Back to Browse Journals » Biologics: Targets and Therapy » Volume 2 » Issue 2

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

Authors Paul N Knoebl

Published Date June 2008 Volume 2008:2(2) Pages 285—296

DOI http://dx.doi.org/10.2147/BTT.S1954

Published 30 June 2008

Paul N Knoebl

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

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

Download Article [PDF] 

Creative Commons License This work is published by Dove Medical Press Limited, and licensed under Creative Commons Attribution - Non Commercial (unported, v3.0) License. The full terms of the License are available at http://creativecommons.org/licenses/by-nc/3.0/. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. Permissions beyond the scope of the License are administered by Dove Medical Press Limited. Information on how to request permission may be found at: http://www.dovepress.com/permissions.php

Readers of this article also read:

Nanocomplexation of thrombin with cationic amylose derivative for improved stability and hemostatic efficacy

Zhuang B, Li Z, Pang J, Li W, Huang P, Wang J, Zhou Y, Lin Q, Zhou Q, Ye X, Ye H, Liu Y, Zhang LM, Chen R

International Journal of Nanomedicine 2015, 10:939-947

Published Date: 29 January 2015

Lower irritation microemulsion-based rotigotine gel: formulation optimization and in vitro and in vivo studies

Wang Z, Mu HJ, Zhang XM, Ma PK, Lian SN, Zhang FP, Chu SY, Zhang WW, Wang AP, Wang WY, Sun KX

International Journal of Nanomedicine 2015, 10:633-644

Published Date: 14 January 2015

Effects of Arthro-7® in relieving symptoms of osteoarthritis with mild to moderate arthralgia [Corrigendum]

Xie Q, Zhou T, Yen L, Shariff M, Nguyen T, Kami K, Gu P, Liang L, Rao J, Shi R

Nutrition and Dietary Supplements 2013, 5:7-8

Published Date: 16 May 2013

Retraction

Singhai R

Breast Cancer: Targets and Therapy 2012, 4:33-34

Published Date: 8 March 2012

Corrigendum

Sailasuta N, Harris K, Tran T, Ross B

Neuropsychiatric Disease and Treatment 2011, 7:707-708

Published Date: 25 November 2011

Corrigendum: Softec HD hydrophilic acrylic intraocular lens: biocompatibility and precision

Espandar L, Sikder S, Moshirfar M

Clinical Ophthalmology 2011, 5:159-160

Published Date: 6 February 2011

Role of aliskiren in cardio-renal protection and use in hypertensives with multiple risk factors

Eduardo Pimenta, Suzanne Oparil

Vascular Health and Risk Management 2009, 5:453-463

Published Date: 19 May 2009