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.
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| http://dx.doi.org/10.1016/j.clinbiochem.2011.03.136 | DOI Listing |
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