• 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.36 no.1
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• pp.96-101
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• 2009

Regional odontodysplasia(ROD) is relatively rare localized developmental anomaly of tooth formation in which hard tissue is affected. The maxilla is typically affected than the mandible, and especially the maxillary left quadrant is the most commonly involved. Females are affected twice as often as males, and there is no association with race. Its etiology remains undetermined, but local circulatory disorders, somatic mutations, virus infections, local trauma, hyperpyrexia, irradiation, metabolic disturbances, and hereditary transmission are considered as possible etiologic factors. The affected teeth are likely to be small, hypoplastic, brown, and grooved. Eruption failure or delay is frequently seen as well as abscess or fistulae formation in absence of caries. Radiographically, there is a lack of contrast between the enamel and dentin, both of which are less radiopaque than unaffected counterparts. Moreover, enamel and dentin layers are thin, giving the teeth a “ghost-like appearance”. The pulp chambers and canals are large, the roots seem like to be short and indistinct. A 2-year-3-month old boy came to the department of pediatric dentistry, Yonsei University, with the chief complaint of delayed eruption and abnormal tooth shape on the lower left quadrant. He was diagnosed as regional odontodysplasia based on the clinical and radiographic findings.

• Journal of the korean academy of Pediatric Dentistry
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• v.41 no.4
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• pp.322-327
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• 2014

Regional odontodysplasia (RO) is a rare and nonhereditary dental malformation. It is a dental alteration of unknown etiology, involving both mesodermal and ectodermal dental components, which is characterized by clinical, radiographic, and histologic features. The maxilla is more often involved than the mandible (especially the left side), and there is no racial predilection, but females are affected twice as often as males. The affected teeth are clinically hypoplastic and hypocalcified, presenting a "ghost-like" appearance radiographically. The present case features a male patient aged 4 years and 6 months who was diagnosed with regional odontodysplasia in the maxilla on the right side, confirmed by clinical and radiographic examination, with a follow up of 5 years. Since teeth affected by RO have a poor prognosis due to the fragile tooth surface and open apices, the long-term treatment strategy depends on periodic clinical and radiological observations.

• 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.

• Journal of Chest Surgery
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• v.43 no.3
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• pp.235-245
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• 2010

Background: Our goal was to evaluate anti-calcification effects of decellularization and diverse fixing methods including preincubation of the bovine pericardium with ethanol. We also assessed changes in mechanical properties. Material and Method: Harvested bovine pericardium was decellularized with 0.25% sodim dodecysulfate and then treated with 5 methods of fixation: (1) 0.5% glutaraldehyde (GA) for 14 days, (2) 0.5% GA for 5 days, 2% GA for 2 days and 0.25% GA for 7 days, (3) 0.5% GA for 5 days, 2% GA for 2 days, 0.25% GA for 7 days, and then 70% ethanol for 2 days, (4) 0.5% GA for 5 days, a mixture of 2% GA and 70% ethanol for 2 days, and 0.25% GA for 7 days, (5) 0.5% GA for 5 days, a mixture of 2% GA, 65% ethanol, and 5% octanediol for 2 days and then 0.25% GA for 7 days. All treated bovine pericardia were tested for histological variables, lipid content, and mechanical properties including tensile strength and thermal stability. A total 10 kinds of differently treated bovine pericardia were implanted into rat subdermis and harvested 8 weeks later. Harvested pericardia were evaluated for calcium content. Result: No protein denaturation was observed microscopically after decellularization. There was a 32% mean decrease in tensile strength index after decellularization in the bovine pericardium group fixed. Octanediol preincubation attenuated the decrease in tensile strength and maintained thermal stability. TG and cholesterol were not affected by decellularization but were decreased by organic solvent. Calcium content was decreased after decellularization, and organic solvent preincubation decreased calcification in the non-decellularized bovine pericardium group. Conclusion: Decellularization and organic solvent preincubation have anti-calcification effects but decellularization may cause mechanical instability. A method of decellularization and fixation that does not cause damage to matrices will be needed for evaluation of the next step in using tissue-engineering for replacement of cardiac valves.

• Clinical and Experimental Pediatrics
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• v.46 no.1
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• pp.91-94
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• 2003

Raine syndrome was described as an unknown syndrome in 1989. It is characterized by severe craniofacial anomalies with microcephaly, hypoplastic nose, depressed nasal bridge, exophthamos/protosis, gum hypertrophy, cleft palate, low-set ears, small mandible, narrow chest, wide cranial sutures and choanal atresia or stenosis, by generalized osteosclerosis with subperiosteal thickening of ribs, clavicles and diaphysis of long bones, and by intracranial calcifications in the particularly periventricular area. It undergoes an autosomal recessive inheritance. Twelve cases of Raine syndrome have been reported in the literature. However, a case of Raine syndrome in Korea has not been reported yet. Therefore, we describe a female newborn with Raine syndrome with a brief review of the literatures.

• 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.

• Journal of Chest Surgery
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• v.30 no.8
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• pp.819-822
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• 1997

Aortic valve replacement with aortic allograft has been considered a treatment of choice for aortic valve disease secondary to bacterial endocarditis because of its good homodynamic performance and higher resistance to infection. The aortic root replacement technique might be superior to the subcoronary allograft implantation technique with regard to aortic regurgitation. A 46 yea,rs old male patient had acute aortic regurgitation with progressing heart failure secondary to acute bacterial endocarditis. The patient underwent emergent aortic root replacement using 20 mm aortic allograft. At operation, right coronary cusp perforation and heavy calcification of commissure between right and left coronary cusp were observed. The patient recovered well and postoperative echocardiography demonstrated no aortic regurgitation. Inflammatory signs were subsided after 8 weeks of antibiotics therapy. Medically uncontrolled acute bacterial endocarditis was treated successfully by aortic root replacement using aortic homograft.

• 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.35 no.4
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• pp.737-743
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• 2008

Regional odontodysplasia is a relatively rare developmental anomaly of dental hard tissue with characteristic clinical, radiographic and histologic features. It requires a continuous and multidisciplinary approaches, and the aim of treatment for these patients should include aiding mastication, improving aesthetics, maintaining normal vertical dimension and space, allowing normal jaw growth and eruptional management of affected teeth. This report describes three cases of regional odontodysplasia with 2-5 years of follow-up. Conservative treatment is chosen to preserve the affected teeth as long as possible, and periodic radiographic and clinical examination was done. During this time, all teeth except one showed progressive development. An interesting finding observed in our cases was that each tooth even in the same person showed different degree of tooth development and eruption rate. Thus, we colcluded that the treatment plan for regional odontodysplasia should be conservative and individualized and based on the assessment of each tooth.