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Open Access Research article

Adaptive behavior in Chinese children with Williams syndrome

Chai Ji, Dan Yao, Weijun Chen, Mingyan Li and Zhengyan Zhao*

Author Affiliations

Department of child health care, The Children’s Hospital of Zhejiang University School of Medicine, 57# zhugan road, Hangzhou, Zhejiang Province, China

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BMC Pediatrics 2014, 14:90  doi:10.1186/1471-2431-14-90

Published: 4 April 2014

Abstract

Background

Williams syndrome (WS) is a neurodevelopmental disease characterized by compelling psychological phenotypes. The symptoms span multiple cognitive domains and include a distinctive pattern of social behavior. The goal of this study was to explore adaptive behavior in WS patients in China.

Methods

We conducted a structured interview including the Infants-Junior Middle School Students Social-life Abilities Scale in three participant groups: children with WS (n = 26), normally-developing children matched for mental age (MA, n = 30), and normally-developing children matched for chronological age (CA, n = 40). We compared the mean scores for each domain between the three groups.

Results

Children with WS had more siblings than children in the two control groups. The educational level of the caregivers of WS children was lower than that of the control children. We found no differences in locomotion, work skill, socialization, or self-management between the WS and MA groups. WS children obtained higher scores of self-dependence (df = 54, Z = −2.379, p = 0.017) and had better communication skills (df = 54, Z = −2.222, p = 0.026) compared with MA children. The CA children achieved higher scores than the WS children for all dimensions of adaptive behavior.

Conclusions

WS children have better adaptive behavior skills regarding communication and self-dependence than normal children matched for mental age. Targeted intervention techniques should be designed to promote social development in this population.

Keywords:
Williams syndrome; Children; Adaptive behavior