• Maxillofacial Plastic and Reconstructive Surgery
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• v.27 no.6
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• pp.559-564
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• 2005

Beckwith-Wiedemann syndrome is an autosomal dominant growth excess disorder, which occurs with a reported incidence of 1 in 13,700 to 1 in 17,000 live births. It constitutes a discrete clinicopathologic entity characterized by macroglossia, abdominal wall defects (omphalocele), visceromegaly, gigantism, hemihypertrophy, hypoglycemia, and the increased risk of solid tumor development from multiple cell lines. A macroglossia is a key component of the syndrome, and can lead to cosmetic, functional and psychologic disorder. This report shows a 5-year-old patient with Beckwith-Wiedemann syndrome, who had macroglossia and received reduction glossectomy.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.33 no.6
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• pp.540-548
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• 2011

Proteus syndrome is a congenital hamartomatous malformation that is characterized by a wide range of deformities, including craniofacial deformities. Proteus syndrome features partial gigantism and asymmetry of the limbs, plantar hyperplasia, hemangiomas, lipomas, lymphangiomas, varicosities, verrucous epidermal nevi, macrocephaly, cranial hyperostosis, and long bone overgrowth. We diagnosed Proteus syndrome in a male patient who visited our hospital with a chief complaint of limited mouth opening and report the case because we obtained a good healing outcome after treating the condition with a corrective osteotomy.

• Fisheries and Aquatic Sciences
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• v.15 no.1
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• pp.43-47
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• 2012

Growth hormone (GH) transgenesis in fish has the potential to improve aquaculture efficiency and capacity. However, many fast-growing transgenic fish have experienced side effects caused by excess GH expression. To overcome this unwanted issue associated with several GH transgenic mud loach Misgurnus mizolepis lines carrying GH construct driven by a strong ${\beta}$-actin regulator ($pml{\beta}$-actGH), we performed an alternative version of GH autotransgenesis using a weaker but more stable regulator, the mud loach lectin promoter. GH transgenesis with a pmlectGH construct consisting of the mud loach GH gene driven by the 2.3-kb lectin promoter exhibited significant growth stimulation. However, the extent of the growth acceleration in pmlectGH transgenics (six times maximum when assessed 2 months post hatching) was much less than that in transgenic individuals carrying the $pml{\beta}$-actGH construct. Additionally, the extraordinary gigantism that was common in $pml{\beta}$-actGH-transgenic mud loaches was diminished in transgenic loaches harboring the pmlectGH construct. Transgenic founders (pmlectGH) successfully transmitted their transgene into the next generation with up to 41% frequency. Growth stimulation also persisted in the transgenic F1 strains, with a seven-fold increase in maximum body weight at 6 months of age.

• Journal of the Korean housing association
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• v.18 no.5
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• pp.133-141
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• 2007

The purpose of this study is to secure an evacuation time and to ensure safety by using an Ubiquitous Sensor Network computing when a fire breaks out at a super high-rise apartment housing. A super high-rise apartment housing that is a type of building to solve the problem of separation of the urban function and the phenomenon of hollowing out downtown has been on the increase, high-rise apartment housings occupying 52.7% of whole housings in 2005. However, if a fire breaks out, there would be serious damage since it accommodates many people and facilities as existence of vertical gigantism in the city. The architectural law in force has no clause on it which is universally applicable to general building, it is difficult to be applicable to a densely super high-rise apartment housing and there would be in danger of a resident's evacuation in the fire. Therefore, as a previous study to introduce an USN-based fire-warning facility and evacuation equipment, this study shows the improvement way after analyzing factors that are barriers to evacuaee's behavior of a super high-rise apartment housing and also shows establishment of the grade of vertical evacuation rank by SimuleX, one of the best computer simulation program.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.12 no.2
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• pp.92-95
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• 2016

Beckwith-Wiedemann syndrome comprises multiple congenital anomalies with a risk of development of childhood tumors. Macroglossia is the predominent finding in Beckwith-Wiedemann syndrome and other findings include postnatal somatic gigantism, abdominal wall defects, nevus flammeus of the face, congenital heart defects, and cleft palate. The aim of this case presentation is to describe the dental treatment of a child with Beckwith-Wiedemann syndrome. A 3-year-old boy with Beckwith-Wiedemann syndrome visited to the Seoul National University Dental Hospital for dental evaluation and treatment. Due to the multiple caries and poor cooperation, we planned to perform the dental procedure under general anesthesia. The dental procedure was successfully performed and there was no airway complications after extubation. This case suggests that general anesthesia may be useful for the patient with uncooperative Beckwith-Wiedemann syndrome, since macroglossia and laryngomalacia may exacerbates airway compromise during dental sedation.

• Proceedings of the Korean Institute Of Construction Engineering and Management
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• 2007.11a
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• pp.539-544
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• 2007

Owing to diversification of social desire and increase of demand as economic growth, construction industry recently is trending toward diversification, complication, gigantism. It means that is changing to difficult environment as existing construction company operation. Specially, some big construction companies are promoting get down to construction business risk management skill & development. Enterprise risk management system, recognized to risk portfolio, is suggested. instead of individually risk management. This study indicates ERM basic model, considering construction risk character, for apply to ERM to field of construction . And it is including analysis of recognition level and reality about ERM in construction company.