Perioperative Management of a Patient With Arginase 1 Deficiency Undergoing a Liver Transplant: A Case Report.

We describe the case of a 24-year-old patient with arginase 1 deficiency who underwent a liver transplant for treatment of his disorder. This case is noteworthy as it provides support for a perioperative management strategy described in one other report of a patient with this rare metabolic disorder undergoing a liver transplant. The primary management goals are to (1) reduce the risk for hyperammonemia by administering sodium phenylbutyrate and sodium benzoate to promote nitrogen excretion and (2) prevent catabolism by infusing carbohydrates and lipids. Vigilant monitoring for sodium derangements is necessary because these infusions may lead to hyper- or hyponatremia.