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Knoebl PN. Severe congenital protein C deficiency: the use of protein C concentrates (human) as replacement therapy for life-threatening blood-clotting complications. Biologics. 2008;2(2):285-96doi: 10.2147/btt.s1954.
Knoebl, P. N. (2008). Severe congenital protein C deficiency: the use of protein C concentrates (human) as replacement therapy for life-threatening blood-clotting complications. Biologics : targets & therapy, 2(2), 285-96. https://doi.org/10.2147/btt.s1954
Knoebl, Paul N. "Severe congenital protein C deficiency: the use of protein C concentrates (human) as replacement therapy for life-threatening blood-clotting complications." Biologics : targets & therapy vol. 2,2 (2008): 285-96. doi: https://doi.org/10.2147/btt.s1954
Knoebl PN. Severe congenital protein C deficiency: the use of protein C concentrates (human) as replacement therapy for life-threatening blood-clotting complications. Biologics. 2008 Jun;2(2):285-96. doi: 10.2147/btt.s1954. PMID: 19707361; PMCID: PMC2721356.
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Biologics. 2008 Jun;2(2):285-96. doi: 10.2147/btt.s1954.

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

Biologics : targets & therapy

Paul N Knoebl

Affiliations

  1. Department of Medicine 1, Division Hematology and Hemostasis, Medical University of Vienna, Vienna, Austria.

PMID: 19707361 PMCID: PMC2721356 DOI: 10.2147/btt.s1954

Abstract

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: coagulopathy; deficiency; protein C; purpura fulminans; replacement therapy

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