asparaginase. The role of protein C in these diseases to trigger thrombosis is not yet established.
D000818:Animals; D000911:Antibodies, Monoclonal; D000941:Antigens / Q000032:analysis; D004797:Enzyme-Linked Immunosorbent Assay; D030342:Genetic Diseases, Inborn / Q000175:diagnosis / Q000235:genetics / Q000628:therapy; D005838:Genotype; D006023:Glycoproteins / Q000032:analysis / Q000172:deficiency / Q000276:immunology / Q000378:metabolism / Q000502:physiology*; D006041:Goats / Q000276:immunology; D006470:Hemorrhage / Q000139:chemically induced; D006493:Heparin / Q000627:therapeutic use; D006579:Heterozygote; D006720:Homozygote; D006801:Humans; D009336:Necrosis / Q000139:chemically induced; D011486:Protein C; D011817:Rabbits / Q000276:immunology; D011863:Radioimmunoassay; D012871:Skin Diseases / Q000139:chemically induced; D013924:Thrombophlebitis / Q000150:complications / Q000517:prevention & control
D000911:Antibodies, Monoclonal; D000941:Antigens; D006023:Glycoproteins; D011486:Protein C; D006493:Heparin