牙本质发育不全的硬组织研究与进展

背景：牙本质发育不全是一种牙本质发育异常的常染色体显性遗传病,目前的研究大多集中在致病基因和临床治疗上,硬组织方面的研究相对较少。目的：就牙本质发育不全的硬组织研究进展做一综述。方法：以＂牙本质发育不全,表面形态,动物模型,釉牙本质界,牙本质小管＂为关键词,应用计算机检索1999/2011CNKI数据库、PubMed数据库,OVID数据库。结果与结论：多数学者研究发现牙本质发育不全的釉质结构正常,病变主要表现在釉牙本质界和牙本质。其釉牙本质界大多表现为直线型外观,牙本质结构紊乱,钙化不规则,牙本质小管数目减少,胶原纤维形态和排列异常。这些异常结构的成因尚不清楚,有待深入研究。

Research and progress of dental hard tissues with dentinogenesis imperfecta

BACKGROUND：Dentinogenesis imperfecta is an autosomal dominant genetic disease with dysplastic dentin.Currently,most studies on dentinogenesis imperfecta are focused on phenotype analysis and clinical therapy,while the reports on dental hard tissue of dentinogenesis imperfecta are rare.OBJECTIVE：To summarize the research progress of dental hard tissue with dentinogenesis imperfecta.METHODS：A computer-based online search of CNKI,PubMed and OVID databases was performed for related articles published between 1999 and 2011,with the keywords of ＂dentinogenesis imperfecta,surface morphology,animal model,enamel dentinal junction,dentinal tubule＂ in English and Chinese.RESULTS AND CONCLUSION：Most researchers recognize that the enamel of dentinogenesis imperfecta has normal structure.The lesions are located in enamel dentinal junction.The enamel dentinal junction represents a linear appearance.The dentin represents a structure disturbance with irregular calcification.The number of dentinal tubules reduces.The shape and arrangement of collagen fibers are abnormal.The reasons for the abnormal structures are not clear and need further research.