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联体双胎:对胚胎发育的影响

Conjoined twins: implications for blastogenesis.

作者信息

Machin G A

机构信息

Department of Pathology, University of Alberta, Edmonton, Canada.

出版信息

Birth Defects Orig Artic Ser. 1993;29(1):141-79.

PMID:8280870
Abstract

It is difficult to draw sweeping general conclusions about the blastogenesis of CT, principally because so few thoroughly studied cases are reported. It is to be hoped that methods such as painstaking gross or electronic dissection will increase the number of well-documented cases. Nevertheless, the following conclusions can be proposed: 1. Most CT can be classified into a few main anatomic types (or paradigms), and there are also rare transitional types that show gradation between the main types. 2. Most CT have two full notochordal axes (Fig. 5); the ventral organs induced along these axes may be severely disorientated, malformed, or aplastic in the process of being arranged within one body. Reported anatomic types of CT represent those notochordal arrangements that are compatible with reasonably complete embryogenesis. New ventro-lateral axes are formed in many types of CT because of space constriction in the ventral zones. The new structures represent areas of "mutual recognition and organization" rather than "fusion" (Fig. 17). 3. Orientations of the pairs of axes in the embryonic disc can be deduced from the resulting anatomy. Except for dicephalus, the axes are not side by side. Notochords are usually "end-on" or ventro-ventral in orientation (Fig. 5). 4. A single gastrulation event or only partial duplicated gastrulation event seems to occur in dicephalics, despite a full double notochord. 5. The anatomy of diprosopus requires further clarification, particularly in cases with complete crania rather than anencephaly-equivalent. Diprosopus CT offer the best opportunity to study the effects of true forking of the notochord, if this actually occurs. 6. In cephalothoracopagus, thoracopagus, and ischiopagus, remarkably complete new body forms are constructed at right angles to the notochordal axes. The extent of expression of viscera in these types depends on the degree of noncongruity of their ventro-ventral axes (Figs. 4, 11, 15b). 7. Some organs and tissues fail to develop (interaction aplasia) because of conflicting migrational pathways or abnormal concentrations of morphogens in and around the neoaxes. 8. Where the cardiovascular system is discordantly expressed in dicephalus and thoracopagus twins, the right heart is more severely malformed, depending on the degree of interaction of the two embryonic septa transversa. 9. The septum transversum provides mesenchymal components to the heawrt and liver; the epithelial components (derived fro the foregut[s]) may vary in number from the number of mesenchymal septa transversa contributing to the liver of the CT embryo.(ABSTRACT TRUNCATED AT 400 WORDS)

摘要

关于联体双胎的胚胎发育过程,很难得出具有广泛适用性的一般性结论,主要原因是已报道的经过充分研究的病例极少。希望诸如细致的大体解剖或电子解剖等方法能够增加记录完备的病例数量。尽管如此,仍可提出以下结论:1. 大多数联体双胎可分为几种主要的解剖类型(或模式),也有罕见的过渡类型,显示出主要类型之间的渐变。2. 大多数联体双胎有两条完整的脊索轴(图5);沿这些轴诱导形成的腹侧器官在排列于一个体内的过程中可能严重错位、畸形或发育不全。已报道的联体双胎解剖类型代表了那些与合理完整的胚胎发育过程相兼容的脊索排列方式。由于腹侧区域的空间受限,许多类型的联体双胎中会形成新的腹外侧轴。这些新结构代表“相互识别和组织”的区域,而非“融合”区域(图17)。3. 胚胎盘中轴对的方向可从最终的解剖结构推断出来。除双头联体双胎外,这些轴并非并列。脊索的方向通常是“端对端”或腹对腹(图5)。4. 尽管有完整的双脊索,但双头联体双胎似乎只发生了一次原肠胚形成事件或仅部分重复的原肠胚形成事件。5. 双面联体双胎的解剖结构需要进一步阐明,特别是在具有完整颅骨而非无脑儿等效情况的病例中。如果脊索真的发生了真正的分叉,双面联体双胎为研究其影响提供了最佳机会。6. 在头胸联体双胎、胸腹联体双胎和坐骨联体双胎中,会以与脊索轴成直角的方式构建出非常完整的新身体形态。这些类型中内脏的表达程度取决于其腹腹轴的不一致程度(图4、11、15b)。7. 由于新轴内和周围迁移途径相互冲突或形态发生素浓度异常,一些器官和组织无法发育(相互作用发育不全)。8. 在双头联体双胎和胸腹联体双胎中,当心血管系统表达不一致时,右心畸形更严重,这取决于两个胚胎横隔的相互作用程度。9. 横隔为心脏和肝脏提供间充质成分;上皮成分(源自前肠)的数量可能与为联体双胎胚胎肝脏提供成分的间充质横隔数量不同。(摘要截选至400字)

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