• Title/Summary/Keyword: X-RD

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A case of mucolipidosis II presenting with prenatal skeletal dysplasia and severe secondary hyperparathyroidism at birth

  • Heo, Ju Sun;Choi, Ka Young;Sohn, Se Hyoung;Kim, Curie;Kim, Yoon Joo;Shin, Seung Han;Lee, Jae Myung;Lee, Juyoung;Sohn, Jin A;Lim, Byung Chan;Lee, Jin A;Choi, Chang Won;Kim, Ee-Kyung;Kim, Han-Suk;Kim, Beyong Il;Choi, Jung-Hwan
    • Clinical and Experimental Pediatrics
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    • v.55 no.11
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    • pp.438-444
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    • 2012
  • Mucolipidosis II (ML II) or inclusion cell disease (I-cell disease) is a rarely occurring autosomal recessive lysosomal enzyme-targeting disease. This disease is usually found to occur in individuals aged between 6 and 12 months, with a clinical phenotype resembling that of Hurler syndrome and radiological findings resembling those of dysostosis multiplex. However, we encountered a rare case of an infant with ML II who presented with prenatal skeletal dysplasia and typical clinical features of severe secondary hyperparathyroidism at birth. A female infant was born at $37^{+1}$ weeks of gestation with a birth weight of 1,690 g (<3rd percentile). Prenatal ultrasonographic findings revealed intrauterine growth retardation and skeletal dysplasia. At birth, the patient had characteristic features of ML II, and skeletal radiographs revealed dysostosis multiplex, similar to rickets. In addition, the patient had high levels of alkaline phosphatase and parathyroid hormone, consistent with severe secondary neonatal hyperparathyroidism. The activities of ${\beta}$-D-hexosaminidase and ${\alpha}$-N-acetylglucosaminidase were moderately decreased in the leukocytes but were 5- to 10-fold higher in the plasma. Examination of a placental biopsy specimen showed foamy vacuolar changes in trophoblasts and syncytiotrophoblasts. The diagnosis of ML II was confirmed via GNPTAB genetic testing, which revealed compound heterozygosity of c.3091C>T (p.Arg1031X) and c.3456_3459dupCAAC (p.Ile1154GlnfsX3), the latter being a novel mutation. The infant was treated with vitamin D supplements but expired because of asphyxia at the age of 2 months.

Association between HSP70 Genotypes and Oocytes Development on In vitro Maturation/Fertilization in Pig

  • Wee, M.S.;Park, C.K.;Cho, S.R.;Lee, S.S.;Yeon, S.H.;Kim, C.D.;Cho, C.Y.;Choi, S.H.;Sang, B.D.;Son, D.S.;Li, Z.D.;Jin, H.J.
    • Asian-Australasian Journal of Animal Sciences
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    • v.21 no.10
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    • pp.1404-1410
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    • 2008
  • This study was performed to clarify whether the variation of stress related heat shock protein 70 (HSP70) (GenBank X68213) gene was associated with the nuclear morphological change of in vitro maturation and in vitro capacitation in oocytes of pig ovaries obtained at the slaughterhouse. The nucleic acid substitution of C to G at the 483rd position was found out in HSP70 K1 (290-512) from X68213. The ovaries were categorized into CC, CG, and GG genotypes using polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) (BsiHKA I). After the second in vitro maturation of immature fresh oocytes, the relation of nuclear morphological change in oocytes with the genotype of HSP70 K1 gene was such that the MII ratios of the genotype GG and CG (46.93% and 42.20%, respectively) were significantly higher than that of the CC genotype (10.71%) (p<0.05). With respect to in vitro maturation of frozen-thawed oocytes by an open pulled straw (OPS) method, the percentage of oocytes matured to MII stage of the CG genotype showed a higher trend than CC and GG genotypes. After the in vitro maturation of immature fresh oocytes and frozen-thawed oocytes by the OPS method, the relation of the pronuclei change in oocytes matured in vitro with HSP70 genotype was assessed, and the result showed that the enlarged sperm heads (ESH) of matured fresh oocytes and frozen-thawed oocytes were 80.0% and 60.0% in the CC genotype, respectively. The CC genotype group had a significantly higher rate of ESH than the CG and the GG genotype group (p<0.05). The ratios of polyspermic invasion were not different among HSP70 of the three genotypes. It was considered that the rate of in vitro maturation of fertilized oocytes was expected to differ according to genotype of the stress related gene.

A Study on Perceptions by College Students of Radiology about the Knowledge, Attitudes and Behaviors of Radiation Exposure Management (방사선과 대학생이 방사선피폭관리에 대한 지식, 태도, 행위에 관한 연구)

  • Yeo, Jindong;Ko, Inho;Kim, Hye-Sook
    • Journal of the Korean Society of Radiology
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    • v.9 no.2
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    • pp.79-99
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    • 2015
  • Participants of this study were students of radiology who were attending colleges or universities located in Daegu and Gyeongbuk. This researcher conducted a questionnaire survey of those students from Feb. 3rd to 21st, 2014. The findings of the study can be summarized as follows. 1. Concerning the knowledge of radiation exposure management, the respondents' scores were highest in two items, or 'Materials based on lead or concrete may shield X-rays' and 'The sexual gland is very sensitive to radiation' and lowest in the item which says' 'Occupational radiation exposure dose should not exceed 20mSv a year in average on a 5-year period basis'. 2. The participants' scores for the attitudes of radiation exposure management were higher in two items, or 'Health examination should be made regularly in relation to radiation exposure' and 'Those who work within the area of irradiation should wear protective clothes' and lowest in the item which says 'Radiation exposure dose should be regularly measured for the calibration of the radiation system'. 3. For the behaviors of radiation exposure management, the surveyed students showed highest scores in two items, or 'When irradiating the patient, the radiator should be behind the protective barrier(plate)' and 'It is needed to receive the education of radiation exposure management regularly' While, their score for a behavior described in the item saying 'Before using the radiation system, it is needed to check whether the machine works normally.

Calculates of GPS Satellite Coordinates Using Rapid and Ultra-Rapid Precise Ephemerides (신속정밀제도력과 초신속정밀궤도력을 이용한 GPS 위성좌표 계산)

  • Park Joung Hyun;Lee Young Wook;Lee Eun Soo
    • Journal of the Korean Society of Surveying, Geodesy, Photogrammetry and Cartography
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    • v.22 no.4
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    • pp.383-390
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    • 2004
  • IGS provides so accute a final precise ephmerides which is offered in the 13rd, and it also offers a rapid precise ephmerides for more prompt application and an ultra-rapid precise ephmerides for real-time application. The purpose of this study is to analyze the accuracy of a rapid precise ephemerides and an ultra-rapid precise ephemerides based on a final precise ephmerides and determine the degree of the Lagrange Interpolation which needs to decide the location of a satellite. As the result of this study, the root mean square error of x,y,z coordinates of a rapid precise ephemerides was $\pm$0.0l6m or so, and the root mean square error of an observed ultra-rapid precise ephemerides was approximately $\pm$0.024m. The root mean square error of an ultra-rapid precise ephemerides predicted for 24 hours was $\pm$0.07m or so and the one of an ultra-rapid precise ephemerides predicted for 6 hours was $\pm$0.04m or so. Therefore, I could figure out that it had higher accuracy than a broadcast ephemerides. Also, in case that the location of a satellite was calculated with the method of the Lagrange Interpolation, it was confirmed that using the 9th order polynomial was efficient.

흉총창에 의한 심방파열 치험 2례

  • Lee, Doo-Yun;Kwack, Sang-Ryong
    • Journal of Chest Surgery
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    • v.13 no.1
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    • pp.60-65
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    • 1980
  • We have experienced 2 cases of the hunshot wound sof the chest involving cardiac injuries at department of the thoracic surgery, Capital Armed Forces General Hospital during I year from April I 1979 to Jan. 1980. In one case of two patients , he was a 22 years old man who was transported to this emergency room 4 hour 10 minutes after having gunshot wound of the left chest by helicopter. Physical examination showed small inlet in left 3rd ICS and left parasternal border, large outlet in left 8th ICS and left scapular line, no breath sound on left side and distant heart sound. chest roentgenography demonstrated marked pleural effusion in left side and mediastinum shifted to right. As soon as chest X-ray was taken, the bleeding through penetrating wound became profuse and cardiac arrest ensued. Closed chest cardiac massage was started and vigorous transfusion continued, but no effective cardiac activity could not be obtained. The patient was pronounced dead due to exsanguinating hemorrhage from wuwpected cardiac wounds. In this critically injured patient with evidence of intrathoracic hemorrhage and suspected cardiac penetration, only emergency thoracic exploration and immediate surgical control of bleeding points might offer the maximum possibility of survival. The other case was a 23 years old man who was transferred to the emergency room 4 hours 50 minutes after having kmultiple communicated fractures of sternum and linear fracture of right mandible by a missile. Examination revealed about 30% skin loss of the anterior chest wall, weak pulse of 96 beats/min., distant heart sound and decreased breath sounds bilaterally. finding on the chest X-ray films showed multiple sternal fractures, marked pericardial effusion indicating hemopericardium. So, the patient was moved immediately to the operation room where, after endotracheal tube inserted, a median sternotomy was performced. A hemorrhagic congestion of the right upper lobe and marked bulging pericardium were disclosed. The pericardium was opened anterior to right phrenic nerve and exsanguinating hemorrhage ensued from the 0.5cm lacerated wound in the auricle of right atrium. The rupture site of right atrium was occluded with non-crushing vascular clamps and then was over sewn with interrupted sutures. It was thought to be highly possible that he was alive long enough to have cardiorrhaphy because of cardiac tamponade, which prevented exsanguinating hemorrhage. He was taken closed reduction for linear fracture of right mandible 2 weeks after repair of ruptured right auricle in dental clinic. This patient's post-operative course was not eventful.

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Analysis on Spectral Regrowth of Bandwidth Expansion Module by Quadrature Modulation Error in Digital Chirp Generator (디지털 첩 발생기에서의 직교 변조 오차에 의한 대역 확장 모듈에서의 스펙트럴 재성장 분석)

  • Kim, Se-Young;Sung, Jin-Bong;Lee, Jong-Hwan;Yi, Dong-Woo
    • The Journal of Korean Institute of Electromagnetic Engineering and Science
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    • v.21 no.7
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    • pp.761-768
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    • 2010
  • This paper presents an effective method to achieve the wideband waveform for high resolution SAR(Synthetic Aperture Radar) using the frequency multiplication technique. And also this paper analyzes the root causes for the spectral regrowth due to 3rd-order intermodulation in chirp bandwidth expansion scheme using quadrature modulator and frequency multipliers. The amplitude and phase imbalance requirement are defined based on the simulation results in terms of quadrature channel imbalance. This minimizes the degradation of range resolution, peak sidelobe ratio and integrated sidelobe ratio. The wideband chirp generator using the frequency multiplier and memory map scheme was manufactured and the compensation technique was presented to reduce the spectral regrowth of SAR waveform by minimizing the amplitude and phase imbalance. After I and Q channel imbalance adjustment, the carrier level reduces -28.7 dBm to -53.4 dBm. Chirp signal with 150 MHz bandwidth at S-band expands to 600 MHz bandwidth at X-band. The sidelobe levels are reduced by about 8 to 9 dB by compensating the amplitude balance between I and Q channels.

Metallic Mineralogical Characteristics of Forged Iron Axe from the Wood-framed Tomb at the Hwangseongdong, Gyeongju, Korea (경주 황성동 목곽묘 출토 단조 철부의 금속광물학적 특성)

  • Kim, Jeong-Hun;Yi, Ki-Wook;Lee, Chan-Hee
    • Journal of the Mineralogical Society of Korea
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    • v.20 no.4
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    • pp.231-245
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    • 2007
  • The forged iron axe of the middle 3rd Century found in the No. 2 wood-framed tomb from the Hwangseongdong site, Gyeongju is rectangular on the plane level. The iron axe shines in met-allic luster, which is light grey with pale creamy tint. The result of X-ray diffraction analysis shows that the axe consists of magnetite and geothite, which can explain why the composition and texture of the original ore has been kept intact. There are fine-grained quartz, calcite, mica, magnetite, amphibole, unknown tungsten minerals, pyroxene and olivine inside the axe. Those must be the impurities that they failed to remove in the thermal treatment process. Generally, the iron axe consists mainly of pearlite texture coexisting ferrite and cementite, and show high carbon contents with homogeneous distribution. It can be interpreted the axe was carburized after the material was made to resemble pure iron. The decarbonization work didn't go well along the process marks. Crude ores of the iron axe are possible utilized by magnetite from the Ulsan mine on the basis of the occurrences and inclusions. It's estimated that the original ore was bloom produced in low-temperature reduction and formed around in $727^{\circ}C$, which is eutetic temperature.

Experimental Evaluation of Hydrate Formation and Mechanical Properties of Limestone Calcined Clay Cement (LC3) According to Calcination Temperature of Low-Quality Kaolin Clay in Korea (국산 저품질 고령토의 소성온도에 따른 석회석 소성점토 시멘트(LC3)의 수화물 생성 및 기계적 특성 평가)

  • Moon, Jae-Geun;Her, Sung-Wun;Cho, Seong-Min;Bae, Sung-Chul
    • Journal of the Korean Recycled Construction Resources Institute
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    • v.10 no.3
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    • pp.252-260
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    • 2022
  • In Korea, low-quality kaolin has significantly greater reserves and superior economic efficiency than high-purity kaolin. However, the utilization is low because it does not match the demand conditions of the market, and it is difficult to find a suitable source of demand. The purpose of this study is to derive the possibility and optimal calcination temperature of domestic low-quality kaolin that can be used as a raw material for limestone plastic clay cement (LC3). Isothermal calorimetry, X-ray diffraction analysis, Thermogravimetric Analysis, and compressive strength tests were conducted to evaluate hydrate generation and mechanical properties of LC3 paste according to calcination temperatures (600 ℃, 700 ℃, 800 ℃, 900 ℃). As a result, although 50 % of the clinker was replaced, the domestic low-quality kaolin clay produced calboaluminate hydrate and C(A)SH from the 3rd day of hydration, showing almost equal or higher strength to OPC, and there was a big difference in strength depending on the firing temperature.

Susceptibility of Medically Important Mosquito Larvae and Larvivorous Fishes to Abate and Abate-S in Korea (국내 주요 모기유충과 천적어류의 Abate 와 Abate-S 에 대한 감수성)

  • 이동규;유효석
    • Korean journal of applied entomology
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    • v.38 no.2
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    • pp.165-169
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    • 1999
  • A study was performed on the susceptibility of Abate@ and Abate-S@ against 3rd instar larvae of 5 species of mosquitoes including Anopheles sinensis, Aedes dorsalis, Culex inatomii, Cx. pipiens pallens, Cx. tritaeniorhynchus and mosquito larvivorous fishes such as Chinese muddy loaches (Misgurnus muzolepis) and crucian carps (Carassius carassi~4s) in September, 1998. Cx. pipiens larvae showed that the LC5os of both Abatea and Abate-Sa were 0.006 ppm. The LC95 values of this mosquito species appeared 0.070 pprn of Abate@ and 0.035 pprn of Abate-S@ which were more susceptible than the dosage (1.0 ppm) recommended from WHO. An. sinensis larvae appeared 0.009 pprn of LC50 and 0.025 pprn of LCgs. The larvae of Cx. inatomii and Ae. dorsalis showed the most highly susceptible to Abate-S@ among the 5 mosquito species with LC50 values of 1.9 x pprn and 1.1 x pprn respectively, and the LC95 values were 5.2 x 10-hppm and 1.4 x ppm, respectively. On the other hand, Cx. tritaeniorhynchus appeared least affected by Abate-Sa. and was brought 0.048 pprn of LC50 and 0.808 pprn of LCg5. These two insecticides were much less toxic to the fishes than mosquito larvae showing the LC, value of Chinese muddy loaches at 24.145 pprn of Abate and 10.750ppm of Abate-S@. This fish species showed that the LCsos of Abate@ and Abate-S@ were 27.567 pprn and 14.775 ppm, respectively. In case of crucian carps, their LC, values were 7.914 pprn of Abate@ and 6.480 pprn of Abate-S@. Therefore, the maximum safe dosages of the insecticides to the fishes were 8 times as high as the values of LCg5 of the mosquito larvae. This study suggests that Abate@ and Abate-S@ demonstrated highly potential insecticides for further larger scale operational integrated control in some proper aquatic places if other aquatic invertebrates are also safe to the insecticides.

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Clinical Features in Primary Mediastinal Tuberculous Lymphadenitis (조직학적으로 증명된 결핵성 림프절염에 대한 임상적 고찰)

  • Ahn, Chul-Min;Yoo, Kwang-Ha;Park, Kwang-Ju;Kim, Hyung-Joong;Kim, Sung-Kyu;Lee, Won-Young
    • Tuberculosis and Respiratory Diseases
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    • v.46 no.6
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    • pp.767-774
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    • 1999
  • Background : Tuberculous mediastinal lymphadenitis (TML) is a relatively commonly encountered in Korea. However, there were no datas available on TML without other combined tuberculous infections in Korea. We retrospectively analyzed clinical manifestations, radiologic findings, Chest CT scan findings, the duration of treatment, and follow up Chest CT scan findings of 23 cases who had only TML. Method : 23 cases from 1991 to 1997 with TML confirmed by biopsy and had no other combined tuberculous infections were studied retrospectively. Results : Of the 23 cases, 7 cases were male and 16 female. The male to female ratio was 1 : 2.4. Mean age was 31 years and the most prevalent age group was the 3rd decade(43%). The most common presenting symptoms were fever(39%) followed by no symptom, cough, swallowing difficulty, and chest discomfort. On simple chest X-ray, mediastinal enlargement were noted in 20 cases(90%). The most frequently involved site was the paratracheal node in 11 cases with the right to left side involvement ratio being 4.6 : 1. On chest CT scan, the most commonly enlarged node was the paratracheal node(33%) followed by the subcarinal(20%), hilar(13%), tracheobronchial (8%), subaortic(8%), supraclavicular(8%) and anterior. mediastinal nodes. 6 cases were dropt out due to incomplete follow up. Thirteen cases were treated with HERZ regimen and the mean duration of treatment was 14 months. Three cases were treated with second line drug regimens(Tarivid, Pyrazinamide, Streptomycin plus Ethambutol or Para-aminosalicylic acid) for 18 months. In HERZ groups, one case was recurred after 10 months later and retreatment was done by same HERZ regimen during 12 months. Follow up chest CT scan after completion of treatment were done in 13 cases and that revealed more than a 50% decrease in size in 77% of the cases and no interval change in 23% of the cases. Conclusion : In cases of TML without other combined tuberculous infection, the minimal duration of treatment was required 12 months by HERZ regimen and 18 months by a 2nd line regimen or more. Further studies will be needed to confirm the treatment duration for TML without other combined tuberculous infections.

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