https://scholars.lib.ntu.edu.tw/handle/123456789/159896
標題: | 威廉斯氏症兒童的氣質特徵 The Temperament of Children with Williams-Beuren Syndrome |
作者: | 簡淑 Chien, Shu |
關鍵字: | 威廉斯氏症;彈力蛋白基因;氣質;Williams-Beuren syndrome;Elastin gene deletion;Temperament | 公開日期: | 2006 | 摘要: | 威廉斯氏症(Williams-Beuren Syndrome; WBS)兒童具有特殊的臨床表徵、身心發展與基因上的特徵,本研究因此推論WBS兒童的氣質特徵與一般兒童亦應有差異。採橫斷研究法,以35位WBS兒童為實驗組,再以其性別和年齡為條件進行配對,選出77位的一般兒童為對照組。由兩組兒童的主要照顧者,依兒童年齡填寫4個月-3歲、4-7歲、與8-12歲3種氣質問卷。結果發現,整體而言,WBS兒童在氣質項目中的活動量與趨避性兩項有顯著較高的趨勢,而堅持度則顯著較一般兒童為低。依據年齡的分組比較,發現這3項氣質上的差異於4-7歲組與8-12歲組是顯著的,但在4個月-3歲組則不顯著。於性別上,則是男性的比較結果於3個項目上均有顯著差異,女性的比較僅在堅持度有顯著差異。兩組並依年齡組別來看各年齡組的發展上的變化,結果發現WBS兒童與一般兒童相同的是活動量是隨著各年齡組別上升而下降;情緒本質自4個月-3歲組到4-7歲組是上升的,而在8-12歲組下降;堅持度是到4-7歲組下降,而在8-12歲組上升;適應度是到4-7歲組上升,在8-12歲組下降;注意力分散度是到4-7歲組下降,在8-12歲組上升。WBS兒童與一般兒童不同的是趨避性隨著年齡而上升,反應閾是到4-7歲組上升,在8-12歲組下降。可見WBS兒童與一般兒童有相似的身心發展,亦有其獨特的發展狀況。 另外,以WBS兒童求診最初的主要症狀的面貌特徵、心雜音、與發育遲緩等3個項目,以及診斷上的主要特徵項目中的彈力蛋白基因(Elastin gene; ELN)缺失、心臟異常與發育遲緩等3個項目,分組比較各氣質項目之差異,以Mann-Whitney U test 檢定均未達統計上顯著差異。WBS兒童的氣質與其身體各器官疾病表現的影響似無相關。此研究結果將有助於因材施教與預防處理其行為所衍生之問題,提供照顧WBS者之參考。 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. |
URI: | http://ntur.lib.ntu.edu.tw//handle/246246/51367 | 其他識別: | zh-TW |
顯示於: | 分子醫學研究所 |
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ntu-95-P92448003-1.pdf | 23.31 kB | Adobe PDF | 檢視/開啟 |
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