Article
Severe congenital protein C deficiency: description of a new mutation and prophylactic protein C therapy and in vivo pharmacokinetics.
Journal of pediatric hematology/oncology - 1 Feb 2008
Tcheng Wendy Y, Dovat Sinisa, Gurel Zafer, Donkin Jennifer, Wong Wing-Yen
Abstract excerpt
Severe congenital protein C deficiency is a rare life-threatening disorder that presents with purpura fulminans, disseminated intravascular coagulation, and thrombotic complications during the neonatal period. Affected children require acute replacement therapy with fresh frozen plasma or protein C concentrate, for example, Ceprotin (Baxter AG, Vienna). Long-term management and outcome is dependent on effective...
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