Steinhausen Hans-Christoph, Von Gontard Alexander, Spohr Hans-Ludwig, Hauffa Berthold P, Eiholzer Urs, Backes Margitta, Willms Judith, Malin Zeno
Department of Child and Adolescent Psychiatry, University of Zurich, Switzerland.
Am J Med Genet. 2002 Sep 1;111(4):381-7. doi: 10.1002/ajmg.10627.
Behavioral phenotypes were studied in four mental retardation syndromes using the Developmental Behavior Checklist (DBC). The four samples comprised fetal alcohol syndrome (FAS), Prader-Willi syndrome (PWS), fragile X syndrome (FRAX), and tuberosis sclerosis (TSC). Both on the item and the subscale level, there were clear behavioral differentiations across the four syndromes. FAS and FRAX proved to be most clearly differentiated from the other two samples, with PWS and TSC showing lower scores and less abnormal behavior profiles. Neither intelligence nor gender nor age contributed to variations in the number of behavior abnormalities. It was concluded that the DBC as a quantitative approach contributes significantly to the differentiation of behavioral phenotypes in various mental retardation syndromes.
使用发育行为检查表（DBC）对四种智力障碍综合征的行为表型进行了研究。这四个样本包括胎儿酒精综合征（FAS）、普拉德-威利综合征（PWS）、脆性X综合征（FRAX）和结节性硬化症（TSC）。无论是在项目层面还是子量表层面，这四种综合征之间都存在明显的行为差异。事实证明，FAS和FRAX与其他两个样本的差异最为明显，PWS和TSC的得分较低，行为异常特征较少。智力、性别和年龄均未导致行为异常数量的差异。研究得出结论，DBC作为一种定量方法，对区分各种智力障碍综合征的行为表型有显著贡献。
