Adaptive behavior in Chinese children with Williams syndrome

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. © 2014 Ji et al.; licensee BioMed Central Ltd.