Protein C exerts anticoagulant effects by inactivating factor Va and VIIIa and stimulating fibrinolysis. The homozygous protein C deficiency is extremely rare and often results in life threatening thrombosis and purpura fulminans with necrotic cutaneous lesions. A child with homozygous protein C deficiency was treated at 6 months by a living-related liver transplantaion. After induction of anesthesia, we started an FFP infusion for protein C replacement and a low molecular weight heparin continuous infusion to prevent thrombosis. A complete reconstitution of protein C activity and resolution of the thrombotic condition occured postoperatively. So we report this case with a brief review of the literature.
Anesthesia* ; Child ; Factor Va ; Fibrinolysis ; Heparin, Low-Molecular-Weight ; Humans ; Liver Transplantation* ; Liver* ; Protein C Deficiency* ; Protein C* ; Purpura Fulminans ; Thrombosis

Protein C (PC) is a vitamin K-dependent plasma protein synthesized as a zymogen by liver. It's active form has an anti-thrombotic activity by cleaving the coagulation factor Va and VIIIa. During the last few years, a large number of cases with protein C deficiency (PCD) which contain variable mutations have been documented. Those with hereditary protein C deficiency tend to have an increased risk of thromboembolism. Hypertension, diabetes, hyperlipidemia and glaucoma are the main underlying diseases of the retinal vein occlusion (RVO). Rarely, thrombophilia (antithrombin III, protein S, protein C deficiency) results in the RVO which usually occurs after adolescent period. We recently experienced a case of central retinal vein occlusion by a PCD.
Adolescent ; Factor Va ; Glaucoma ; Humans ; Hyperlipidemias ; Hypertension ; Liver ; Plasma ; Protein C ; Protein C Deficiency ; Protein S ; Retinal Vein ; Retinal Vein Occlusion ; Thromboembolism ; Thrombophilia ; Vitamins