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| DC 欄位 | 值 | 語言 |
|---|---|---|
| dc.contributor.advisor | 吳英璋 | |
| dc.contributor.author | Shu Chien | en |
| dc.contributor.author | 簡淑 | zh_TW |
| dc.date.accessioned | 2021-06-13T03:31:51Z | - |
| dc.date.available | 2006-08-03 | |
| dc.date.copyright | 2006-08-03 | |
| dc.date.issued | 2006 | |
| dc.date.submitted | 2006-07-28 | |
| dc.identifier.citation | 1. Williams JCP, Barratt-Boyes BG, Lowe JB. Supravalvular aortic stenosis. Circulation. 1961;24:1311-8.
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Williams-Beuren syndrome in monozygotic twins with variable expression. American Journal of Medical Genetics. 1993;47:475-7. 30. Osborne LR, Li M, Pober B, Chitayat D, Bodurtha J, Mandel A, et al. A 1.5 million-base pair inversion polymorphism in families with Williams-Beuren syndrome. Nature Genetics. 2001;29:321-5. 31. UCSC Human Gene Sorter on March 2006.Available from: http://genome.cse.ucsc.edu/cgi-bin/hgNear?hgsid=71783178&org=Human&db=hg18&near_search=WBSCR&submit=Go%21&near_order=expGnfAtlas2&near.count=50) 32. Bayes M, Magano LF, Rivera N, Flores R, Perez Jurado LA. Mutational mechanisms of Williams-Beuren syndrome deleeions. American Journal of Human Genetics. 2003;73:131-51. 33. Osborne LR. Willims-Beuren syndrome: unraveling the mysteries of a microdeletion disorder. Molecular Genetics and Metabolism. 1999;67:1-10. 34. Urbán Z, Riazi S, Seidl TL, Katahira J, Smoot LB, Chitayat D, et al. Connection between Elastin Haploinsufficiency and Increased Cell Proliferation in Patients with Supravalvular Aortic Stenosis and Williams-Beuren Syndrome. The American Journal of Human Genetics. 2002 July, 2002;71(1):30-44. 35. Smoot L, Zhang H, Klaiman C, Schultz R, Pober B. Medical overview and genetics of Williams-Beuren syndrome. Progress in Pediatric Cardiology. 2005;20:195-205. 36. Heller R, Rauch A, Luttgen S, Schroder B, Winterpacht A. Partial deletion of the critical 1.5Mb interval in Williams-Beuren syndrome. Journal of Medical Genetics. 2003;40(8):e99. 37. Frangiskakis JM, Ewart AK, Morris CA, Mervis CB, Bertrand J, Robinson BF, et al. LIM-kinase 1 hemizygosity implicated in impaired visuospatial constructive cognition. Cell. 1996;86:59-69. 38. Karmiloff-Smith A, Grant J, Ewing S, Carette MJ, Metcalfe K, Donnai D, et al. Using case study comparisons to explore genotype-phenotype correlations in Williams-Beuren syndrome. Journal of Medical Genetics. 2003;40:136-40. 39. Bee H. The developing child. 9 ed. MA: Needham Heights: Allyn and Bacon;2000. 40. Waches TD, Bates JE. Temperament. In: Gavin Bremner& Alan Fogel, editors. Blackwell handbook of infant development. MA: Blackwell publisher; 2001. p. 465-501. 41. Carey WB. A simplified method for measuring infant temperament. The Journal of Pediatrics. 1970;77(2):188-94. 42. Fox NA, Henderson HA, Rubin KH, Susan D C, Schmidt LA. Continuity and discontinuity of behavioral inhibition and exuberance: psychophysiological and behavioral influences across the first four years of life. Child Development. 2001;72(1):1-21. 43. Frick PJ, Morris AS. Temperament and developmental pathways to conduct problems. Journal of Clinical Child and Adolescent Psychology. 2004;33(1):54-68. 44. Eklund M, Hansson L, Bengtsson-Tops A. The influence of temperament and character on functioning and aspects of psychological health among people with schizophrenia. European Journal Psychiatry. 2004 ;19(1):34-41. 45. 李美瑩. 學齡兒童氣質,家庭氣氛與學業成績之關係. [碩士論文]. 台北市: 國立政治大學教育研究所.1994. 46. Galen C. Available from: http://en.wikipedia.org/wiki/Galen 47. Thomas A, Chess S, Birch HG. Temperament and Behavior Disorders in Children. New York: New York University 1969. 48. Rutter M. Temperament, personality and personality disorder. British Journal of Psychiatry. 1987(150):443-58. 49. 王珮玲. 兒童氣質:基本特性與社會構成. 初版一刷. 台北市: 心理出版社; 2003. 50. Caspi A, Silva PA. Temperamental qualities at age three predict personality traits in young adulthood: longitudinal evidence from a birth conhort.Child Development. 1995;66:486-98.. 51. Guerin DW, Gottfried AW, Thomas CW. Difficult temperament and behaviour problems: a longitudinal study from 1.5 to 12 years.Internal Journal of Behavior Development. 1997;21(1):71-90. 52. Oniszczenko W, Zawadzki B, Streau J, Riemann R, Angleitner A, Spinath FM. Genetic and environmental determinants of temperament: a comparative study based on Polish and German samples. European Journal of Personality. 2003;17(17):207-20. 53. Hsu C-C, Soong W-T, Stigler JW, Hong C-C, Liang C-C. The temperamental charateristics of Chinese babies. Child Development. 1981;52:1337-40.. 54. Buss AH, Plomin R. Temperqment: early developing personality traits. NJ:Hillsdal, Erlbaum; 1984. 55. Lengua LJ, West SG, Sandler IN. Temperament as a predictor of symptomatology in children:addressing contamination of measures. Child Development. 1998;69(1):164-81. 56. Rothbart MK. Commentary: differentiated measures of temperament and multiple pathways to childhood disorders. Journal of Clinical Child and Adolescent Psychology. 2004;33(1):82-7. 57. Lahey BB. Commentary: role of temperament in developmental models of psychopathology. Journal of Clinical Child and Adolescent Psychology. 2004;33(1):88-93. 58. Tomc SA, Williamson NK, Pauli RM. Temperament in Williams syndrome. American Journal of Medical Genetics. 1990;36:345-52. 59. Gosch A, Pankau R. Personality characteristics and behavior problems in individuals of different ages with Williams syndrome. Developmental Medicine and Child Neurology. 1997;39:527-33. 60. Carey WB, McDevitt SC. Revision of the infant temperament questionnaire. Pediatrics. 1978;61(5):735-9. 61. 洪奇昌, 黃富源, 粱鏡秋, 沈慶村, 宋維村, 徐澄清. 中國嬰幼兒氣質特徵之初步研究. 中華民國小兒科醫學會雜誌. 1979;20(4):217-27. 62. 黃靜微. 母親對低出生體重兒與足月兒氣質評估比較. [碩士論文]. 台北市: 國立台灣大學醫學院護理學研究所; 1995. 63. 陳玉華. 台北市中山區及台北縣泰山鄉三歲至七歲兒童氣質特徵之初步研究. [碩士論文]. 台北市: 國立台灣大學醫學院公共衛生研究所; 1981. 64. 劉千琪. 學齡前期住院兒童的氣質與其住院,前,後的行為反應之相關性探討. [碩士論文]. 台北市: 國立台灣大學醫學院護理學研究所; 1994. 65. Waches TD, Bates JE. Temperament. In: Gavin Bremner AF, editor. Blackwell handbook of infant development MA: Blackwell publisher; 2001. p. 465-501. 66. Bellugi U, Lai Z, Wang P. Languagae, communication, and neural systems in Williams syndrome.Mental Retardation and Developmental Disorders Research Reviews. 1997;3:334-42. | |
| dc.identifier.uri | http://tdr.lib.ntu.edu.tw/jspui/handle/123456789/32103 | - |
| dc.description.abstract | 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。 | zh_TW |
| dc.description.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. | en |
| dc.description.provenance | Made available in DSpace on 2021-06-13T03:31:51Z (GMT). No. of bitstreams: 1 ntu-95-P92448003-1.pdf: 572204 bytes, checksum: 8fbc6573f0488e2a78c5384908abd62c (MD5) Previous issue date: 2006 | en |
| dc.description.tableofcontents | 致謝 3
目錄 4 中文摘要 6 英文摘要 7 第一章 緒論 8 第一節 威廉斯氏症的臨床表徵：生理、體型與心智特徵 9 第二節 威廉斯氏症的遺傳與基因變異 11 第三節 基因與臨床表徵的關係 12 第四節 威廉斯氏症者是否有其特殊的氣質之探討 13 第五節 本研究的目的與假說 15 第二章 研究方法與研究工具 16 第一節 研究對象 16 第二節 兒童年齡與問卷類別 17 第三節 研究對象的基本資料 17 第四節 研究對象父母與家庭的基本資料 18 第五節 研究工具 21 第六節 氣質評估量表的信度 24 第七節 資料處理 24 第三章 研究結果 26 第一節 威廉斯氏症兒童與一般兒童的氣質比較 26 第二節 威廉斯氏症兒童的臨床病徵與氣質之相關分析 32 第四章 結論 35 第五章 討論 36 參考文獻 39 附錄一：氣質項目與量表題目一覽表 44 附錄二：威廉斯氏症兒童氣質調查問卷-(4-7歲)為例 51 附錄三：氣質評估問卷使用意書 61 | |
| dc.language.iso | zh-TW | |
| dc.title | 威廉斯氏症兒童的氣質特徵 | zh_TW |
| dc.title | The Temperament of Children with Williams-Beuren Syndrome | en |
| dc.type | Thesis | |
| dc.date.schoolyear | 94-2 | |
| dc.description.degree | 碩士 | |
| dc.contributor.oralexamcommittee | 黃璉華,胡務亮 | |
| dc.subject.keyword | 威廉斯氏症,彈力蛋白基因,氣質, | zh_TW |
| dc.subject.keyword | Williams-Beuren syndrome,Elastin gene deletion,Temperament, | en |
| dc.relation.page | 62 | |
| dc.rights.note | 有償授權 | |
| dc.date.accepted | 2006-07-28 | |
| dc.contributor.author-college | 醫學院 | zh_TW |
| dc.contributor.author-dept | 分子醫學研究所 | zh_TW |
| 顯示於系所單位： | 分子醫學研究所 | |
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