1996 ~ 2020年北京佑安医院遗传代谢性肝病住院患者流行病学特征分析  

Partial epidemiological analysis of patients with inherited metabolic liver disease admitted in Beijing You'an Hospital between 1996 and 2020

作　　者：白洁 任艳[1] 梁晨 汤珊 刘念晨 白丽 刘霜 郑素军[2] 段钟平[1] Bai Jie;Ren Yan;Liang Chen(Fourth Department of Liver Disease,You'an Hospital Affiliated to Capital Medical University,Beijing 100069,China)

机构地区：[1]首都医科大学附属北京佑安医院肝病中心四科,北京市100069 [2]首都医科大学附属北京佑安医院肝病中心一科,北京市100069 [3]北京市肝衰竭与人工肝治疗研究重点实验室

出　　处：《实用肝脏病杂志》2021年第2期164-167,共4页Journal of Practical Hepatology

基　　金：北京市医院管理局消化内科学科协同发展中心项目(编号:XXZ0503);科技创新服务能力建设-高精尖学科建设项目(编号:11920703);北京市医院管理中心重点医学专业发展计划(扬帆计划)资助项目(编号:ZYLX202125)。

摘　　要：目的了解1996~2020年北京佑安医院住院的遗传代谢性肝病(IMLD)患者的病因构成和变化趋势。方法1996年1月~2020年12月期间我院收治的IMLD患者,分析疾病分布及患者性别和年龄分布特征。结果共收治IMLD患者552人次,占住院肝病患者的3.6‰;在实际393例IMLD患者中,男性患病率高于女性,平均年龄为29岁;肝豆状核变性和先天性高胆红素血症分别为223例(56.7%)和136例(34.6%),共占比为91.3%;前后10年对比,近10年较为少见的病种住院人数逐渐增多,发病年龄略有升高(24.9岁对31.4岁,P<0.01),中、老年患者所占比例呈上升趋势。结论成人IMLD并不罕见,疾病谱逐渐丰富,应该引起肝病医生的重视。Objective The aim of this study was to analyze partial epidemiological characteristics of patients with inherited metabolic liver disease(IMLD)of Beijing You'an Hospital from 1996 to 2020.Methods All patients with IMLD admitted in Beijing You'an Hospital from January 1996 to December 2020 were included,and the prevalence of disease catalogues,gender and ages of patients were analyzed.Results There were 552 cases of IMLD(some admitted more than one time)presented in our hospital during this period,accounting for 3.6‰of inpatients with liver disease;out of the 393 patients with IMLD,the percentage of male patients was higher than that of female,with an average age of 29 years;among all patients with IMLD,223(56.7%)had Wilson's disease and 136(34.6%)had congenital hyperbilirubinemia,both accounting for 91.3%;compared with in the previous 10 years,the number of rare diseases increased in recent 10 years and the age of onset increased slightly from 24.9 years to 31.4 years(P<0.01),with middle-aged and elderly patients also increasing.Conclusion Adult inherited metabolic liver disease is not rare,and the disease spectrum is gradually rich,to which should be paid more attention by hepatologists.

关 键 词：遗传代谢性肝病 流行病学 疾病谱 

分 类 号：R575[医药卫生—消化系统]