Investigation into the Medical, Developmental, and Adaptive Behavior Phenotype of Infants and Toddlers with Williams Syndrome
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Date
2015-05
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The Ohio State University
Abstract
Williams syndrome (WS) is a neurodevelopmental disorder that causes cardiac abnormalities, difficulties eating and sleeping, cognitive delays, and hypersociability. Although researchers have conducted characterizations of children and adults with WS, less is known about younger children with this disorder. This study will characterize the medical, developmental, and adaptive behavior features of infants and children with WS aged 3 months - 5 years. Data for this project was obtained from 16 infants and toddlers who came to the Nationwide Children’s Hospital/Nisonger Center Williams Syndrome Clinic from 2007-2014, including parent reported developmental history, medical 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. Delays were noted in the acquisition of developmental milestones, particularly in language development. Levels of adaptive behavior were in the Mildly Delayed range. Within the Practical domain of adaptive behavior (Home Living, Community Use, Health and Safety, Self Care), Community Use skills were significantly stronger than Self Care skills. A significant main effect was also found between the three composite scores on the Bayley Scales of Infant and Toddler Development (Motor, Cognitive, and Language), with lower scores appearing in the Motor Domain. The data collected thus far highlights the need for early intervention in these young children.
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Keywords
Williams Syndrome, Adaptive Behavior, development, disability