Journal of the Korean Academy of Pediatric Dentistry 2000;27(1):1-6.
Published online February 29, 2000.
A CASE REPORT OF DENTINOGENESIS IMPERFECTA
 
상아질 형성 부전증에 대한 증례보고
김재곤, 이두철, 이승영, 이승익, 백병주
전북대학교 치과대학 소아치과학교실 및 구강생체과학연구소
Abstract
Dentinogenesis imperfecta is an inherited disorder of dentin formation, usually exhibiting an autosomal dominant mode of transmission. Type I dentinogenesis imperfecta occurs in patients afflicted with osteogenesis imperfecta. Type II dentinogenesis imperfecta is not associated with osteogenesis imperfecta. Type III dentinogenesis imperfecta (Brandywine type) occurs in a racial isolate area in the state of Maryland. In all three types, teeth of both dentitions are affected with variable clinical appearances. The teeth are opalescent with the color ranging from bluish-gray to brown to yellowish. The dentin is abnormally soft, providing inadequate functional support to the overlying enamel. Although the enamel is normal, it fractures or chips away easily, exposing the occlusal and incisal dentin. The exposed soft dentin often undergoes rapid and severe functional attrition. The teeth exhibit bulb-shaped crowns with constricted cementoenamel junctions and thin roots. The teeth will exhibit varying stages of obliteration of the coronal and root pulpal chambers. The cementum, periodontal ligament and supporting alveolar bone appear normal. The enamel is normal. The mantle dentin remains nearly normal, whereas the remaining dentin is severely dysplastic. The dentinal tubules are disoriented, irregular, widely spaced, and usually larger than normal.
Key Words: Dentinogenesis imperfecta, Osteogenesis impefecta, Enamel, Dentinal tubule


ABOUT
BROWSE ARTICLES
EDITORIAL POLICY
FOR CONTRIBUTORS
Editorial Office
Seoul National University, Dental Hospital, B1-166 101, Daehak-ro, Jongno-gu, Seoul 03080, Republic of Korea
Tel: +82-70-4145-8875    Fax: +82-2-745-8875    E-mail: info@kapd.org                

Copyright © 2024 by Korean Academy of Pediatric Dentistry.

Developed in M2PI

Close layer
prev next