Department of Psychiatry, Dokkyo Medical University Saitama Medical Center, Saitama, Japan; Department of Psychiatry, Dokkyo Medical University School of Medicine, Tochigi, Japan.
Department of Psychiatry, Dokkyo Medical University Saitama Medical Center, Saitama, Japan.
Res Dev Disabil. 2018 Feb;73:126-134. doi: 10.1016/j.ridd.2017.12.020. Epub 2018 Jan 8.
This study aims to explore the differences of age as well as genotype in regards to the severity of behavioral symptoms in Prader-Willi syndrome (PWS), with emphasis on the comparison between youngadults and adults.The Food Related Problem Questionnaire (FRPQ), the Aberrant Behavior Checklist Japanese Version (ABC-J), and the Pervasive Developmental Disorders Autism Society Japan Rating Scale (PARS) were administered to 46 PWS patients, including 33 young adults (ages 18-28) and 13 adults(ages 30-45). To examine the differences between young adults and adults, Mann-Whitney U tests were conducted. Statistically significant differences were found in ABC-J (p = .027) and PARS (p = .046), with higher scores in young adults than adults. Such differences between the two age groups were still true for the subgroups having a paternal chromosome 15q deletion (DEL) for ABC-J (p = .050) and part of PARS ("Problematic behavior"; p = .007). By contrast, there was no significant differences between young adults and adults regarding FRPQ (p = .65).These results suggest that aberrant behaviors decline from around the ages of thirty, in PWS patients in general and in DEL subgroups in particular, while food-related behaviors give no indication of diminishing in spite of developmental growth.
本研究旨在探讨 Prader-Willi 综合征（PWS）患者行为症状严重程度的年龄和基因型差异，重点比较青年人和成年人。我们对 46 名 PWS 患者进行了问卷调查，包括 33 名青年人和 13 名成年人，使用了食物相关问题问卷（FRPQ）、日本异常行为检查表（ABC-J）和日本广泛性发育障碍自闭症协会评定量表（PARS）。为了检验青年人和成年人之间的差异，我们进行了曼-惠特尼 U 检验。结果发现 ABC-J（p=0.027）和 PARS（p=0.046）有统计学差异，青年组的得分高于成年组。对于携带父源 15q 染色体缺失（DEL）的亚组，ABC-J（p=0.050）和 PARS 的部分项目（“问题行为”；p=0.007）的两组，这种差异仍然存在。相反，FRPQ 青年人和成年人之间没有显著差异（p=0.65）。这些结果表明，在一般的 PWS 患者和 DEL 亚组中，行为异常从 30 岁左右开始下降，而与发育相关的行为则没有减少的迹象。
