摘要
目的分析儿童自身免疫性肝炎(AIH)的临床特点及治疗转归。方法回顾性分析2008年12月-2017年12月在首都医科大学附属北京佑安医院住院治疗的10例儿童AIH患者的临床、肝脏病理、治疗转归及随访资料。结果患儿年龄3~16岁,中位年龄10岁,女性占70%。1型5例,2型5例。3例(30%)急性起病,2例(20%)亚急性起病,5例(50%)慢性起病。转氨酶升高8例(80%),胆红素升高7例(70%); GGT升高7例(70%); IgG升高6例(60%)。7例接受肝脏病理检查的患儿中炎症程度≥G3者4例,纤维化分期≥S3者4例,界面性炎症4例,浆细胞浸润5例,玫瑰花环4例;淋巴细胞浸润7例。1例患者死亡,1例失访,有效6例,复发1例,疗效不佳1例。5例2型AIH患者中,4例采用糖皮质激素联合硫唑嘌呤治疗,病情得到有效控制。结论儿童AIH临床表现复杂,个别患者病情重。2型患者多需要糖皮质激素联合硫唑嘌呤治疗。
Objective To investigate the clinical features and treatment outcome of children with autoimmune hepatitis（ AIH）. Methods A retrospective analysis was performed for the clinical data,liver pathology,treatment outcome,and follow-up data of 10 children with AIH who were treated in Beijing YouAn Hospital,Capital Medical University,from December 2008 to December 2017. Results The children were aged 3-16 years（ median 10 years）,and girls accounted for 70%. Of all patients,5 had type 1 AIH,and 5 had type 2 AIH; 3（ 30%） had an acute onset,2（ 20%） had a subacute onset,and 5（ 50%） had a chronic onset. Elevation of aminotransferases was found in 8 children（ 80%）,and elevations of bilirubin,gamma-glutamyl transpeptidase,and immunoglobulin G were found in 7 children（ 70%）,7 children（ 70%）,and 6 children（ 60%）,respectively. Among the 7 children who underwent liver biopsy,3 had grade ≥3 liver inflammation,4 had stage ≥3 liver fibrosis,5 had interface hepatitis,5 had plasma cell infiltration,4 had rosette-like annulation of hepatocytes,and 7 had lymphocyte infiltration. One child died,and one was lost to follow-up; among the other 8 children,6 had good response,1 experienced recurrence,and one had poor response. 4 patients with type 2 AIH were treated with glucocorticoids combined with azathioprine,the disease was effectively controlled. Conclusion Children with AIH have diverse clinical manifestations,and some children have serious conditions. Most patients with type 2 AIH need the treatment of glucocorticoids combined with azathioprine.
作者
陈杰
刘燕敏
黄春洋
黄云丽
张小丹
任美欣
杜晓菲
边新渠
廖慧钰
韩莹
单晶
CHEN Jie, LIU Yanmin, HUANG Chunyang, et al.(Department of Hepatology and hnmunology, Beijing YouAn Hospital, Capital Medical University, Beijing 100069, China)
出处
《临床肝胆病杂志》
CAS
北大核心
2018年第10期2164-2168,共5页
Journal of Clinical Hepatology
关键词
肝炎
自身免疫性
疾病特征
儿童
hepatitis
autoimmune
disease attributes
child
