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Blood ammonia and glutamine as predictors of hyperammonemic crises in patients with urea cycle disorder. 2015
Brendan Lee, and
George A Diaz, and
William Rhead, and
Uta Lichter-Konecki, and
Annette Feigenbaum, and
Susan A Berry, and
Cindy Le Mons, and
James A Bartley, and
Nicola Longo, and
Sandesh C Nagamani, and
William Berquist, and
Renata Gallagher, and
Dennis Bartholomew, and
Cary O Harding, and
Mark S Korson, and
Shawn E McCandless, and
Wendy Smith, and
Stephen Cederbaum, and
Derek Wong, and
J Lawrence Merritt, and
Andreas Schulze, and
Jerry Vockley, and
Gerard Vockley, and
David Kronn, and
Roberto Zori, and
Marshall Summar, and
Douglas A Milikien, and
Miguel Marino, and
Dion F Coakley, and
Masoud Mokhtarani, and
, and
Bruce F Scharschmidt
1] Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas, USA [2] Howard Hughes Medical Institute, Chevy Chase, Maryland, USA.
OBJECTIVE 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 concentrations <0.5 upper limit of normal (ULN), 0.5 to <1.0 ULN, and ≥1.0 ULN, the probability of a normal average daily ammonia value was 87, 60, and 39%, respectively, and 10.3, 14.1, and 37.0% of these patients, respectively, experienced ≥1 hyperammonemic crisis over 12 months. Time to first hyperammonemic crisis was shorter (P = 0.008) and relative risk (4.5×; P = 0.011) and rate (~5×, P = 0.006) of hyperammonemic crises were higher in patients with fasting ammonia ≥1.0 ULN vs. <0.5ULN; relative risk was even greater (20×; P = 0.009) in patients ≥6 years old. A 10- or 25-µmol/l increase in ammonia exposure increased the relative risk of a hyperammonemic crisis by 50 and >200% (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. CONCLUSIONS 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.
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