Use of human protein C concentrates in the treatment of patients with severe congenital protein C deficiency
Sabine Kroiss, Manuela AlbisettiDivision of Hematology, University Children’s Hospital, Zurich, SwitzerlandAbstract: Protein C is one of the major inhibitors of the coagulation system that downregulate thrombin generation. Severe congenital protein C deficiency leads to a hypercoagulab...
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| Autores principales: | , |
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| Formato: | article |
| Lenguaje: | EN |
| Publicado: |
Dove Medical Press
2010
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| Materias: | |
| Acceso en línea: | https://doaj.org/article/08281d4ded334a03a102e25ce501da85 |
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| Sumario: | Sabine Kroiss, Manuela AlbisettiDivision of Hematology, University Children’s Hospital, Zurich, SwitzerlandAbstract: Protein C is one of the major inhibitors of the coagulation system that downregulate thrombin generation. Severe congenital protein C deficiency leads to a hypercoagulability state that usually presents at birth with purpura fulminans and/or severe venous and arterial thrombosis. Recurrent thrombotic events are commonly seen. From the 1990’s, several virus-inactivated human protein C concentrates have been developed. These concentrates currently constitute the therapy of choice for the treatment and prevention of clinical manifestations of severe congenital protein C deficiency. This review summarizes the available information on the use of human protein C concentrates in patients with severe congenital protein C deficiency.Keywords: Congenital protein C deficiency, protein C concentrate, purpura fulminans |
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