Preparation of L-Arginine and Sodium Benzoate Tablets for Patients with Hyperammonemia Caused by Inborn Errors of Urea Synthesis, and their Clinical Applications.

Accession number;99A0450013
Title;Preparation of L-Arginine and Sodium Benzoate Tablets for Patients with Hyperammonemia Caused by Inborn Errors of Urea Synthesis, and their Clinical Applications.
Author; KOORI NAONORI (Hokkaidoyakudai Byoin) ITAGAKI FUMIO (Hokkaido Univ., Sch. of Med., Hosp.) NAGASAKA HIRONORI (Hokkaido Univ., Sch. of Med.) KISHINO SATOSHI (Hokkaido Univ., Sch. of Med., Hosp.) ISEKI KEN (Hokkaido Univ., Sch. of Med., Hosp.) KOBAYASHI KUNIHIKO (Hokkaido Univ., Sch. of Med.) MIYAZAKI KATSUMI (Hokkaido Univ., Sch. of Med., Hosp.)
Journal Title;Japanese Journal of Hospital Pharmacy
Journal Code:Y0888A
ISSN:0389-9098
VOL.25;NO.2;PAGE.118-122(1999)
Figure&Table&Reference;FIG.4, TBL.1, REF.8
Pub. Country;Japan
Language;Japanese
Abstract;Hyperammonemia which is caused by inherited urea cycle enzyme deficiencies, is often fatal when acute symptoms of hyperammonemic coma(vomiting, unconsciousness convulsion, respiratory paralysis, et al.) appear and continue without any medical treatment. L-Arginine or sodium benzoate has been used for the treatment of hyperammonemia. However, it is difficult for children to take such reagents orally in powder form due to the bulky dose volume and their offensive bitter taste. A significant decrease in medication compliance has thus led to hyperammonemic coma or liver transplantation. In this study, we prepared tablets containing a high percentage of L-arginine or sodium benzoate by the wetgranulation method, and administered them to several children with hyperammonemia. Remarkable improvements in the compliance and clinical effects, as well as decreases in the occurance of comas and the serum ammonium levels, have been observed in all patients. (author abst.)