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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<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. © 1992 Mosby-Year Book, Inc.




Maestri, N. E., McGowan, K. D., & Brusilow, S. W. (1992). Plasma glutamine concentration: A guide in the management of urea cycle disorders. The Journal of Pediatrics, 121(2), 259–261. https://doi.org/10.1016/S0022-3476(05)81200-4

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