Curfs L M, Hoondert V, van Lieshout C F, Fryns J P
Clinical Genetics Center, Maastricht, The Netherlands.
J Intellect Disabil Res. 1995 Jun;39 ( Pt 3):241-8. doi: 10.1111/j.1365-2788.1995.tb00506.x.
For the study of the personality profile of youngsters with Prader-Willi syndrome (PWS), a PWS group was compared with a matched group of youngsters attending regular school. The PWS group consisted of 28 youngsters (12 males and 16 females; mean age 11 years, 11 months). These youngsters were matched on gender and age level with 28 youngsters out of a group of 333 youngsters in regular school. Behaviour and personality characteristics of each youngster in both groups were described by both parents, using a Dutch version of the California Child Q-set (CCQ; Block & Block 1980). The present authors compared the scores of both groups on eight personality dimensions, derived from the aggregated CCQ-descriptions for fathers and mothers. The personality dimensions were Extraversion, Agreeableness, Conscientiousness, Emotional Stability, Openness, Motor Activity, Irritability and Dependency. The authors investigated further individual differences of PWS youngsters on the same eight CCQ personality dimensions, considering gender and age level as well as IQ level and the presence or absence of a 15q11-q13 deletion. The personality profile of PWS youngsters was markedly different from youngsters in regular school. Some personality characteristics were related to gender and IQ. The lower level of physical activity in PWS girls without 15q11-q13 deletion needs further study.
为了研究普拉德-威利综合征(PWS)患儿的人格特征,将一个PWS患儿组与一组在普通学校就读的匹配患儿组进行了比较。PWS患儿组由28名患儿组成(12名男性和16名女性;平均年龄11岁11个月)。这些患儿在性别和年龄水平上与333名普通学校患儿中的28名进行了匹配。两组中每个患儿的行为和人格特征均由其父母使用荷兰语版的加利福尼亚儿童Q分类表(CCQ;Block & Block,1980)进行描述。本文作者比较了两组在从父亲和母亲汇总的CCQ描述中得出的八个人格维度上的得分。这些人格维度包括外向性、宜人性、尽责性、情绪稳定性、开放性、运动活动、易怒性和依赖性。作者进一步研究了PWS患儿在相同的八个CCQ人格维度上的个体差异,同时考虑了性别、年龄水平以及智商水平和是否存在15q11-q13缺失。PWS患儿的人格特征与普通学校的患儿明显不同。一些人格特征与性别和智商有关。无15q11-q13缺失的PWS女孩身体活动水平较低这一情况需要进一步研究。