School of Education, University College Dublin, Belfield, Dublin 4, Ireland.
J Intellect Disabil Res. 2012 Oct;56(10):929-46. doi: 10.1111/j.1365-2788.2012.01542.x. Epub 2012 Apr 4.
A number of genetic conditions with associated intellectual disability and/or special educational needs have increasingly well-defined behavioural phenotypes. Thus, the concept of 'behavioural phenotype' and aetiology of intellectual disability may be important with regard to school-based interventions.
The evidence for distinctive cognitive and behavioural aspects of five of the most common genetic syndromes (Down syndrome, fragile X syndrome, Williams syndrome, Prader-Willi syndrome and velo-cardio-facial syndrome) associated with special educational needs is reviewed with respect to key studies and findings. The possible utility of aetiology-related interventions in education is discussed with reference to arguments for and against such approaches with respect to published guidelines and published research.
Behavioural phenotypes are probabilistic and many children with a specific genetic syndrome will share commonalities with other children with other genetic syndromes and within syndrome variability is not uncommon. There is evidence that teachers and parents have limited knowledge of aspects of the proposed cognitive and behaviour profiles associated with the reviewed syndromes. While there are published guidelines in the area of learning and behaviour for each of the five reviewed syndromes there is a limited amount of evidence of the efficacy of such approaches in school settings.
It is likely that knowing the aetiology of a child's special educational needs will be helpful for staff who work in school settings in relation to cognitive and behavioural implications. However, how such knowledge might inform teaching practice or behavioural interventions has not been studied. A model is proposed that might help inform educators about the possible role of aetiology in the classroom.
越来越多与智力障碍和/或特殊教育需求相关的遗传疾病具有明确的行为表型。因此,“行为表型”和智力障碍的病因可能与基于学校的干预措施有关。
本文回顾了与特殊教育需求相关的五种最常见的遗传综合征(唐氏综合征、脆性 X 综合征、威廉姆斯综合征、普拉德-威利综合征和心脏面部血管发育不良综合征)的独特认知和行为方面的证据,重点介绍了关键研究和发现。根据发表的指南和研究,讨论了病因相关干预措施在教育中的可能应用,同时也讨论了支持和反对这些方法的论据。
行为表型是概率性的,许多患有特定遗传综合征的儿童与其他患有其他遗传综合征的儿童有共同之处,而且综合征内的变异性也并不罕见。有证据表明,教师和家长对所审查综合征相关的提议认知和行为特征的某些方面知之甚少。虽然针对所审查的五种综合征中的每一种,都有学习和行为方面的发表指南,但在学校环境中采用这些方法的效果证据有限。
了解儿童特殊教育需求的病因对于在学校环境中工作的工作人员在认知和行为方面可能会有所帮助。然而,这种知识如何影响教学实践或行为干预尚未得到研究。本文提出了一个模型,可以帮助教育工作者了解病因在课堂中的可能作用。