Severe congenital protein C deficiency: description of a new mutation and prophylactic protein C therapy and in vivo pharmacokinetics.

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...

Topics

• Base Sequence
• Female
• Humans
• Infant, Newborn
• Molecular Sequence Data
• Mutation
• Protein C
• Protein C Deficiency