Title

Blood ammonia and glutamine as predictors of hyperammonemic crises in patients with urea cycle disorder.

Document Type

Article

Publication Date

7-1-2015

Institution/Department

Pediatrics

Journal Title

Genetics in medicine : official journal of the American College of Medical Genetics

MeSH Headings

Adolescent, Adult, Ammonia, Child, Child, Preschool, Female, Glutamine, Humans, Hyperammonemia, Infant, Male, Middle Aged, Predictive Value of Tests, Urea Cycle Disorders, Inborn, Young Adult

ISSN

1530-0366

Abstract

PURPOSE: The aim of this study was to examine predictors of ammonia exposure and hyperammonemic crises in patients with urea cycle disorders.

METHODS: The relationships between fasting ammonia, daily ammonia exposure, and hyperammonemic crises were analyzed in >100 patients with urea cycle disorders.

RESULTS: Fasting ammonia correlated strongly with daily ammonia exposure (r = 0.764; P < 0.001). For patients with fasting ammonia concentrations200% (P < 0.0001), respectively. The relationship between ammonia and hyperammonemic crisis risk seemed to be independent of treatment, age, urea cycle disorder subtype, dietary protein intake, or blood urea nitrogen. Fasting glutamine correlated weakly with daily ammonia exposure assessed as 24-hour area under the curve and was not a significant predictor of hyperammonemic crisis.

CONCLUSION: Fasting ammonia correlates strongly and positively with daily ammonia exposure and with the risk and rate of hyperammonemic crises, suggesting that patients with urea cycle disorder may benefit from tight ammonia control.

First Page

561

Last Page

568

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