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
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Publicado: Dove Medical Press 2008
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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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