• Title/Summary/Keyword: arched

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Monosomy 21 Mosaicism in a Child with Dyserythropoiesis (적혈구 이형성증(Dyserythropoiesis)의 소견을 보이는 부분 21-monosomy 증후군)

  • Lim, Jae-Young;Seo, Ji-Hyoun;Choi, Myoung-Bum;Park, Chan-Hoo;Woo, Hyang-Ok;Youn, Hee-Shang
    • Clinical and Experimental Pediatrics
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    • v.46 no.4
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    • pp.397-399
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    • 2003
  • All complete monosomy 21 appear to be lethal early in their development in humans and only survive in mosaic forms. Complete monosomy 21 is a very rare and usually debilitating genetic disorder. Partial monosomy 21 is also rare and is thought to constitute a clinical syndrome consisting of peculiar faces, hypertonia, psychomotor retardation, and slow growth. We experienced a case of monosomy 21 mosaicism. Chromosome analysis demonstrated mosaicism for cell lines in the lymphocytes examined; 45, XX, -21/46, XX. The main clinical features were craniofacial dysmorphism including high arched palate, submucosal cleft, micrognathia and arthrogryposis-like symptoms including flexion deformity of fingers. And hematological findings were revealed dyserythropoiesis, thrombocytopenia and eosinophilia. Currently, the patient has nearly compatible growth, but a mild degree of mental retardation. We report here an 8 years old female child with apparent monosomy 21 mosaicism associated with dyserythropoiesis, thrombocytopenia and eosinophilia, with a review of the associated literatures.

Assessment of Visual Characteristics on Arch Bridge Using Landscape Simulation (경관시뮬레이션을 이용한 아치교량의 시각적 특성평가)

  • Jung, Sung-Gwan;Park, Young-Eun;Park, Kyung-Hun;You, Ju-Han;Kim, Kyung-Tae;Lee, Woo-Sung
    • Journal of the Korean Institute of Landscape Architecture
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    • v.35 no.4
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    • pp.48-56
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    • 2007
  • This study was to understand the component that affects the formative beauty and to present the direction of bridge design for improving the image of urban landscape to survey the visual effect and landscape Preference by the change of bridge type. The results of this study are as follows. In the results of image analysis by bridge types, the images of one-arch bridges are unique and interesting, whereas more than two successive arched bridge were harmonize, stable, consecutive and regular. In the case of the arch rib, braced-rib arch bridge was assessed that complicated, diverse and interesting more than solid-rib arch bridge. The results of factor analysis on the psychological factor were classified into three categories: orderliness, aesthetic and symbolism. In the results of analysis on psychological factors by bridge types, the orderliness and symbolism were different in the position of path, and the number of arches, too. In case of arch rib, symbolism was different. In the preference analysis, they showed a sensitive reaction in the background of building. In the results of the relativity preference and psychological factor, according to aesthetic, symbolism and orderliness, there was an effect on the background of building. And, there showed the high effect in order of aesthetic, orderliness and symbolism in the background of mountain and building. This study should be objective raw data of the arch bridge design for improving the urban landscape. In the future, aesthetic variables like colors or textures should be considered for more exact evaluation.

Optimizing Planting Distance and Labor-Saving Efficiency for Head Lettuce Using a Transplanter for Summer Season Cultivation in the Alpine Area (고랭지 결구상추의 기계정식시 적정 재식거리 및 노력절감효과)

  • Jang, Suk-Woo;Kim, Won-Bae;Kim, Jin-Young
    • Horticultural Science & Technology
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    • v.18 no.6
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    • pp.787-791
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    • 2000
  • Lettuce seedlings used in this investigation of planting distance and labor-saving efficiency were first grown in 100-hole paper trays for 30 days. Seedling height for transplanting ranged from 3 cm to 6 cm and plants had 3 to 5 leaves. The beds prepared for transplanting were of the arched type and were 35-40 cm in width, 15-20 cm in bed height, and between-bed furrow width was 20 30 cm. Typical seedling planting depth with the transplanter was 4-5 cm, although depth was quite variable because of the irregularities of the ground. Total transplanting time with the transplanter varied from 2.6 to 2.7 hours per 10a, while it took 38.1 hours per 10a with conventional planting. It was critical that the condition of both the seedlings and the bed be adjusted to the transplanter before planting. Considering yield and inter-plant distance, optimal transplanter performance resulted with $60{\times}20cm$ or $60{\times}25cm$spacing, and the labor-saving efficiency using the transplanter was improved by over 93% of that of conventional planting by hand.

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NOONAN SYNDROME : A CASE REPORT (누난 증후군 : 증례보고)

  • Lee, Soo-Eon;Choi, Sung-Chul;Kim, Kwang-Chul;Na, Sung-Sik;Park, Jae-Hong
    • The Journal of Korea Assosiation for Disability and Oral Health
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    • v.7 no.2
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    • pp.123-126
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    • 2011
  • Noonan syndrome (NS) is a developmental disorder characterized by dysmorphic facial features in association with short stature, mental retardation and congenital heart disease. NS may be sporadic or inherited as an autosomal dominant or recessive trait. The children with NS usually have ocular hypertelorism, downslanting palpebral fissures, low-set ears and a webbed neck, chest deformity. In addition, oral features include micrognathia, high arched palate, dental malocclusion, dental anomalies and rarely, cleft palate. The phenotype of NS bears similarities to that of Turner syndrome. However, NS occurs in both males and females with a normal sex chromosome 46, XX and 46, XY constitution. This case presents the intermittent treatment of an 8-year-old girl who was referred from a local clinic for the extraction of supernumerary teeth and treatment of dental caries. The focus of this case report is the oral aspects on NS and particularities of the dental treatment in subjects affected by this genetic disease.

A Case Report of Cleidocranial Dysostosis (쇄골 두개 이골증의 증례보고)

  • Lim C.Y.;Kim S.W.;Ahn H.K.;You D.S.;Park T.W.
    • Journal of Korean Academy of Oral and Maxillofacial Radiology
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    • v.7 no.1
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    • pp.49-52
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    • 1977
  • The authors had observed a case of cleidocranial dysostosis of 42 years old unmarried woman. The observation was founded upon roentgenogram of skull P-A view, orthopantomograph, cephalometric view, chest P-A view and wrist X-ray view. The patient gave a dwarfic impression with 145㎝ body height and concaved mandibular prognastic facial feature. The palate was narrowed and extremely high arched. The eruption state of teeth were extremely poor, only four teeth were erupted, composed of upper third molar and lower three deformed teeth. The skull P-A view of roentgenogram reveals overlying metopic suture, extends from the nasion to the sagittal suture, and the suture are delayed up to date and many wormian bones are formed. On the cephalometric view the maxilla is underdeveloped and produce the false mandibular prognathism. Twenty four impacted teeth can be detected by orthopantmograph, twelve in upper jaw and twelve in lower jaw. There are many supernumerary teeth, one in upper jaw and seven in lower jaw. On the chest P-A view, the clavicles are totally aplastic, but a vestige of clavicle is visible in the right side. On the wrist roentgenogram, the epiphyseal ossification of distal phalanx, mesial phalanx and proximal phalanx is delayed.

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MANDIBULAR EXPANSION IN A CONGENITAL AGLOSSIA PATIENT (선천적 무설증 환자에서의 하악골 확장)

  • Cho, Jae-Yong;Kim, Jung-Wook;Jang, Ki-Taeg;Kim, Chong-Chul;Hahn, Se-Hyun;Lee, Sang-Hoon
    • Journal of the korean academy of Pediatric Dentistry
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    • v.30 no.4
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    • pp.673-677
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    • 2003
  • Congenital aglossia is a very rare condition. The oral manifestations of an aglossia include micrognathia, high arched or cleft palate, defects of the lower lip, an absence of lateral incisors and a mandibular growth deficiency. Although the etiology of congenital aglossia is unclear, both genetic and teratogenic mechanisms have been proposed. Treatment of aglossia patients depends on the nature and severity of the condition which includes surgical rehabilitation of the tongue tip to some extent, orthopedic expansion of the mandible to guide mandibular growth, and mandibular expansion by a distraction osteogenesis. In the present case, a 6 year old female aglossia patient with situs inversus was treated. A bonded hyrax screw was used to increase her mandibular primary intercanine width and intermolar width. A second phase orthodontic and surgical treatment will be possible after some retention phase.

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A Study on the Reuse Plan of Architectural Heritage as a Townscape Resource - Focused on an old office building of Leaf tobacco Producer' cooperation, Jecheon - (도시경관 자원으로서 건축문화재의 활용 방안에 관한 연구 - 제천 엽연초생산조합 구사옥을 중심으로 -)

  • Lee, Wan-Geon
    • Korean Institute of Interior Design Journal
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    • v.23 no.1
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    • pp.69-77
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    • 2014
  • This study aims to find the optimum method to reuse as townscape resource and to preserve the architectural heritage. And, it wants to contribute the policy of the architectural heritage and townscape. This research is targeted at an old office building of Leaf tobacco Producer' cooperation, designated as a cultural assets in Jecheon. It investigated the design characteristics of an old office building of Leaf tobacco Producer' cooperation and the streetscape of Uibyeong-daero 12-gil. It was conducted using the research methods of the field survey, and it was to analyze and to compare the various existing data and a current status. The result are as followings. Firstly, we should be consider the policy of architectural heritage that a sense of place can be preserved. Secondly, we should be consider the linear method of the conservation and reuse in urban street that the architectural heritage is located at least. Thirdly, if it will be to maintain the facade of the building adjacent to the street using the design characteristics of an old office building of Leaf tobacco Producer' cooperation, Uibyeong-daero 12-gil will be made a unique street that will be represented the history of leaf tobacco produce. Lastly, it has been analyzed that the design characteristics of an old office building of Leaf tobacco Producer' cooperation are the arch of entrance, the arched window, the rectangular window, the entabature, the horizontal joint, the finish of cement mortar, a pitched roof, etc. And, the design guideline and the maintenance plan of Uibyeong-daero 12-gil have made.

A Study on the Repair Method for Performance Degradation Cause of Korean Arch Bridge -Focused on the Seonamsa Seungseonggyo, Songgwangsa Geukrockgyo- (홍예교 성능저하 원인에 따른 보수방안 고찰 - 선암사 승선교·송광사 극락교를 중심으로 -)

  • Kim, Jeong-Eon;Cheon, Deuk-Youm
    • Journal of architectural history
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    • v.23 no.1
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    • pp.7-19
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    • 2014
  • This study considers the proper repair techniques by examining the most representative repair cases of the Korean arch bridges and proposes the constructional manual which can apply similar occasions. The cases are Seonamsa Seungseongyo and Songgwangsa Geukrockgyo where this researcher had taken part in the repair works. This Study proposes the maintenance construction manual about the performance degradation drew by performance degradation of the both Korean arch bridges in the maintenance process. First, arch bridge maintenance should be carried out in the dry season, when water is impermeable in the bottom surface of the bridge. Moreover, risk factors of the maintenance should be excluded to secure the water vally flow, the bypass and the temporary bridge. Second, prior to repair, it has to precede (1)3D shooting (2)formal examination (3)structure safety test (4)geological and lithic surveys (5)arch curvature establishment and makeshift frame settlement before transformation (6)relationship expert comments. Third, if the baduk and the foundation stones are inevitable to replace due to performance degradation on the foundation, it should use the high quality stones and secure greater stress by extending the standard range. The foundation on irregular rock needs to be flattened and underside on the replaced materials require Grengyijil to deliver the equal loads. Fourth, In the process of dismantling the stones of the arched bridge, it could make heavy weathering degree and not reuse the materials. Charge should converge the expert advices to choose the reuseable, the conservate and the alternative materials, and increase the reutilization of the raw materials by preservation and reinforcement treatments. Fifth, the side wall should be repaired by the rubble work technique which is not able to pile compost satiety, so it must use long depth of masonary stones for reinforcement. It is considered to reinforce the stone wall in shore as much as possible and protect the abutment and the side wall on the upstream for the arch bridge maintenance works.

Theoretical and experimental study on damage detection for beam string structure

  • He, Haoxiang;Yan, Weiming;Zhang, Ailin
    • Smart Structures and Systems
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    • v.12 no.3_4
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    • pp.327-344
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    • 2013
  • Beam string structure (BSS) is introduced as a new type of hybrid prestressed string structures. The composition and mechanics features of BSS are discussed. The main principles of wavelet packet transform (WPT), principal component analysis (PCA) and support vector machine (SVM) have been reviewed. WPT is applied to the structural response signals, and feature vectors are obtained by feature extraction and PCA. The feature vectors are used for training and classification as the inputs of the support vector machine. The method is used to a single one-way arched beam string structure for damage detection. The cable prestress loss and web members damage experiment for a beam string structure is carried through. Different prestressing forces are applied on the cable to simulate cable prestress loss, the prestressing forces are calculated by the frequencies which are solved by Fourier transform or wavelet transform under impulse excitation. Test results verify this method is accurate and convenient. The damage cases of web members on the beam are tested to validate the efficiency of the method presented in this study. Wavelet packet decomposition is applied to the structural response signals under ambient vibration, feature vectors are obtained by feature extraction method. The feature vectors are used for training and classification as the inputs of the support vector machine. The structural damage position and degree can be identified and classified, and the test result is highly accurate especially combined with principle component analysis.

Shprintzen-Goldberg syndrome with a novel missense mutation of SKI in a 6-month-old boy

  • Jeon, Min Jin;Park, Seul Gi;Kim, Man Jin;Lim, Byung Chan;Kim, Ki Joong;Chae, Jong Hee;Kim, Soo Yeon
    • Journal of Genetic Medicine
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    • v.17 no.1
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    • pp.43-46
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    • 2020
  • The Shprintzen-Goldberg syndrome (SGS) is an extremely rare genetic disorder caused by heterozygous variant in SKI. SGS is characterized by neurodevelopmental impairment with skeletal anomaly. Recognition of SGS is sometimes quite challenging in practice because it has diverse clinical features involving skeletal, neurological, and cardiovascular system. Here we report a case of a 6-month-old boy who initially presented with developmental delay and marfanoid facial features including prominent forehead, hypertelorism, high arched palate and retrognathia. He showed motor developmental delay since birth and could not control his head at the time of first evaluation. His height was above 2 standard deviation score. Arachnodactyly, hypermobility of joints, skin laxity, and pectus excavatum were also noted. Sequencing for FBN1 was negative, however, a novel missense variant, c.350G>A in SKI was identified by sequential whole exome sequencing. To our knowledge, this is the first case with SGS with phenotypic features of SGS overlapping with those of the Marfan syndrome, diagnosed by next generation sequencing in Korea.