• Journal of the Korean Academy of Esthetic Dentistry
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• v.12 no.2
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• pp.31-38
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• 2003

Amelogenesis imperfecta patients suffered common clinical problems of poor esthetics, teeth sensitivity, and loss of occlusal vertical dimension. Amelogenesis imperfecta is a group of inherited disorders primarily affecting dental enamel. Variants of amelogenesis imperfecta generally classified hypoplastic, hypocalcified, or hypomaturation types based on the primary enamel defects. The mildest problems were found in the pitted hypoplastic type whereas the most severe problems were encountered in the hypocalcified type amelogenesis imperfecta. Management stragies include composite resin veneer and jacket crowns for anterior teeth as well as steel crowns for posterior teeth. Knowledge of the clinical features and dental complications of each variants if amelogenesis impecta helps in the diagnosis of the condition and allows institution of early preventive measures. The objective of this paper is to provide a review of the current concepts of the wide spectrum of etiological factors involved in the pathogenesis of this significance clinical entity in the primary dentition.

• Imaging Science in Dentistry
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• v.45 no.3
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• pp.181-185
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• 2015

Numerous cases of enamel renal syndrome have been previously reported. Various terms, such as enamel renal syndrome, amelogenesis imperfecta and gingival fibromatosis syndrome, and enamel-renal-gingival syndrome, have been used for patients presenting with the dental phenotype characteristic of this condition, nephrocalcinosis or nephrolithiasis, and gingival findings. This report describes a case of amelogenesis imperfecta of the enamel agenesis variety with nephrolithiasis in a 21-year-old male patient who complained of small teeth. The imaging modalities employed were conventional radiography, cone-beam computed tomography, and renal sonography. Such cases are first encountered by dentists, as other organ or metabolic diseases are generally hidden. Hence, cases of amelogenesis imperfecta should be subjected to advanced diagnostic modalities, incorporating both dental and medical criteria, in order to facilitate comprehensive long-term management.

• Journal of the korean academy of Pediatric Dentistry
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• v.25 no.4
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• pp.704-709
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• 1998

Amelogenesis imperfecta represents a group of hereditary conditions that manifest enamel defects without evidence of generalized or systemic disorders. These enamel disorders are apparently heterogeneous in the basic chemical structure, resulting in a diverse presentation of clinical characteristics. The reported prevalence of amelogenesis imperfecta varies from 1 in 14,000-16,000 to 1.4 in 1,000 depending on specific population studied with the autosomal dominant hypocalcification type of amelogenesis imperfecta believed to be the least prevalent. The most widely accepted current classification system for delineating the amelogenesis imperfecta types considers the mode of inheritance and clinical manifestations. Three major groups are recognized; hypoplastic, hypocalcified, and hypomaturation types. Delineating specific types of amelogenesis imperfecta can be confusing due to the phenotypical similarity of many forms and that the most recent classification lists 14 different types. A 12 year-old female patient came to our pediatric dentistry clinic complaining of the ugly shape and color of her teeth, especially the upper front area. Although the goal of the treatment was mainly focused on the improvement of patient's esthetics, longevity of the restorations was also considered in selecting the appropriate restorative system, resin jacket crown, which can satisfy the both aspects.

• Journal of the korean academy of Pediatric Dentistry
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• v.37 no.4
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• pp.467-471
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• 2010

Amelogenesis imperfecta, one of the dental genetic disease, is clinically and genetically complex disease. Amelogenesis imperfecta can be classified into three major categories according to clinical phenotype; hypoplastic, hypomaturation, and hypocalcification. Recently a novel gene, Fam83h, was identified to cause autosomal dominant hypocalcification amelogenesis imperfecta, however its functional role in the pathogenesis of enamel defect is not known yet. So this study was aimed to identify the knockdown effect of Fam83h gene on the amelogenin mRNA expression via shRNA transfection into immortalized ameloblast cell line. The result showed that the knockdown of Fam83h did not influence the amelogenin expression. Further study of the functional role of Fam83h gene should be performed to understand the complex nature of amelogenesis as well as molecular pathogenesis of amelogenesis imperfecta.

• Journal of the korean academy of Pediatric Dentistry
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• v.35 no.3
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• pp.562-570
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• 2008

Amelogenesis imperfecta is a group of hereditary defects of enamel, unassociated with any other generalized defects. It is classified into 14 subtypes according to different clinical and genetic features. According to its clinical features, it is classified into hypoplastic type, hypocalcified type and hypomaturation type. However, these features tend to co-exist often. Dental features associated with amelogenesis imperfecta include quantitative and qualitative enamel deficiencies, pulpal calcification, root malformations, abnormal eruption, impaction of permanent teeth, progressive resorption of root and crown, congenital missing teeth and anterior and posterior open bite occlusions. The first case patient is a 16 month-old child with discoloration of deciduous teeth. All of her deciduous and permanent dentition has shown amelogenesis imperfecta. The restorational, orthodontic and recent prosthodontic treatments have been completed. Another patient is a 9 year and 3 month-old child with amelogenesis imperfecta in both deciduous and permanent dentition. The restoration has been done and the prosthodontic treatment is planned after the completion of growth. Above cases indicate that amelogenesis imperfecta occurs both in deciduous and permanent dentition, and it requires the long term treatment and care.

• Journal of Korean Academy of Oral and Maxillofacial Radiology
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• v.27 no.1
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• pp.203-216
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• 1997

The purpose of this study was to investigate the radiation effect on the stages of amelogenesis. Twenty ll-day-old rats which were irradiated by 4Gy of garmna radiation on the 19th prenatal day were used for the experimental group and twenty ll-day-old rats which were not irradiated were used for the control group. The length of each zone of amelogenesis were measured on the sagittal section using a light microscopic enlargement at 400 x the normal view while the morphologic changes of ameloblasts of each zone were observed electron­microscopically. The obtained results were as followed : 1. The length of the region of facing pulp and facing dentin of the zone of presecretion were increased by 11.5％(P<0.05) and 17.7％(P<0.01), respectively. 2. The length of the zone of secretion was increased by 17.3％(P<0.01), but the zone of maturation was decreased by 15.3％(P<0.01). 3. The total length of the zone of amelogenesis was not changed significantly(P>0.05). 4. Electron-microscopically, enlargement of the cell membrane, rER, mitochondria, and nuclear membrane were observed. These changes were mostly severe in the zone of maturation.

• 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.

• Journal of the korean academy of Pediatric Dentistry
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• v.25 no.3
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• pp.598-603
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• 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.

• International Journal of Oral Biology
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• v.43 no.4
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• pp.177-183
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• 2018

The objective of this study was to examine the expression pattern of Kelch-like ECH-associated protein 1 (Keap1) in the maxillary $2^{nd}$ molar germs of rats. We used the maxillary $2^{nd}$ molar germs in rats' pup at postnatal day 3 (bell stage), 6 (crown formation stage) and 9 (root formation stage). The investigation on mRNA and protein levels were done using reverse transcription - polymerase chain reaction and western blot. Localization of Keap 1 in the maxillary $2^{nd}$ molar germs were revealed through immunofluorescence staining. Keap1 from the maxillary 2nd molar germs were mostly manifested on postnatal day 3 and dramatically decreased on postnatal day 6 and 9 at mRNA and protein levels, while amelogenin and ameloblastin increased during the development of maxillary 2nd molar germs. During immunofluorescence analysis, the strong immunoreactivity against Keap1 was detected in the apical side of ameloblasts at the presecretory and secretory stages. However, Keap1 expression was hardly observed in the ameloblasts at the maturation stage. These results shows that Keap1 is strongly expressed in the presecretory and secretory ameloblasts of amelogenesis, and suggest that Keap1 may be a crucial molecule for the regulatory mechanisms tasked with the formation of enamel layer.

• The Journal of Korean Academy of Prosthodontics
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• v.55 no.4
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• pp.410-418
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• 2017

Amelogenesis imperfecta characterized as abnormally formed enamel is caused by a defect of unique group of genes. Patients affected by this disease might have difficulties in social and psychological aspects due to non-esthetic teeth as well as functional problems caused by enamel detachment and tooth wear from their early ages. Adult patients with amelogenesis imperfecta can be treated with full-mouth restorations, which make functional and esthetic rehabilitations of severely worn tooth. However, the anterior open bite and lack of occlusal clearance for posterior teeth restorations due to compensatory extrusion are the intervening factors in the prosthetic treatment. Therefore, the determination of anterior tooth lengths, vertical dimension, and anterior guidance should be set carefully. Recently, computer-aided design and computer-aided manufacturing (CAD/CAM) techniques help systematic approaches and enable dentists to reduce time-consuming procedures in the diagnosis and treatment of full-mouth rehabilitation. This case report demonstrates the successful full mouth rehabilitation using a CAD/CAM system in a young adult patient with amelogenesis imperfecta and anterior open bite.