Plasma glutamine concentration: a guide in the management of urea cycle disorders

J Pediatr. 1992 Aug;121(2):259-61. doi: 10.1016/s0022-3476(05)81200-4.


Because increases in plasma glutamine concentrations are almost always associated with hyperammonemia in patients with urea cycle disorders, we determined the correlation between these two variables for 2 years in a child with ornithine transcarbamylase deficiency. A correlation coefficient of 0.77 (p less than 0.0001) was found. Hyperammonemia was rarely observed when plasma glutamine levels were near normal. These data suggest that one goal of therapy is the maintenance of plasma glutamine levels at or near normal values.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Amino Acid Metabolism, Inborn Errors / blood*
  • Amino Acid Metabolism, Inborn Errors / therapy
  • Ammonia / blood*
  • Glutamine / blood*
  • Humans
  • Infant, Newborn
  • Male
  • Ornithine Carbamoyltransferase Deficiency Disease*
  • Urea / metabolism


  • Glutamine
  • Ammonia
  • Urea