• Journal of the korean academy of Pediatric Dentistry
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• v.26 no.3
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• pp.492-498
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• 1999

Oculodentodigital syndrome(ODD) was first reported by Lohmann in 1920 and termed by Meyer Schwicketath, which they called "dysplasia oculo-dento-digitalis" in 1957. It is somewhat rare heritable disease. ODD is generally inherited in an autosomal dominant pattern with a complex phenotype. The characteristic features are : (1) unique facial features, (2) microphthalmos, (3) syndactyly and camptodactyly of 4th and 5th fingers, (4) osseous anomalies of the middle phalanges of 5th fingers and toes, (5) enamel hypoplasia, (6) dry lusterless hair. We found several occlusal wearing and yellow discoloration of succedaneous teeth, multiple caries lesions, premature loss and pulpal involvement of primary teeth with associated enamel abnormalities caused by generalized enamel hypoplasia in a fairly constant oral finding. Occasionally partial anodontia, microdontia and cleft lip and palate can be manifested. This case, a 9-year-old female with repaired bilateral syndactyly was referred to pediatric dental clinic, Pusan National University Hospital for evaluation of severe attrition of teeth and caries lesions. She had most of the above mentioned typical manifestations of the syndrome. Dental treatment including caries control, stainless steel crown were performed.

• 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.31 no.2
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• pp.262-266
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• 2004

The parathyroid hormone plays a major role in the metabolism of calcium and phosphorus. In hypoparathyroidism the parathyroid glands are atrophied or absent associated with autoantibodies against parathyroid tissue. Pseudohypoparathyroidism is a metabolic disease caused by the disturbance in peripheral action of parathormone, but parathormone level is normal. In general, patients with pseudohypoparathyroidism have short stature, round face, brachydactylia, obesity, mental retardation, cataracts & ectopic calcifications on soft tissues. Dental manifestations are enamel hypoplasia, delayed eruption, blunting of root apex, hypodontia, pulp calcification, thickened lamina dura, excessive caries & malocclusion. In this case, intraoral examination showed enamel hypoplasia on the erupted permanent teeth & hypermobility on the remaining deciduous teeth. From the radiographic view severe dental anomalies were observed on canines and shortening and blunting of root apex was observed on mandibular incisors. Pathologic root resorption was also observed on deciduous teeth.

• 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 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.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 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.

• The Journal of Korean Academy of Prosthodontics
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• v.50 no.3
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• pp.210-215
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• 2012

Some patients with generalized attrition and teeth discoloration may want their anterior teeth to be treated just for esthetic improvement. Ameologenesis imperfecta, however, should be considered for such patients prior to any treatment with thorough clinical and radiographic examination. If a patient is diagnosed with amelogenesis imperfecta, the treatment on anterior teeth just for esthetic purpose is not advisable. In this case, a young man with amelogenesis imperfecta was treated with metal-ceramic restorations. The patient had generalized attrition, teeth discoloration, crown fracture, and cross-bite on the left teeth. The ultimate objective of this treatment was to enhance esthetics and masticatory function. The cross-bite on the left anterior teeth was treated with restorations, whereas the reverse horizontal overlap was maintained on the posterior. The patient was satisfied with the result esthetically and functionally, and the third month recall examination revealed no pathologic changes associated with the treatment.

• 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.27 no.1
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• pp.70-76
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• 2000

Congenital biliary atresia with progressive sclerosis of the intra- and extra-hepatic duct system occurs in 1 : 10,000 live births, and has a poor prognosis with an expected survival of less than 5 years. Etiology of biliary atresia is unclear, however, it is believed a genetic or developmental cause. The clinical characteristics include pronounced jaundice, hepatosplenomegaly, pruritus, steatorrhea, xanthomas, growth retardation, portal hypertension, bleedings, ascites and respiratory infections. Oral manifestations have seldom been reported in patients with biliary atresia, but there may be enamel hypoplasia, delayed tooth eruption, and green teeth. Early diagnosis and surgical intervention have decreased morbidity. returned growth and development to normal and improved the prognosis for survival. Authors report the clinical and radiologic characteristics, proper managements about two cases with biliary atresia.