Blood ammonia and glutamine as predictors of hyperammonemic crises in patients with urea cycle disorder.
Brendan LeeGeorge A DiazWilliam RheadUta Lichter-KoneckiAnnette FeigenbaumSusan A BerryCindy Le MonsJames A BartleyNicola LongoSandesh C NagamaniWilliam BerquistRenata GallagherDennis BartholomewCary O HardingMark S KorsonShawn E McCandlessWendy E SmithStephen CederbaumDerek WongJ Lawrence MerrittAndreas SchulzeJerry VockleyGerard VockleyDavid KronnRoberto ZoriMarshall L SummarDouglas A MilikienMiguel MarinoDion F CoakleyMasoud Mokhtaraninull nullBruce F ScharschmidtPublished in: Genetics in medicine : official journal of the American College of Medical Genetics (2014)
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.