Glutamine and hyperammonemic crises in patients with urea cycle disorders
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- 作者：B. Leea ; blee@bcm.edu" class="auth_mail" title="E-mail the corresponding author ; G.A. Diazb ; W. Rheadc ; U. Lichter-Koneckid ; A. Feigenbaume ; S.A. Berryf ; C. Le Monsg ; J. Bartleyh ; N. Longoi ; S.C. Nagamania ; W. Berquistj ; R.C. Gallagherk ; C.O. Hardingl ; S.E. McCandlessm ; W. Smithn ; A. Schulzeo ; M. Marinol ; R. Rowellp ; D.F. Coakleyq ; M. Mokhtaraniq ; B.F. Scharschmidtq
- 关键词：Glycerol phenylbutyrate ; RAVICTI ; Ammonia ; Metabolic disorders
- 刊名：Molecular Genetics and Metabolism
- 出版年：2016
- 出版时间：January 2016
- 年：2016
- 卷：117
- 期：1
- 页码：27-32
- 全文大小：601 K
文摘
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Glutamine is a weaker predictor of HA crises in UCD patients than is ammonia.
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Blood glutamine varies several-fold less over 24-h than does ammonia.
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Morning ammonia after an overnight fast is a strong predictor of HA crises.