摘要
目的了解自身免疫性肝炎(AIH)患儿临床特点与预后。方法回顾性分析2016年8月至2019年8月于湖北省襄阳市中心医院收治的138例AIH患儿的临床资料,分析临床特征及治疗、预后。结果138例患儿中AIH-1型96例(69.57%),AIH-2型42例(30.43%)。发热6例(4.35%),恶心、乏力12例(8.70%),转氨酶异常39例(28.26%),黄疸81例(58.70%)。AIH-1型患儿合并炎症性肠病、原发性硬化性胆管炎、系统性红斑狼疮、原发性胆汁性胆管炎分别为6例(4.35%)、9例(6.52%)、3例(2.17%)、3例(2.17%)。入院时所有患儿均有肝功能异常,AIH-1型者γ球蛋白、免疫球蛋白G显著高于AIH-2型者(P<0.05)。117例行肝活组织检查,提示界面性肝炎108例(92.31%),玫瑰花结9例(7.69%),浆细胞或淋巴细胞浸润69例(58.97%);肝脏炎症分级G≥3级66例(56.41%),纤维化分期S≥3级78例(66.67%)。120例行糖皮质激素规范治疗,完全缓解87例(72.50%),部分缓解30例(25.00%),无应答3例(2.50%)。完全缓解后激素减量期45例(51.72%)复发。结论儿童AIH多见于AIH-1型,临床表现复杂,发病时多数患儿肝脏炎症及纤维化已明显进展,激素单用或联合硫唑嘌呤治疗后可改善患儿生化指标及病理,但易复发。
Objective To study the clinical characteristics and prognosis of children with autoimmune hepatitis(AIH).Methods Clinical data of 138 children with AIH admitted to our hospital from August 2016 to August 2019 were retrospectively analyzed to analyze clinical features,treatment and prognosis.Results Among the 138 cases,96 cases(69.57%)had AIH-1 type and 42 cases(30.43%)had AIH-2 type;Fever in 6 cases(4.35%),nausea and fatigue in 12 cases(8.70%),abnormal transaminase in 39 cases(28.26%),and jaundice in 81 cases(58.70%);Children with AIH-1 complicated with inflammatory bowel disease,primary sclerosing cholangitis,systemic lupus erythematosus and primary biliary cholangitis were 6 cases(4.35%),9 cases(6.52%),3 cases(2.17%)and 3 cases(2.17%),respectively.At admission,all children had abnormal liver function,and the levels of gamma globulin and immunoglobulin G in AIH-1 group were significantly higher than those in AIH-2 group(P<0.05).117 cases of liver tissue examination showed that 108 cases of interfacial hepatitis(92.31%),9 cases of rosette(7.69%),69 cases of plasma cell or lymphocyte infiltration(58.97%);There were 66 cases(56.41%)with liver inflammation grade G≥grade 3,and 78 cases(66.67%)with fibrosis stage S≥grade 3.120 cases were treated with glucocorticoid,87 cases(72.50%)were completely relieved,30 cases(25.00%)were partially relieved and 3 cases(2.50%)were unresponsive.45 cases(51.72%)relapsed after complete remission.Conclusion AIH-1 type is more common in children AIH.Its clinical manifestations are complex.Liver inflammation and fibrosis in most children have progressed significantly.Hormone alone or combined with azathioprine can improve the biochemistry and pathology of children,but it is easy to recur.
作者
赵梦
陈明
王丽
胡孙裔
ZHAO Meng;CHEN Ming;WANG Li;HU Sun-yi(Department of Pediatrics,Xiangyang Central Hospital,affiliated Hospital of Hubei University of Arts and Science,Xiangyang,441021;Department of Pediatrics,Hubei provincial maternal and child health hospital,Tongji Medical College,Huazhong University of Science and Technology,Wuhan 430070)
出处
《肝脏》
2020年第4期379-381,386,共4页
Chinese Hepatology
基金
湖北省自然科学基金项目(2015CFB660)。
关键词
儿童
自身免疫性肝炎
临床特点
预后
Children
Autoimmune hepatitis
Clinical characteristics
Prognosis
