RESUMEN
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.