Anesthesia for Living Related Liver Transplantation in Homozygous Protein C Deficiency. |
Mi Ae Cheong, Kyu Sam Hwang, Kyu Taek Choi, Yoon Choi, Eun Ju Lee, Eun Jung Chung |
1Department of Anesthesiology, College of Medicine, University of Ulsan, Seoul, Korea. 2Department of Anesthesiology, College of Medicine, University of Hanyang, Seoul, Korea. |
|
|
Abstract |
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. |
Key Words:
Anesthesia: pediatric; Blood: coagulation; protein C; Liver: transplantation |
|