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脊髓纵裂的发病机制:鸡胚手术模型能否为人类畸形提供一些线索?

Pathogenesis of diastematomyelia: can a surgical model in the chick embryo give some clues about the human malformation?

作者信息

Rilliet B, Schowing J, Berney J

机构信息

Départment d'Oto-neuro-ophtalmologie, Hôpital Cantonal Universitaire, Geneva, Switzerland.

出版信息

Childs Nerv Syst. 1992 Sep;8(6):310-6. doi: 10.1007/BF00296560.

DOI:10.1007/BF00296560
PMID:1394277
Abstract

To reproduce diastematomyelia, a sagittal incision was carried out at the level of the rhomboidal sinus of 36- to 40-h-old chick embryos. A small piece of membrane shell, a small agar screen, or a piece of quail isochronous isotopic notochord was inserted into the gap. The embryos were killed and fixed after 9 days' incubation. Diastematomyelia was obtained in several embryos treated with interposition of a membrane screen or a piece of quail notochord. Microscopic examination revealed two hemicords, each containing its own central canal; in some cases one of the cords showed hydromyelia. Absence of the rump was seen in association with experimental diastematomyelia. The interposition of a resorbable agar screen did not succeed in reproducing diastematomyelia. The results of these surgical manipulations suggest that diastematomyelia cannot be explained by a primary disorder of neurulation. It supports the theory of noninvolution of a firm midline structure (probably the neurenteric canal, rapidly surrounded by mesodermal cells originating from the notochord), which prevents the fusion of the separated parts.

摘要

为了复制脊髓纵裂,在36至40小时龄鸡胚的菱形窦水平进行矢状切口。将一小片膜壳、一个小琼脂屏障或一块鹌鹑同步同位素脊索插入间隙中。孵化9天后处死并固定胚胎。在用膜屏障或一块鹌鹑脊索进行插入处理的几个胚胎中获得了脊髓纵裂。显微镜检查显示有两条半脊髓,每条都含有自己的中央管;在某些情况下,其中一条脊髓显示有脊髓积水。在实验性脊髓纵裂中可见臀部缺失。插入可吸收琼脂屏障未能成功复制脊髓纵裂。这些手术操作的结果表明,脊髓纵裂不能用神经胚形成的原发性障碍来解释。它支持了一个坚实的中线结构(可能是神经肠管,迅速被源自脊索的中胚层细胞包围)未退化的理论,该结构阻止了分离部分的融合。

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A case of spina bifida combined with diastematomyely, the anomaly of Chiari and hydrocephaly.一例脊柱裂合并脊髓纵裂、Chiari畸形和脑积水。
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