• 제목/요약/키워드: Dominant delay

검색결과 92건 처리시간 0.024초

Bannayan-Riley-Ruvalcaba Syndrome in a Patient with a PTEN Mutation Identified by Chromosomal Microarray Analysis: A Case Report

  • Lee, Sun Hwa;Ryoo, Eell;Tchah, Hann
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • 제20권1호
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    • pp.65-70
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    • 2017
  • Bannayan-Riley-Ruvalcaba syndrome (BRRS) is one of the phosphatase and tensin homolog hamartoma tumor syndrome with a PTEN gene mutation. It is a rare dominant autosomal disorder characterized by cutaneous lipomas, macrocephaly, intestinal polyps, and developmental delay. Diagnosing this syndrome is important, because it may represent the pediatric phenotype of Cowden syndrome, in which there is an increased risk for malignant tumors in children. Until now, the prevalence of BRRS is unknown. Several dozen cases have been reported in the medical literature, but no case has been reported in Korea. Here we report a case of a 19-year-old girl who was diagnosed with BRRS because of macrocephaly, intellectual disability, and intestinal polyps. Her mother had similar findings and a PTEN mutation. Neither patient had mutations detected by conventional mutation-detection techniques, but a PTEN gene deletion was demonstrated by chromosomal microarray analysis.

Learning in the WTO/DDA Negotiations?: An Experimental Study

  • Sung, Hankyoung
    • East Asian Economic Review
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    • 제19권3호
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    • pp.243-273
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    • 2015
  • The purpose of this paper is to identify learning in games in experimental economic settings, and apply their results to real multilateral trade negotiations, such as the Doha Development Agenda (DDA) in the World Trade Organizations (WTO). This paper argues that the structure of games including a veto player (Veto games) is similar to the WTO/DDA negotiations in that the players do not possess identical power. This paper's main contribution to the literature involves showing that learning about power is dominant over learning from simple repetition in Veto games. Additionally, this paper shows that players are concerned about how much they have gained in previous games in Veto games, although their memories generally do not last beyond the next game, and thus they tend to be selfish as they have less shares. Based on these results, there is a possibility to be more generous in the distribution of benefits by allowing players without veto power to retain special rights so that they would not be totally powerless. It also shows the necessity of having "respite" in the process of negotiations and policy options for choosing partners for winning coalitions.

부분 모드만을 고려하여 성형된 입력을 이용한 잔류 진동의 감소 효과 (Reducing Effect of Residual Vibration Through Command Input Shaped Considering Partial Modes)

  • 정광석
    • 융복합기술연구소 논문집
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    • 제1권1호
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    • pp.18-23
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    • 2011
  • Shaping an input command through considering the resonant modes of multi degrees of freedom system, it is possible to realize the wanted motion, without exciting the uncontrollable modes of the flexible system. But, an increase of modes to be considered brings inevitably about the time delay due to an excessive rising time. On the purpose of reducing the rising time, only the interesting and dominant modes can be considered to determine the timing pulses of input shaper. In this paper, an effect of shaper by the partial modes is analysed for a specific system and the input shapers by the partial modes are analysed for three d.o.f damped system, using Matlab simulation.

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A Korean case of CTCF related neurodevelopmental disorders

  • Seong Ryeong Kang;Soo Hyun Seo;Kyunghoon Kim;Hee Bum Yang;Hye Ran Yang;Anna Cho
    • Journal of Genetic Medicine
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    • 제20권2호
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    • pp.70-74
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    • 2023
  • CCCTC-binding factor (CTCF) is a transcriptional regulator that binds to a complex DNA motif in various orientations and plays a crucial role in regulating gene expression, chromatin restructuring, and developmental processes. Mutations in the CTCF are associated with neurodevelopmental disorders. Here we report the first Korean case with a de novo heterozygous variant in the CTCF (c.1025G>A; p.Arg342His). She showed global developmental delay, failure to thrive, and dysmorphic face, which are phenotypes consistent with previous reports in the autosomal dominant intellectual developmental disorder 21 (MIM 615502). She also showed clinical features not previously reported, such as antral web and tracheobronchomalacia. Our case follows suit and expands understanding of this rare disorder by reporting common features and, on the other hand, unreported concomitant congenital anomalies.

PDV를 이용한 가상 버퍼상의 AAL2 패킷 폐기 알고리즘과 성능분석 (Performance Analysis of AAL2 Packet Dropping Algorithm using PDV on Virtual Buffer)

  • 정다위;조영종
    • 대한전자공학회논문지TC
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    • 제39권1호
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    • pp.20-33
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    • 2002
  • 패킷을 이용한 음성 서비스는 무선 망을 통한 음성 서비스 등으로 구현되고 있으며, 미래의 모든 음성 서비스는 패킷 기반형으로 진화할 것이다. 이러한 측면에서 ATM의 AAL2를 이용한 음성 패킷의 전달은 백본 망에서 음성 패킷의 전달 효율을 증가시키는 대표적인 방법이다. AAL2를 이용한 음성 서비스의 경우, 망의 자원이 허락되면 새로운 호의 연결을 수락한다. 하지만 새로운 호에 의해 발생되는 패킷으로 기존의 AAL2 연결의 전송 지연이 급격하게 증가할 가능성이 존재한다. 따라서 본 논문에서는 AAL2를 이용한 음성 서비스를 제공하는데 있어, AAL2 버퍼 관리 매카니즘으로 N개의 호에 대해 N개의 가상 버퍼를 통하여 각각의 호에 대한 전송지연변이를 계산하고, 전송 지연 변이에 따른 패킷 폐기 알고리즘을 제시한다. 이러한 패킷 폐기 알고리즘은 새로운 호의 수락으로 인해 발생하는 전송지연변이의 증가때문에 기존의 호의 QoS가 급격하게 악화되는 것을 방지한다. 그리고 종단 대 종단 지연에 영향을 미치는 AAL2 패킷 구성과 ATM 셀 구성의 요소를 분석하고, 음성 서비스를 ATM 셀로 만드는 과정을 K-정책을 갖는 M/D/1 큐잉시스템과 MIN(K, Tc) 정책을 갖는 M/D/1 큐잉 시스템으로 모델링함으로써, AAL2 패킷을 ATM 셀로 만드는 과정에서의 ATM 셀 밀집도와 AAL2 버퍼에서의 지연시간을 분석하였다. 제안하는 알고리즘을 모의 실험하여서 큐잉 시스템 모델링 결과와 비교하고 성능 결과를 분석하였다. 성능 분석 결과, AAL2 패킷 생성 시간과 ATM셀 생성 시간은 음성 소스의 갯수가 증가할 경우 전송 지연에 영향을 미치지 못하였으며, 제안하는 알고리즘은 음성 소스의 QoS를 저하하지 않은 수준에서 전송 지연을 낮게 유지할 수 있었다.

Diagnostic exome sequencing을 통한 KBG 증후군의 조기 진단 (Early Diagnosis of KBG Syndrome Using Diagnostic Exome Sequencing)

  • 홍준호;김세희;이승태;최종락;강훈철;이준수;김흥동
    • 대한소아신경학회지
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    • 제26권4호
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    • pp.272-275
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    • 2018
  • KBG 증후군은 특징적인 얼굴 기형 및 발달 장애, 저 신장 등을 보이는 희귀한 질환이며, ANKRD11유전자의 변이가 KBG 증후군을 일으킨다고 알려져 있다. 그 임상 양상의 스펙트럼은 넓은 편이며, 발달 장애와 신경학적 이상의 경우 개인마다 다양한 정도로 보고되고 있다. 본 증례의 환자들 역시 서로 다른 정도의 발달 장애를 보였으며, 그 중 더 심한 정도의 발달 장애를 가진 환자에서는 뇌전증이 동반되었다. 기존의 KBG증후군의 진단 기준에서 macrodontia는 매우 중요한 요소였으며, 대부분의 KBG 증후군 환자에서 나타난다고 보고되었다. 본 증례의 환자들은 발달장애를 보여 시행한 diagnostic exome sequencing을 통해 ANKRD11 유전자 이상을 확인하였지만 macrodontia는 관찰되지 않았다. 이는 KBG 증후군이 현재까지 밝혀진 것 보다 더 흔할 수 있으며, 특징적인 얼굴 기형이 없는 경우에도 발달장애가 있는 환자들에서는 더욱 적극적인 유전자 검사를 시행하여 KBG 증후군을 진단 할 필요가 있음을 시사한다.

미국의 사법형 ADR제도와 그 함의에 대한 연구 (A Study of the Court-Annexed ADR and Its Implications in the United States)

  • 김진현;정용균
    • 한국중재학회지:중재연구
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    • 제21권3호
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    • pp.55-87
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    • 2011
  • This paper is to illustrate a variety of court-annexed ADR programs and vindicate its implications of court-annexed ADR in United States. It has been almost three decades since Frank Sender articulated his vision of the multi-door courthouse. The court-annexed ADR originated from the concept of multi-door court house. Professor Sander argued that the court must transform from the court that provides litigation, only one type of dispute resolution, to the multi-door courthouse which provides a variety of dispute resolution methods including a number of ADR programs. The types of court-annexed ADR on which this paper focus are court-annexed mediation, court-annexed arbitration, mini trial, early neutral evaluation(ENE), summary jury trial, rent-a-judge, and med-arb in United States. The findings of this paper is as follows. First, the ADR movement is the irreversible and dominant phenomenon in the US court. The motivation of incorporating ADR into court is to reduce the cost of court to handle the civil disputes and to eliminate the delay of litigation process in the court. At the same time, a couple of studies of ADR revealed that the ADR program satisfied users of ADR. Second, the landscape of ADR has not been fixed. In 1970's, the court-annexed arbitration has been popular. In 1980's, the diverse kinds of ADR programs were introduced into the federal court as well as state courts, such as mini trial, early neutral evaluation(ENE), summary jury trial, and court-annexed mediation. But in 2000s, the court-annexed mediation has been the dominant type of ADR in United States. Third, the each type of ADR program has its own place for the dispute resolution. Since Korean society enters into the stage in which diverse kind of disputes occur in the areas of environment, construction, medicare, etc, it is desirable to take into consideration of the introduction of ADR to dispute resolution in Korea.

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Influence of microbial additive on microbial populations, ensiling characteristics, and spoilage loss of delayed sealing silage of Napier grass

  • Cai, Yimin;Du, Zhumei;Yamasaki, Seishi;Nguluve, Damiao;Tinga, Benedito;Macome, Felicidade;Oya, Tetsuji
    • Asian-Australasian Journal of Animal Sciences
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    • 제33권7호
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    • pp.1103-1112
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    • 2020
  • Objective: To measure whether a microbial additive could effectively improve the fermentation quality of delayed-sealing (DS) silage, we studied the effects of inoculants of lactic acid bacteria (LAB) and cellulase enzyme on microbial populations, ensiling characteristics, and spoilage loss of DS silage of Napier grass in Africa. Methods: Quick-sealing (QS) and DS silages were prepared with and without LAB (Lactobacillus plantarum) inoculant, cellulase enzymes, and their combination. The QS material was directly chopped and packed into a bunker silo. The DS material was packed into the silo with a delay of 24 h from harvest. Results: In the QS silage, LAB was dominant in the microbial population and produced large amounts of lactic acid. When the silage was treated with LAB and cellulase, the fermentation quality was improved. In the DS silage, aerobic bacteria and yeasts were the dominant microbes and all the silages were of poor quality. The yeast and mold counts in the DS silage were high, and they increased rapidly during aerobic exposure. As a result, the DS silages spoiled faster than the QS silages upon aerobic exposure. Conclusion: DS results in poor silage fermentation and aerobic deterioration. The microbial additive improved QS silage fermentation but was not effective for DS silage.

고령자 남녀의 발목관절 근육 반응시간의 비교 (Comparison of Reaction Times of Ankle Joint Muscles in the Elderly Men and Women)

  • 김지원;정홍영;김효희;권유리;엄광문;이정환;김경섭;전재훈;박병규
    • 대한의용생체공학회:의공학회지
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    • 제33권4호
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    • pp.163-168
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    • 2012
  • Elderly women are reported to have greater risk of falls. The purpose of this paper was to investigate the possible gender differences in the reaction performance of ankle joint muscles, which have dominant role in the control of sagittal plane balance. Twenty-six elderly men and women with comparable mean age participated in this study. Reaction times to the audible beeps were measured in the tibialis anterior muscle and gastrocnemius muscle. Reaction time variables included premotor time, electromechanical delay and total reaction time. Gender difference in each reaction time was investigated by independent t-test. In both muscles, premotor time was longer in men but the electromechanical delay was longer in women (p < 0.05). Resulting total reaction time was longer in men in tibialis anterior muscle (p < 0.01) and it tended to be longer in men also in gastrocnemius muscle (p = 0.25). The results demonstrates that the overall reaction performances of elderly women is better than or comparable to those of elderly men in ankle joint muscles. This suggests that the reaction performance of ankle muscles is hardly the cause of the greater risk of falls in elderly women.

DYNAMICAL CHARACTERISTICS OF SUNSPOT CHROMOSPHERES II. ANALYSIS OF CA II H, K AND ${\lambda}8498$ LINES OF A SUNSPOT (SPO 5007) FOR OSCILLATORY MOTIONS

  • Yoon, Tae-Sam;Yun, Hong-Sik;Kim, Jeong-Hoon
    • 천문학회지
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    • 제28권2호
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    • pp.245-253
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    • 1995
  • We have analyzed the time series of Ca II H,K and ${\lambda}8498$ line profiles taken for a sunspot (SPO 5007) with the Echelle spectrograph attached to Vacuum Tower Telescope at Sacramento Peak Solar Observatory. Each set of spectra was taken simultaneously for 20 minutes at a time interval of 30 seconds. A total of 40 photographic films for each line was scanned by a PDS at Korea Astronomy Observatory. The central peak intensity of Ca II H ($I_{max}$), the intensity measured at ${\Delta}{\lambda}=-0.1{\AA}$ from the line center of ${\lambda}8498(I_{{\lambda}8489})$, the radial velocity ($V_r$) and the Doppler width (${\Delta}{\lambda}_D$) estimated from Ca II H have been measured to study the dynamical behaviors of the sunspot chromosphere. Fourier analysis has been carried out for these measured quantities. Our main results are as follows: (1) We have confirmed the 3-minute oscillation being dominant throughout the umbra. The period of oscillations jumps from 180 sec in the umbra to 500 to 1000 sec in the penumbra. (2) The nonlinear character of the umbral oscillation is noted from the observed sawtooth shaped radial velocity fluctuations with amplitudes reaching up to $5{\sim}6\;km/sec$. (3) The spatial distribution of the maximum powers shows that the power of oscillations is stronger in the umbra than in the penumbra. (4) The spatial distributions of the time averaged < $I_{max}$ > and < $V_r$ > across the spot are found to be nearly axially symmetric, implying that the physical quantities derived from the line profiles of Ca II H and ${\lambda}8498$ are inherently associated with the geometry of the magnetic field distribution of the spot. (5) The central peaks of the CaII H emission core lead the upward motions of the umbral atmosphere by $90^{\circ}$, while no phase delay is found in intensities between $I_{max}$ and $I_{{\lambda}8498}$, suggesting that the umbral oscillation is of standing waves.

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