• Journal of the korean academy of Pediatric Dentistry
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• v.33 no.2
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• pp.323-328
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• 2006

Dentinogenesis imperfecta is an inheritable disease of dentinal defect, generally is inherited as a single autosomal dominant trait. It has a prevalence of 1 in 8000 with the trait, and no significant difference between male and female, with involvement of the primary and permanent teeth. Shields proposed three types of Dentinogenesis imperfecta. Affected teeth have various discoloration, separation of enamel rapid destruction of underlying dentin, and severe attrition. Radiographically, the teeth have cervical constriction, bulbous crown, thin root, obliteration of the root canals and pulp chambers, and periapical lesions in a sound tooth. The objective of treatment is rehabilitation of the esthetics, the masticatory function, and the vertical dimension of occlusion. In these cases, two pediatric patients reported to the Kyungpook University Pediatric clinic, with a chief complaint of discolored teeth and severe attrition. As a result of clinical and radiographic exam, it was diagnosed as Dentinogenesis imperfecta. The posterior teeth were restored with Stainless Steel Crown, and the anterior teeth were restored with composite resin veneering.

• Journal of Korean Academy of Oral and Maxillofacial Radiology
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• v.22 no.2
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• pp.215-219
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• 1992

The author ovserved a case of dentinogenesis imperfecta in a 8-year-old and a 9-year-old brother with complaints of abnormal morphologic changes of the teeth. 1. Clinically, yellowish brown colored teeth, fractured crown and numerous retained root tips of deciduous teeth with severe attrition were observed in the both deciduous and permanent dentitions. 2 Radiographically, small-sized teeth, partial or complete obliteration of the pulp chambers and pulp canals in the anterior teeth, partial obliteration of the pulp chambers and thin enamel in the permanent tooth germs were observed. 3. The familial history was their father has been lose his teeth early.

• Journal of Yeungnam Medical Science
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• v.4 no.1
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• pp.157-163
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• 1987

Osteogenesis imperfecta has been categorized as a heritable disorder of connective tissue affecting both bone and soft tissues and is characterized by fragility of bone, blue sclerae, and deafness, less frequently by dentinogenesis imperfecta and laxity of ligament. The goals of orthopedic management with osteogenesis imperfecta are the treatment of acute fractures and long-term rehabilitation in an effort to maintain ambulation. We report a case of osteogenesis imperfecta with anterior bowing of tibia which was successfully corrected by multiple osteotomies and intramedullary fixation by Sofield method and reviewed literatures.

• Journal of the korean academy of Pediatric Dentistry
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• v.48 no.2
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• pp.237-244
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• 2021

Dentinogenesis imperfecta (DI) is a hereditary disorder of dentinal defect. It is generally inherited as a single autosomal dominant trait. DI usually affects both the primary and permanent dentition. Affected teeth have various types of discolorations, rapid destruction of the dentin, and severe attrition. In radiologic view, the affected teeth have bulbous crowns, short roots and narrow or closed pulp chambers. The treatment objective is to prevent additional attrition and recover the vertical dimension of occlusion. The aim of this report was to present the long-term prognosis in 15 years in a pair of siblings. Both the patients had DI with tooth attrition and discoloration. Different treatment procedures were used, depending on the difference in the timing of intervention. The first patient saved most of his teeth. The second patient had all of her teeth extracted. This report could be helpful for early diagnosis and overall treatment of DI.

• The Journal of Korean Academy of Prosthodontics
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• v.52 no.4
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• pp.317-323
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• 2014

Dentinogenesis Imperfecta, with a high incidence rate of 1 : 6 - 8000, is inherited by autosomal dominant genetic transmission. This dental disorder causes discoloration of the teeth and the enamel and dentin show hypoplastic or hypocalcified defects which lead to frequent fractures and rapid attrition. Therefore, timely treatment is necessary for the preservation of the remaining teeth. In this particular case, a 19-year-old patient suffering from Type 1 dentinogenesis imperfecta showed signs of brownish hued teeth with multiple fractures, a loss of vertical dimension, excessive interdental space in the maxillary anterior teeth, and a lack of 5 posterior teeth. To improve the esthetic appearance of the anterior teeth, the vertical dimension was increased. Resin caps were used to alleviate the difficulty of taking an impression of multiple teeth at once. Monolithic zirconia materials used in this case showed high fracture strength and the ability to mask the discoloration of the teeth and therefore, functionally and esthetically favorable results were achieved.

• Journal of the korean academy of Pediatric Dentistry
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• v.44 no.2
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• pp.164-169
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• 2017

Dentinogenesis imperfecta type II (DGI-II) is an inherited disorder affecting the dentin matrix and is related to mutations in the dentin sialophosphoprotein (DSPP) gene. The protein encoded by the DSPP gene undergoes extensive posttranslational modifications. Dentin phosphoprotein (DPP), one of the DSPP expressed products, has unique composition with highly repetitive Asp-Ser-Ser amino acid residues and is related to the maturation of dentin mineralization. We aimed to identify mutation in DSPP, including the DPP coding region, contributing to inherited dentin defects in a Korean family with DGI-II. Clinical and radiographic examinations were performed, and all five exons and exon-intron boundaries of the DSPP gene were sequenced. Additionally, allele-specific cloning for highly repetitive DPP region was performed. By sequencing and cloning, a heterozygous single nucleotide deletion (c.2688delT) was identified. The identified mutation caused a frameshift in the DPP coding region. This frameshift mutation would introduce hydrophobic amino acids instead of hydrophilic amino acids and would result in a change in the characteristics of DPP.

• Journal of the korean academy of Pediatric Dentistry
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• v.21 no.2
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• pp.599-604
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• 1994

Very few reports are available on the occurrence of abnormality of both enamel and dentin. This case has some characteristiced of both amelogenesis imperfecta and dentinogenesis imperfecta. Clinically, the enamel of primary dentition was completely absent and when the permanent teeth came to the pediatric dept. of Dankook University Dental Hopital for treatment. Fixed-removable type resin plate was delivered to increase vertical dimension and to solve esthetic and functional defects. Hypoplastic teeth were restored either stainless steel crown or composite resin restoration or both. The periodic recall check and oral hygiene education are recommended.

• Imaging Science in Dentistry
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• v.44 no.1
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• pp.7-13
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• 2014

Purpose: This study was performed to evaluate the prevalence of all types and subtypes of dental anomalies among 7- to 35-year-old patients by using panoramic radiographs. Materials and Methods: This cross-sectional study was conducted on 1649 people in Hamadan City, in 2012-2013. The prevalence of four types and 12 subtypes of dental anomalies was evaluated by two observers separately by using panoramic radiography. Dental anomalies were divided into four types: (a) shape (including fusion, taurodontism, and dens invagination); (b) number (including hypodontia, oligodontia, and hyperdontia); (c) structure (including amelogenesis imperfecta, dentinogenesis imperfecta, and dentin dysplasia); and (d) position (including displacement, impaction, and dilacerations). Results: The reliability between the two observers was 79.56% according to the Kappa statistics. The prevalence of dental anomalies diagnosed by panoramic radiographs was 29%. Anomalies of position and number were the most common types of abnormalities, and anomalies of shape and structure were the least in both genders. Anomalies of impaction (44.76%), dilacerations (21.11%), hypodontia (15.88%), taurodontism (9.29%), and hyperdontia (6.76%) were the most common subtypes of dental anomalies. The anomalies of shape and number were more common in the age groups of 7-12 years and 13-15 years, respectively, while the anomalies of structure and position were more common among the other age groups. Conclusion: Anomalies of tooth position were the most common type of dental anomalies, and structure anomalies were the least in this Iranian population. The frequency and type of dental anomalies vary within and between populations, confirming the role of racial factors in the prevalence of dental anomalies.

• BMB Reports
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• v.42 no.2
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• pp.86-90
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• 2009

Deletion of smpd3 induces osteogenesis and dentinogenesis imperfecta in mice. smpd3 is highly elevated in the parietal bones of developing mouse calvaria, but not in sutural mesenchymes. Here, we examine the mechanism of smpd3 regulation, which involves BMP2 stimulation of Runx2. smpd3 mRNA expression increased in response to BMP2 treatment and Runx2 transfection in C2C12 cells. The Runx2-responsive element (RRE) encoded within the -562 to -557 region is important for activation of the smpd3 promoter by Runx2. Electrophoretic mobility shift assays revealed that Runx2 binds strongly to the -355 to -350 RRE and less strongly to the -562 to -557 site. Thus, the smpd3 promoter is activated by BMP2 and is directly regulated by the Runx2 transcription factor. This novel description of smpd3 regulation will aid further studies of bone development and osteogenesis.

• Journal of the korean academy of Pediatric Dentistry
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• v.35 no.2
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• pp.313-318
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• 2008

Dens invaginatus is a rare malformation resulting from invagination of the enamel before calcification has occurred. It is mostly found in permanent maxillary lateral incisors and mandibular teeth are rarely affected by this anomaly. The malformation is estimated to affect between 0.04 % and 10 % of people and has been associated with other abnormalities such as taurodontism, microdontia, gemination and dentinogenesis imperfecta. Dens invaginatus is classified in three types with respect to the depth of invaginatus and has a broad spectrum of morphologic variations. Invagination frequently allows the entry of irritants and microorganism, which usually lead to caries, pulp infection and pulp necrosis. Root canal treatment on such invaginatus tooth may present severe problems because of its complex anatomy of the tooth. Therefore, the early diagnosis of such malformation is crucial and preventive approach is strongly recommended.