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威廉姆斯综合征作为基因决定的右半球优势模型。

Williams syndrome as a model of genetically determined right-hemisphere dominance.

作者信息

Bogdanov N N, Solonichenko V G

机构信息

Institute of Higher Nervous Activity and Neurophysiology, Russian Academy of Sciences, Moscow.

出版信息

Neurosci Behav Physiol. 1997 May-Jun;27(3):264-7. doi: 10.1007/BF02462891.

Abstract

Studies were carried out on the dermatoglyphics (skin ridge marks) on the hands of children with Williams syndrome; this is an inherited disease with cardiovascular pathology and a characteristic facial phenotype ("elf" facies), along with specific mental and cognitive disturbances. The results suggest a characteristic dermatoglyphic type with the presence of complex whorls on the fingers and a clear predominance of marks of greater complexity on the left hand; this is a very rare trait in normal people and in those with other inherited nervous system disorders. The features of the dermatoglyphic pattern serve as a characteristic marker of a genetically determined state of the human central nervous system, and suggests directions for neurophysiological studies of children with Williams syndrome as a unique model for analysis of higher nervous function in humans.

摘要

对患有威廉姆斯综合征儿童的手部皮纹(皮肤嵴纹)进行了研究;这是一种遗传性疾病,伴有心血管病变和特征性面部表型(“小精灵”面容),以及特定的精神和认知障碍。结果表明存在一种特征性皮纹类型,手指上有复杂的涡纹,且左手明显以更复杂的纹路为主;这在正常人和患有其他遗传性神经系统疾病的人中是非常罕见的特征。皮纹模式的特征可作为人类中枢神经系统遗传决定状态的特征性标志物,并为将威廉姆斯综合征儿童作为分析人类高级神经功能的独特模型进行神经生理学研究指明了方向。

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