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Assessment of plasma ammonia and glutamine concentrations in urea cycle disorders. 2011
M Serrano, and
A Ormazábal, and
M A Vilaseca, and
N Lambruschini, and
R Garcia-Romero, and
S Meavilla, and
B Perez-Dueñas, and
M Pineda, and
A Garcia-Cazorla, and
J Campistol, and
R Artuch
Neuropediatric Gastroenterology and Clinical Biochemistry Departments, Hospital Sant Joan de Déu, Barcelona, Spain. mserrano@hsjdbcn.org
OBJECTIVE To analyze the association between ammonia and glutamine used for metabolic control in inherited urea cycle disorders (UCD) in a large series of patients. 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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A Ormazábal, and
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M Pineda, and
A Garcia-Cazorla, and
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