Abstract: Acute metabolic crises in the patients with organic acidemias are critical conditions of inherited metabolic disorders，where the severity of hyperammonemia directly associates with the prognosis. Except for restricting protein intake to reduce ammonia generation，maintaining adequate energy supply is important to prevent exacerbation of hyperammonemia due to increased breakdown of endogenous proteins. Correction of the underlying metabolic disturbances is also necessary. Carglumic acid，a synthetic analogue of N-acetylglutamate，can increase substrates for the urea cycle，restarting the urea cycle and accelerating ammonia metabolism. This article summarizes the application of carglumic acid in the management of acute metabolic crises due to organic acidemia，providing insights for clinical treatment.

Key words: carglumic acid, organic acidemia, metabolic crisis, hyperammonemia

摘要： 有机酸代谢病急性代谢危象是遗传代谢病中的危急重症，其伴发的高氨血症的严重程度直接决定了患者的预后。治疗中不仅需要限制蛋白质，降低血氨生成，同时需要保证能量供应，以防止自身蛋白分解加重高氨血症，才能纠正代谢紊乱。卡谷氨酸是人工合成的N-乙酰谷氨酸类似物，可以增加尿素循环底物，改善肝脏尿素循环，加速血氨代谢。文章总结卡谷氨酸在防治有机酸代谢病急性代谢危象中的应用，为临床治疗提供参考。

关键词: 卡谷氨酸, 有机酸代谢病, 代谢危象, 高氨血症