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牙体硬组织发育异常是非龋性疾病的重要内容，主要包括釉质发育不全和牙本质发育异常。这些疾病如果得不到及时有效的干预和治疗，往往会造成牙体组织敏感、缺损，甚至牙缺失，影响咀嚼功能和面部美观。目前很多口腔医师对此类疾病认识不足，亟需全面关注和重视此类疾病和患病人群。本文在总结目前研究进展的基础上，重点对此类疾病的临床分类、疾病表型及突变基因和致病机制等进行阐述和分析，以期为广大口腔医师和患病人群提供帮助。“,”Developmental disorders of dental hard tissues are important components of non-carious diseases, which mainly include amelogenesis imperfecta and hereditary dentin disorders with various subtypes. In the absence of effective intervention, these disorders would lead to tooth sensitivity, defects of tooth structure or even loss of tooth, affecting the masticatory function and facial aesthetic configuration. At present, many dental clinicians may not have sufficient understanding of the diseases, and it is urgent to pay attention to the diseases per se and the patients affected. Based on the summary of the current research progresses, this article focuses on the clinical classification, the disease phenotype and the pathogenesis of gene mutations, in order to provide reference and help for the dental clinicians as well as the patients.