• 제목/요약/키워드: Gigantism

검색결과 6건 처리시간 0.02초

Beckwith-Wiedemann 증후군 환자에서의 거대설 절제술 (REDUCTION GLOSSECTOMY OF MACROGLOSSIA IN BECKWITH-WIEDEMANN SYNDROME : A CASE REPORT)

  • 김학균;김은석;고영권;김수관
    • Maxillofacial Plastic and Reconstructive Surgery
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    • 제27권6호
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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.

악골의 편측 비대를 보이는 프로테우스 증후군의 치료: 증례보고 (Management of Proteus Syndrome with Craniofacial Hemihyperostosis: Case Report)

  • 윤영은;설가영;오민석;최정구;김상중;이동근;강지연
    • Maxillofacial Plastic and Reconstructive Surgery
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    • 제33권6호
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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.

Growth Performance of Transgenic Mud Loach Misgurnus mizolepis Carrying a GH Transgene Driven by Mud Loach C-Type Lectin Regulator

  • Song, Ha-Yeon;Kim, Dong-Soo
    • Fisheries and Aquatic Sciences
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    • 제15권1호
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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.

초고층 공동주택의 USN기반 피난시스템 도입을 위한 수직적 피난위계 설정에 관한 연구 (A Study on Setting the Grade of Vertical Evacuation Rank to Introduce an USN-based Evacuation System into Super High-rise Apartment Housing's)

  • 홍원화;전규엽;최준호
    • 한국주거학회논문집
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    • 제18권5호
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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.

Beckwith-Wiedemann syndrome 환아의 치아우식 치료 : 증례보고 (DENTAL CARIES TREATMENT OF A PATIENT WITH BECKWITH-WIEDEMANN SYNDROME : A CASE REPORT)

  • 허선재;신터전;현홍근;김정욱;장기택;이상훈;김종철;김영재
    • 대한장애인치과학회지
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    • 제12권2호
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    • pp.92-95
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    • 2016
  • Beckwith-Wiedemann syndrome은 다양한 임상적 특징을 보이는 질환이다. 거대설은 BWS 환아에서 가장 흔히 관찰되는 특징으로 그로 인한 섭식 문제와 호흡곤란이 야기될 수 있어 치과 치료 시, 주의를 요하게 된다. 또한 거대설로 야기될 수 있는 골격적인 문제에 대한 장기적인 평가가 필요하다.

건설기업의 전사적 리스크 관리 체계 적용을 위한 고려 사항 및 추진 방안에 대한 연구 (A Study of Considerations and Way to promote Enterprise Risk Management in Construction Company)

  • 김승원;이재호;유정호;김창덕
    • 한국건설관리학회:학술대회논문집
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    • 한국건설관리학회 2007년도 정기학술발표대회 논문집
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    • pp.539-544
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    • 2007
  • 최근 건설 산업은 경제성장에 따른 사회적 욕구의 다양화와 이로 인한 수요의 증대로 인하여 점차 다양화, 복잡화, 거대화되는 추세이다. 이는 기존의 건설사업의 운영으로는 감당하기 어려운 환경으로 변화하고 있음을 의미한다. 건설 산업에 내재된 불확실성과 위험요인을 체계적으로 관리하고자 하는 움직임이 대형 건설 회사들을 중심으로 나타나기 시작하였고, 특히 일부 대형 건설 회사들은 본격적인 건설사업 리스크 관리기법 도입 및 개발을 적극추진하고 있다. 이에 개별적으로 행해오던 리스크 관리를 넘어서 기업이 직면한 다양한 위험들을 전사적인 시각에서 통합하여 하나의 리스크 포트폴리오로 인식, 관리하는 새로운 리스크 관리 방식인 전사적 리스크 관리(Enterprise Risk Management)시스템이 두각 되기 시작하였다. 이에 본 연구는 전사적 리스크 관리를 건설 분야에 적용시키기 위한 첫 번째 단계료 ERM에 대한 건설 기업의 인식수준과 현 실태를 분석하고 건설 분야에서의 리스크 특성을 고려한 ERM 기본 모형을 제시하고자 한다.

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