Abstract: Congenital urea cycle disorders are severe inherited metabolic diseases caused by the enzyme deficiency with low morbidity and high mortality. Due to different pathogenic causes and personal conditions，the patients present with different clinical manifestations. About half of the patients with urea cycle disorders have hyperammonemia，which results in acute or chronic brain and liver damage. Individualized diet，ammonia lowering drugs or liver transplantation should be considered for the treatment. The main treatment principle of urea cycle disorders is to lower blood ammonia by reducing ammonia generation and promoting ammonia excretion. As the core drugs of urea cycle disorders，nitrogen scavengers consume the excess ammonia in the body by combining with glycine or glutacrylamide. The pharmacological action，pharmacokinetic characteristics and clinical study of nitrogen scavenger and other ammonia lowering drugs were reviewed in this paper in order to provide reference for rational clinical use of nitrogen scavenger.

Key words: nitrogen scavenger, urea cycle disorders, hyperammonemia, sodium benzoate, sodium butyrate

摘要： 先天性尿素循环障碍是一类严重的遗传代谢病，由于尿素循环中的酶缺陷所致，发病率低，致残率及致死率高。由于病因及个体生活状态不同，患者临床表现各异，半数患者发生高氨血症，造成急性或慢性脑损伤及肝损害，需要个体化饮食、药物治疗及肝移植治疗。尿素循环障碍的主要治疗原则是减少体内氨生成，促进氨排泄，降低血氨，尽可能防治高氨血症。氮清除剂作为尿素循环障碍治疗的核心药物，通过与甘氨酸或谷氨酰胺结合，消耗体内多余的氨。文章对氮清除剂等降氨药物的药理作用、药代动力学特征及临床应用等方面进行阐述，以期为临床合理用药提供参考。

关键词: 氮清除剂, 尿素循环障碍, 高氨血症, 苯甲酸钠, 苯丁酸钠