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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Dove Medical Press
2010
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oai:doaj.org-article:08281d4ded334a03a102e25ce501da852021-12-02T01:15:07ZUse of human protein C concentrates in the treatment of patients with severe congenital protein C deficiency1177-54751177-5491https://doaj.org/article/08281d4ded334a03a102e25ce501da852010-03-01T00:00:00Zhttp://www.dovepress.com/use-of-human-protein-c-concentrates-in-the-treatment-of-patients-with--a4043https://doaj.org/toc/1177-5475https://doaj.org/toc/1177-5491Sabine 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 Sabine KroissManuela AlbisettiDove Medical PressarticleMedicine (General)R5-920ENBiologics: Targets & Therapy, Vol 2010, Iss default, Pp 51-60 (2010) |
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Medicine (General) R5-920 |
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Medicine (General) R5-920 Sabine Kroiss Manuela Albisetti Use of human protein C concentrates in the treatment of patients with severe congenital protein C deficiency |
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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 |
format |
article |
author |
Sabine Kroiss Manuela Albisetti |
author_facet |
Sabine Kroiss Manuela Albisetti |
author_sort |
Sabine Kroiss |
title |
Use of human protein C concentrates in the treatment of patients with severe congenital protein C deficiency |
title_short |
Use of human protein C concentrates in the treatment of patients with severe congenital protein C deficiency |
title_full |
Use of human protein C concentrates in the treatment of patients with severe congenital protein C deficiency |
title_fullStr |
Use of human protein C concentrates in the treatment of patients with severe congenital protein C deficiency |
title_full_unstemmed |
Use of human protein C concentrates in the treatment of patients with severe congenital protein C deficiency |
title_sort |
use of human protein c concentrates in the treatment of patients with severe congenital protein c deficiency |
publisher |
Dove Medical Press |
publishDate |
2010 |
url |
https://doaj.org/article/08281d4ded334a03a102e25ce501da85 |
work_keys_str_mv |
AT sabinekroiss useofhumanproteincconcentratesinthetreatmentofpatientswithseverecongenitalproteincdeficiency AT manuelaalbisetti useofhumanproteincconcentratesinthetreatmentofpatientswithseverecongenitalproteincdeficiency |
_version_ |
1718403176781053952 |