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芬兰重度和极重度智力残疾的患病率、病因及共病情况。

Prevalence, aetiology and comorbidity of severe and profound intellectual disability in Finland.

作者信息

Arvio M, Sillanpää M

机构信息

Pääjärvi Centre for the Mentally Retarded, Lammi, and Department of Child Neurology, Turku University, Turku, Finland.

出版信息

J Intellect Disabil Res. 2003 Feb;47(Pt 2):108-12. doi: 10.1046/j.1365-2788.2003.00447.x.

Abstract

BACKGROUND

The aim of the present study was to describe the aetiology, associated impairments and prevalence of severe and profound intellectual disability (SPID) in Finland.

METHODS

The number of people with SPID in the catchment area of the Pääjärvi Centre for the Mentally Retarded, Lammi, Finland, (total population = 341,227) was calculated from the client register of this centre. Aetiological factors and background diagnoses for all subjects with SPID were analysed retrospectively.

RESULTS

The number of people with SPID was 461, giving a prevalence of 0.13%. The aetiology of their SPID was genetic or congenital in 235 (50.9%) individuals, acquired in 89 (19.3%), genetic and/or acquired in 84 (18.3%), and unknown in 53 (11.5%) subjects. Out of the 53 individuals with an SPID of unknown origin, 48 (90.6%) had an associated impairment; the remaining five were the only members of the study group showing normal growth, and having neither dysmorphic features, physical abnormalities nor family members with ID. Out of the 461 subjects, 422 (91.5%) had between one and six associated impairments (total = 954), and the remaining 39 (8.5%) had SPID as their only impairment. Uncomplicated SPID was mainly of genetic or congenital origin, whereas all subjects with acquired encephalopathy had multiple disabilities. Speech defects, epilepsy and cerebral palsy were the most common associated impairments.

CONCLUSIONS

Severe and profound ID almost always occurs concomitantly with other severe neurological or psychiatric impairments. The proportion of people with SPID described in the present study is similar to that found in Finland in 1966. The aetiology of SPID in the vast majority of cases is biopathological.

摘要

背景

本研究旨在描述芬兰重度和极重度智力残疾(SPID)的病因、相关障碍及患病率。

方法

从芬兰拉米市派亚尔维智障中心(总人口 = 341,227)的客户登记册中计算该中心服务区域内SPID患者的数量。对所有SPID患者的病因因素和背景诊断进行回顾性分析。

结果

SPID患者数量为461人,患病率为0.13%。其SPID的病因在235人(50.9%)中为遗传或先天性,89人(19.3%)为后天性,84人(18.3%)为遗传和/或后天性,53人(11.5%)病因不明。在53名病因不明的SPID患者中,48人(90.6%)有相关障碍;其余5人是研究组中仅有的生长正常、无畸形特征、无身体异常且家庭成员无智力残疾的成员。在461名受试者中,422人(91.5%)有1至6种相关障碍(共计954种),其余39人(8.5%)仅有SPID这一障碍。单纯性SPID主要为遗传或先天性起源,而所有患有后天性脑病的受试者都有多种残疾。言语缺陷、癫痫和脑瘫是最常见的相关障碍。

结论

重度和极重度智力残疾几乎总是与其他严重的神经或精神障碍同时出现。本研究中描述的SPID患者比例与1966年在芬兰发现的比例相似。绝大多数情况下,SPID的病因是生物病理学的。

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