Waltimo J
Department of Pedodontics and Orthodontics, University of Helsinki, Finland.
J Oral Pathol Med. 1994 Oct;23(9):389-93. doi: 10.1111/j.1600-0714.1994.tb00082.x.
Dentin matrix of deciduous teeth from two patients affected by dentinogenesis imperfecta (DI) associated with types IB and IVB osteogenesis imperfecta (OI) displayed previously undescribed structures in transmission electron microscopic examination. Vesicles were seen in dentin of both patients, and abnormally thick collagen fibers (hyperfibers) were found in dentin of the patient with the rare type IB OI. Both vesicles and hyperfibers were situated in abnormal, atubular areas of dentin. Matrix vesicles, which have normally been identified in mantle dentin only, were abundant in selected areas of the affected dentin, thereby supporting the concept that dentin matrix in OI is elaborated by successive cell generations. The hyperfibers, not previously described in either normal or abnormal human dentin, have possibly been formed by fusion of several collagen fibers. Further ultrastructural studies of dentin in DI with OI may help to clarify the marked clinical variation in teeth of patients affected by OI.
在透射电子显微镜检查中，两名患有与IB型和IVB型成骨不全症（OI）相关的牙本质生成不全（DI）的患者的乳牙牙本质基质呈现出先前未描述的结构。在两名患者的牙本质中均可见到囊泡，并且在患有罕见的IB型OI的患者的牙本质中发现了异常粗大的胶原纤维（超纤维）。囊泡和超纤维均位于牙本质的异常无小管区域。通常仅在罩牙本质中鉴定出的基质小泡在受影响牙本质的选定区域中大量存在，从而支持了OI中的牙本质基质是由连续几代细胞形成的这一概念。超纤维在正常或异常人类牙本质中均未曾描述过，可能是由几根胶原纤维融合形成的。对患有OI的DI患者的牙本质进行进一步的超微结构研究可能有助于阐明OI患者牙齿中明显的临床差异。
