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Korean Journal of Anesthesiology 2001;40(5):671-676.
DOI: https://doi.org/10.4097/kjae.2001.40.5.671   
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


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