目的　了解儿童急性肝衰竭（ALF）的病因及生化指标特点。方法　回顾性分析2011年1月至2014年12月收治的ALF患儿的病因及生化指标结果。结果 共67例患儿入选，男36例、女31例。根据病因分为非遗传代谢病组29例（43.28%），包括药物性肝损伤12例，瑞氏综合征5例，噬血细胞综合征3例，单纯疱疹病毒感染3例，自身免疫性肝炎2例，毒蕈中毒1例，甲型肝炎病毒感染1例，巨细胞病毒感染1例，脓毒症1例；遗传代谢病组14例（20.90%），包括肝豆状核变性6例，肝糖原累积症2例，进行性家族性肝内胆汁淤积症2例，citrin缺陷症2例，极长链酰基辅酶A脱氢酶缺乏症1例，原发性肉碱缺乏症1例；病因不明组24例（35.82%）。遗传代谢病组与非遗传代谢病组及病因不明组比较，血清谷丙转氨酶(ALT)、谷草转氨酶(AST)、白蛋白、血糖水平以及AST/ALT差异有统计学意义（P均<0.05），其中遗传代谢病组的血清(ALT)、AST、白蛋白水平最低，而AST/ALT最高。结论　儿童 ALF病因复杂，如ALT升高不显著，AST/ALT比值显著升高，并存在低白蛋白血症、低血糖时，需警惕遗传代谢疾病所致。

Objective　To explore the etiology and biochemical markers of acute liver failure (ALF) in children. Methods
　 The cause and the biochemical markers of ALF in children who were treated in December 2014 to January 2011 were analyzed
retrospectively. Results　A total of 67 children were enrolled, including 31 females and 36 males. According to the cause
of the disease, the children were divided into non-genetic metabolic group, genetic metabolic group, and cryptogenic group. In the non-genetic metabolic group (29 cases, 43.28%) there were 12 cases of drug-induced ALF, 5 cases of Reye syndrome, 3 cases of hemophagocytic syndrome, 3 cases of herpes simplex virus infection, 2 cases of autoimmune hepatitis, one of case mushroom poisoning one case of hepatitis A virus infection, one case of cytomegalovirus infection and one case of sepsis respectively. In the genetic metabolic group (14 cases, 20.90%) there were 6 cases of Wilson’s disease, 2 case of glycogen storage disease, 2 of cases progressive familial intrahepatic cholestasis, 2 cases of neonatal intrahepatic cholestasis caused by citrin deficiency, one case of very long-chain acyl coenzyme A dehydrogenase deficiency and one case of primary carnitine deficiency. In the cryptogenic group there were 24 cases (35.82%). The serum alanine aminotransferase (ALT), aspartate aminotransferase (AST), albumin, blood glucose level and AST/ALT were statistically significantly different in genetic metabolic group from in non-genetic metabolism disease group and cryptogenic group, (P<0.05). The genetic metabolic group had the lowest levels of serum ALT, AST, albumin and glucose while the genetic metabolic group had the highest ratio of AST/ALT. Conclusions　The etiology of ALF in children are complex. Genetic metabolic disease should be considered when the child with ALF has no significantly elevated ALT, extremely high ratio of AST/ALT, combined with hypoproteinemia and hypoglycemia.