Abstract | OBJECTIVE: STUDY DESIGN: Twelve patients with ASA were enrolled in a double-blind, placebo-controlled, cross-over study design. Subjects were randomized to receive either a low-dose of arginine therapy (100 mg · kg(-1) · d(-1)) combined with sodium phenylbutyrate (500 mg · kg(-1) · d(-1)) (LDA arm) or a high-dose of arginine alone (500 mg · kg(-1) · d(-1)) (HDA arm) for one week. At the end of one week of therapy, liver function tests were assessed and metabolite fluxes were measured using a multi-tracer stable isotope protocol. RESULTS: CONCLUSIONS: Administering higher doses of arginine in subjects with ASA results in increases in AST and ALT levels, especially in the subset of patients with elevated baseline aminotransferases. Hence, low-dose arginine sufficient to normalize arginine levels in plasma combined with nitrogen scavenging therapy should be considered as a therapeutic option for treatment of ASA in patients with elevations of hepatic aminotransferases.
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Authors | Sandesh C S Nagamani, Oleg A Shchelochkov, Mary A Mullins, Susan Carter, Brendan C Lanpher, Qin Sun, Soledad Kleppe, Ayelet Erez, E O'Brian Smith, Juan C Marini, Members of the Urea Cycle Disorders Consortium, Brendan Lee |
Journal | Molecular genetics and metabolism
(Mol Genet Metab)
Vol. 107
Issue 3
Pg. 315-21
(Nov 2012)
ISSN: 1096-7206 [Electronic] United States |
PMID | 23040521
(Publication Type: Journal Article, Randomized Controlled Trial, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't)
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Copyright | Copyright © 2012 Elsevier Inc. All rights reserved. |
Chemical References |
- Phenylbutyrates
- Placebos
- Argininosuccinic Acid
- Arginine
- Aspartate Aminotransferases
- Alanine Transaminase
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Topics |
- Adolescent
- Alanine Transaminase
(blood)
- Arginine
(blood, therapeutic use)
- Argininosuccinic Acid
(blood)
- Argininosuccinic Aciduria
(blood, drug therapy)
- Aspartate Aminotransferases
(blood)
- Child
- Child, Preschool
- Cross-Over Studies
- Double-Blind Method
- Drug Administration Schedule
- Drug Therapy, Combination
- Female
- Humans
- Liver Function Tests
- Male
- Phenylbutyrates
(blood, therapeutic use)
- Placebos
- Young Adult
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