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在五名患有进行性精神运动发育迟缓、癫痫、反应亢进和皮纹异常独特综合征的无关患者中,额外的双着丝粒15号染色体短臂导致了15号染色体长臂2区1带/2区2带的异常。

Extra dicentric 15 pter leads to q21/22 chromosomes in five unrelated patients with a distinct syndrome of progressive psychomotor retardation, seizures, hyper-reactivity and dermatoglyphic abnormalities.

作者信息

Zannotti M, Preto A, Giovanardi P R, Dallapiccola B

出版信息

J Ment Defic Res. 1980 Dec;24 Pt 4:235-42. doi: 10.1111/j.1365-2788.1980.tb00077.x.

Abstract

Five unrelated patients with a supernumerary chromosome derivative of chromosome 15 are described. The clinical findings in the present series of cases show a gross concordance with the data previously reported in subjects with similar aberrations and allow the delineation of a distinct syndrome. Although undetermined variation in the structure of these extra chromosomes may contribute significantly to phenotypic heterogeneity, the patients display a rather common constellation of findings, which include: absence of major malformations, mental and developmental retardation, seizures, hypotonia, behavioural disturbances, and reduced total ridge count on fingertips. Patients with partial trisomy 15q- resulting from dicentric chromosomes bear little resemblance to patients carrying 15q- chromosomes arising de novo or due to unbalanced translocations.

摘要

本文描述了5例与15号染色体衍生的额外染色体无关的患者。本系列病例的临床发现与先前报道的具有类似畸变的受试者的数据总体一致,并有助于明确一种独特的综合征。尽管这些额外染色体结构的未确定变异可能显著导致表型异质性,但患者表现出相当常见的一组症状,包括:无 major 畸形、智力和发育迟缓、癫痫发作、肌张力减退、行为障碍以及指尖总嵴数减少。由双着丝粒染色体导致的部分15q-三体患者与新发或因不平衡易位携带15q-染色体的患者几乎没有相似之处。 (注:“major”这里可能是“严重”等意思,原文表述不太准确,可能是医学专业术语相关特定指代,因缺乏更多背景难以精准翻译)

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