• The Journal of the Korean dental association
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• v.46 no.7
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• pp.440-445
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• 2008

Dentinogenesis imperfecta is one of the most common autosomal dominant traits experienced in humans. It generally affects both the deciduous and permanent dentitions. There are 3 forms of dentinogenesis imperfecta that have been classified into type I(osteogenesis imperfecta associated), type II(hereditary opalescent dentin), and type III(Brandywine isolate opalescent dentin).1,2 The prevalence for all types of dentinogenesis imperfecta is approximately 1 per 8000 subjects. Early diagnosis and treatment are therefore, fundamental, aiming at obtaining a favourable prognosis since late intervention makes treatment more complex. This clinical report describes a treatment solution to the problems encountered by a dentinogenesis imperfecta patient with minimally invasive techniques.

• Imaging Science in Dentistry
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• v.47 no.2
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• pp.129-133
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• 2017

Dentinogenesis imperfecta is a dominant autosomal hereditary disorder of dentin formation that affects the deciduous and permanent teeth. Its etiology is characterized by inadequate cell differentiation during odontogenesis. The clinical characteristics of dentinogenesis imperfecta are discolored teeth with a translucency that varies from gray to brown or amber. Radiographically, the teeth exhibit pulp obliteration, thin and short roots, bell-shaped crowns, and periapical bone rarefaction. The aim of this report was to present a case of dentinogenesis imperfecta type II that was followed up over a 17-year period. This report also presents scanning electron microscopy images of the enamel and dentin, showing that both were altered in the affected teeth. The disease characteristics and the treatments that were administered are reported in this study to guide dentists with respect to the need for early diagnosis and adequate follow-up to avoid major sequelae.

• Journal of the korean academy of Pediatric Dentistry
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• v.27 no.1
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• pp.1-6
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• 2000

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.

• Journal of the korean academy of Pediatric Dentistry
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• v.48 no.4
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• pp.376-383
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• 2021

The aim of this study was to determine the prevalence and incidence and evaluate the current status of dental treatment of Amelogenesis imperfecta (AI) and Dentinogenesis imperfecta (DI) in South Korea. The data was based on National Health Insurance Service (NHIS)-National Sample Cohort Database (2002 - 2015) and Jeonbuk National University (JBNU) Dental Hospital (2011 - 2020). The NHIS data analysis showed prevalence of AI and DI were 11.6 and 2.4 per 100,000 people, respectively. The annual incidence of AI and DI for 2013 - 2015 were 2.2 and 0.5 per 100,000. There were no statistically significant differences regarding the number of visits, the reimbursable cost among AI, DI patients and others. In the patient analysis of the JBNU dental hospital, proportion of the reimbursable and non-reimbursable cost for AI patients were 12.1% and 87.9%, while DI patients accounted for 18.6% and 81.4%.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.14 no.1
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• pp.31-35
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• 2018

Dentinogenesis imperfecta is a hereditary disorder that causes abnormal dentin formation and tooth morphology. Affected teeth show very thin dentin and large pulp chamber. Immediately after their eruption, the teeth tend to be destroyed rapidly. Early dental intervention is recommended to improve the feeding capacity and quality of life for the child, to maintain vertical dimension, and to promote healthy and functional dentition. This case report describes a treatment for a 4-year-2-month-old girl whose primary and permanent dentition were affected by Dentinogenesis Imperfecta. Shell teeth were observed in the entire primary dentition. The pulp of most teeth was exposed and the patient complained of pain. Most paternal family members were transferred to the Dentinogenesis Imperfecta. She needed an extensive dental treatment, but her compliance could not be expected because she was very young and anxious. So we decided to treat her under general anesthesia. All molar teeth are restored with Stainless Steel crown. Maxillary anterior teeth were extracted and mandibular anterior teeth are restored with composite resin. The patient's pain disappeared and masticatory function was restored.

• Journal of the korean academy of Pediatric Dentistry
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• v.28 no.4
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• pp.654-660
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• 2001

Dentinogenesis imperfecta is an example of an inheritable dentinal defect originating during the histodifferentiation stage of tooth development, with involvement of the primary and permanent teeth. Shields, Bixler and El-Kafrawy proposed three types of Dentinogenesis imperfecta : Type I, II, III. Witkop reported a prevalence of 1 in 8000 with the trait, and no significant difference between male and female. Affected teeth have red-brown discoloration often with distinctive wearness of occlusal surface of posterior teeth and incisal surface of anterior teeth. Once enamel seperated from underlying defective dentin, the dentin demonstrates significantly acclerated attrision. Radiographically, the teeth have thin roots, bulbous crown, cervical constriction, and obliteration of the root canals and pulp chambers. In primary dentition periapical lesions or multiple root fractures are often observed. In successive generations the phenotypes of discoloration and wearness of teeth occurred, and one of the patient's subships, 10 year-old sister, showed general discoloration of her teeth and mild wearness. In this case, a 4 year-old male reported to the Yonsei University Pedodontics clinic, with a chief complaint of discolored teeth. The teeth showed generally yellowish-brown discoloration and moderate wearness. In radiographic features, obliteration of pulp, bulbous crown, and short roots were observed. It was diagnosed as Dentinogenesis imperfecta. The posterior teeth were restored with Stainless Steel Crown, and defective incisors including left upper primary central incisor which was extracted due to a root fracture with Open-faced Stainless Steel crown.

• Proceedings of the KACD Conference
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• 2007.05a
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• pp.277-277
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• 2007

• Journal of the korean academy of Pediatric Dentistry
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• v.33 no.4
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• pp.738-742
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• 2006

• The Journal of the Korean dental association
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• v.22 no.3 s.178
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• pp.184-184
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• 1984

• The Journal of the Korean dental association
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• v.37 no.6 s.361
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• pp.402-406
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• 1999