[The Binder syndrome and goniodysgenesis. Apropos of a case].

A Bron, A M Bertrand, C Chabod, J Royer
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Abstract

A 15 years old boy was deferred to our department with growth hormone deficiency, deafness with middle ear bones involvement, and a facial dysmorphy called Binder syndrome. He presented ocular abnormalities with juvenile glaucoma and iris anterior insertion and goniodysgenesis. All these pathologic features are linked with the development of the neural crests. In our knowledge, it is the first so complete clinical association reported, which emphasizes the important role of the neural crest cells in early organogenesis.

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宾德综合征与性腺发育。关于一个案例]。
一名15岁男孩因生长激素缺乏、耳聋伴中耳骨受损伤和面部畸形被称为宾德综合征而被推迟到我科就诊。他的眼部异常表现为幼年型青光眼、虹膜前插入和性腺发育。所有这些病理特征都与神经嵴的发育有关。据我们所知,这是首次报道如此完整的临床关联,强调了神经嵴细胞在早期器官发生中的重要作用。
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