protein C deficiency


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protein C deficiency

A condition characterized by a deficiency of vitamin K dependent plasma protein C and protein S, both natural anticoagulants; PCD is either AD of variable penetration, or acquired, and due to DIC, warfarin therapy, hepatic disease and postoperatively
References in periodicals archive ?
Reported causes of thrombophilia in HIV-infected subjects include antiphospholipid syndrome [1], increased platelet activation [1], elevated homocysteinemia [2], elevated plasma factor VII activity [2], lupus anticoagulant [3], activated protein C resistance [3], protein C deficiency [3,4], and acquired protein S deficiency [4-10].
Isolated protein S deficiency was seen in 9 patients, and combined protein S plus protein C deficiency occurred in 3 subjects.
But HIV is also associated with a variety of acquired coagulopathies that increase the incidence of venous and arterial thrombosis, including antiphospholipid-anticardoplipin antibodies, increased platelet activation, elevated serum homocysteine levels, lupus anticoagulant, elevated plasma factor VII activity, activated protein C resistance, protein C deficiency, and protein S deficiency [1-10].
These protein deficiencies/abnormalities are genetic and include entities such as antithrombin deficiency, protein C deficiency, protein S deficiency, Factor V Leiden mutation, prothrombin 20210, and dysfibrinogenemia, among others.
Food and Drug Administration (FDA) has approved CEPROTIN [Protein C Concentrate (Human)], a plasma-derived Protein C concentrate for use as replacement therapy in patients with life-threatening blood-clotting complications related to severe congenital Protein C deficiency.
Severe congenital Protein C deficiency results in a hypercoagulable state, meaning there is an abnormal tendency for blood clotting.
It is indicated for patients with severe congenital Protein C deficiency for the prevention and treatment of venous thrombosis and purpura fulminans (PF) (a severe skin and systematic blood clotting disorder).
This is a significant day for patients with severe congenital Protein C deficiency and their families, as this offers a much needed treatment option for these patients.
Protein C deficiency was first reported in 1981 by Griffin et al.
However, genetic testing has been used successfully for prenatal diagnosis of homozygous protein C deficiency.
Because of biological variation and gene-environment interactions, there is a large overlap in, for instance, protein C values between proven carriers of a protein C deficiency (heterozygotes) and non-protein C-deficient family members [8].
ProC Global represents an easy-to-perform screening test that is highly sensitive for FV Leiden and protein C deficiency.