Maestri N E, McGowan K D, Brusilow S W
Department of Pediatrics, Johns Hopkins School of Medicine, Baltimore, Maryland.
J Pediatr. 1992 Aug;121(2):259-61. doi: 10.1016/s0022-3476(05)81200-4.
Because increases in plasma glutamine concentrations are almost always associated with hyperammonemia in patients with urea cycle disorders, we determined the correlation between these two variables for 2 years in a child with ornithine transcarbamylase deficiency. A correlation coefficient of 0.77 (p less than 0.0001) was found. Hyperammonemia was rarely observed when plasma glutamine levels were near normal. These data suggest that one goal of therapy is the maintenance of plasma glutamine levels at or near normal values.
由于尿素循环障碍患者血浆谷氨酰胺浓度升高几乎总是与高氨血症相关，我们对一名鸟氨酸转氨甲酰酶缺乏症患儿进行了为期2年的研究，以确定这两个变量之间的相关性。结果发现相关系数为0.77（p小于0.0001）。当血浆谷氨酰胺水平接近正常时，很少观察到高氨血症。这些数据表明，治疗的一个目标是将血浆谷氨酰胺水平维持在正常或接近正常的值。
