β1-adrenergic antagonists improve sleep and behavioural disturbances in a circadian disorder, Smith-Magenis syndrome

β1-adrenergic antagonists improve sleep and behavioural disturbances in a circadian disorder, Smith-Magenis syndrome
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DOI:
10.1136/jmg.38.9.586
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发表时间:
2001-09-01
影响因子:
4
通讯作者:
Munnich, A
Munnich, A
中科院分区:
医学1区
文献类型:
--
作者:
De Leersnyder, H;de Blois, MC;Munnich, A

文献摘要

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Smith-Magenis综合征(SMS)是一种临床上可识别的连续基因综合征,归因于染色体17p11.2的间质性缺失。患者的褪黑激素昼夜节律发生相移,激素的昼夜分泌矛盾。研究了9名SMS儿童(年龄4至17岁)的血清褪黑激素水平和昼夜行为,这些儿童在清晨服用选择性β(1)-肾上腺素能拮抗剂醋丁洛尔(10 mg/kg)。在给药前后监测心脏检查、血清褪黑激素、运动活动记录和睡眠日记。目前的研究表明,一个单一的早晨剂量的醋丁洛尔抑制不适当的分泌褪黑激素的SMS。还注意到不适当行为的显著改善,包括注意力集中、延迟入睡、睡眠时间增加和延迟醒来。这些结果表明,β(1)-肾上腺素能拮抗剂有助于管理多动,提高认知能力,并减少睡眠障碍的SMS。
Smith-Magenis syndrome (SMS) is a clinically recognisable contiguous gene syndrome ascribed to interstitial deletions of chromosome 17p11.2. Patients have a phase shift of their circadian rhythm of melatonin with a paradoxical diurnal secretion of the hormone. Serum melatonin levels and day-night behaviour were studied in nine SMS children (aged 4 to 17 years) given acebutolol, a selective beta (1)-adrenergic antagonist (10 mg/kg early in the morning). Cardiac examination, serum melatonin, motor activity recordings, and sleep diaries were monitored before and after drug administration. The present study shows that a single morning dose of acebutolol suppressed the inappropriate secretion of melatonin in SMS. A significant improvement of inappropriate behaviour with increased concentration, delayed sleep onset, increased hours of sleep, and delayed waking were also noted. These results suggest that beta (1)-adrenergic antagonists help to manage hyperactivity, enhance cognitive performance, and reduce sleep disorders in SMS.