Pharmacological treatment of disruptive behavior in Smith-Magenis syndrome.
Pharmacological treatment of disruptive behavior in Smith-Magenis syndrome.
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DOI：
10.1002/ajmg.c.30282
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发表时间：
2010-11-15
影响因子：
3.1
通讯作者：
Smith, Ann C. M.
中科院分区：
文献类型：
--
作者：
Laje, Gonzalo;Bernert, Rebecca;Morse, Rebecca;Pao, Maryland;Smith, Ann C. M.
关键词：
Smith-Magenis综合征（SMS）是由染色体17p11.2的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失调。我们收集了62名SMS患者的父母或其他照顾者的回顾性精神药物使用信息，这些患者被问及从常用精神药物列表中使用精神药物的情况。对于那些确定的药物，受访者被要求使用Likert类型的量表对特定药物的体验进行评分。药物分为七大类：（1）兴奋剂;（2）抗抑郁药;（3）抗精神病药;（4）助眠药;（5）情绪稳定剂;（6）α 2激动剂;（7）苯二氮卓类。推导出每种药物类别与年龄和药物效应相关的相对频率、平均值和标准差。检查的7种药物类别中有6种显示与“无变化”评分无有意义的偏离。苯二氮卓类药物组显示出轻微的有害作用。疗效无性别差异。精神药物的使用始于生命早期（平均年龄5岁），特别是睡眠辅助剂。尽管在SMS中未发现有效的药物类别，但本文报告的所有类别均可视为短暂症状缓解的一种选择。
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.
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影响因子：
4
作者：
Potocki, L;Glaze, D;Lupski, JR
通讯作者：
Lupski, JR
DOI：
10.1046/j.1365-2788.1998.4260481.x
发表时间：
1998-12-01
影响因子：
3.6
作者：
Dykens, EM;Smith, ACM
通讯作者：
Smith, ACM
影响因子：
5.1
作者：
De Leersnyder, H;de Blois, MC;Munnich, A
通讯作者：
Munnich, A
影响因子：
4
作者：
De Leersnyder, H;de Blois, MC;Munnich, A
通讯作者：
Munnich, A
影响因子：
3.9
作者：
Dykens, EM;Finucane, BM;Gayley, C
通讯作者：
Gayley, C