华西口腔医学杂志 ›› 2025, Vol. 43 ›› Issue (4): 530-533.doi: 10.7518/hxkq.2025.2025068

• 儿童口腔医学诊疗新进展 • 上一篇    下一篇

遗传性乳光牙本质1例及文献回顾

宋经娥(), 南燕, 张瑞()   

  1. 兰州大学口腔医院儿童口腔科,兰州 730000
  • 收稿日期:2025-02-24 修回日期:2025-05-21 出版日期:2025-08-01 发布日期:2025-08-29
  • 通讯作者: 张瑞 E-mail:songje2023@lzu.edu.cn;zhangrui@lzu.edu.cn
  • 作者简介:宋经娥,主治医师,硕士,E-mail:songje2023@lzu.edu.cn
  • 基金资助:
    兰州大学口腔医学院(口腔医院)科研基金(lzukqky-2023-t05)

Case report of dentinogenesis imperfecta and review of literature

Song Jing’e(), Nan Yan, Zhang Rui()   

  1. Dept. of Pediatric Dentistry, Hospital of Stomatology, Lanzhou University, Lanzhou 730000, China
  • Received:2025-02-24 Revised:2025-05-21 Online:2025-08-01 Published:2025-08-29
  • Contact: Zhang Rui E-mail:songje2023@lzu.edu.cn;zhangrui@lzu.edu.cn
  • Supported by:
    Research Fund of School/Hospital of Stomatology, Lanzhou University(lzukqky-2023-t05)

摘要:

遗传性乳光牙本质是一种常染色体显性遗传性牙本质发育缺陷疾病,发病率低,临床罕见。本文报道1例就诊于兰州大学口腔医院儿童口腔科的遗传性乳光牙本质的病例,对先证者的4代家系谱进行调查并进行相关文献回顾,发现遗传性乳光牙本质男女均可发病,乳恒牙均可受累,家族发病率高。通过分析遗传性乳光牙本质的临床表现,探讨早期管理策略,以期提高临床医师对遗传性乳光牙本质的认识和管理,提高患者的生活质量。

关键词: 遗传性乳光牙本质, 常染色体显性遗传, 牙本质发育疾病

Abstract:

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.

Key words: dentinogenesis imperfecta, autosomal dominant inheritance, dentin development disorder

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