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首页> 外文期刊>Pediatrics: Official Publication of the American Academy of Pediatrics >Behavioral and social phenotypes in boys with 47,XYY syndrome or 47,XXY klinefelter syndrome
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Behavioral and social phenotypes in boys with 47,XYY syndrome or 47,XXY klinefelter syndrome

机译：47，XYY综合征或47，XXY klinefelter综合征男孩的行为和社交表型

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OBJECTIVE: To contrast the behavioral and social phenotypes including a screen for autistic behaviors in boys with 47,XYY syndrome (XYY) or 47,XXY Klinefelter syndrome (KS) and controls and investigate the effect of prenatal diagnosis on the phenotype. METHODS: Patients included 26 boys with 47,XYY, 82 boys with KS, and 50 control boys (ages 4-15 years). Participants and parents completed a physical examination, behavioral questionnaires, and intellectual assessments. RESULTS: Most boys with XYY or KS had Child Behavior Checklist parental ratings within the normal range. On the Child Behavior Checklist, mean problem behaviors t scores were higher in the XYY versus KS groups for the Problem Behavior, Externalizing, Withdrawn, Thought Problems, and Attention Problems subscales. On the Conners' Parent Rating Scale-Revised, the XYY versus KS group had increased frequency of hyperactive/impulsive symptoms (P < .006). In addition, 50% and 12% of the XYY and KS groups, respectively, had scores >15 for autism screening from the Social Communication Questionnaire. For the boys with KS, prenatal diagnosis was associated with fewer problem behaviors. CONCLUSIONS: A subset of the XYY and KS groups had behavioral difficulties that were more severe in the XYY group. These findings could guide clinical practice and inform patients and parents. Boys diagnosed with XYY or KS should receive a comprehensive psychoeducational evaluation and be screened for learning disabilities, attention-deficit/hyperactivity disorder, and autism spectrum disorders.
机译：目的：比较行为和社会表型，包括筛查47，XYY综合征（XYY）或47，XXY Klinefelter综合征（KS）和对照男孩的自闭症行为，并调查产前诊断对表型的影响。方法：患者包括26名47，XYY男孩，82名KS男孩和50名对照男孩（4-15岁）。参与者和父母完成了身体检查，行为问卷和智力评估。结果：大多数患有XYY或KS的男孩的儿童行为清单父母的评分都在正常范围内。在儿童行为清单上，对于问题行为，外在化，撤回，思维问题和注意问题子量表，在XYY与KS组中，平均问题行为t分数较高。在修订的Conners父母评分量表上，XYY与KS组的多动/冲动症状发生率增加（P <.006）。此外，来自社交交流问卷的XYY和KS组分别有50％和12％的孤独症筛查得分大于15。对于患有KS的男孩，产前诊断与较少的问题行为相关。结论：XYY和KS组的子集的行为困难在XYY组更为严重。这些发现可以指导临床实践，并告知患者和父母。被诊断患有XYY或KS的男孩应接受全面的心理教育评估，并应筛查学习障碍，注意力缺陷/多动障碍和自闭症谱系障碍。

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