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• W2040485280 abstract "Abstract PROTEIN C IS a vitamin K-dependent plasma protein that is an important natural antithrombotic component. After activation by a thrombin-thrombomodulin complex on the endothelial cells, activated protein C inhibits coagulation by inactivating factors V and VIII and leads to inhibition of thrombin formation. Protein S serves as a cofactor for protein C in its anticoagulant properties.1 Protein C and S deficiencies are rare diseases, characterized by recurrent venous thrombosis. Their deficiency may be inherited, either homozygous (purpura fulminans in neonates) or heterozygous or acquired. Inherited protein C and S deficiencies are transmitted in an autosomal codominant fashion and present during infancy. If not treated, homozygous individuals die of thrombosis in early infancy.2 An infant is described with a highly unusual case of combined congenital homozygous protein C and S deficiency who also had cardiac defects (transposition of the great arteries and subpulmonic stenosis) and inferior vena cava thrombosis." @default.
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• W2040485280 date "2000-04-01" @default.
• W2040485280 modified "2023-10-12" @default.
• W2040485280 title "Cardiac operation in a patient with combined homozygous protein C and protein S deficiency" @default.
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• W2040485280 doi "https://doi.org/10.1016/s1053-0770(00)90017-x" @default.
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