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

• Journal of the korean academy of Pediatric Dentistry
• /
• v.47 no.3
• /
• pp.344-351
• /
• 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
• /
• v.52 no.4
• /
• pp.317-323
• /
• 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
• /
• v.27 no.1
• /
• pp.70-76
• /
• 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.

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

• Maxillofacial Plastic and Reconstructive Surgery
• /
• v.29 no.1
• /
• pp.91-96
• /
• 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
• /
• v.48 no.4
• /
• pp.376-383
• /
• 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
• /
• v.33 no.2
• /
• pp.323-328
• /
• 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 the korean academy of Pediatric Dentistry
• /
• v.25 no.3
• /
• pp.598-603
• /
• 1998

Amelogenesis imperfecta is a group of hereditary defects of enamel unassociated with any other generalized defects. The prevalence of this condition has been estimated to range from 1 in 14,000 to 1 in 16,000, depending on the population studied. It may be differentiated into three general types : hypoplastic, hypocalcified, and hypomaturation, depending on the clinical presentation of the defects and the likely stage of enamel formation that is primarily affected. The dentin and root form are usually normal, but the enamel may lack the normal prismatic structure, being laminated throughout its thickness or at the periphery, with the result that these teeth are more resistant to decay. This case is that of an six-year-old girl brought to the pediatric dentistry department by her parents for esthetic reasons and also because of slight dental sensitivity. Clinical and radiographic examinations confirmed amelogenesis imperfecta. The author has treated with the crowning of the primary molars, using prefomed NiCr crowns and periodic fluoride application on whole dentition.

• Journal of the korean academy of Pediatric Dentistry
• /
• v.26 no.3
• /
• pp.486-491
• /
• 1999

Hypoparathyroidism has abnormally decreased secretion of parathyroid hormon which responds to the blood calcium level. Wherease, Pseudohypoparathyroidism has normal activity of thyroid hormon, but end-organs, such as urinary tract and osteoclast, do not respond to parathyroid hormon. The cause of this disease is due to the mutation of Guanine stimulating(Gs) protein regulating Gs gene, which is the receptor to this hormon. Pseudohypoparathyroidism is usually noted before 20 years old on average of 8-9 years old. The clinical features of this disease includes delayed growth and development, round face, obesity, soft tissue calcification, ectopic ossification, shortening of metacarpals and metatarsals by epiphyseal closure in advance of age. The mutation of Gs gene which are found in brain, endocrine organs, and chondrocytes is the cause of those features. Reaction to Glucagon, gonadal hormon, and thyroid stimulating hormon is not expected in both cases. The common dental manifestations include enamel hypoplasia, delayed eruption, agenesis of tooth, hypodontia, dysplastic short roots, widened pulpal space, microdontia, intrapulpal calcification, and malocclusion are also often reported. This case which is diagnosed to Pseudohypoparathyroidism showed short and under-developed root of permanent troth, delayed eruption, and non-eruption of premolars and molars. And morphogenesis imperfecta of first and second premolars were also found.