Clarke D J, Boer H
University of Birmingham, United Kingdom.
Am J Ment Retard. 1998 Nov;103(3):264-71. doi: 10.1352/0895-8017(1998)103<0264:PBAWDP>2.0.CO;2.
Problem behaviors of individuals who had one of three chromosome deletion disorders (5p- cri-du-chat, 15q- Prader-Willi, or 17p- Smith-Magenis) were investigated. The Aberrant Behavior Checklist was used. Results were contrasted with those of two groups of people with mental retardation who were described in other studies. The checklist rates many, but not all, potentially relevant behaviors. Eating abnormalities, known to be problematic in Prader-Willi syndrome, and sleep abnormalities, believed to be problematic in Smith-Magenis syndrome, were not included in the survey. All three disorders were associated with greater ratings of problem behaviors than the comparison groups on at least one subscale of the checklist. The results lend support to the partial specificity model of behaviors associated with genetically determined syndromes.
对患有三种染色体缺失疾病（5p-猫叫综合征、15q-普拉德-威利综合征或17p-史密斯-马吉尼斯综合征）之一的个体的问题行为进行了调查。使用了异常行为检查表。将结果与其他研究中描述的两组智力障碍者的结果进行了对比。该检查表对许多但并非所有潜在相关行为进行评分。普拉德-威利综合征中已知存在问题的饮食异常以及史密斯-马吉尼斯综合征中被认为存在问题的睡眠异常未纳入调查。在检查表的至少一个子量表上，所有这三种疾病与问题行为的评分均高于对照组。这些结果支持了与基因决定的综合征相关的行为的部分特异性模型。
