• 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.44 no.2
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• pp.164-169
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• 2017

Dentinogenesis imperfecta type II (DGI-II) is an inherited disorder affecting the dentin matrix and is related to mutations in the dentin sialophosphoprotein (DSPP) gene. The protein encoded by the DSPP gene undergoes extensive posttranslational modifications. Dentin phosphoprotein (DPP), one of the DSPP expressed products, has unique composition with highly repetitive Asp-Ser-Ser amino acid residues and is related to the maturation of dentin mineralization. We aimed to identify mutation in DSPP, including the DPP coding region, contributing to inherited dentin defects in a Korean family with DGI-II. Clinical and radiographic examinations were performed, and all five exons and exon-intron boundaries of the DSPP gene were sequenced. Additionally, allele-specific cloning for highly repetitive DPP region was performed. By sequencing and cloning, a heterozygous single nucleotide deletion (c.2688delT) was identified. The identified mutation caused a frameshift in the DPP coding region. This frameshift mutation would introduce hydrophobic amino acids instead of hydrophilic amino acids and would result in a change in the characteristics of DPP.

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

• Journal of the korean academy of Pediatric Dentistry
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• v.28 no.1
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• pp.175-179
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• 2001

Regional odontodysplasia is a relatively rare condition in which both enamel and dentin are hypoplastic and hypocalcified. The result is localized arrest in tooth development. The etiology of regional odontodysplasia is uncertain, but, disturbance in vascular supply, somatic mutation, latent virus infection, trauma, hyperpyrexia, irradiation, nutrition, metabolic disorders and hereditary transmission are supported to be etiologic factors. Females are more often affected than males. (1.4 : 1). The maxillary arch is more often affected than the mandibular arch with the maxillary left quadrant being the most commonly involved. Affected teeth are hypoplastic, typically discolored yellow or yellowish brown, smaller in size and display a variety of surface marking including pitting and grooving. Radiographically, the teeth affected have been described to have a "ghost like" appearance or "fuzzy" appearance. Pulp calcification and denticles may be present within the pulp chambers of the affected teeth. In it's case, a 2 years old male visited for a treatment of uneruption of lower right teeth. Partial eruption of lower right deciduous central incisor and unerupted deciduous lateral incisor, deciduous canine and deciduous first molar showed severly delayed eruption state. On radiographic appearance, "Ghost like appearance", shortened root and opened apexes on lower right region were observed. It was suspected regional odontodysplasia with clinical and radiographic condition.

• Journal of Chest Surgery
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• v.30 no.7
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• pp.656-662
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• 1997

Aortic valvuloplasty has recently gained attention as an attractive alternative procedure for aortic valvular disease. Between March 1995 to August 1996, 14 patients with pure aortic regurgitation(AR) underwent aortic alvuloplasty using leaflet extension with glutaraldehydepreserved autologous pericardium. There were 11 males and 3 females, and the mean age was 34.8 $\pm$ 15.3 years. Preoperative echocardiography and cardiac catheterization revealed that the degree of AR was mean 3.4$\pm$0.65, and more than moderate degree of mitral regurgitation(MR) were detected in 4 patients. In 12 patients, 3 leaflets were extended and in another 2 patients only one deformed leaflet was extended. Concomitant mitral valvuloplasty (MVP) was performed in 4 patients. The competency of the aortic valve after completion of repair was evaluated by the transesophageal echocardiography in operating theater, and there was no aortic and mitral stenosis or regurgitation. In an early postoperative echocardiography, trivial AR was detected in 3 patients and mild MR in 1 patient. The end-systolic and end-diastolic dimensions of the left ventricle were decreased significantly(p<0.05) as compared with those of preoperative values. T ere was no mortality and no significant postoperative complication encountered. Late complication developed in 2 patients during the follow-up period(mean 7.9$\pm$ 5.9 months). One patient underwent AVR on postoperative 7th month due to endocarditis, and the another patient with Behcet's disease underwent Ross operation at postoperative 4th month. In conclusion, AVP of leaflet extension technique offers an excellent early clinical result and represents a good alterna!ivy surgical treatment for the pure AR especially in young age group, although long-term follow-up is necessary to determine the durability of glutaraldehyde-preserved autologous pericardium as a valve leaflet.

• Journal of Chest Surgery
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• v.30 no.7
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• pp.670-676
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• 1997

The structural failure of the glutaraldehyde-treated xenograft valves has been the primary concern about the limited durability as predicted from the begimling of clinical use, and long-term follow-up has shown a significant incidence of primary tissue failure(PTF) from both biological and mechanical reasons. Twenty-seven patients with the low-profile lonescu-Shiley valves explanted from mitral position for PTF(Group III) were studied on the patient characteristics and valve pathology, and the results were compared with the matched observations of the Haycock(Group I) and of the standard-profile lonesiu-Shiley valves(Group II). Patients were aged 16 to 56 years(mean, 38.0$\pm$ 11.0 years), and the size of the failed mitral bioprosthesis was 30.8$\pm$ 1.3 mm. The hemodynamic consequences were stenosis in 29.6%, insufficiency in 44.4%, mixed steno-insufficiency in 14.8%, together with normal function for the rest of patients of prophylactic re-replacement. Pathology revealed calcification with or without tissue damage in 63.0% and tissue damage with or without calcification in 58.l%, in contrast with the observations of predominant tissue damage(76.8%) over calcification in Group I and of calcification(76.1%) over tissue damage in group II. Although dystrophic calcification has long and repeatedly dealt with patient's young age as a determinant of valve durability, such a characteristic evidence was not reached even in patients with calcified valves. Moreover, the prolonged explantation p riods from the studied on the previous report suggested strongly yet possibly evolving destructive processes among the valves in the remaining patients, and awaits further follow-up. In conclusion, PTF of the xenograft valves seems to result from more complicated biologic and metabolic reasons as well as more complex mecharical factors than the reported, and newer generation prostheses, with tissue preservation with glutaraldehyde, do not likely to provide decisive improvement in the occurrence of structural failurebioprostheses is generally limited to the highly aged.

• Journal of Chest Surgery
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• v.36 no.7
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• pp.510-513
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• 2003

Annuloaortic ectasia, aortic regurgitation(AR), and ventricular septal defect(VSD) in patients with right ventricular hypoplasia is a very rare condition. We report a patient who underwent aortic root replacement with a composite graft for annuloaortic ectasia associated with VSD and AR in right ventricular hypoplasia. The patient was a 19 year-old male. Transthoraic echocardiogram and cardiac catheterization revealed a perimembranous VSD (2 cm in diameter), severe AR, annuloaortic ectasia, bipartite right ventricle with hapoplasia, and hypoplastic tricuspid valve. Operative findings showed that free margins of the right and noncoronary cusps were markedly elongated, thickened, and retracted, and commissure between the right coronary cusp and the noncoronary cusp was fused and calcified. VSD was closed with an autologous pericardial patch and composite graft aortic root replacement using direct coronary button reimplantation was performed, and the hypertrophic muscle of the right ventricular outflow tract was resected. The patient had transient weaning failure of cardiopulmonary bypass and was discharged at the postoperative 14 days without any problems.

• Journal of the korean academy of Pediatric Dentistry
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• v.37 no.2
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• pp.260-266
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• 2010

Trauma to the primary teeth may have an effect on the successive permanent teeth. Thus, the treatment plans should be focused on safety of their permanent teeth. The degree of developmental disturbances in those permanent teeth depends upon many factors including the age of the children, developmental conditions of the affected teeth, types and severities of trauma and time interval from the moment of trauma to the initial treatment the patients received. The complications of the effected permanent teeth vary from the mild enamel hypoplasia to the premature finishing of teeth development. In this study, children whose ages were 33 months, 5 years, and 26 months presented the developmental disturbances to their permanent teeth, which resulted from the trauma to the primary teeth: the intrusion, the avulsion, and the subluxation. There showed a mild complication in the case of subluxation resulting in enamel hypoplasia, but, the more severe complication of the root dilacerations occurred in the case of intrusion. Furthermore, the 5-year-old patient whose primary dentition was near in transition period to the permanent one, the more complicated problem such as stop of root development of the permanent teeth was resulted from the avulsion. Thus, one should assume that the types of trauma and the age of the children at the moment of trauma have different effects on those successive permanent teeth. In conclusion, when the trauma that causes changes in the position of primary teeth happened, it is important to arrange an appropriate treatment procedure considering the types of trauma and the developmental conditions of the permanent teeth.

• Korean Journal of Clinical Laboratory Science
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• v.51 no.2
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• pp.270-275
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• 2019

The incidence of degenerative valve disease also increasing with the increasing life expectancy of the elderly population. Rheumatic valve disease is decreasing gradually and the incidence of calcified degenerative aortic stenosis (AS) is growing. Echocardiography is a very important tool for evaluating the prognosis and treatment method as well as the time of operation and diagnosis of heart valve disease. When evaluating valvular heart disease, 2-dimensional echocardiography, which observes all heart valves in detail, should take precedence. Understanding the clinical findings of degenerative valve disease and performing precise echocardiography are extremely important. In addition, an assessment of the severity of aortic stenosis is necessary to determine the surgical indications. An assessment of the severity by echocardiography was explained with three cases of degenerative aortic stenosis. To perform echocardiography accurately, it is necessary to understand degenerative valve disease and its clinical findings accurately.

• Journal of Chest Surgery
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• v.29 no.10
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• pp.1111-1117
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• 1996

Structural deterioration of the bioprosthetic xenograft valves due to primary tissue failure occurs in two modes: from fatigue lesions with tear and wear with or without calcification and from calcification with obstruction. Two groups of consecutive 56 patients with the Hancock porcine ortic valve(HM) and of 1 13 patients with the standard-profile onescu-Shiley bovine pericardial valve(ISM) explanted from mitral position at the time of re-replacement surgery for primary tissue failure at Seoul national University Hospital until 1994, were studied for clinical and pathological features. Their ages at primary implant were 31.9 $\pm$9.2 years In HM and 30.4$\pm$ 12.5 years in ISM. Hemodrnamic dysfunction of the failed mitral bloprostheses were predominantly insufficiency in HM(64.3%) and stenosis in ISM(51.3%)(p<0.001). Pathologic findings of the explanted mitral valves reflected these hemodynamic changes, revealing failure more often from tissue damage(tears and wears) in HM and more often from calcification in ISM(p< 0.001). Explant period(from primary implant to explant) was relatively short in ISM(8.7$\pm$2.6years), compared with the one in HM(10.4 $\pm$2.6 years)(p<0.001). In conclusion, both the Hancock and the lonescu-shiley valves would fail from calcification as well as issue damage. However, while the Hancock porcine valves in mitral position failed more frequently from tissue failure and insufficiency, the standard-profile lonescu-Shiley pericardial valves did from calcification and stenosis, especially in young pAtients . Although the possibility of less occurrence of valve failure from mechanical reasons may be expected with newer generation bloprostheses, it does not seem to Improve durability significantly unless further refinement in antimineralization is achieved. Therefore, clinical use of the glutaraldehyde-treated bioprosthetic valves is, at present, limited to the patients of advanced age groups.