Korean J Anesthesiol.  2001 May;40(5):671-676. 10.4097/kjae.2001.40.5.671.

Anesthesia for Living Related Liver Transplantation in Homozygous Protein C Deficiency

• Cheong MA
• Hwang KS
• Choi KT
• Choi Y
• Lee EJ
• Chung EJ

• KMID: 2091627
• DOI: http://doi.org/10.4097/kjae.2001.40.5.671

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.

Keyword

Anesthesia: pediatric; Blood: coagulation; protein C; Liver: transplantation

MeSH Terms

Anesthesia*
Child
Factor Va
Fibrinolysis
Heparin, Low-Molecular-Weight
Humans
Liver Transplantation*
Liver*
Protein C Deficiency*
Protein C*
Purpura Fulminans
Thrombosis
Factor Va
Heparin, Low-Molecular-Weight
Protein C