title: Use of human protein C concentrates in the treatment of patients with severe congenital protein C deficiency.
abstract: 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.
authors.affiliation: Division of Hematology, University Children's Hospital, Zurich, Switzerland.
authors.forename: Sabine
authors.initials: S
authors.lastname: Kroiss
authors.forename: Manuela
authors.initials: M
authors.lastname: Albisetti