Maeda E, Matsuo M, Saiki K, Nakamura H, Matsuo T, Takemine H
Acta Paediatr Jpn. 1989 Dec;31(6):721-6. doi: 10.1111/j.1442-200x.1989.tb01386.x.
Two unusual peaks were found on gas chromatography of urine from four hyperammonemic patients treated with sodium benzoate. These peaks were identified by gas chromatography/mass spectrometric analyses as methylsuccinate and mesaconate. Of the two unusual substances, methylsuccinate was found to be a contaminant of sodium benzoate administered for the treatment of hyperammonemia. However, mesaconate was not a contaminant of sodium benzoate, though it could be detected in all urine samples from hyperammonemic patients treated with sodium benzoate. Mesaconate can be produced from methylsuccinate in vivo. Considering that mesaconate is an inhibitor of fumarase, the toxic effects of sodium benzoate may be attributable to the mesaconate. It is recommended that methylsuccinate-free sodium benzoate should be used for the treatment of hyperammonemia.
在对四名接受苯甲酸钠治疗的高氨血症患者的尿液进行气相色谱分析时发现了两个异常峰。通过气相色谱/质谱分析，这些峰被鉴定为甲基琥珀酸和中康酸。在这两种异常物质中，甲基琥珀酸被发现是用于治疗高氨血症的苯甲酸钠的污染物。然而，中康酸并非苯甲酸钠的污染物，尽管在用苯甲酸钠治疗的高氨血症患者的所有尿液样本中都能检测到它。中康酸可在体内由甲基琥珀酸产生。考虑到中康酸是富马酸酶的抑制剂，苯甲酸钠的毒性作用可能归因于中康酸。建议使用不含甲基琥珀酸的苯甲酸钠来治疗高氨血症。
