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Stable isotopes in the diagnosis and treatment of inherited hyperammonemia.

Authors :
Mew, Nicholas Ah
Yudkoff, Marc
Tuchman, Mendel
Source :
Journal of Pediatric Biochemistry. 2014, Vol. 4 Issue 1, p57-63. 7p.
Publication Year :
2014

Abstract

Stable isotopes have greatly contributed to our understanding of nitrogen metabolism and the urea cycle. The measurement of urea flux via isotopic methods has traditionally been utilized to determine total body protein synthesis in subjects with an intact urea cycle. However, isotopic studies of nitrogen metabolism are also a useful adjunct to conventional clinical investigations in the diagnosis and management of the inherited hyperammonemias. Such studies offer a safe non-invasive method of measuring the reduction of in vivo hepatic ureagenesis, and thus may provide a more accurate measure of phenotypic severity in affected patients. In addition, isotopic methods are ideally suited to evaluate the efficacy of novel therapies to augment urea production. [ABSTRACT FROM AUTHOR]

Details

Language :
English
ISSN :
18795390
Volume :
4
Issue :
1
Database :
Academic Search Index
Journal :
Journal of Pediatric Biochemistry
Publication Type :
Academic Journal
Accession number :
94081202
Full Text :
https://doi.org/10.3233/JPB-140106