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目的探讨分析河南省中部地区三级医院中2003-2013年罕见病住院患者分布情况,了解罕见病的分布特征,为进一步进行罕见病流行病学研究提供科学依据。方法采集河南省38家三级医院中2003-2013年罕见病住院患者病历68 544例,进行描述性研究分析。结果先天畸形、变性和染色体异常是最主要的罕见病类型,占30.12%。河南省中部地区排名前10位的罕见病依次为心间隔先天畸形,紫癜及其他出血情况,新生儿呼吸窘迫,胰岛素依赖型糖尿病,帕金森病,肛门及直肠区的裂和瘘,面神经疾病,脑的其他疾病,腭裂及大动脉先天性畸形,其中心间隔先天畸形与紫癜最为常见,分别占11.89%和11.85%。2003-2013年河南省中部地区三级医院罕见病住院患者中,0~5岁患者病例数最高,占24.99%;15岁以下儿童占46.86%。男性罕见病患者所占比例高于女性患者,二者比值为1.2。在罕见病类型方面,男性和女性的罕见病分布情况相似,最主要的罕见病种类均为先天畸形、变性和染色体异常,男性为28%,女性为32%。罕见病在豫中地区三地的分布相似。结论罕见病在人群中的分布有较大差异,不同年龄段主要罕见病类型不同,不同性别主要罕见病类型相似,不同性别主要罕见病发病率不同,儿童主要罕见病为先天畸形、变性和染色体异常,且儿童罕见病住院患者人数远高于其他年龄段患者。
Objective To investigate the distribution of rare inpatients from 2003 to 2013 in tertiary hospitals in central area of Henan Province and understand the distribution characteristics of rare diseases so as to provide a scientific basis for further epidemiological study of rare diseases. Methods A total of 68 544 cases of rare inpatients from 2003 to 2013 in 38 tertiary hospitals in Henan Province were collected for descriptive analysis. Results Congenital malformations, degeneration and chromosomal abnormalities were the most common types of rare diseases, accounting for 30.12%. The top 10 rare diseases in central Henan Province were congenital heart defects, Purpura and other bleeding, neonatal respiratory distress, insulin-dependent diabetes, Parkinson’s disease, anus and rectum fissures and fistulas, facial nerve diseases, Other diseases of the brain, cleft palate and aortic congenital malformations, the most common central congenital malformations and purpura, accounting for 11.89% and 11.85%. Among the patients with rare diseases admitted to tertiary hospitals in the central region of Henan Province from 2003 to 2013, the number of patients aged 0 ~ 5 years was the highest, accounting for 24.99%; children under 15 accounted for 46.86%. The proportion of males with rare diseases is higher than that of females, with a ratio of 1.2. In terms of rare disease types, the distribution of rare diseases in both men and women is similar. The most common types of rare diseases are congenital malformations, degenerations and chromosomal abnormalities, 28% for males and 32% for females. The distribution of rare diseases in three regions of Henan is similar. Conclusions The distribution of rare diseases in the population is quite different. The main types of rare diseases are different in different age groups. The types of major rare diseases in different genders are similar. The incidence rates of major rare diseases in different genders are different. The main rare diseases in children are congenital malformations, degeneration and chromosomes Abnormal, and the number of children with rare diseases inpatients is much higher than other age groups.