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200例病因不明的智力发育迟缓及多发先天性异常患儿的细胞遗传学研究结果

Cytogenetic findings in 200 children with mental retardation and multiple congenital anomalies of unknown cause.

作者信息

Coco R, Penchaszadeh V B

出版信息

Am J Med Genet. 1982 Jun;12(2):155-73. doi: 10.1002/ajmg.1320120206.

Abstract

We report a cytogenetic study of 200 children with mental retardation and three or more major or minor congenital anomalies. In all cases, the chromosomes were studied with conventional staining methods (nonbanding) and with at least one of the following techniques: Q, G, or R banding. In a few patients, C banding and in vitro differentiation with BUDR and acridine orange (R banding) were also used. In patients with structural abnormality, parental chromosomes were studied using the same techniques. A chromosomes abnormality was found in 42 patients (21%). Of these, 16(8%) had complete or mosaic aneuploidies (11 autosomal and 5 gonosomal); and 26 (13%) had structural defects. In 21 of the latter the structural abnormality occurred as a de novo rearrangement, and in 5 the defect was inherited from a parent carrier of a balanced rearrangement. The contribution of chromosome aberrations to the cause of (idiopathic) MCA/MR syndromes is discussed.

摘要

我们报告了一项对200名智力发育迟缓且伴有三种或更多主要或次要先天性异常的儿童进行的细胞遗传学研究。在所有病例中,均采用传统染色方法(非显带)以及以下技术中的至少一种对染色体进行研究:Q显带、G显带或R显带。在少数患者中,还使用了C显带以及用5-溴脱氧尿苷和吖啶橙进行的体外分化(R显带)。对于结构异常的患者,采用相同技术研究其父母的染色体。在42名患者(21%)中发现了染色体异常。其中,16名(8%)有完全或嵌合的非整倍体(11例常染色体和5例性染色体);26名(13%)有结构缺陷。在后者中,21例结构异常为新发重排,5例缺陷是从平衡重排的父母携带者遗传而来。本文讨论了染色体畸变在(特发性)多发先天性异常/智力发育迟缓综合征病因中的作用。

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