The Temperament of Children with Williams-Beuren Syndrome
Date Issued
2006
Date
2006
Author(s)
Chien, Shu
DOI
zh-TW
Abstract
Williams-Beuren syndrome (WBS) is a developmental disorder affecting connective tissue and central nervous system. It also involves a contiguous gene deletion by hemizygous microdeletion of 7q11.23. Children with WBS are observed to have friendly nature, affectionateness and sociability. Based on these characteristics, this study hypothesized that WBS children would have distinct temperaments and carried on the study. Both the primary carers of 35 children with WBS and 77 normal children match with sex and chronological age were asked to complete a Chinese version of temperament questionnaires for ages 4 months to 3 years, for ages 4-7years, and for ages 8-12 years. Each questionnaire has a similar evaluating format. The results showed that, as a whole, children with WBS appeared to display higher Activity, greater Approach, and less Persistence than normal children. These 3 behavioral styles are prominent in boys with different age group. When separated by age groups, these 3 behavioral styles were prominent in 4-7 years group and 8-12 years group. As for the differences by sex, these 3 behavioral styles were prominent in boys with different age group. Girls were found to be less Persistence with different age group. Developmentally, WBS children had the same trends as normal children on Activity, Emotion, Persistence, Adaptation, and Distraction, while differed on Approach and Threshold. No differences regarding facial features, heart murmur, developmental delay, elastin gene deletion, cardiovascular defects and inguinal hernia were found. It referred that the temperament of WBS may mainly come from the genetic composition.
Subjects
威廉斯氏症
彈力蛋白基因
氣質
Williams-Beuren syndrome
Elastin gene deletion
Temperament
SDGs
Type
other
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