Objective To study the manufacture and clinical application of the fixed⁃removable prostheses for hereditary opalescent dentin. Methods The pedigree of a patient suffered from hereditary opalescent dentin found in Nanjing was tracked, and the heredi⁃tary features were assessed. The most appropriate jaw position was established by try⁃in and occlusal adjustment of the temporary pros⁃theses for three months, where the fixed⁃removable prostheses were designed and manufactured. The patient was followed up regularly for five years, and the fixed⁃removable prostheses were evaluated from safety, strength and fitness. The assessment was referred to the improved prostheses evaluation standards by United States Public Health Service. Results Three sequential generations with hereditary opalescent dentin were found in this family, and the prevalence of later generation was 50%, the findings were compatible with an auto⁃somal dominant inheritance. According to the follow⁃up, the patient was satisfied with the masticatory function, fitness, facial form, aesthetics and comfortableness of temporomandibular joint. Clinical examination showed the marginal fitness, gingival health, color sta⁃bility and strength of crowns was satisfactory, and no complications such as fracture, exfoliation, dropping or abutment crown fracture were found. Conclusion The occlusal reconstruction of the fixed⁃removable prostheses for hereditary opalescent dentin is satisfactory.%目的：探讨应用固定－可摘联合修复技术对遗传性乳光牙本质患者进行咬合重建的制作方法及效果评价。方法对南京发现的1例遗传性乳光牙本质患者调查家族史，比对该病的遗传特征。对患者通过3个月暂时性修复体的试戴与咬合调整建立最适下颌位，在此位置进行固定－可摘联合修复体的设计和制作。修复后定期随访5年，可摘局部义齿主要从安全性、强度、适合性三方面进行评价。固定义齿参照改良的美国公共卫生署评价标准对修复体的临床修复效果进行综合评价。结果该患者家族中遗传性乳光牙本质连续三代出现，子代患病率50％，符合常染色体显性遗传特征。该患者进行固定－可摘联合修复后主观感觉咀嚼功能提高、舒适性良好、面型改善、恢复美观、无颞下颌关节不适。修复体检查结果显示固定部分边缘密合，牙龈颜色形态自然，无牙龈炎，修复体色泽自然逼真，未出现破裂、崩瓷、脱落、基牙冠折等并发症。结论采用固定－可摘联合修复技术治疗遗传性乳光牙本质患者，进行咬合重建的效果令人满意。
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