评估尿素循环障碍患者的血浆氨和谷氨酰胺浓度。
Assessment of plasma ammonia and glutamine concentrations in urea cycle disorders.
机构信息
Neuropediatric Gastroenterology and Clinical Biochemistry Departments, Hospital Sant Joan de Déu, Barcelona, Spain.
出版信息
Clin Biochem. 2011 Jun;44(8-9):742-4. doi: 10.1016/j.clinbiochem.2011.03.136. Epub 2011 Apr 8.
OBJECTIVES
To analyze the association between ammonia and glutamine used for metabolic control in inherited urea cycle disorders (UCD) in a large series of patients.
DESIGN AND METHODS
Paired plasma amino acid-ammonia data from 26 UCD patients were analyzed (n=921).
RESULTS
Increased plasma glutamine values were consistently observed in UCD patients, despite normal plasma ammonia concentrations, especially for mitochondrial UCD.
CONCLUSIONS
Further therapeutic efforts are probably needed to control increased glutamine values, considering their potentially neurotoxic effect.
目的
分析在大量遗传性尿素循环障碍（UCD）患者中，用于代谢控制的氨和谷氨酰胺之间的关联。
设计和方法
分析了 26 例 UCD 患者的配对血浆氨基酸-氨数据（n=921）。
结果
尽管血浆氨浓度正常，但 UCD 患者的血浆谷氨酰胺值始终升高，尤其是线粒体 UCD。
结论
鉴于其潜在的神经毒性作用，可能需要进一步的治疗努力来控制升高的谷氨酰胺值。
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