Suppr超能文献

同胞兄弟姐妹出现肱桡关节融合、小头畸形、胼胝体短小及生殖器异常。

Humero-radial synostosis, microcephaly, short corpus callosum, and abnormal genitalia in sibs.

作者信息

Guilherme Romain, Baumann Clarisse, Garel Catherine, Huten Yolène, Oury Jean-François, Delezoide Anne-Lise

机构信息

Department of Developmental Biology, Robert Debré Hospital, AP-HP, Paris Diderot University, Paris, France.

出版信息

Am J Med Genet A. 2008 Jul 15;146A(14):1775-80. doi: 10.1002/ajmg.a.32380.

Abstract

We report on two male sib fetuses with humero-radial synostosis and thumb hypoplasia, microcephaly with simplified gyral pattern, short corpus callosum and ambiguous genitalia. The main clinical, anatomopathological and imaging findings are presented and compared with previous cases of humero-radial synostosis as a prominent manifestation and with the X-linked lissencephaly with ambiguous genitalia syndrome (X-LAG). To our knowledge, this combination of anomalies has never been described before, and we propose that this disorder comprises a new humero-radial synostosis syndrome with an autosomal recessive or X-linked pattern of inheritance.

摘要

我们报告了两例男性同胞胎儿,他们患有肱桡关节融合和拇指发育不全、脑回模式简化的小头畸形、胼胝体短小以及生殖器模糊。本文呈现了主要的临床、解剖病理学和影像学发现,并与之前以肱桡关节融合为突出表现的病例以及伴有生殖器模糊的X连锁无脑回综合征(X-LAG)进行了比较。据我们所知,这种异常组合此前从未被描述过,我们提出这种疾病构成了一种新的具有常染色体隐性或X连锁遗传模式的肱桡关节融合综合征。

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验