Anesthetic management of a patient with arginase deficiency undergoing liver transplantation.

A 20-year-old female underwent orthotopic liver transplantation for arginase deficiency, a urea cycle disorder. A hyperammonemic state was prevented by the administration of lipid and carbohydrate substrate and avoidance of protein loading (including human albumin) and prolonged fasting. Caval cross-clamping may have been tolerated poorly owing to the potential interaction between hyperargininemia (a nitric oxide precursor) and the lack of collateral venous drainage. Ammonia and arginine levels improved in parallel with hepatic function after reperfusion of the hepatic graft.