title: Assessment of plasma ammonia and glutamine concentrations in urea cycle disorders.
abstract: 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.
authors.affiliation: Neuropediatric Gastroenterology and Clinical Biochemistry Departments, Hospital Sant Joan de Déu, Barcelona, Spain. mserrano@hsjdbcn.org
authors.forename: M
authors.initials: M
authors.lastname: Serrano
authors.forename: A
authors.initials: A
authors.lastname: Ormazábal
authors.forename: M A
authors.initials: MA
authors.lastname: Vilaseca
authors.forename: N
authors.initials: N
authors.lastname: Lambruschini
authors.forename: R
authors.initials: R
authors.lastname: Garcia-Romero
authors.forename: S
authors.initials: S
authors.lastname: Meavilla
authors.forename: B
authors.initials: B
authors.lastname: Perez-Dueñas
authors.forename: M
authors.initials: M
authors.lastname: Pineda
authors.forename: A
authors.initials: A
authors.lastname: Garcia-Cazorla
authors.forename: J
authors.initials: J
authors.lastname: Campistol
authors.forename: R
authors.initials: R
authors.lastname: Artuch
mesh_terms: D000641:Ammonia / Q000097:blood*; D005973:Glutamine / Q000097:blood*; D006801:Humans; D007231:Infant, Newborn; D056806:Urea Cycle Disorders, Inborn / Q000097:blood*
chemical_list: D005973:Glutamine; D000641:Ammonia