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Korean Journal of Anesthesiology 2005;49(4):563-566.
DOI: https://doi.org/10.4097/kjae.2005.49.4.563   
Anesthesia for Living Related Liver Transplantation in Argininosuccinic Acidemia: A case report.
So Young Ban, Bon Nyeo Koo, Jong Ho Lee, Soon Ho Nam
1Department of Anesthesiology and Pain Medicine, Yonsei University College of Medicine, Seoul, Korea. nsh66@yumc.yonsei.ac.kr
2Anesthesia and Pain Research Institute, Yonsei University College of Medicine, Seoul, Korea.
Abstract
We describe our initial experience of the perioperative anesthetic care provided to 8 years old female child with argininosuccinic acidemia undergoing living-related liver transplantation because it is the only available therapy for end-stage liver disease. Induction and maintenance of anesthesia has been conventional method. Arterial catheterized at radial and femoral arteries for continuous blood pressure monitoring and sampling. 18 G central vein catheterization was placed in left subclavian vein for fluid, drug infusion and CVP monitoring. EKG, pulse oxymetry, end-tidal CO2, urine output and body temperature were monitored. CBC, PT, aPTT, serum electrolyte were checked at preanhepatic, anhepatic phase and just after hepatic artery anastomosis. ABGA was checked every 1 hour. The level of serum ammmonia returned to normal range without protein restriction. We describe this case and a brief review of the literature.
Key Words: argininosuccinic acidemia; liver transplantation


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