Kirchner Rebecca M, Martens Marilee A, Andridge Rebecca R
Department of Neuroscience, The Ohio State University, ColumbusOH, USA; Department of Psychology, The Ohio State University, ColumbusOH, USA.
Department of Psychology, The Ohio State University at Newark, NewarkOH, USA; Nisonger Center, The Ohio State University, ColumbusOH, USA.
Front Psychol. 2016 Apr 28;7:598. doi: 10.3389/fpsyg.2016.00598. eCollection 2016.
Williams syndrome (WS) is a neurodevelopmental disorder that causes deficits in adaptive behavior, difficulties eating and sleeping, cognitive delays, and delayed development. Although researchers have conducted characterizations of children and adults with WS, less is known about young children with this disorder. This study characterizes the developmental and adaptive behavior features of 16 infants and toddlers with WS aged 3 months - 5 years. Data for this project was obtained from 2007 to 2014, and includes parent report data and standardized developmental testing. Thirty-one percent (31.3%) of parents reported that their infant/toddler with WS had sleeping problems and 58.3% reported feeding difficulties. Levels of adaptive behavior were in the Mildly Delayed range as measured by the Adaptive Behavior Assessment System, Second Edition. Self-care skills such as feeding or dressing oneself were significantly weaker than skills needed to function in the community, such as recognizing his/her home or throwing away trash. The difficulty with self-care skills is hypothesized to be related to the reported difficulties with eating and sleeping. Motor skills were significantly lower than both cognitive and language skills on the Bayley Scales of Infant and Toddler Development, Third Edition. The current study highlights the need for early intervention in these young children across all areas of development, particularly in self-care skills.
威廉姆斯综合征(WS)是一种神经发育障碍,会导致适应性行为缺陷、饮食和睡眠困难、认知延迟以及发育迟缓。尽管研究人员已对患有WS的儿童和成人进行了特征描述,但对于患有这种疾病的幼儿了解较少。本研究描述了16名年龄在3个月至5岁之间的患有WS的婴幼儿的发育和适应性行为特征。该项目的数据收集于2007年至2014年,包括家长报告数据和标准化发育测试。31.3%的家长报告称他们患有WS的婴幼儿存在睡眠问题,58.3%的家长报告存在喂养困难。根据《适应性行为评估系统(第二版)》测量,适应性行为水平处于轻度延迟范围内。诸如自己进食或穿衣等自我照顾技能明显弱于在社区中发挥功能所需的技能,比如认出自己的家或扔垃圾。据推测,自我照顾技能方面的困难与所报告的饮食和睡眠困难有关。在《贝利婴幼儿发展量表(第三版)》上,运动技能显著低于认知和语言技能。当前研究强调了对这些幼儿在所有发育领域进行早期干预的必要性,尤其是在自我照顾技能方面。