Objectives: To analyze the association between ammonia and glutamine used for metabolic control in inherited urea cycle disorders (UCD) in a large series of patients.
Design and methods: Paired plasma amino acid-ammonia data from 26 UCD patients were analyzed (n=921).
Results: Increased plasma glutamine values were consistently observed in UCD patients, despite normal plasma ammonia concentrations, especially for mitochondrial UCD.
Conclusions: Further therapeutic efforts are probably needed to control increased glutamine values, considering their potentially neurotoxic effect.
Copyright © 2011 The Canadian Society of Clinical Chemists. Published by Elsevier Inc. All rights reserved.