Severe congenital protein C deficiency: the use of protein C concentrates (human) as replacement therapy for life-threatening blood-clotting complications

Paul N KnoeblDepartment of Medicine 1, Division Hematology and Hemostasis, Medical University of Vienna, Vienna, AustriaAbstract: The protein C pathway has an important function in regulating and modulating blood coagulation and ensuring patency of the microcirculation. Protein C deficiency leads to...

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Autor principal:	Paul N Knoebl
Formato:	article
Lenguaje:	EN
Publicado:	Dove Medical Press 2008
Materias:	Medicine (General) R5-920
Acceso en línea:	https://doaj.org/article/6f065675ace44e00bf83afdf9e8dc21a
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spelling	oai:doaj.org-article:6f065675ace44e00bf83afdf9e8dc21a2021-12-02T02:40:11ZSevere congenital protein C deficiency: the use of protein C concentrates (human) as replacement therapy for life-threatening blood-clotting complications1177-54751177-5491https://doaj.org/article/6f065675ace44e00bf83afdf9e8dc21a2008-06-01T00:00:00Zhttp://www.dovepress.com/severe-congenital-protein-c-deficiency-the-use-of-protein-c-concentrat-a1733https://doaj.org/toc/1177-5475https://doaj.org/toc/1177-5491Paul N KnoeblDepartment of Medicine 1, Division Hematology and Hemostasis, Medical University of Vienna, Vienna, AustriaAbstract: The protein C pathway has an important function in regulating and modulating blood coagulation and ensuring patency of the microcirculation. Protein C deficiency leads to macro- and microvascular thrombosis. Congenital severe protein C deficiency is a life-threatening state with neonatal purpura fulminans and pronounced coagulopathy. Patients with heterozygous protein C deficiency have an increased risk for thromboembolic events or experience coumarin-induced skin necrosis during initiation of coumarin therapy. Replacement with protein C concentrates is an established therapy of congenital protein C deficiency, resulting in rapid resolving of coagulopathy and thrombosis without reasonable side effects. This article summarizes the current knowledge on protein C replacement therapy in congenital protein C deficiency.Keywords: protein C, deficiency, replacement therapy, purpura fulminans, coagulopathy Paul N KnoeblDove Medical PressarticleMedicine (General)R5-920ENBiologics: Targets & Therapy, Vol 2008, Iss Issue 2, Pp 285-296 (2008)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine (General) R5-920
spellingShingle	Medicine (General) R5-920 Paul N Knoebl Severe congenital protein C deficiency: the use of protein C concentrates (human) as replacement therapy for life-threatening blood-clotting complications
description	Paul N KnoeblDepartment of Medicine 1, Division Hematology and Hemostasis, Medical University of Vienna, Vienna, AustriaAbstract: The protein C pathway has an important function in regulating and modulating blood coagulation and ensuring patency of the microcirculation. Protein C deficiency leads to macro- and microvascular thrombosis. Congenital severe protein C deficiency is a life-threatening state with neonatal purpura fulminans and pronounced coagulopathy. Patients with heterozygous protein C deficiency have an increased risk for thromboembolic events or experience coumarin-induced skin necrosis during initiation of coumarin therapy. Replacement with protein C concentrates is an established therapy of congenital protein C deficiency, resulting in rapid resolving of coagulopathy and thrombosis without reasonable side effects. This article summarizes the current knowledge on protein C replacement therapy in congenital protein C deficiency.Keywords: protein C, deficiency, replacement therapy, purpura fulminans, coagulopathy
format	article
author	Paul N Knoebl
author_facet	Paul N Knoebl
author_sort	Paul N Knoebl
title	Severe congenital protein C deficiency: the use of protein C concentrates (human) as replacement therapy for life-threatening blood-clotting complications
title_short	Severe congenital protein C deficiency: the use of protein C concentrates (human) as replacement therapy for life-threatening blood-clotting complications
title_full	Severe congenital protein C deficiency: the use of protein C concentrates (human) as replacement therapy for life-threatening blood-clotting complications
title_fullStr	Severe congenital protein C deficiency: the use of protein C concentrates (human) as replacement therapy for life-threatening blood-clotting complications
title_full_unstemmed	Severe congenital protein C deficiency: the use of protein C concentrates (human) as replacement therapy for life-threatening blood-clotting complications
title_sort	severe congenital protein c deficiency: the use of protein c concentrates (human) as replacement therapy for life-threatening blood-clotting complications
publisher	Dove Medical Press
publishDate	2008
url	https://doaj.org/article/6f065675ace44e00bf83afdf9e8dc21a
work_keys_str_mv	AT paulnknoebl severecongenitalproteincdeficiencytheuseofproteincconcentrateshumanasreplacementtherapyforlifethreateningbloodclottingcomplications
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Severe congenital protein C deficiency: the use of protein C concentrates (human) as replacement therapy for life-threatening blood-clotting complications

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