Takeda E, Kuroda Y, Toshima K, Watanabe T, Naito E, Miyao M
Clin Pediatr (Phila). 1983 Mar;22(3):206-8. doi: 10.1177/000992288302200309.
An 8-year-old girl with partial ornithine carbamoyl transferase deficiency was treated with sodium benzoate (200 mg/kg/day) for 13 months. Before administration of sodium benzoate, her protein intake was reduced to 1.0 to 1.5 g/kg/day and her caloric intake fluctuated. Hyperammonemic attacks were frequently observed in winter. After the start of administration of sodium benzoate, the severity and frequency of these attacks decreased, although her protein intake was increased to 1.5 to 2.0 g/kg/day. No adverse effect of sodium benzoate were detected by clinical and laboratory examinations. It is concluded that long-term oral administration of sodium benzoate was effective in reducing the frequency and severity of hyperammonemic attacks in this patient. Sodium benzoate therapy in combination with dietary manipulation may improve the growth and development of these patients by allowing reduced dietary protein restriction.
一名患有部分鸟氨酸氨甲酰基转移酶缺乏症的8岁女孩接受了苯甲酸钠（200毫克/千克/天）治疗，为期13个月。在给予苯甲酸钠之前，她的蛋白质摄入量降至1.0至1.5克/千克/天，热量摄入波动。冬季经常观察到高氨血症发作。开始给予苯甲酸钠后，尽管她的蛋白质摄入量增加到1.5至2.0克/千克/天，但这些发作的严重程度和频率有所降低。临床和实验室检查未发现苯甲酸钠的不良反应。结论是，长期口服苯甲酸钠对减少该患者高氨血症发作的频率和严重程度有效。苯甲酸钠治疗与饮食调整相结合，通过减少饮食蛋白质限制，可能改善这些患者的生长发育。
