P092: Hyperammonemia in a child with short bowel syndrome: urea cycle disorder or complication of altered gastrointestinal function?

Initial management of the patient’s hyperammonemia consisted of rifaximin, lactulose, 10% dextrose at 1.5 times maintenance rate, intralipids 1g/kg/day, and an IV ammonia scavenger. He was later transitioned to an enteral ammonia scavenger and supplementation with enteral citrulline. In regards to his dietary management, original enteral protein goals were 1.5g/kg/day in order to balance the need for low protein with a potential urea cycle disorder and malabsorption from short bowel syndrome. However, plasma amino acid levels remained low until TPN was initiated and the amino acid concentration in the TPN alone was increased to 1g/kg/day.