儿童肝病原因分析

Analysis of the causes of liver disease in children under 14 years old

目的研究14岁以下患儿肝病的发生率及病因。方法 2016年1月至2019年12月南京市第二医院门诊及住院肝病患儿676例,按年龄分为六个年龄段,分析不同年龄段的肝病发生率和病因。结果 676例患儿,男性554例占81.95%,女性122例占18.05%,以男性发病率高;3~6岁年龄组肝病发生例数最多199例,占27.96%。患儿主要病种分布:慢性乙型肝炎407例,占60.21%;非酒精性脂肪肝74例,占10.95%;感染累及肝功能异常36例,占5.32%;药物性肝炎19例,占2.81%;CMV肝炎18例,占2.66%;慢性丙型肝炎11例,占1.63%;甲肝8例,占1.18%;EBV肝炎6例,占0.88%;急性乙型肝炎3例,占0.44%;肝母细胞瘤1例,占0.15%。经过常规血清学检查及影像学检查后,仍未明确肝病病因的患儿93例,占13.76%。66例做肝穿病理或(和)基因检测后,Gilbert综合征21例,占22.58%;肝豆状核变性11例,占11.83%;进行性肌营养不良9例,占9.68%;自身免疫性肝炎6例,占6.45%;糖原累积症4例,占4.30%;DubinJohnson 3例,占3.23%;葡萄糖-6-磷酸脱氢酶缺陷症、进行性家族胆汁淤积、Alagille综合征各2例,各占2.15%;重叠综合征、硬化性胆管炎、Rotor综合征、遗传性球形红细胞增多症各1例,各占1.075%。仍有2例未能明确病因。结论 14岁以下肝病患儿病因较多,以慢性乙型肝炎为主;不明原因肝功能异常患儿中,遗传代谢性异常导致肝病是主要原因。

Objective To study the incidence and etiology of liver disease in children under 14 years old.Methods From January 2016 to December 2019,676 outpatients and inpatients with liver disease in our hospital were collected and divided into six groups according to their ages.The incidence of liver disease in different age groups was analyzed,and the causes of 676 patients were analyzed.Results Among 676 cases,554 cases were male(81.95%),122 were female(18.05%),and the incidence rate was high in male.199 cases were diagnosed as liver diseases in 3~6 years old group(27.96%),and the Uvalue was 3.18(P<0.01).There were 407 cases of chronic hepatitis B(60.21%),74 cases of nonalcoholic fatty liver(10.95%),36 cases of abnormal liver function(5.32%).There were 19 cases with drug-induced hepatitis(2.81%),18 with CMV hepatitis(2.66%),11 with chronic hepatitis C(1.63%),8 with hepatitis A(1.18%),6 with EBV hepatitis(0.88%),3 with acute hepatitis B(0.44%)and 1 with hepatoblastoma(0.15%).After routine serological examination and imaging examination,93 cases(13.76%)were still unclear about the cause of liver disease.After liver biopsy or/and related gene detection in66 cases,there were 21 cases of Gilbert’s syndrome(22.58%),11 cases of hepatolenticular degeneration(11.83%),9 cases of progressive muscular dystrophy(9.68%),6 cases of autoimmune hepatitis(6.45%),4 cases of glycogen accumulation(4.30%)and 3 cases of Dubin Johnson(6.45%)There were 2 cases of glucose-6-phosphate dehydrogenase deficiency,2 cases of progressive familial cholestasis and 2 cases of Alagille syndrome,accounting for 2.15% respectively;there were 1 case of overlap syndrome,1 case of sclerosing cholangitis,1 case of rotor syndrome and 1 case of hereditary spherocytosis,accounting for 1.075%respectively.There were still 2 cases of unknown etiology.Conclusion There were many causes of liver disease in children under 14 years old,mainly chronic hepatitis B;and children with unexplained abnormal liver function;genetic metabolic abnormalities leading to liver disease was the main reason.