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CASE REPORT OF RUBINSTEIN-TABYI SYNDROME (Bubinstein-Tabyi syndrome에 관한 증례보고)

  • Jung, Sung-Ho;Yang, Kyu-Ho;Lee, Tae-Hee
    • Journal of the korean academy of Pediatric Dentistry
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    • v.28 no.1
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    • pp.166-170
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    • 2001
  • Rubinstein-Tabyi syndrome(RTS) is a congenital multisystem dysmorphic syndrome with many non-specific features, making diagnosis occasionally difficult. The major features of this syndrome include mental retardation, characteristic facial appearance, short stature, microcephaly, and broad thumbs and halluces. This syndrome was first described by Rubinstein and Tabyi in 1963, and many studies have been continued about this syndrome, but specific pathogenesis of the Rubinstein-Tabyi syndrome phenotype is still not clear. High arched palate, micrognathia and multiple caries etc have been reported in Rubinstein-Tabyi syndrome. In this report, a 6-year and 5-month-old boy visited at our department due to multiple dental caries, who showed broad thumbs, mental and physical development retardation, and characteristic facial appearance including both ptosis and ear deformity. This patient was diagnosed as a Rubinstein-Tabyi syndrome, and treated the multiple dental caries under general anesthesia. This study was aimed to observe the relationship between medical and dental characteristics.

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MESIODENS EXTRACTION OF A PATIENT WITH ROBINOW SYNDROME UNDER GENERAL ANESTHESIA (로비노 증후군(Robinow syndrome) 환자의 전신마취 하 과잉치 발치)

  • Park, Sung-Hee;Shin, Teo-Jeon;Hyun, Hong-Keun;Kim, Young-Jae;Kim, Jung-Wook;Lee, Sang-Hoon;Kim, Chong-Chul;Jang, Ki-Taeg
    • The Journal of Korea Assosiation for Disability and Oral Health
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    • v.12 no.1
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    • pp.1-5
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    • 2016
  • Robinow syndrome is skeletal dysplasia with both autosomal dominant and recessive inheritance patterns. It is characterized by short-limbed dwarfism, abnormalities in the head and face, as well as vertebral segmentation. A 2-year-7-month old boy with Robinow syndrome had visited Seoul National University Dental Hospital, for the evaluation of tooth palatal eruption on maxilla. He had micrognathia, delayed tooth eruption, cleft lip with bifid uvula. He also had an erupted mesiodens on the palatal side of maxillary primary incisors, which was tuberculated and 8mm in major diameter. The patient was scheduled for mesiodens extraction under general anesthesia. He was a young child with delayed development, so general anesthesia was inevitable. General anesthesia was induced and maintained with inhalation agent, Sevoflurane. There were no postoperative complications related to anesthesia and dental treatment. Robinow syndrome patients have craniofacial dysmorphism and eruption disorders. Therefore, he requires regular check-ups as well as dental managements.

Cell Biological Study on Factors Affecting Brain Formation at Early Chick Embryo (1) The Effect of Serotonin (초기 계배의 뇌형성에 미치는 몇가지 요인에 관한 세포 생물학적 연구 (1) Serotonin의 영향)

  • 최임순;주상옥;주충노;오억수;신길상
    • The Korean Journal of Zoology
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    • v.32 no.1
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    • pp.55-73
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    • 1989
  • The effect of tryptophan or serotonin on the early stage of chick brain development has been morphologically investigated using an electron microscope. The electron micrographs of neural plate cells of 1-day chick embryo treated with tryptophan or serotonin showed irregularity, evagination and disruption of nuclear membrane and nuclear chromatin condenstation, nucleolar margination and segregation. Hypertrophy of stalks, vesicles and vaculoes were seen and dilated and disrupted rough endoplasmic reticulum and underdeveloped neurotubules were also observed. In mesenchyme cells of tryptophan or serotonin administered 18 hr embryo, irregular nuclear membrane, swollen mitochondria, dilated rough endoplasmic reticulum and very large yolk granules were observed. Furthermore, DNA, RNA and protein contents of the embryos treated with typtophan or serotonin were considerably lower than those of control group. The amount of tubulin of the experimental groups was also greatly lower than that of control, suggesting that the impairment of microtubule formation occurred. Tryptophan or serotonin administration might depress the biosynthesis, of nucleic acid and protein including some enzymes tested. It seems that the serotonin formed from exogeneous tryptophan might inhibit the degradation of yolk granule by feedback regulation mechanism so as to impair microtububle and microvilli formation followed by a malformation of chick embryos.

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A CASE OF CORENELIA DE LANGE SYNDROME WITH MENTAL RETARDATION AND AUTISTIC DISORDER (정신지체와 자폐장애를 보이는 Cornelia De Lange 증후군 1예)

  • Kim, Se-Joo;Choi, Nak-Kyoung;Song, Jung-Eun
    • Journal of the Korean Academy of Child and Adolescent Psychiatry
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    • v.14 no.1
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    • pp.123-127
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    • 2003
  • Cornelia de Lange syndrome is a dysmorphogenic disorder characterized by multiple congenital abnormalities, mental retardation, growth retardation and neurodevelopmental abnormalities. Diagnosis for the Cornelia de Lange syndrome is dependent on the clinical observation because neither definite biological marker nor definite chromosomal abnormality have been investigated. Clinical observation is important for the diagnosis, so we report a case of Corenelia de Lange syndrome with mental retardation and autistic disorder. The patient is a 6-year old girl. Her motor development and language development have been delayed. She could say no meaningful word and understood simple command partially. She showed poor eye contact and poor emotional interaction. Social interaction was impaired and she Showed stereotypic behaviors. Thus we diagnosed her as mental retardation with autistic disorder. She had vesicoureteral reflux, frequent upper respiratory infection and pneumonia. She had experienced febrile convulsions 4 times. She had short stature, confluent eyebrows, long eyelashes, and upturned nose with anteverted nostrils. She also showed low hairline and hypertrichosis in body and extremities. Her finger was short. In this case, we diagnosed Cornelia de Lange syndrome by her characteristic face, hypertrichosis and medical and behavioral problems that were frequently showed in this syndrome.

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Study on the neurodevelopmental predictors for the results of the Bayley Scales of Infant Development II in high-risk neonates (고위험 신생아에서 Bayley 발달 검사 결과에 영향을 미치는 신경발달 예후인자에 대한 연구)

  • Woo, Mi Kyeong;Kim, Dong Wook;Huh, Kyoung;Shim, Gyu Hong;Chey, Myoung Jae
    • Clinical and Experimental Pediatrics
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    • v.52 no.11
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    • pp.1221-1227
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    • 2009
  • Purpose:To identify the risk factors for poor neurodevelopmental outcomes in high-risk neonates. Methods:We studied 94 infants admitted to the neonatal intensive care unit at the Sanggye Paik Hospital between January 2002 and November 2005 and evaluated the follow-up data. The following events were considered as risk factors: ${\leq}32$ weeks of gestation, very low birth weight, Apgar scores of less than 5, neonatal resuscitation, neonatal seizure, congenital infection, and abnormalities in cranial ultrasound or magnetic resonance imaging (MRI). The infants who had any one of these risk factors were included. They were evaluated at their mean corrected age of 13.84${\pm}$8.03 months. Mental and Psychomotor Developmental Indices (MDI, PDI) were determined by a clinical psychologist using the Bayley Scales of Infant Development II. Results:The mean MDI and PDI of all the patients were 96.28${\pm}$26.70 and 94.00${\pm}$22.80, respectively. Abnormalities on cranial ultrasound or MRI were significant predictors of both low MDI (P<0.05) and low PDI (P<0.001). These abnormalities showed a stronger association with low PDI than with low MDI. The infants with periventricular leukomalacia had the lowest MDI and PDI ($70.10{\pm}28.68$ and $69.70{\pm}24.91$, respectively). Apgar score at 1 minute and very low birth weight were also significant predictors for low PDI (P<0.05, P<0.05, respectively). Conclusion:Image findings with cranial ultrasound or MRI were the strongest predictors of neurodevelopmental outcome. Periventricular leukomalacia was the best predictive factor for mental and psychomotor development.

Growth and Yield Response of Rice Cultivars to Different Night illumination Intensities (야간조명 광도에 따른 벼 품종별 생육 및 수량 반응)

  • 김충국;조현숙;서종호;최성호;변종영
    • KOREAN JOURNAL OF CROP SCIENCE
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    • v.47 no.6
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    • pp.471-474
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    • 2002
  • This experiment was conducted to investigate the response of rice growth and grain yield to night illumination. Three cultivars Heugjubyeo (early maturing), Hwaseongbyeo (medium maturing), and Ilpumbyeo(mid-late maturing) were tested under different light intensities ranging 1.0 to 20.0 lux (0.3 to 5.6 $1\mu\textrm{W}$ $cm^{-2}$). Awn length of all cultivars became longer as night illumination intensity increased; 4.09 cm under 10-20 lux compared to 1.93cm under 2 lux in Hwaseongbyeo. However culm and spike length ere not affected by night illumination. Night illumination delayed flowering by 3 days in Heugjinjubyeo, 5 days in Hwaseongbyeo, and 9 days in Ilpumbyeo at 10-20 lux compared to that at 2 lux. Grain yield was reduced by 10.0% in Heugjinjubyeo, 17.7% in Hwaseongbyeo, and 20.7% in Ilpumbyeo at 10-20 lux compared to 2 lux condition.

An Offloading Decision Scheme Considering the Scheduling Latency of the Cloud in Real-time Applications (실시간 응용에서 클라우드의 스케줄링 지연 시간을 고려한 오프로딩 결정 기법)

  • Min, Hong;Jung, Jinman;Kim, Bongjae;Heo, Junyoung
    • KIISE Transactions on Computing Practices
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    • v.23 no.6
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    • pp.392-396
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    • 2017
  • Although mobile device-related technologies have developed rapidly, many problems arising from resource constraints have not been solved. Computation offloading that uses resources of cloud servers over the Internet was proposed to overcome physical limitations, and many studies have been conducted in terms of energy saving. However, completing tasks within their deadlines is more important than saving energy in real-time applications. In this paper, we proposed an offloading decision scheme considering the scheduling latency in the cloud to support real-time applications. The proposed scheme can improve the reliability of real-time tasks by comparing the estimated laxity of offloading a task with the estimated laxity of executing a task in a mobile device and selecting a more effective way to satisfy the task's deadline.

Analysis Simultaneously Switching Density Using Ring Oscillator (Ring Oscillator를 이용한 신호의 동시 스위칭 밀도 분석)

  • Jeong, Sang-Nam;Baeg, Sang-Hyeon
    • Journal of the Institute of Electronics Engineers of Korea SD
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    • v.45 no.9
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    • pp.79-84
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    • 2008
  • Switching speeds increase in both frequency and the transition rate of edges. Inadequate forecast for simultaneous switching signals may cause designing the power planes without sufficient current capability. The delay of critical signals in a chip can be therefore inadvertently increased and the situation makes it hard to debug issues. It is important to find the degree of increased switching during the debugging or chip characterization phases. This paper proposes the interpolation method to predict the switching density in a design. The interpolation was achieved by utilizing the dependencies between switching frequency and the delay appeared in a ring oscillator. The ring oscillator was primarily used to accumulate the effects of the ground bounce by higher switching. The result of interpolation was demonstrated using DongBu Hitec 0.18um CMOS technology.

Interconnection of P2PSIP Overlay and IMS Network and Its Characteristics (P2PSIP Overlay와 IMS 네트워크간 상호접속 및 특성)

  • Kim, Hyun-Ji;Han, Chi-Moon
    • Journal of the Institute of Electronics Engineers of Korea TC
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    • v.47 no.10
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    • pp.57-66
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    • 2010
  • Today the various types of communication and application service are provided by the development of Internet and IP technologies. It is expected to be extended the service domain of two networks through the interconnecting P2PSIP overlay which is highlighted as an important technologies for Internet based services and IMS(IP Multimedia Subsystem) which is the new architecture adopted in the evolution of NGN. Therefore, this paper explains the possible methods of the service expansion with interconnecting P2PSIP overlay and IMS network. Specially this paper suggests the interconnection architectures of P2PSIP overlay and IMS network as subscriber's types and analyzes the traffic analysis model and session set up delay characteristics by simulation model. As a results, this paper shows that the interconnection architecture using gateway AS(Application Server) is the excellent method to interconnect the IMS in case of P2PSIP overlay only subscriber, and that interconnection architecture using I-CSCF is good method to interconnect the IMS in case of P2PSIP overlay and IMS subscriber.

Oscillating Boundary Layer Flow and Low Frequency Instability in Hybrid Rocket Combustion (하이브리드 로켓 연소에서의 경계층 진동 변화와 저주파수 연소불안정)

  • Kim, Jina;Lee, Changjin
    • Journal of the Korean Society for Aeronautical & Space Sciences
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    • v.47 no.10
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    • pp.720-727
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    • 2019
  • Resonating thermal lags of solid fuel with heat transfer oscillations generated by boundary layer oscillation is the primary mechanism of the occurrence of the LFI (Low Frequency Combustion Instability) in hybrid rocket combustion. This study was experimentally attempted to confirm that how the boundary layer was perturbed and led to the LFI. Special attention was also made on oxidizer swirl injection to investigate the contribution to combustion stabilization. Also the overall behavior of fluctuating boundary layer flow and the occurrence of the LFI was monitored as swirl intensity increased. Fluctuating boundary layer was successfully monitored by the captured image and POD (Proper Orthogonal Decomposition) analysis. In the results, oscillating boundary layer became stabilized as the swirl intensity increases. And the coupling strength between high frequency p', q' diminished and periodical amplification of RI (Rayleigh Index) with similar frequency band of thermal lag was also decreased. Thus, results confirmed that oscillating axial boundary layer triggered by periodic coupling of high frequency p', q' is the primary mechanism to excite thermal resonance with thermal lag characteristics of solid fuel.