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Smith–Magenis syndrome is a microdeletion syndrome associated with a clinically recognizable behavioral and physical phenotype due to an interstitial deletion of 17p11. 2. Recent evidence suggests that haploinsufficiency of the retinoic acid induced 1 gene (RAI1) is probably responsible for the behavioral, neurological, otolaryngological, and craniofacial aspects of SMS, whereas more variable features such as heart and renal defects are probably due to hemizygosity of other genes in the 17p11. 2 region [Slager et al., 2003]. Neurobehavioral manifestations typical of this syndrome include mental retardation, sleep dysfunction with inverted circadian rhythm of melatonin, hyperactivity, stereotypy, selfhugging behavior, and self-injurious behaviors [Chen et al., 1996; Smith et al., 2002a]. Common physical manifestations include short stature, brachycephaly, broad face, midface hypoplasia, bowed or tented upper lip …