Abstract: Objective　To explore the etiology and clinical features of pediatric cirrhosis，to understand its prognosis，and to improve the levels of diagnosis and treatment of it.Methods　The clinical data of 36 cases of childhood cirrhosis treated in Tongji Hospital，Tongji Medical College，Huazhong University of Science and Technology from October 2016 to December 2020 were studied.The clinical data included，gender，age，past history，etiology，clinical manifestations，complications，nutritional status at diagnosis，laboratory test results，genetic results，post-treatment follow-up.Compare the related indicators between the good prognosis group and the death group.Results　Among the 36 patients with cirrhosis，18 were male and 18 were female with，the age was 51.7（5，84）months；13 cases had biliary atresia，6 were Wilson diseases，Carolis disease had 2 cases，progressive familial intrahepatic cholestasis（PFIC）2 cases；there were 7 patients with cryptogenic cirrhosis，one hereditary hemorrhagic telangiectasia（HHT），one case of Niemann-Pick disease，one case of congenital bile acid synthesis defect，case of hepatitis B cirrhosis，case of neonatal intrahepatic cholestasis（NICCD），and one case of Shwachman-Diamond syndrome（SDS）.The main clinical features were splenomegaly in 18 cases（50%），skin jaundice in 16 cases（44.4%），fever in 10 cases（27.7%），abdominal distension in 10 cases（27.7%），gastrointestinal bleeding in 6 cases（16.6%）and vomiting in 4 cases（11.1%），etc.At the time of diagnosis of cirrhosis，the number of children with underweight and growth retardation was 8（22.2%）and 12（33.3%）respectively.Ultrasonography was performed in all 36 cases to diagnose cirrhosis，abdominal CT examination showed cirrhosis in 14 cases，and 15 patients were diagnosed through histopathology.The main complications included esophagogastric fundus varicose veins in 9 cases（26.4%），hypersplenia and/or cytopenia in 8 cases（23.5%），and ascites in 6 cases（16.6%），etc.Genetic tests were performed in 10 children，and pathogenic genes were detected in 6 cases，including NPC1，ABCB4，SADM4，ATP7B，SBDS and SLC25A13.All the children were treated according to etiology，and the symptomatic supportive treatment such as liver protection and antixanthosis was added.Among them，27（75%）were in good general condition，7（19.4%）died，8 had been treated with liver transplantation，and 2 were currently awaiting liver transplantation.There was no significant difference in alanine aminotransferase（ALT），aspartate aminotransferase（AST），γ -glutamyl transpeptidase（GGT），prothrombin time（PT），platelet or total bilirubin（TBIL）between the good prognosis group and the death group（P＞0.05）.There was statistically significant difference in age，albumin（ALB）and direct bilirubin（DBIL）at the time of diagnosis（P＜0.05）.Conclusion　The etiology of cirrhosis in children is very different from that in adults. biliary atresia is the most common etiology；B-ultrasonography is the most common examination method for its diagnosis；malnutrition is easy to occur，so it is suggested to strengthen nutritional supplements；pediatric cirrhosis with younger age，lower albumin and higher direct bilirubin has poor prognosis and high mortality.

Key words: pediatric cirrhosis, etiology, B-ultrasound, malnutrition

摘要： 目的　探讨儿童肝硬化的病因及临床特征，了解其预后，提高对其认识和诊治水平。方法　回顾性分析华中科技大学同济医学院附属同济医院儿童消化专科2016年10月至2020年12月收治的36例儿童肝硬化患儿的临床资料，包括性别、年龄、既往史、病因、临床表现、并发症、确诊时营养状况、实验室检查结果、基因结果和治疗后随访情况等，比较预后良好组与死亡组患儿的相关指标差异。结果　36例肝硬化患儿，男18例，女18例，年龄为51.7（5，84）月，其中胆道闭锁13例，肝豆状核变性6例，先天性肝内胆管扩张症（Caroli病）2例，进行性家族性肝内胆汁淤积症（PFIC）2 例，遗传性出血性毛细血管扩张症（HHT）1例，尼曼-匹克病1例，先天性胆汁酸合成障碍 1例，新生儿肝内胆内淤积症（NICCD）1例、SDS综合征1例，乙肝肝硬化1例，隐源性肝硬化7例。临床特征主要为脾大18例（50%）、皮肤黄染16例（44.4%）、发热10例（27.7%）、腹胀10例（27.7%）、消化道出血6例（16.6%）、呕吐4例（11.1%）等。确诊肝硬化时8例（22.2%）患儿存在体重低下，12例（33.3%）患儿存在生长迟缓。36例全部行肝胆B超检查诊断肝硬化，其中14例腹部CT检查诊断肝硬化，15例组织病理诊断肝硬化。并发症主要包括食管胃底静脉曲张9例（26.4%），脾功能亢进和（或）血细胞减少8例（23.5%），腹腔积液6例（16.6%）等。10例完善基因相关检查，6例检测出致病基因，分别为NPC1、ABCB4、SADM4、ATP7B、SBDS和SLC25A13；所有患儿优先根据病因治疗，在此基础上加用护肝和退黄药等对症支持治疗，其中27（75%）例患儿一般情况良好，7（19.4%）例死亡，8例患儿已行肝移植治疗，2例患儿目前等待肝移植。预后良好组与死亡组两组比较，在丙氨酸转氨酶（ALT）、天冬氨酸转氨酶（AST）、γ-谷氨酰转肽酶（GGT）、凝血酶原时间（PT）、血小板、总胆红素（TBIL）方面差异无统计学意义（P均＞0.05）；在患儿确诊时年龄、白蛋白（ALB）、直接胆红素（DBIL）方面差异有统计学意义（P＜0.05）。结论　儿童肝硬化的病因与成人大不相同，胆道闭锁是其最常见的病因；B超是诊断其最常用的检查方法；肝硬化患儿易出现营养不良，建议加强营养补充；患儿起病年龄越小，伴白蛋白水平越低、直接胆红素水平越高时其预后差、病死率高。

关键词: 儿童肝硬化, 病因, B超, 营养不良