摘要：
目的:了解四川省病残儿先天性疾病顺位及变化趋势.方法:基于标准化的国际疾病分类(ICD-10)编码自动归类流程,对四川省2004年-2015年地市级医学鉴定的18 897例先天性病残儿21 059个鉴定诊断进行疾病编码,应用SAS 9.2、R3.3.3统计软件对鉴定资料进行基本人口学特征,总体、地区和年度的疾病顺位及趋势分析.结果:四川省2004年-2015年先天性病残儿平均鉴定审批率为83.3％,鉴定时患儿平均年龄7.7岁,男性占59.2％,农村占77.5％.先天性病残儿鉴定诊断共有131种,心脏的其他先天性畸形,心间隔先天性畸形,脑的其他先天性畸形,睾丸未降,肌肉骨骼的其他先天性变形,唐氏综合征,头、面、脊柱和胸的先天性肌肉骨骼变形,上肢短小缺陷,髋先天性变形,腭裂伴有唇裂为高发的前10位先天性疾病.地区顺位显示除泸州市首位高发先天性疾病为脑先天性畸形外,其余市(州)均为先天性心脏病.年度趋势分析结果表明唐氏综合征、心间隔先天性畸形和地中海贫血呈上升趋势,而肌肉骨骼的先天性变形、脑先天性畸形和睾丸未降呈下降趋势.结论:先天性心脏病、脑先天性畸形,睾丸未降,肌肉骨骼先天性变形,唐氏综合征等是四川省先天性病残儿的高发病种,需要针对唐氏综合征、心间隔先天性畸形和地中海贫血等呈上升趋势的先天性疾病制定切实有效的重点防治策略,降低儿童先天性疾病的发生率和致残率.%Objective:To analysis medical authentication data of congenital disabled children,and to understand the cis -position and trend of congenital diseases.Methods:Based on standard ICD-10 diseases automatic classification process,21059 diagnosed congenital diseases were coded corresponding to 18897 disabled children applied for medical authentication by prefectural level between 2004 and 2015 in Sichuan province,and SAS 9.2 and R 3.3.3 statistics software were used for analyzing the basic demographic characteristics,as well as disease cis-position and trend of these congenital disabled children during 12 years by general,region and annual.Results:Average approval ratio of medical authentication for congenital disabled children in Sichuan province between 2004 and 2015 was 83.3 ％,and average age of them when applied for medical authentication was 7.7 years old,male accounted for 59.2％,and children from rural area accounted for 77.5 ％.There were 131 congenital diseases diagnosed,and the most popular ten congenital diseases were congenital malformations of heart,congenital malformations of heart septum,congenital malformations of brain,undescended testicle,congenital musculoskeletal deformities,Down's syndrome,congenital musculoskeletal deformities of head,face,spine and chest,reduction defects of upper limb,congenital deformities of hip and cleft palate with cleft lip.The region cis-position showed that the most popular congenital disease in Luzhou city was congenital malformations of brain,but the most popular congenital disease in the other cities of Sichuan province were congenital heart disease.Annual trend analysis results showed that the proportion of Down's syndrome,congenital malformations of heart septum,and thalassaemia were in the rising trend,while the proportion of congenital musculoskeletal deformities,congenital malformations of brain,and undescended testicle were in the downward trend.Conclusion:Congenital heart disease,congenital malformations of brain,undescended testicle,congenital musculoskeletal deformities,Down's syndrome were high incidence congenital diseases in disabled children in the medical authentication of Sichuan province.In order to reduce the incidence and disability rates of children's congenital diseases,it is necessary to work out effective prevention and cure strategies for the congenital diseases in the rising trend such as Down's syndrome,congenital malformations of heart septum,and thalassaemia.
