Abstract |
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.
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Authors | H De Leersnyder, M C de Blois, M Vekemans, D Sidi, E Villain, C Kindermans, A Munnich |
Journal | Journal of medical genetics
(J Med Genet)
Vol. 38
Issue 9
Pg. 586-90
(Sep 2001)
ISSN: 1468-6244 [Electronic] England |
PMID | 11546826
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
- Adrenergic beta-Antagonists
- Acebutolol
- Melatonin
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Topics |
- Acebutolol
(pharmacology, therapeutic use)
- Adolescent
- Adrenergic beta-Antagonists
(pharmacology, therapeutic use)
- Behavior
(drug effects, physiology)
- Child
- Child, Preschool
- Chromosomes, Human, Pair 17
(genetics)
- Chronobiology Disorders
(drug therapy, genetics, physiopathology)
- Circadian Rhythm
(drug effects, physiology)
- Cognition
(drug effects)
- Female
- Humans
- Hyperkinesis
(drug therapy, genetics, physiopathology)
- In Situ Hybridization, Fluorescence
- Male
- Melatonin
(blood)
- Sleep
(drug effects, genetics, physiology)
- Sleep Wake Disorders
(drug therapy, genetics, physiopathology)
- Syndrome
- Wakefulness
(drug effects, genetics, physiology)
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