دورية أكاديمية
Severe congenital protein C deficiency: the use of protein C concentrates (human) as replacement therapy for life-threatening blood-clotting complications
| العنوان: | 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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| المؤلفون: | Knoebl,Paul N |
| بيانات النشر: | Dove Press |
| سنة النشر: | 2008 |
| المجموعة: | Dove Medical Press |
| مصطلحات موضوعية: | Biologics: Targets and Therapy |
| الوصف: | 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 |
| نوع الوثيقة: | article in journal/newspaper review |
| وصف الملف: | text/html |
| اللغة: | English |
| العلاقة: | https://www.dovepress.com/severe-congenital-protein-c-deficiency-the-use-of-protein-c-concentrat-peer-reviewed-fulltext-article-BTTTest |
| الإتاحة: | https://doi.org/10.2147/BTT.S1954Test https://www.dovepress.com/severe-congenital-protein-c-deficiency-the-use-of-protein-c-concentrat-peer-reviewed-fulltext-article-BTTTest |
| حقوق: | info:eu-repo/semantics/closedAccess |
| رقم الانضمام: | edsbas.32647119 |
| قاعدة البيانات: | BASE |
| الوصف غير متاح. |