• Journal of the korean academy of Pediatric Dentistry
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• v.35 no.1
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• pp.181-185
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• 2008

• 대한치과보철학회:학술대회논문집
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• 2000.04a
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• pp.31-31
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• 2000

• The Journal of the Korean dental association
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• v.22 no.2 s.177
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• pp.94-94
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• 1984

• The Journal of the Korean dental association
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• v.36 no.3 s.346
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• pp.173-175
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• 1998

• Journal of the korean academy of Pediatric Dentistry
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• v.27 no.4
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• pp.499-504
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• 2000

Amelogenesis imperfecta is defined as a genetically determined effect affecting enamel formation and may be associated with other ectodermal or systemic disorders. It is entirely an ectodermal disturbance, since the mesodermal components of the teeth are basically normal. The presentation of diverse clinical manifestations in 1:14,000 to 1:16,000. Classification of the AI types considers mode of inheritance and clinical manifestations. The most widely accepted classification system recognize three major groups; i.e., hypoplastic(thin enamel), hypocalcified(primary mineralization defect), hypomaturation(defect in enamel maturation). The treatment is that at first, genetic counselling must be practiced, and in anterior teeth, composite resin veneer or jacket crown for esthetics, and in posterior teeth, stainlees steel crown or gold onlay.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.29 no.1
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• pp.91-96
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• 2007

Amelogenesis imperfecta (AI) is a hereditary disease that affects enamel formation. The patients with AI have esthetic and functional problems due to damage of multiple teeth. So most AI patients resolve these problem through the conservative and prosthodontic treatments. In our case, It was difficult to obtain good results in means of conservative and prosthodontic treatments, because the AI patient had skeletal Class III malocclusion. Moreover, because of vertical dimension loss due to severe dental caries and maxillofacial skeletal disharmony, the ordinary prosthodontic treatment was troublesome. So we planned orthognathic surgery to resolve these problems. After the endodontic treatment, temporary restoration was delivered for stable post-operative occlusion. Then orthognathic surgery was done, and final restoration was delivered in stable period. We obtained satisfactory results in esthetic and functional aspects through multidisciplinary management(conservative treatment, prosthodontics and orthognathic surgery).

• 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%.

• Journal of dental hygiene science
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• v.8 no.2
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• pp.89-96
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• 2008

Effects of sodium fluoride exposure on the amelogenesis during fetal formation were investigated using 11 days rat incisor of control group and two experimental groups. According to results of morphological analysis using an electron microscope, enamel organ in the rat incisor consisted of presecretory, secretory, and maturation zone, especially maturation zone had ruffle-ended ameloblasts (rAB) that additionally supply inorganic ions and smooth-ended ameloblasts (sAB) that remove water and organic compounds. Such a histological composition was same in fetal and adult rats. According to experimental results using calcein (green fluorescence) in order to reveal the modulation cycle of ameloblast, modulation cycle of experimental group decreased on an average one time than control group, as increase of density of sodium fluoride indicated that thickness of smooth-ended ameloblast decreased. Also ratio of thickness on sagittal total length of sAB increased than rAB in experimental groups than control group. In total length of teeth, an injected 100 ppm sodium fluoride group was similar control group but as injected 200 ppm group became short. In experimental group, thickness of sAB and rAB became narrow to the tip of cutting edge. According to concentration of sodium fluoride grows, the modulation cycle and total length of teeth were decreased, finally it prevented teeth growth.

• Journal of the korean academy of Pediatric Dentistry
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• v.47 no.3
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• pp.344-351
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• 2020

Amelogenesis imperfecta (AI) occurs either in isolation or in association with other dental abnormalities and systemic disorder. A rare syndrome associating AI with nephrocalcinosis was named as Enamel Renal Syndrome (ERS; OMIM #204690). This syndrome is characterized by severe enamel hypoplasia, failed tooth eruption, intra pulpal calcifications, enlarged gingiva, and nephrocalcinosis. Nephrocalcinosis is a condition where calcium salts are deposited in renal tissue, and this may lead to critical kidney complications. This rare syndrome shows pathognomonic oral characteristics that are easily detectable at an early age, which proceeds the onset of renal involvement. Pediatric dentists are the first oral health practitioners whom ERS patients will meet at early age. The role of pediatric dentists is critically important for early diagnosis and referral of patients to both nephrologists for renal assessment and geneticists for identification of causative mutation and diagnosis. Early detection of renal involvement may provide chances to prevent further undesired renal complications.

• International Journal of Oral Biology
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• v.34 no.1
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• pp.21-28
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• 2009

Mutations in DLX3 are associated with both autosomal dominant hypoplastic hypomaturation amelogenesis imperfecta (ADHHAI) and tricho-dento-osseous (TDO) syndrome. ADHHAI is caused by a c.561_562delCT (2bp-del DLX3) mutation whereas TDO syndrome is associated with a c.571_574delGGGG (4bp-del DLX3) mutation. However, although the causal relationships between DLX3 and an enamel phenotype have been established, the pathophysiological role of DLX3 mutations in enamel development has not yet been clarified. In our current study, we prepared expression vectors for wild type and deletion mutant DLX3 products (4bp-del DLX3, 2bp-del DLX3) and examined the effects of their overexpression on the expression of the enamel matrix proteins and proteases. Wild type DLX3 enhanced the expression of matrix metalloprotease 20 (MMP20) mRNA and protein in murine ameloblast-like cells. However, neither a 4bp-del nor 2bp-del DLX3 increased MMP20 expression. Wild type DLX3, but not the above DLX3 mutants, also increased the activity of reporters containing 1.5 kb or 0.5 kb of the MMP20 promoter. An examination of protein stability showed that the half-life of wild type DLX3 protein was less than 12 h whilst that of both deletion mutants was longer than 24 h. Endogenous Dlx3 was also found to be continuously expressed during ameloblast differentiation. Since inactivating mutations in the gene encoding MMP20 are associated with amelogenesis imperfecta, the inability of 4bp-del or 2bp-del DLX3 to induce MMP20 expression suggests a possible involvement of such mutations in the enamel phenotype associated with TDO syndrome or ADHHAI.