Barshop B A, Breuer J, Holm J, Leslie J, Nyhan W L
Department of Pediatrics, University of California San Diego, La Jolla 92093.
J Inherit Metab Dis. 1989;12(1):72-9. doi: 10.1007/BF01805533.
In patients with non-ketotic hyperglycinaemia and two patients with urea cycle disorders treated with varying doses of sodium benzoate there was linear correlation between intake of benzoate and excretion of hippurate. Patients with non-ketotic hyperglycinaemia excreted significantly more benzoate in the form of hippurate than patients with urea cycle disorders (74 +/- 7.0 vs 41 +/- 3.6%). The plasma concentration of glycine decreased following benzoate treatment only in the patients with non-ketotic hyperglycinaemia. The observed difference between the two groups in the excretion of hippurate seems to support the concept that glycine availability may be limiting in benzoate therapy for some patients.
在患有非酮症高甘氨酸血症的患者以及两名接受不同剂量苯甲酸钠治疗的尿素循环障碍患者中，苯甲酸盐摄入量与马尿酸盐排泄之间存在线性相关性。非酮症高甘氨酸血症患者以马尿酸盐形式排泄的苯甲酸盐显著多于尿素循环障碍患者（分别为74±7.0%和41±3.6%）。仅在非酮症高甘氨酸血症患者中，苯甲酸盐治疗后血浆甘氨酸浓度降低。两组在马尿酸盐排泄方面观察到的差异似乎支持这样一种观点，即对于某些患者，在苯甲酸盐治疗中甘氨酸的可利用性可能是有限的。
