Plasma glutamine and ammonia concentrations in ornithine carbamoyltransferase deficiency and citrullinaemia
Plasma glutamine and ammonia concentrations in ornithine carbamoyltransferase deficiency and citrullinaemia
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DOI：
10.1023/a:1012995701589
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发表时间：
2001-11-01
影响因子：
4.2
通讯作者：
Leonard, JV
中科院分区：
文献类型：
--
作者：
Wilson, CJ;Lee, PJ;Leonard, JV
本文对 7 名鸟氨酸氨基甲酰转移酶 (OCT) 缺乏症患者和 3 名瓜氨酸血症患者的长期治疗期间血浆氨和谷氨酰胺浓度进行了回顾性研究。氨和谷氨酰胺的血浆浓度之间的关系不是简单的线性关系，两种疾病之间存在显着差异。与 OCT 缺乏的患者相比，对于给定的血浆谷氨酰胺浓度，瓜氨酸血症患者的血浆氨浓度往往较高。讨论了这些差异的可能原因以及对管理的影响。
A retrospective review of the plasma ammonia and glutamine concentrations during the long-term management of 7 patients with ornithine carbamoyltransferase (OCT) deficiency and three with citrullinaemia is presented. The relationship between the plasma concentrations of ammonia and glutamine is not a simple linear one and there are significant differences between the two disorders. Patients with citrullinaemia tend to have higher plasma ammonia concentrations for a given plasma glutamine concentration compared to those with OCT deficiency. The possible reasons for these differences and the implications for management are discussed.