Song Jing'e, Nan Yan, Zhang Rui
Dept. of Pediatric Dentistry, Hospital of Stomatology, Lanzhou University, Lanzhou 730000, China.
Hua Xi Kou Qiang Yi Xue Za Zhi. 2025 Aug 1;43(4):530-533. doi: 10.7518/hxkq.2025.2025068.
Dentinogenesis imperfecta is a dentin development disorder inherited in an autosomal dominant manner. It is rarely seen in clinical with a low incidence rate. We reported a case of dentinogenesis imperfecta referred to the Department of Pediatric Dentistry, Hospital of Stomatology, Lanzhou University. Investigation of the four-generation pedigree of the proband and review of relevant literature indicated that dentinogenesis imperfecta equally affects both genders and involves deciduous and permanent teeth with a high familial prevalence. By analyzing the clinical manifestations of dentinogenesis imperfecta and exploring early management strategies, this case study aims to enhance dentists' understan-ding and management of this condition to improve patients' quality of life.
牙本质发育不全是一种以常染色体显性方式遗传的牙本质发育障碍。临床上很少见,发病率较低。我们报告了一例转诊至兰州大学口腔医院儿童牙科的牙本质发育不全病例。对先证者的四代家系进行调查并复习相关文献表明,牙本质发育不全对男女的影响相同,累及乳牙和恒牙,家族患病率较高。通过分析牙本质发育不全的临床表现并探索早期管理策略,本病例研究旨在提高牙医对这种疾病的认识和管理能力,以改善患者的生活质量。