INTRAVENOUS ARGININE DRAMATICALLY IMPROVED HYPERAMMONEMIA IN A PATIENT WITH LATE-ONSET ORNITHINE TRANSCARBAMYLASE DEFICIENCY

We describe a 12 year-old male patient with late-onset ornithine trans carbamylase deficiency, in whom infusion of arginine alone dramaticall y improved intercurrent hyperammonemia. The plasma glutamine level als o decreased while the urea nitrogen level increased with arginine infu sion, indicating that accumulated nitrogen was metabolized to urea in response to the arginine infusion.