Blood ammonia and glutamine as predictors of hyperammonemic crises in patients with urea cycle disorder.
Blood ammonia and glutamine as predictors of hyperammonemic crises in patients with urea cycle disorder.
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DOI：
10.1038/gim.2014.148
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发表时间：
2015-07
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影响因子：
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中科院分区：
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探讨尿素循环障碍（ucd）患者氨暴露和高氨血症危象（HAC）的预测因素。分析了100例UCD患者空腹氨、每日氨暴露与HACs的关系。空腹氨与每日氨暴露密切相关（r=0.764, p<0.001）。空腹氨氮水平<0.5 ULN、0.5 ~ <1.0 ULN和≥1.0 ULN的患者，平均每日氨氮值正常的概率分别为87%、60%和39%，其中10.3%、14.1%和37.0%的患者在12个月内经历≥1次HAC。空腹氨氮≥1.0 ULN的患者发生首次HAC的时间较短（p=0.008），发生HAC的相对危险度（4.5×， p=0.011）和发生率（~5×， p=0.006）高于<0.5ULN的患者；年龄≥6岁的患者相对风险更大（20×； p=0.009）。当氨暴露量增加10或25 μmol/L时，HAC的相对危险度分别增加50%和200% （p<0.0001）。氨与HAC风险之间的关系与治疗、年龄、UCD亚型、膳食蛋白质摄入量或血尿素氮无关。空腹谷氨酰胺与AUC0-24相关性较弱，并不是HACs的显著预测因子。空腹氨与每日氨暴露以及HACs的风险和发生率呈正相关，表明UCD患者可能受益于严格的氨控制。
To examine predictors of ammonia exposure and hyperammonemic crises (HAC) in patients with urea cycle disorders (UCDs). The relationships between fasting ammonia, daily ammonia exposure, and HACs were analyzed in >100 UCD patients. Fasting ammonia correlated strongly with daily ammonia exposure (r=0.764, p<0.001). For patients with fasting ammonia levels <0.5 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 experienced ≥1 HAC over 12 months. Time to first HAC was shorter (p=0.008) and relative risk (4.5×; p=0.011) and rate (~5×, p=0.006) of HACs 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. A 10 or 25 μmol/L increase in ammonia exposure increased the relative risk of a HAC by 50% and >200% (p<0.0001), respectively. The relationship between ammonia and HAC risk appeared independent of treatment, age, UCD subtype, dietary protein intake, or blood urea nitrogen. Fasting glutamine correlated weakly with AUC0-24 and was not a significant predictor of HACs. Fasting ammonia correlates strongly and positively with daily ammonia exposure and with the risk and rate of HACs, suggesting that UCD patients may benefit from tight ammonia control.
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影响因子：
3.8
作者：
Mokhtarani, M.;Diaz, G. A.;Rhead, W.;Berry, S. A.;Lichter-Konecki, U.;Feigenbaum, A.;Schulze, A.;Longo, N.;Bartley, J.;Berquist, W.;Gallagher, R.;Smith, W.;McCandless, S. E.;Harding, C.;Rockey, D. C.;Vierling, J. M.;Mantry, P.;Ghabril, M.;Brown, R. S., Jr.;Dickinson, K.;Moors, T.;Norris, C.;Coakley, D.;Milikien, D. A.;Nagamani, S. C.;LeMons, C.;Lee, B.;Scharschmidt, B. F.
通讯作者：
Scharschmidt, B. F.
影响因子：
3.8
作者：
Berry, Susan A.;Lichter-Konecki, Uta;Lee, Brendan
通讯作者：
Lee, Brendan
影响因子：
4.2
作者：
Wilson, CJ;Lee, PJ;Leonard, JV
通讯作者：
Leonard, JV
影响因子：
5.1
作者：
Smith, Wendy;Diaz, George A.;Lee, Brendan
通讯作者：
Lee, Brendan
影响因子：
3.7
作者：
KAPLAN, EL;MEIER, P
通讯作者：
MEIER, P