CAJ | 학술논문

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。
목적：료해인동급성간쇠갈（ALF）적병인급생화지표특점。방법회고성분석2011년1월지2014년12월수치적ALF환인적병인급생화지표결과。결과공67례환인입선，남36례、녀31례。근거병인분위비유전대사병조29례（43.28%），포괄약물성간손상12례，서씨종합정5례，서혈세포종합정3례，단순포진병독감염3례，자신면역성간염2례，독심중독1례，갑형간염병독감염1례，거세포병독감염1례，농독증1례；유전대사병조14례（20.90%），포괄간두상핵변성6례，간당원루적증2례，진행성가족성간내담즙어적증2례，citrin결함증2례，겁장련선기보매A탈경매결핍증1례，원발성육감결핍증1례；병인불명조24례（35.82%）。유전대사병조여비유전대사병조급병인불명조비교，혈청곡병전안매(ALT)、곡초전안매(AST)、백단백、혈당수평이급AST/ALT차이유통계학의의（P균<0.05），기중유전대사병조적혈청(ALT)、AST、백단백수평최저，이AST/ALT최고。결론인동 ALF병인복잡，여ALT승고불현저，AST/ALT비치현저승고，병존재저백단백혈증、저혈당시，수경척유전대사질병소치。
ObjectiveTo 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 ana-lyzed retrospectively.ResultsA 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 cas-es progressive familial intrahepatic cholestasis, 2 cases of neonatal intrahepatic cholestasis caused by citrin deifciency, one case of very long-chain acyl coenzyme A dehydrogenase deifciency and one case of primary carnitine deifciency. 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 signiifcantly different in genetic metabolic group from in non-genetic me-tabolism 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.ConclusionsThe etiology of ALF in children are complex. Genetic metabolic disease should be considered when the child with ALF has no signiifcantly elevated ALT, extremely high ratio of AST/ALT, combined with hypoproteinemia and hypoglycemia.