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缝生物学与颅缝早闭的相关因素

Sutural biology and the correlates of craniosynostosis.

作者信息

Cohen M M

机构信息

Department of Oral Biology, Faculties of Dentistry, Dalhousie University, Halifax, Nova Scotia, Canada.

出版信息

Am J Med Genet. 1993 Oct 1;47(5):581-616. doi: 10.1002/ajmg.1320470507.

DOI:10.1002/ajmg.1320470507
PMID:8266985
Abstract

The purpose of this paper is to provide a new perspective on craniosynostosis by correlating what is known about sutural biology with the events of craniosynostosis per se. A number of key points emerge from this analysis: 1) Sutural initiation may take place by overlapping, which results in beveled sutures, or by end-to-end approximation, which produces nonbeveled, end-to-end sutures. All end-to-end sutures occur in the midline (e.g., sagittal and metopic) probably because embryonic biomechanical forces on either side of the initiating suture tend to be equal in magnitude. A correlate appears to be that only synostosed sutures of the midline have pronounced bony ridging. 2) Long-term histologic observations of the sutural life cycle call into question the number of layers within sutures. The structure varies not only in different sutures, but also within the same suture over time. 3) Few, if any, of the many elegant experimental research studies in the field of sutural biology have increased our understanding of craniosynostosis per se. An understanding of the pathogenesis of craniosynostosis requires a genetic animal model with primary craniosynostosis and molecular techniques to understand the gene defect. This may allow insight into pathogenetic mechanisms involved in primary craniosynostosis. It may prove to be quite heterogeneous at the basic level. 4) The relationship between suture closure, cessation of growth, and functional demands across sutures poses questions about various biological relationships. Two conclusions are provocative. First, cessation of growth does not necessarily, or always lead to fusion of sutures. Second, although patent sutures aid in the growth process, some growth can take place after suture closure. 5) In an affected suture, craniosynostosis usually begins at a single point and then spreads along the suture. This has been shown by serial sectioning and calls into question results of studies in which the affected sutures are only histologically sampled. 6) Craniosynostosis is etiologically and pathogenetically heterogeneous. Known human causes are reviewed. Is craniosynostosis simply normal suture closure commencing too early?(ABSTRACT TRUNCATED AT 400 WORDS)

摘要

本文的目的是通过将已知的缝合生物学知识与颅缝早闭本身的事件相关联,为颅缝早闭提供一个新的视角。从这一分析中得出了一些关键点:1)缝合起始可能通过重叠发生,这会导致倾斜的缝合线,或者通过端对端近似发生,这会产生非倾斜的端对端缝合线。所有端对端缝合线都出现在中线(例如矢状缝和额缝),可能是因为起始缝合线两侧的胚胎生物力学力在大小上趋于相等。一个相关的现象似乎是,只有中线的融合缝合线有明显的骨嵴。2)对缝合线生命周期的长期组织学观察对缝合线内的层数提出了质疑。其结构不仅在不同的缝合线中有所不同,而且在同一缝合线内也会随时间变化。3)在缝合生物学领域,很少有(如果有的话)优雅的实验研究能增进我们对颅缝早闭本身的理解。对颅缝早闭发病机制的理解需要一个具有原发性颅缝早闭的遗传动物模型以及分子技术来理解基因缺陷。这可能有助于深入了解原发性颅缝早闭所涉及的发病机制。在基础层面上,它可能证明是相当异质性的。4)缝合线闭合、生长停止以及跨缝合线的功能需求之间的关系引发了关于各种生物学关系的问题。有两个结论很有启发性。第一,生长停止不一定,也不总是导致缝合线融合。第二,虽然开放的缝合线有助于生长过程,但在缝合线闭合后仍可发生一些生长。5)在受影响的缝合线中,颅缝早闭通常从一个点开始,然后沿着缝合线扩散。这已通过连续切片得到证实,并对那些仅对受影响缝合线进行组织学取样的研究结果提出了质疑。6)颅缝早闭在病因和发病机制上是异质性的。对已知的人类病因进行了综述。颅缝早闭仅仅是过早开始的正常缝合线闭合吗?(摘要截断于400字)

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