Ly T M, Hodapp R M
Graduate School of Education & Information Studies, University of California, Los Angeles, CA 90095, USA.
J Intellect Disabil Res. 2005 Dec;49(Pt 12):929-39. doi: 10.1111/j.1365-2788.2005.00782.x.
Genetic disorders predispose individuals to exhibit characteristic behaviours, which in turn elicit particular behaviours from others. In response to the strength of Prader-Willi syndrome (PWS) and weakness of Williams syndrome (WS) in visual-spatial tasks such as jigsaw puzzles, parents' behaviours can be affected by their child's level of puzzle ability or syndrome diagnosis.
Children were asked to complete two jigsaw puzzles (one with the experimenter and the other with the parent) for 5 min each. Frequencies of parental helping and reinforcement behaviours, along with ratings of parental directiveness, were examined as parents interacted with their children on a jigsaw puzzle task. Within each aetiological group, correlates of parental behaviours with child characteristics were also examined.
Compared to parents of children with PWS, parents of children with WS engaged in a more directive style of interaction, and provided more help and reinforcement. Relative to parents of children with higher puzzle abilities (from both aetiologies), parents of children with lower abilities also showed the same pattern. Both the child's aetiology and puzzle abilities were important in predicting parents' directiveness and helping and reinforcement behaviours. Within the PWS group, parents' behaviours correlated negatively with the child's puzzle abilities and general cognitive functioning; no such relations occurred in the WS group.
Parents' behaviours were affected by both the child's diagnosis and actual puzzle abilities, suggesting important implications for understanding and intervening with parents and children with different genetic syndromes.
遗传疾病使个体易于表现出特定行为，这些行为反过来又会引发他人的特定行为。针对普拉德-威利综合征（PWS）患者在拼图等视觉空间任务中的优势以及威廉姆斯综合征（WS）患者的劣势，父母的行为可能会受到孩子拼图能力水平或综合征诊断结果的影响。
要求儿童完成两幅拼图（一幅与实验者一起完成，另一幅与父母一起完成），每幅拼图限时5分钟。在父母与孩子一起完成拼图任务时，观察父母给予帮助和强化行为的频率，以及父母指令性的评分。在每个病因组内，还研究了父母行为与孩子特征之间的相关性。
与PWS患儿的父母相比，WS患儿的父母采用了更具指令性的互动方式，并提供了更多的帮助和强化。相对于拼图能力较高的孩子（来自两种病因组）的父母，能力较低的孩子的父母也表现出相同的模式。孩子的病因和拼图能力在预测父母的指令性以及帮助和强化行为方面都很重要。在PWS组内，父母的行为与孩子的拼图能力和一般认知功能呈负相关；而在WS组中则没有这种关系。
父母的行为受到孩子的诊断结果和实际拼图能力的影响，这对于理解和干预患有不同遗传综合征的父母和孩子具有重要意义。
