目的 探讨自身免疫性肝炎（AIH）的早期诊断、规范治疗。方法 回顾分析2例AIH患儿的临床诊治过程， 并结合相关文献进行分析。结果 2例均是男性，分别为6岁1个月、 6个月18 天，均以黄疸为首发症状，伴转氨酶升高、 自身抗体阳性；其中例1血清IgG升高，肝脏病理表现为界面性肝炎，均排除其他病因，对激素联合硫唑嘌呤应答良好； 例2患儿单用激素效果欠佳。结论 儿童AIH临床少见，对糖皮质激素及免疫抑制剂治疗应答良好，应早期诊断、及时治 疗。

Objective To explore the early diagnosis and standard treatment of autoimmune hepatitis (AIH). Methods  The process of clinical diagnosis and treatment in 2 children with AIH was retrospectively analyzed. The related literatures were reviewed. Results Two males were 6 years and 1 month old and 6 monthes and 18 days old, respectively. Both of them had the symptom of jaundice at onset, accompanied with the elevate aminotransferase and positive autoantibodies. One case showed elevated serum IgG and interface hepatitis in liver pathology, and all other causes were excluded. He had a good response to the treatment of hormone combined with azathioprine. The other case had a poor response to the treatment of single hormone. Conclusions Childhood AIH is rare in clinic. Its response to the therapy of glucocorticoid and immune inhibitors is good. So it should be diagnosised and treated early.