• Title/Summary/Keyword: 무접합

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A Case of Neonatal Intrahepatic Cholestasis Caused by Citrin Deficiency (NICCD) Confirmed by SLC25A13 Mutation

  • Son, Yeong-Bae;Jang, Ju-Yeong;Park, Hyeong-Du;Lee, Su-Yeon
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.14 no.2
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    • pp.186-190
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    • 2014
  • Citrullinemia type 2 (citrin deficiency) is an autosomal recessive inborn error metabolism, caused by the SLC25A13 gene mutation. Citrin deficiency is associated with two clinical phenotype; neonatal-onset type II citrullinemia (CTLN2), also known as neonatal intraphepatic cholestasis caused by citrin deficiency (NICCD) and adult-onset CTLN2. Clinical manifestations of NICCD include poor growth, intrahepatic cholestasis, liver dysfunction and increased plasma citrulline, methionine, threonine, arginine. The molecular diagnosis could be confirmed by SLC25A13 gene mutation analysis. A 3-month-old male infant with persistent jaundice was referred for evaluation. Newborn screening was normal at birth. Mild elevation of serum ammonia and AST/ALT were observed. Plasma amino acid analysis showed significantly elevated citrulline, methionine, threonine. DNA sequence analysis of the SLC25A13 gene revealed two compound heterozygous mutations, c.[852_855del]($p.Met285Profs^*2$) and [1180+1G>A]. We suggest that NICCD should be considered as one of the cause of in infants with cholestatic jaundice, although the newborn screening was normal.

Experimental Study on the Flexural Performance of Steel Beams Reinforced by AFRP Sheets (아라미드 섬유 쉬트를 이용한 철골 보 부재의 휨 보강 성능에 관한 실험적 연구)

  • Kim, Kang Seok;Nah, Hwan Seon;Kim, Kang Sik;Lee, Hyeon Ju;Lee, Kang Min
    • Journal of the Korea institute for structural maintenance and inspection
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    • v.15 no.2
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    • pp.61-69
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    • 2011
  • Fiber Reinforced Plastic (FRP) sheets have been widely used to retrofit and rehabilitate RC structures, while in case of retrofitting steel structures, there are no codes and researches. It stems from configuration of member and characteristics of bonding behavior. This study focused on the static behavior of steel beams reinforcement by AFRP sheets. The main objective of the experimental programme was the evaluation of the force transfer mechanism, the increment of the beam load carrying capacity and the bending stiffness. A bending test was conducted on a H-shaped steel beam, with aramid FRP sheets bonded to its flanges. The mid-span deflection and the strain from three points along AFRP sheets were recorded Test results exhibit that the increment of the load-carrying capacity with reference to a mid-span deflection level of 15 mm(1/125mm of the clear span) was equal to 9.4% and for the two layers case, an elastic stiffness increment is slightly higher than one layer case.

Behavior of the herbicide metolachlor in carps (제초제 metolachlor의 잉어체내 행적)

  • Kyung, Kee-Sung;Kim, Jin-Wha;Lee, Byung-Moo;Oh, Byung-Youl;Jeong, Young-Ho;Lee, Jae-Koo
    • The Korean Journal of Pesticide Science
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    • v.3 no.2
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    • pp.54-59
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    • 1999
  • In order to investigate the behavior of the herbicide metolachlor [2-chloro-6'-ethyl-N-(2-methoxy-1-methylethyl)aceto-O-toluidide] in fish, carps (Cyprinus carpio L.) were exposed to the herbicide at $LC_{10}$(1.93 mg/L) for 4 days. Metolachlor dissolved in water was absorbed rapidly into carps to mark the maximum concentration 6 hours after exposure. The amounts of the $^{14}C$-metolachlor and its metabolites absorbed in gall were much higher than those in the other parts, strongly suggesting that biliary excretion involving enterohepatic recirculation could be an important route for the elimination of metolachlor. The $^{14}C$-radioactivity distributed into aqueous phase fraction in test water and in carp extract was increased in time-dependent manner. Extraction rate of $^{14}C$ absorbed in carp tissues was decreased remarkably up to 6 hours after exposure, suggesting that the possible polar metabolites of metolachlor were transformed into the conjugates to form non-extractable bound residues.

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Seam-line Determination in Image Mosaicking using Adaptive Cost Transform and Dynamic Programming (동적계획법과 적응 비용 변환을 이용한 영상 모자이크의 seam-line 결정)

  • Chon, Jae-Choon;Suh, Yong-Cheol;Kim, Hyong-Suk
    • Journal of the Korean Association of Geographic Information Studies
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    • v.7 no.2
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    • pp.16-28
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    • 2004
  • A seam-line determination algorithm is proposed to determine image border-line in mosaicing using the transformation of gray value differences and dynamic programming. Since visually good border-line is the one along which pixel differences are as small as possible, it can be determined in association with an optimal path finding algorithm. A well-known effective optimal path finding algorithm is the Dynamic Programming (DP). Direct application of the dynamic programming to the seam-line determination causes the distance effect, in which seam-line is affected by its length as well as the gray value difference. In this paper, an adaptive cost transform algorithm with which the distance effect is suppressed is proposed in order to utilize the dynamic programming on the transformed pixel difference space. Also, a figure of merit which is the summation of fixed number of the biggest pixel difference on the seam-line (SFBPD) is suggested as an evaluation measure of seamlines. The performance of the proposed algorithm has been tested in both quantitively and visually on various kinds of images.

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Study of the method of production of excavated arrow bundle and its conservation treatment (발굴 출토 화살다발 제작기법 연구 및 보존처리)

  • Lee, Byeonghoon;Choi, Bobae;Huh, Ilgwon
    • Conservation Science in Museum
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    • v.25
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    • pp.9-26
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    • 2021
  • This paper describes the production methods that were originally used for an arrow bundle excavated from a Bronze Age residential area in Auraji in Jeongseon, Gangwon-do Province and the conservation treatment process that it subsequently underwent. An arrow conventionally consists of an arrowhead and a shaft. It is rare to excavate a shaft along with an arrowhead in a complete form since the shaft is made of organic materials. Notably, the arrow bundle from the Auraji site is of great significance as it shows traces of tangless stone arrowheads attached to charred shafts and offers an important case of the split end of a piece of a tree being inserted into an arrowhead. For a further examination of the characteristics of the arrows from Auraji, microscopic investigation was conducted and the type of wood used for the arrow shafts was examined. The sequence and direction of processing and the particle sizes of the grinding tools were revealed through the analysis of traces of grinding on the stone arrowheads. The shaft is presumed to have been made from a green length of three-year-old willow (Salix spp.). A curing agent with a high degree of waterproofing and reversibility was used during the on-site curing process according to demands of the surrounding environment, and a technique that the authors call the "Bridge" method was used for emergency collection of the relics. Once the bundle was transferred to the conservation treatment lab, reinforcing materials were carefully chosen as it was important not to damage the relics during the process of turning them for the repair of their reverse sides. For this purpose, artificial clay was selected since it can safely bear a load and has excellent physical properties. Finally, detached parts were rejoined, the relics and their surrounding materials were cleaned, and the bottom sides were finished with epoxy resin prior to the display of the relics at the museum.

An Experimental Study on Seismic Performance of Two-story Reinforced Concrete Frames Retrofitted with Internal Steel Frame and Wall Type Friction Damper (내부 철골끼움골조 및 벽체형 마찰댐퍼(WFD)로 보강된 2층 철근콘크리트골조 내진성능에 대한 실험적 연구)

  • Yoo, Chang-Gi;Choi, Chang-Sik
    • Journal of the Korea institute for structural maintenance and inspection
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    • v.26 no.6
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    • pp.64-72
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    • 2022
  • In this study, in order to confirm the seismic performance of reinforced concrete frames retrofitted with Wall Friction Damper(WFD), the test was conducted by setting two-story Reinforced concrete frames (reference specimen, OMF-N and specimen retrofitted with internal H-shaped steel frame and WFD, OMF-ALL(H)) as main variables. The WFD Seismic Retrofit Method is a mixture of strength improvement and energy dissipation methods. To prevent the pre-destruction of existing structure by friction force before sufficient energy dissipation of WFD, the internal H-shaped steel frame and chemical anchor that penetrates the side of the beam were used to install WFD. According to the test results, the OMF-N specimen showed an brittle failure pattern caused by the shear force of the R/C column after the maximum strength was expressed. The OMF-ALL(H) specimen showed that the reduction of pinching effect and the failure of the RC column occurred. Also, the maximum strength, cumulative energy dissipation and ductility of OMF-ALL(H) increased 3.01 times, 7.2 times and 1.72 times for OMF-N. As a results, test results revealed that the WFD Seismic Retrofit Method installed on Reinforced concrete structure improves the seismic performance and the strengthening effect is valid.

A Comparison of Three Suture Techniques on Adhesion in End-to-end Intestinal Anastomosis of Dogs (개에서 세가지 단단장문합 봉합법에 따른 유착비교)

  • Kim, Je-Sun;Jeong, Soon-Wuk;Kim, Joon-Young;Jeong, Man-Bok;Han, Hyun-Jung
    • Journal of Veterinary Clinics
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    • v.20 no.1
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    • pp.12-21
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    • 2003
  • In this study, we evaluated effects of three anastomotic techniques of small intestine on adhesions in the dog. Twenty six healthy mixed dogs were randomly assigned to three groups. Group I(n = 8) was sutured with a simple continuous suture, group II(n = 7) was sutured with a simple interrupted approximating suture and group III(n = 11) was sutured with a single layer continuous Connell suture. On completion of any intestinal anastomosis, a pedicle of greater omentum was wrapped around the suture line in all experimental dogs. One percent sodium carboxymeth-ylcellulose (5ml/kg) was administrated into the abdomen by feeding tube prior to closing the last part of peritoneum in all dogs. Postoperative adhesions were evaluated at 14th day after operation. The adhesions consisted primarily in two dogs in group I, three dogs in group II and group III. There were adhesions between intestinal serosal surfaces in eight dogs in all groups, but there were no intestinal serosa-visceral peritoneum adhesion and intestinal serosa-mesentery adhesion. Mean adhesion scores were less than score 2 in all groups. Between anastomotic site and omental graft, there were 13.13$\pm$4.97 mm (mean$\pm$S.D.) adhesion formation in group I and 17.29$\pm$4.68 mm in group II and 14.64$\pm$3.80mm in group III. A simple continuous suture resulted in the least adhesion formation and a simple interrupted approximating suture resulted in the greatest adhesion formation among the groups. However, there were no significant differences among three suture techniques in the severity of adhesions. Intestinal intussusception only encountered in one dog during the 14 days, the dog operated and survived. Daily monitoring of temperature, activity, appetite, defecation and micturition were done. All of those vital signs were within normal values and there were no obvious differences among the groups. In conclusion, even though there were no significant differences among three groups, a simple continous suture pattern is recommended to prevent adhesions when operating intestinal anastomosis in dogs.

Studies on Genetic Diversity and Phylogenetic relationships of Chikso (Korea Native Brindle Cattle) Using the Microsatellite Marker (Microsatellite marker를 활용한 칡소의 유전적 다양성 및 유연관계 분석)

  • Choy, Yun Ho;Seo, Joo Hee;Park, Byungho;Lee, Seung Soo;Choi, Jae Won;Jung, Kyoung-sub;Kong, Hong Sik
    • Journal of Life Science
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    • v.25 no.6
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    • pp.624-630
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    • 2015
  • This study examined the genetic distance among Chikso (Korea native brindle cattle) in nine regional areas using allele frequencies and a genetic diversity analysis with microsatellite markers. The analysis of the genetic diversity and genetic relationships of 2068 Chikso (383 KW, 180 GG, 52 KN, 129 KB, 332 UL, 24 JN, 198 JB, 148 CN, 622 CB) was carried out using 11 microsatellite markers. The number of alleles, observed heterozygostiy (Hobs), expected heterozygosity (Hexp), and polymorphism information content (PIC) of the 11 microsatellite markers were 8–24, 0.672–0.834, 0.687–0.886, and 0.638–0.876, respectively. The expected probability of identity values in random individuals (PI), random half-sib (PIhalf-sibs), and random sibs (PIsibs) were estimated to be 5.24×10−19, 2.63×10−06, and 2.63× 10−06, respectively, indicating that these markers can be used for traceability systems in Chikso cattle. The results of a phylogenetic tree (neighbor-joining tree), principle component analysis (PCA), and factorial component analysis (FCA) revealed genetic distance among nine Chikso populations. In conclusion, this study provides useful basic data that can be utilized in Chikso breeding and development. In addition, we will have to manage and conserve as a valuable genetic resource, without losing diversity of Chikso.

Effect of Dietary Anti-oxidant Supplementation on Telomere Length and Egg Quality in Laying Hens (산란계에 항산화물질 급여가 텔로미어 함량 및 난질에 미치는 영향)

  • Lee, M.H.;Lee, S.H.;Kim, Y.J.;Ko, Y.H.;Jang, I.S.;Moon, Y.S.;Choi, Y.H.;Sohn, S.H.
    • Korean Journal of Poultry Science
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    • v.35 no.3
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    • pp.267-274
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    • 2008
  • There are many kinds of anti-oxidant materials in natural plant resources. The Siberian ginseng and Eucommia are well known as anti-oxidant and medicinal plants. To investigate the effect of their anti-oxidant-like activity on telomere quantity and egg quality, diets containing Siberian ginseng leaf and Eucommia leaf at 0.5% and 1% were given Hyline Brown commercial laying hens during two periods of age: 20 to 30 wks and 60 to 70 wks. The amount of telomere in lymphocyte, liver, ovary, heart and lung was analyzed by quantitative fluorescence in situ hybridization using telomeric DNA probe. Egg weight, albumin height, Haugh unit, egg yolk color, egg shell color, egg shell thickness, egg shell weight and egg shell density were measured to analyze egg quality. The chickens consuming diets Siberian ginseng and Eucommia had higher telomeric DNA in lymphocytes than control chickens in younger layers whereas no significant differences were detected in all target cells analyzed from older layers. Egg quality was increased in younger hens with dietary supplementation as determined by egg weight, albumin height and Haugh unit but there were no effects in older hens. These results imply that dietary supplementation of Siberian ginseng and Eucommia in layers improves bio-activity and egg quality at early laying stage.

A Newborn Case of Maple Syrup Urine Disease Type 1B Presenting with Lethargy and Central Apnea (기면과 중추성 무호흡으로 나타난 단풍시럽뇨병 Type 1B 신생아 1례)

  • Kang, Youngtae;Choi, Sung Hwan;Ko, Jung Min;Shin, Seung Han;Kim, Ee-Kyung;Kim, Han-Suk
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.18 no.2
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    • pp.43-49
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    • 2018
  • Maple syrup urine disease (MSUD, OMIM#248600) is a rare and autosomal recessively-inherited metabolic disorder that is caused by mutations in the branched-chain ${\alpha}$-ketoacid dehydrogenase (BCKDH) genes. It prevents the normal breakdown of branched-chain amino acids (BCAAs), such as leucine, isoleucine, and valine, and leads to poor feeding, lethargy, abnormal movements, seizure, and death if untreated. Here, we report the case of a Korean newborn of biochemically- and genetically-confirmed MSUD manifesting lethargy and central apnea, the acute state of which was successfully treated. The molecular genetic investigation revealed two novel heterozygous mutations (p.Ala32Phefs*48 and p.Val 130Phe) in BCKDHB, and both parents were confirmed as carriers. We emphasize the importance of early diagnosis and prompt introduction of specific treatment for MSUD in life saving and prognosis.

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