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首页> 外文期刊>American journal of medical genetics, Part A >Cognition and behavior in pre-pubertal children with Prader-Willi syndrome and associations with sleep-related breathing disorders.
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Cognition and behavior in pre-pubertal children with Prader-Willi syndrome and associations with sleep-related breathing disorders.

机译：青春期前患有Prader-Willi综合征的儿童的认知和行为以及与睡眠相关的呼吸障碍的关联。

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摘要

Prader-Willi syndrome (PWS) is characterized by hypotonia, hypogonadism, obesity, and short stature. Neurobehavioral abnormalities, cognitive impairment, and sleep-related breathing disorders (SRBD) are common. In the general population associations between neurobehavioral and cognitive abnormalities and SRBD have been found. We investigated cognition, behavior, and SRBD in children with PWS. Thirty-one pre-pubertal PWS children were evaluated (5 with paternal deletion, 14 with maternal disomy, 4 with imprinting-center mutation, and in 8 the defect was not specified). Cognition was assessed by Wechsler scale subtests, and behavior by parent-questionnaires. Polysomnography was performed. Cognition, behavior, and associations with SRBD were evaluated. All cognitive subtests were significantly below O SDS, with the lowest median (interquartile range) scores for the Block design subtest (-2.7 SDS (-3.0 to -0.3)). In 60%, verbal subtests were less affected than performance subtests. Parents reported problem behavior related to emotions/behavior not adapted to the social situation social information." All children had SRBD, with an Apnea Hypopnea Index of 4.1/hr (2.6-7.9). One performance subtest score was significantly higher in children with better sleep efficiency, and daytime sleepiness was associated with more autistic-like social impairment. In contrast to our expectations, behavior was worse in children with better sleep-related breathing. In pre-pubertal PWS children, cognition is impaired. Neurobehavioral abnormalities are common, particularly autistic-like social impairment. Sleep efficiency was associated with better performance on one of the performance subtests, and neurobehavioral abnormalities were associated with daytime sleepiness. In contrast, we could not confirm a positive association of neurobehavioral abnormalities with SRBD in PWS.
机译：普拉德-威利综合症（PWS）的特点是肌张力低下，性腺功能低下，肥胖和身材矮小。神经行为异常，认知障碍和睡眠相关的呼吸障碍（SRBD）很常见。在一般人群中，已经发现了神经行为和认知异常与SRBD之间的关联。我们调查了PWS患儿的认知，行为和SRBD。对31名青春期前PWS儿童进行了评估（5例因父本缺失，14例因母体畸形，4例有印迹中心突变，8例未明确缺陷）。认知通过Wechsler量表测验进行评估，行为通过父母问卷调查进行评估。进行多导睡眠监测。评估认知，行为和与SRBD的关联。所有认知子测验均显着低于O SDS，Block design子测验的中位数（四分位间距）得分最低（-2.7 SDS（-3.0至-0.3））。在60％的学生中，口语子测验比性能子测验受影响较小。父母报告说，与情绪/行为有关的问题行为与社会状况的社会信息不适应。”所有儿童均患有SRBD，呼吸暂停低通气指数为4.1 /小时（2.6-7.9）。睡眠效率和白天嗜睡与更多的自闭症般的社交障碍有关。与我们的预期相反，睡眠相关呼吸更好的孩子的行为较差;在青春期前PWS的孩子中，认知能力受损。神经行为异常很常见，睡眠效率与一项表现子测验中的较好表现相关，而神经行为异常与日间嗜睡相关，但我们无法确定PWS中神经行为异常与SRBD呈正相关。

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