PEDIATRICS Vol. 73 No. 4 April 1984, pp. 489-492
This Article
Right arrow Full Text (PDF)
Right arrow P3Rs: Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when P3Rs are posted
Right arrow Alert me if a correction is posted
Services
Right arrow E-mail this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My File Cabinet
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via CrossRef
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Kato, T.
Right arrow Articles by Ban, M.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Kato, T.
Right arrow Articles by Ban, M.

Hyperammonemia in Lysinuric Protein Intolerance

Tomoaki Kato MD1, Naoki Mizutani MD1, and Masakazu Ban MD1

1 From the Department of Pediatrics, Chubu-Rosai Hospital; Department of Pediatrics, School of Medicine, Nagoya University; and Department of Pediatrics, Mitsubishi Nagoya Hospital, Nagoya, Japan

Two brothers with hyperdibasicaminoaciduria and postprandial hyperammonemia showed characteristics of lysinuric protein intolerance. Intravenous alanine load produced hyperammonemia that was aborted by oral supplementation with arginine in one brother but not in the other, although both patients had almost the same intestinal malabsorption of arginine. This occurrence suggests that even a small amount of arginine, when absorbed into the blood, can normalize the affected ammonia metabolism of lysinuric protein intolerance. Two patients with cystinuria developed marked hyperammonemia when they received an intravenous alanine load after a 19-hour fast. As both patients displayed a reduced plasma concentration of arginine and ornithine at this time, the hyperammonemia was assumed to arise from the low plasma amino acid level. It seems likely that a decrease in plasma levels of urea cycle substrate causes a failure of the tissue urea cycle metabolism. Thus the impaired ammonia metabolism in lysinuric protein intolerance would be attributed to the low plasma arginine and ornithine levels.

Key Words: arginine • cystinuria • hyperammonemia • hyperdibasicaminoaciduria • ornithine

Submitted on February 3, 1983
Accepted on June 8, 1983




This article has been cited by other articles:


Home page
 ChestHome page
A. S. Clay and B. E. Hainline
Hyperammonemia in the ICU
Chest, October 1, 2007; 132(4): 1368 - 1378.
[Abstract] [Full Text] [PDF]