H. Kodama et al., INTRAVENOUS ARGININE DRAMATICALLY IMPROVED HYPERAMMONEMIA IN A PATIENT WITH LATE-ONSET ORNITHINE TRANSCARBAMYLASE DEFICIENCY, Tohoku Journal of Experimental Medicine, 180(1), 1996, pp. 83-86
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.