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Smith-Magenis 综合征患者的同胞:阳性和阴性行为特征相关因素的研究。

Siblings of individuals with Smith-Magenis syndrome: an investigation of the correlates of positive and negative behavioural traits.

机构信息

Department of Human and Molecular Genetics, Virginia Commonwealth University, Richmond, VA 23298, USA.

出版信息

J Intellect Disabil Res. 2012 Oct;56(10):996-1007. doi: 10.1111/j.1365-2788.2012.01581.x. Epub 2012 Jun 7.

DOI:10.1111/j.1365-2788.2012.01581.x
PMID:22672270
Abstract

BACKGROUND

Smith-Magenis syndrome (SMS) is a neurodevelopmental disorder that affects approximately one out of 25,000 births worldwide. To date, no research has been conducted to investigate how having an individual with SMS in a family is a positive or negative influence on siblings.

METHODS

To investigate this question we conducted a study involving 79 siblings and 60 parents of individuals with SMS to assess perceptions of how having a sibling with SMS positively and negative influence siblings' behavioural traits.

RESULTS

Our findings show that age of siblings of individuals with SMS was associated with a significant increase in positive behavioural traits and a significant decrease in negative behavioural traits. Additionally, siblings who perceive benefits from having a sibling with SMS demonstrate significantly more positive behavioural traits and significantly fewer negative behavioural traits. Parents accurately assess the changes in sibling behavioural traits with age, and parents who perceive their child as having experienced benefits from the sibling relationship report that siblings demonstrate significantly more positive behavioural traits and significantly fewer negative behavioural traits.

CONCLUSIONS

Our research shows that although individuals experience difficulties as a result of having a sibling with SMS, overall, siblings tend to fare well and parents appreciate both the positive and negative behavioural effects that result from having a sibling with SMS.

摘要

背景

Smith-Magenis 综合征(SMS)是一种神经发育障碍,全球每 25000 例出生中就有一例受到影响。迄今为止,尚未有研究调查家中有 SMS 患者对兄弟姐妹的影响是积极的还是消极的。

方法

为了调查这个问题,我们对 79 名 SMS 患者的兄弟姐妹和 60 名父母进行了研究,以评估兄弟姐妹对 SMS 患者的积极和消极影响的看法。

结果

我们的研究结果表明,SMS 患者兄弟姐妹的年龄与积极行为特征的显著增加和消极行为特征的显著减少有关。此外,那些认为从有 SMS 患者的兄弟姐妹中受益的兄弟姐妹表现出更多的积极行为特征和更少的消极行为特征。父母可以准确评估兄弟姐妹随着年龄的变化而发生的行为特征变化,那些认为自己的孩子从兄弟姐妹关系中受益的父母报告说,兄弟姐妹表现出更多的积极行为特征和更少的消极行为特征。

结论

我们的研究表明,尽管个体由于有 SMS 患者的兄弟姐妹而面临困难,但总体而言,兄弟姐妹往往表现良好,父母赞赏兄弟姐妹关系带来的积极和消极行为影响。

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