Levels of protein C, low at birth, physiologically Increase until six months of age and achieve the adult range after puberty. Protein C deficiency may be congenital or acquired. Severe protein C deficiency is a rare autosomal recessive disorder that usually presents in neonatal period with purpura fulminans. Acquired protein C deficiency may be caused by increased consumption (e.g., asphyxia, overt DIC, severe infection without overt DIC, acute VTE) or by decreased synthesis of the active carboxylated protein (e.g. administration of vitamin K antagonists, severe hepatic synthetic disfunction). Two different formulations of protein C are available: recombinant human activated protein C (rhAPC) and human plasma-derived viral-inactivated protein C. It is known that in septic patients replacement therapy with rhAPC reduces mortality but is associated with an increased risk of bleeding. During the neonatal period, when a higher risk of bleeding exists, the human plasma-derived viral-inactivated protein C concentrate may represent an effective therapeutic option. In fact, its administration results effective both in severe congenital and acquired forms of protein C deficiency.
De Carolis, M. P., Use of protein C concentrate in neonatal period., <<MINERVA PEDIATRICA>>, 2010; (Giugno): 29-30 [http://hdl.handle.net/10807/23537]
Use of protein C concentrate in neonatal period.
De Carolis, Maria Pia
2010
Abstract
Levels of protein C, low at birth, physiologically Increase until six months of age and achieve the adult range after puberty. Protein C deficiency may be congenital or acquired. Severe protein C deficiency is a rare autosomal recessive disorder that usually presents in neonatal period with purpura fulminans. Acquired protein C deficiency may be caused by increased consumption (e.g., asphyxia, overt DIC, severe infection without overt DIC, acute VTE) or by decreased synthesis of the active carboxylated protein (e.g. administration of vitamin K antagonists, severe hepatic synthetic disfunction). Two different formulations of protein C are available: recombinant human activated protein C (rhAPC) and human plasma-derived viral-inactivated protein C. It is known that in septic patients replacement therapy with rhAPC reduces mortality but is associated with an increased risk of bleeding. During the neonatal period, when a higher risk of bleeding exists, the human plasma-derived viral-inactivated protein C concentrate may represent an effective therapeutic option. In fact, its administration results effective both in severe congenital and acquired forms of protein C deficiency.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.