Abstract: The acute presentation of urea cycle disorders is characterized by hyperammonemia，with high disability and mortality risks. Patients with urea cycle disorders at acute stage should be identified and diagnosed as early as possible，and the quick control ofthe acute attack would make a great significance in improving the survival and prognosis of the patients with urea cycle disorders.

Key words: urea cycle disorders, acute hyperammonemia, diagnosis, treatment

摘要： 尿素循环障碍急性期以高氨血症发作为主要特征，具有高致残、高病死率风险。应尽早识别和诊断尿素循环障碍急性期患儿，快速控制急性期发作对提高尿素循环障碍患儿生存率和改善疾病预后具有重要意义。

关键词: 尿素循环障碍, 急性高氨血症, 诊断, 治疗