Waltimo J, Ranta H, Lukinmaa P L
Department of Pedodontics, Institute of Dentistry, University of Helsinki, Finland.
Scanning Microsc. 1995 Mar;9(1):185-97; discussion 197-8.
Heritable dentin defects form a group of diseases which exclusively affect dentin among the various dental tissues. While one type is associated with the generalized connective tissue disorder, osteogenesis imperfecta, other types occur as single traits. The clinical manifestations of the dentin defects vary from insignificant to severe enough to cause aesthetical and functional failure of the teeth. Scanning and transmission electron microscopic studies, reviewed in this paper, have markedly clarified the ultrastructure of the aberrant dentin matrix. Both similar and different changes seem to occur in the various forms of heritable dentin defects. Abnormalities in the appearance and organization pattern of collagen fibers in the defective dentin partly resemble those observed in skin in generalized connective tissue diseases. The similarity of ultrastructural findings in dentin defects, which are currently classified as distinct entities, and even in diseases affecting other tissues, could be related to the complicated interactions between the extracellular matrix macromolecules. Thus, many of the changes observed may be secondary in nature. Ultrastructural studies can help us to understand the pathogenesis of the different types of heritable dentin defects as well as aid in diagnostics and classification of these diseases.
遗传性牙本质缺陷是一组仅影响多种牙体组织中牙本质的疾病。其中一种类型与全身性结缔组织疾病成骨不全相关，而其他类型则表现为单一性状。牙本质缺陷的临床表现从轻微到严重不等，严重时可导致牙齿的美观和功能丧失。本文回顾的扫描电镜和透射电镜研究显著阐明了异常牙本质基质的超微结构。在各种形式的遗传性牙本质缺陷中似乎都出现了相似和不同的变化。缺陷牙本质中胶原纤维外观和组织模式的异常部分类似于全身性结缔组织疾病中皮肤所观察到的情况。目前被归类为不同实体的牙本质缺陷，甚至在影响其他组织的疾病中，超微结构发现的相似性可能与细胞外基质大分子之间复杂的相互作用有关。因此，观察到的许多变化可能本质上是继发性的。超微结构研究有助于我们理解不同类型遗传性牙本质缺陷的发病机制，也有助于这些疾病的诊断和分类。
