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Adaptive behavior in Chinese children with Williams syndrome

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. | Ji et al. BMC Pediatrics 2014 14 90 http www.biomedcentral.eom 1471-2431 14 90 BMC Pediatrics RESEARCH ARTICLE Open Access Adaptive behavior in Chinese children with Williams syndrome Chai Ji Dan Yao Weijun Chen Mingyan Li and Zhengyan Zhao 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 Background Williams syndrome WS is a neurogenetic disorder that occurs in about 1 in every 8 000 live births. WS is caused by the contiguous deletion of 26-28 genes on chromosome 7q11.23. Individuals with WS