• 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.

• 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 the korean academy of Pediatric Dentistry
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• v.27 no.1
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• pp.1-6
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• 2000

Dentinogenesis imperfecta is an inherited disorder of dentin formation, usually exhibiting an autosomal dominant mode of transmission. Type I dentinogenesis imperfecta occurs in patients afflicted with osteogenesis imperfecta. Type II dentinogenesis imperfecta is not associated with osteogenesis imperfecta. Type III dentinogenesis imperfecta (Brandywine type) occurs in a racial isolate area in the state of Maryland. In all three types, teeth of both dentitions are affected with variable clinical appearances. The teeth are opalescent with the color ranging from bluish-gray to brown to yellowish. The dentin is abnormally soft, providing inadequate functional support to the overlying enamel. Although the enamel is normal, it fractures or chips away easily, exposing the occlusal and incisal dentin. The exposed soft dentin often undergoes rapid and severe functional attrition. The teeth exhibit bulb-shaped crowns with constricted cementoenamel junctions and thin roots. The teeth will exhibit varying stages of obliteration of the coronal and root pulpal chambers. The cementum, periodontal ligament and supporting alveolar bone appear normal. The enamel is normal. The mantle dentin remains nearly normal, whereas the remaining dentin is severely dysplastic. The dentinal tubules are disoriented, irregular, widely spaced, and usually larger than normal.

• Journal of the korean academy of Pediatric Dentistry
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• v.36 no.4
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• pp.601-606
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• 2009

Amelogenesis Imperfecta (AI) is a genetic disorder which retards the development of enamel and it can be classified into three types: hypoplastic, hypomaturation, hypocalcified type. This can occur both in deciduous and permanent dentition. A 8 year 8 month old patient with a chief complaints of delayed eruption on upper anteriors, calculus deposit on lower anteriors and anterior openbite visited the clinic. Anteriors had thin layer of enamel and were very narrow. Especially lower anteriors had rough surface and were in bad shape. Teeth were very hypersensitive to thermal changes. Upper and lower first molars showed severe attrition on the occlusal surface. Radiographs also verified hypoplastic enamel in the whole dentition including the teeth in the tooth bud. The patient was diagnosed as hypoplastic AI, and is being treated at the pediathc and prosthodontic department of the Kyunghee dental university hospital. To improve the function, esthetics, hypersensitivity of the AI patients, restorations on the posteriors and the anteriors with oral hygiene instruction are necessary, Constant follow-up check is needed until full growth and after full growth, cooperative care with the other department is needed.

• 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.

• Tuberculosis and Respiratory Diseases
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• v.57 no.5
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• pp.489-493
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• 2004

Unilateral pulmonary artery agenesis is a rare congenital anomaly usually associated with other cardiovascular anomalies such as tetralogy of Fallot or septal defect. Unilateral pulmonary artery agenesis without other coexisting cardiovascular abnormality (isolated unilateral pulmonary artery) is extremely rare and often asymptomatic until adulthood. In these patients, diagnostic clue is found in a plain chest roentgenogram, showing a hyperlucent contracted hemithorax. We have recently experienced a case of isolated right pulmonary artery agenesis, which was diagnosed by chest dynamic CT, perfusion scan, echocardiogram and 3-dimensional reconstruction cardiac CT angiography in a 50-year old female who had suffered from mild dyspnea on exertion and improved with conservative treatment. We report this case with a brief review of the relevant literature.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.13 no.1
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• pp.88-94
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• 1991

Maxillofacial deformities are not considered to be a trouble in social life but function. So many maxillofacial plastc surgeons has made efforts to overcome these troubles and bring out more positive life. The proper proportion and shape decide esthetic quality. Lower third of face was consist with lip, cheek, mandibular lower border and mandibular angle. Widening lower third of face give a impression with muscular and recklessness. And lower and wide mandibular angle makes face square shape. Unilateral involvement cause asymmestric face. These face is considered unfavorable, especially in Korea or Japan. We prevent a number of with mandibular angle Bulging which was corrected with mandibular osteotomy or masseter myotomy.

• Journal of Yeungnam Medical Science
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• v.20 no.1
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• pp.71-78
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• 2003

Unilateral absence of a pulmonary artery (UAPA) is a rare congenital anomaly that is frequently associated with other cardiovascular anomalies first reported by Fraentzel in 1968. Most patients who have no associated cardiac anomalies have only minor or absent symptoms. We experienced a case of isolated UAPA in a young female presenting hemoptysis. The chest radiograph showed a small left lung volume and high resolutional CT of chest showed multiple subpleural nodules and centrilobular nodules in parenchyma. The video-assisted thoracoscopic biopsy revealed diffuse dilated vessels in visceral pleura. The pulmonary angiogram confirmed the absence of the left main pulmonary artery.

• 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.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.