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首页> 外文期刊>American journal of medical genetics, Part C. Seminars in medical genetics >Pharmacological treatment of disruptive behavior in Smith-Magenis syndrome.
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Pharmacological treatment of disruptive behavior in Smith-Magenis syndrome.

机译:Smith-Magenis综合征破坏行为的药物治疗。

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Smith-Magenis syndrome (SMS) is a complex genetic syndrome caused by an interstitial deletion of chromosome 17p11.2. Children and adults with SMS appear to have unique neurobehavioral problems that include: sleep disturbance, self-injurious and maladaptive behaviors, stereotypies, and sensory integration disorders. We gathered retrospective psychotropic use information from parents or other caregivers of 62 individuals with SMS who were asked about use of psychotropic medication from a list of commonly used psychiatric medications. For those drugs identified, respondents were asked to rate the experience with the particular medication using a likert-type scale. Drugs were grouped into seven main categories: (1) stimulants; (2) antidepressants; (3) antipsychotics; (4) sleep aides; (5) mood stabilizers; (6) alpha 2 agonists; and (7) benzodiazepines. Relative frequencies, means and standard deviations pertaining to age and medication effect were derived for each medication category. Six of the seven medication categories examined showed no meaningful deviations from the "no change" score. The benzodiazepine group showed a mild detrimental effect. There were no gender differences in efficacy. Use of psychotropic medication started early in life (mean age 5 years), particularly with sleep aides. Although no medication category was identified as efficacious in SMS, all the categories reported herein may be considered as an option for brief symptomatic relief.
机译:Smith-Magenis综合征(SMS)是由染色体17p11.2间质性缺失引起的复杂遗传综合征。患有SMS的儿童和成人似乎具有独特的神经行为问题,包括:睡眠障碍,自我伤害和适应不良行为,刻板印象和感觉统合障碍。我们从62名SMS患者的父母或其他照顾者那里收集了回顾性精神使用信息,这些患者被询问了常用精神药物清单中有关精神药物的使用情况。对于确定的那些药物,要求受访者使用李克特型量表对特定药物的使用经验进行评分。药物分为七个主要类别:(1)兴奋剂; (2)抗抑郁药; (3)抗精神病药; (4)助眠剂; (5)情绪稳定剂; (6)α2激动剂; (7)苯二氮卓类。对于每种药物类别,得出了与年龄和药物作用有关的相对频率,均值和标准差。所检查的七个药物类别中的六个显示出与“无变化”评分无显着偏差。苯二氮卓类药物组显示出轻微的有害作用。疗效没有性别差异。精神药物的使用始于生命的早期(平均年龄5岁),尤其是在睡眠助手的帮助下。尽管没有药物类别在SMS中被确认为有效,但本文报告的所有类别都可以视为短暂症状缓解的一种选择。

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