Anesthesia for Living Related Liver Transplantation in Homozygous Protein C Deficiency.
10.4097/kjae.2001.40.5.671
- Author:
Mi Ae CHEONG
1
;
Kyu Sam HWANG
;
Kyu Taek CHOI
;
Yoon CHOI
;
Eun Ju LEE
;
Eun Jung CHUNG
Author Information
1. Department of Anesthesiology, College of Medicine, University of Ulsan, Seoul, Korea.
- Publication Type:Case Report
- Keywords:
Anesthesia: pediatric;
Blood: coagulation;
protein C;
Liver: transplantation
- MeSH:
Anesthesia*;
Child;
Factor Va;
Fibrinolysis;
Heparin, Low-Molecular-Weight;
Humans;
Liver Transplantation*;
Liver*;
Protein C Deficiency*;
Protein C*;
Purpura Fulminans;
Thrombosis
- From:Korean Journal of Anesthesiology
2001;40(5):671-676
- CountryRepublic of Korea
- Language:Korean
-
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.
