740例罕见病住院病例分布特征分析  

Analysis of Distribution Characteristics of 740 Rare Disease Hospitalized Cases

作　　者：李佳霖 杨琴[1] 明星辰[1] 罗勤[1] Li Jialin;Yang Qin;Ming Xingchen;Luo Qin(Shiyan Taihe Hospital,Shiyan 442000,Hubei Province,China;不详)

机构地区：[1]十堰市太和医院,湖北省十堰市442000

出　　处：《中国病案》2023年第9期74-76,共3页Chinese Medical Record

摘　　要：目的分析某三级甲等综合医院罕见病住院患者分布特征,为加强罕见病诊疗管理提供参考依据。方法收集医院2019年1月1日-2021年12月31日罕见病住院病例资料,对罕见病患者基本情况、病例分布变化趋势、疾病分类及前10位病种分布情况进行描述性分析。结果医院共收治住院罕见病患者740例,占住院患者总例数的0.19%,年罕见病住院患者占比从0.17%上升至0.21%。患者年龄范围0~86岁,平均年龄35.11岁,男女性别比例为1.07:1。罕见病发病涉及21个疾病系统分类中的9个分类,共覆盖51个病种;神经系统疾病包含了13种罕见病,从患者数量和病种广度上均列首位。前10位病种患者占罕见病患者总数的63.11%,以青年组及中年组患者居多;男女比例相差较大的罕见病依次为进行性肌营养不良(17.0:1)、血友病(3.5:1)、自体免疫性脑炎(2.5:1)。结论罕见病住院患者占比逐年上升;罕见病发病年龄跨度大、疾病分布广;不同类型的罕见病中,男女比例差别较大。Objectives This study aims to analyze the distribution characteristics of inpatients with rare diseases in a tertiary general hospital,so as to provide a reference for strengthening the diagnosis and treatment management of rare diseases.Methods Data on inpatient cases of rare diseases in the hospital from January 1,2019 to December 31,2021 were collected.Descriptive analysis was carried out on the basic situation of rare disease patients,the trend of case distribution,disease classification,and the distribution of the top 10 diseases.Results A total of 740 inpatients with rare diseases were admitted to the hospital,accounting for 0.19%of the total number of inpatients.The proportion of inpatients with rare diseases rose from 0.17%to 0.21%per year.The age of the patients ranged from 0 to 86 years old,with an average age of 35.11 years old.The male-to-female ratio was 1.07:1.The incidence of rare diseases involved 9 of the 21 disease classifications,covering a total of 51 diseases.Nervous system diseases included 13 rare diseases,ranking first in terms of the number of patients and the breadth of diseases.Patients with the top 10 diseases accounted for 63.11%of the total number of rare disease patients,most of whom were young and middle-aged patients.The rare diseases with a large difference in male to female ratio were progressive muscular dystrophy(17.0:1),hemophilia(3.5:1),and autoimmune encephalitis(2.5:1).Conclusions The proportion of hospitalized patients with rare diseases is increasing year by year.Rare diseases have a large age span of onset and a wide distribution of diseases.Among different types of rare diseases,the ratio of male to female varies greatly.

关 键 词：罕见病 分布情况 描述性分析 

分 类 号：R197.323[医药卫生—卫生事业管理]