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

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...

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Bibliographic Details
Published inThe Journal of pediatrics Vol. 121; no. 2; pp. 259 - 261
Main Authors Maestri, Nancy E., McGowan, Kathryn D., Brusilow, Saul W.
Format Journal Article
LanguageEnglish
Published New York, NY Mosby, Inc 01.08.1992
Elsevier
Subjects
Amino Acid Metabolism, Inborn Errors - blood
Amino Acid Metabolism, Inborn Errors - therapy
Ammonia - blood
Biological and medical sciences
Errors of metabolism
Glutamine - blood
Humans
Infant, Newborn
Male
Medical sciences
Metabolic diseases
Miscellaneous hereditary metabolic disorders
Ornithine Carbamoyltransferase Deficiency Disease
Urea - metabolism
Human
Correlation
Biochemical analysis
Enzyme
Deficiency
Exploration
Metabolic diseases
Infant
Enzymopathy
Ammonemia
Genetic disease
Ornithine carbamoyltransferase
Blood plasma
Glutamine
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Summary: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.
Bibliography:ObjectType-Case Study-3
ObjectType-Article-1
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ISSN:0022-3476
1097-6833
DOI:10.1016/S0022-3476(05)81200-4