青少年患有普拉德-威利综合征的异常行为检查清单:对横截面和纵向行为特征的初步研究
Soo-Jeong Kim1,2, Lydia Kim1, Waylon Howard3
1Division of Child and Adolescent Psychiatry, University of Washington, Seattle, Washington, USA.
American journal of medical genetics. Part A
|August 20, 2024
概括
普拉德-威利综合征 (PWS) 行为,通过异常行为检查清单 (ABC) 测量,随着年龄的增长显示出变化. 刺激性得分在12岁后下降,但在5-12岁的男性中增加.
科学领域:
- 遗传学 遗传学 是一个
- 神经发育障碍 神经发育障碍
- 行为科学 行为科学
背景情况:
- 普拉德-威利综合征 (PWS) 是一种罕见的遗传疾病,由15号染色体上的父基因丢失引起.
- 异常行为检查清单 (ABC) 是评估发育障碍患者行为问题的关键工具.
研究的目的:
- 在患有普拉德-威利综合征 (PWS) 的年轻人中描述异常行为检查清单 (ABC) 评分.
- 分析PWS人群中随着时间的推移,这些行为评分的变化.
主要方法:
- 分析69名被诊断患有普拉德-威利综合征 (PWS) 的患者的ABC分数.
- 在四个年龄组 (5-8,9-12,13-16,17-22岁) 中,ABC平均分数的比较.
- 纵向分析以追踪随时间变化的行为评分,包括性别和遗传亚型差异.
主要成果:
- 在5-8岁的年龄组,与9-12岁的年龄组相比,易怒度得分显著下降.
- 易怒,惰,刻板印象行为,过度活跃和ABC总分的得分在12岁以后往往会下降.
- 5-12岁之间的男性易怒度得分比女性得分增加了更多;非删除组在12年后显示出比删除组更大的Lethargy减少.
结论:
- 普拉德-威利综合征 (PWS) 的行为模式随着年龄的增长而演变,一些具有挑战性的行为在青春期后可能会减少.
- 了解这些与年龄有关的行为变化对于有针对性的干预和支持至关重要.
- 对PWS行为进行系统的数据收集是至关重要的,因为不适应性行为的终身影响.
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