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A Case of Mucopolysaccharidosis Type 2 Diagnosed Early through Brain MRI (뇌자기공명영상 검사를 통해 조기 발견된 제2형 뮤코다당증 1례)

  • Lee, Yoon kyoung;Cho, Sung Yoon;Kim, Jinsup;Huh, Rimm;Jin, Dong-Kyu
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.15 no.2
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    • pp.87-92
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    • 2015
  • Mucopolysaccharidosis (MPS) is an inherited disease entity associated with lysosomal enzyme deficiencies. MPS type 2, also known as Hunter syndrome, has a characteristic morphology primarily involving x-l inked recessive defects and iduronate-2-sulfatase gene mutation. The purpose of this case report is to provide important clues to help pediatricians identify Hunter syndrome patients earlier (i.e., before the disease progresses). A 30-month-old boy showed developmental delay and decreased speech ability. Physical examinations revealed a flat nose and extensive Mongolian spots. Brain magnetic resonance images (MRIs) showed bilateral multiple patchy T2 hyperintense lesions in the periventricular and deep white matter, several cyst-like lesions in the body of the corpus callosum, and diffuse brain atrophy, which were in keeping with the diagnosis. Based on these findings, the patient was suspected of having MPS. In the laboratory findings, although the genetic analysis of IDS (Iduronate-2-sulfatase) did not show any pathogenic variant, the enzymatic activity of IDS was not detected. We could confirm the diagnosis of MPS, because other sulfatases, such as ${\alpha}$-L-iduronidase, were detected in the normal range. Early enzymatic replacement therapy is essential and has a relatively good prognosis. Therefore, early diagnosis should be made before organ damage becomes irreversible, and brain MRIs can provide additional diagnostic clues to help distinguish the disorder.

Influence and Application of an External Variable Magnetic Field on the Aqueous HCl Solution Behavior: Experimental Study and Modelling Using the Taguchi Method (염산 수용액 거동에 대한 가변 외부 자기장의 적용과 영향: 실험 연구 및 Taguchi 법을 이용한 모델링)

  • Hashemizadeh, Abbas;Ameri, Mohammad Javad;Aminshahidy, Babak;Gholizadeh, Mostafa
    • Applied Chemistry for Engineering
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    • v.29 no.2
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    • pp.215-224
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    • 2018
  • Influences of the magnetic field on 5, 10 and 15 wt% (1.5, 3 and 4.5 M) HCl solution behaviour, which has widespread applications in petroleum well acidizing, were investigated in various conditions. Differences in the pH of magnetized hydrochloric acid compared to that of normal hydrochloric acid were measured. Taguchi design of experimental (DoE) method were used to model effects of the magnetic field intensity, concentration, velocity and temperature of acid in addition to the elapsed time. The experimental results showed that the magnetic field decreases [$H^+$] concentration of hydrochloric acid up to 42% after magnetization. Increasing the magnetic field intensity (with 28% contribution), concentration (with 42% contribution), and velocity of acid increases the effect of magnetic treatment. The results also demonstrated that the acid magnetization was-not influenced by the fluid velocity and heating. It was also displayed that the acid preserves its magnetic memory during time. The optimum combination of factors with respect to the highest change of [$H^+$] concentration was obtained as an acid concentration of 10% and an applied magnetic field of 4,300 Gauss. Due to the reduction of HCl reaction rate under the magnetization process, it can be proposed that the magnetized HCl is a cost effective and reliable alternative retarder in the matrix acidizing of hydrocarbon (crude oil and natural gas) wells.

Microarray Analysis of Gene Expression Profile by Treatment of Schizandrae fructus Extract in Inflammation-induced Human Epithelial A549 Cells (염증이 유발된 인간기관지상피세포에서 오미자가 Microarray를 이용한 유전자 발현 분석에 미치는 영향)

  • Jung, Jin-Yong;Jung, Sung-Ki;Jung, Hee-Jae;Rhee, Hyung-Koo
    • The Journal of Internal Korean Medicine
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    • v.29 no.3
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    • pp.543-553
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    • 2008
  • Objective: The goal of this study was to determine the anti-asthma mechanism of SF on TNF-${\alpha}$ induced activation on A549 (human type II-like epithelial) cells. Using oligonucleotide microarray, we sought to establish the molecular mechanism of the protective effects of SF on A549 cells. Material & Methods : Cells were cultured in three different conditions: 1) negative control group was cultured in normal condition of DMEM, 2) positive control group was activated with TNF-${\alpha}$, IL-4. and IL-1${\beta}$, and 3) SF treated group was previously treated with 0.1${\mu}g/ml$ SF after TNF-${\alpha}$, IL-4. and IL-1 activation. Cells of positive control and SF treated groups were cultured for 30 min, 1hr, 3hr and 6hr. Results : The comparative analysis of the gene expression profile revealed that proinflammatory cytokines such as IL1F8, IL1F9, IL1R1. IL1RN, IL1RAPL1, IL8, TNFRSF4, TNFSF10c, TNFSF13, TRAF5, and TRAF7 and inflammation-related genes including MMP2, MMP11, MMP14, MMP15, MMP16, MMP19, MMP25, and MMP27 were down regulated with SF treatment. Cell adhesion molecule genes such as ITGB1, ITGBL1, selectin P ligand, selectin E, ICAM2, ICAM3, VCAM1, PECAM, FCER1G and MMP28 genes were also down-regulated in SF treated A549 cells. Conclusion : These results suggest that the anti-asthmatic effects of SF could be mediated by regulating specific genes related with cell adhesion, proinflammatory cytokine and inflammation-related genes in A549 cells.

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Measuring Myun Health Worker's Performance by Time-Activity Approach (시간활동 접근법에 의한 면 보건요원의 업무량 분석)

  • Kim, Han-Joong;Kim, Moon-Shik
    • Journal of Preventive Medicine and Public Health
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    • v.10 no.1
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    • pp.34-43
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    • 1977
  • This study attempts to examine the performances of Myun Health Workers-the frontline workers in the Korean rural health care delivery system. The time-activity approach was mainly utilized as a measuring tool. This study was undertaken in September 1976 with 35 Myun Health Workers at the Kang Wha County. The pretested time-activity approach sheets were filled out daily for one month by those Myun Health Workers themselves. Statistical means and variances of analysis were utilized for statistical method in comparing some activities and functions converged into time distribution Findings: 1. The workers's average working hours derived in this study is 8 hours and 48 minutes per day, which takes half an hour longer than normal schedule. 2. They spend 56% working hour for direct services, in other words, the main function, 22% for supportive function, and 22% for other activities, the unrelated health services. 3. Considering the total working hours of main function, out-center activity is far more than in-center services with the ratio of 70% to 30% respectively, which proves, therefore, that the main activity of the workers is home visiting. 4. It takes 20 minutes purely for home visiting and takes 14 minutes for transportation. 5. This research also indicates that such factors as characteristics of the health workers and myun influence in shaping the structures of the worker's function and activity: a. The workers whose working site is located in myun office spend 15% among total working hours in carring out official myun activities, which is incidentally unrelated to health services, while the health subcenter have no rooms for administrative jobs for myun office. b. The workers whose office is in health subcenter contribute much time in doing main function and those working in special project distribute more time in performing supportive function. c. The types of workers are another dominant factor to influence the components of worker's functions and activities. MCH workers and MPW I spend much time for manipulating main function. d. MPW II, whose function is reorganized by special project in 2 myuns shows different pattern of time distribution compared to the TB worker orFP worker in the ordinary area. MPW II distributes their time evenly in performing MCH program, T.B. Program, F.P. program and education activity, while the unipurpose workers engage in carring out only their dominant role. e. Another variables which involve the variation of the worker's activity can be illustrated with the variables like target population, size of myun and convenience for transportation, among which the latter two are remarkable factors in determining the time for out-center service.

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Studies on the Production of Twins in Cattle (소의 쌍자 생산술 개발에 관한 연구)

  • Hwang Woo-Suk
    • Journal of Veterinary Clinics
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    • v.7 no.2
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    • pp.511-515
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    • 1990
  • Induction of twinning was attempted by transfer of two whole- or demi-embryos in Holstein cows and heifers. Cows were superovulated with follicle stimulating hormone(FSH) administered twice daily in intra-muscular injection. On day of 6.5 to 7 post-estrus, embryos were collected nonsurgically, Normal morulae and early balstocysts were obtained from superovulated cows. The embryos were hisected with a micro-blade made from a razor. Twenty seven pairs of half embryos were successfully produced and 23 pairs of half embryos were transferred to recipients( Group A). Twenty cows were treated with low unit of FSH (3mg${\times}$2,2mg${\times}$2 ; Group B1, 2mg${\times}$2, 1mg${\times}$2, 1mg${\times}$2 ; Group 32) to induce double or triple ovulations. The cows of Group B1 and B2 were Inseminated artificially(A1) at following estrus. Twenty four heifers were bred by A1 and received an additional embryo into the uterine horn contralateral to the corpus luteum(CL) 6 days later(Group C). One embryo was transferred into each uterine hem of 16 heifers 6 days after estrus(Group D). Fourteen heifers were received two embryos into the uterine horn ipsilateral to the CL at day 6 of estrous cycle(Group I). Pregnancy rates at 60 days in Group A, B1, B2, C, D and E were 34.8%, 70.0%, 60.0%, 66.7%, 62.5% and 57.1%, respectively. Twinning rates were 8.7%, 20.0%, 10.0%, 16.7%, 18.7% and 21.4%, respectively. The present experiments demonstrate that FSH treatment( Group 31) and ipsilateral transfer( Group I ) of two whole-embryos are more useful methods to produce twins than the others.

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A Case of Superior Vena Cava Syndrome Caused by Klebsiella Pneumonia (폐렴간균 폐렴에 의해 유발된 상대정맥 증후군 1예)

  • Kim, Ju-Young;Lim, Chae-Man;Kim, Seon-Hee;Chu, Yun-Ho;Koh, Youn-Suck;Kim, Woo-Sung;Kim, Won-Dong
    • Tuberculosis and Respiratory Diseases
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    • v.41 no.1
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    • pp.58-62
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    • 1994
  • Superior vena cava(SVC) syndrome is mostly related to a malignant process, but many different benign causes have also been described. We report a case of SVC syndrome caused by Klebsiella pneumonia diagnosed by sputum culture and serial chest X-ray changes. A 27-year-old man had been in stable health until three days before admission, when he complained of pleuritic chest pain, facial flushing, and shortness of breath. Examination of the head and neck disclosed edema of face and both arms, and jugular venous distention to the angle of the jaw. The chest auscultation revealed decreased breath sound without crack1e on right upper lung field. The chest roentgenogram showed homogenous air space consolidation on right upper lobe, asociated with downward displacement of minor fissure and contralateral displacement of trachea, but air bronchogram was not seen. We began antibiotic therapy under impression of pneumonia after available culture was taken from blood and sputum. SVC scintigraphy showed stasis of drain of right brachiocephalic vein at the proximal portion with reflux into the right internal jugular vein and faintly visible SVC via the collaterals. Sputum culture revealed Klebsiella pneumoniae. Antibiotic therapy resulted in a cure of infection and disappearance of facial swelling. Follow-up SVC scintigraphy after 20 days showed normal finding. We first report a case of SVC syndrome caused by klebsiella pneunonia.

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Time-series Mapping and Uncertainty Modeling of Environmental Variables: A Case Study of PM10 Concentration Mapping (시계열 환경변수 분포도 작성 및 불확실성 모델링: 미세먼지(PM10) 농도 분포도 작성 사례연구)

  • Park, No-Wook
    • Journal of the Korean earth science society
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    • v.32 no.3
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    • pp.249-264
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    • 2011
  • A multi-Gaussian kriging approach extended to space-time domain is presented for uncertainty modeling as well as time-series mapping of environmental variables. Within a multi-Gaussian framework, normal score transformed environmental variables are first decomposed into deterministic trend and stochastic residual components. After local temporal trend models are constructed, the parameters of the models are estimated and interpolated in space. Space-time correlation structures of stationary residual components are quantified using a product-sum space-time variogram model. The ccdf is modeled at all grid locations using this space-time variogram model and space-time kriging. Finally, e-type estimates and conditional variances are computed from the ccdf models for spatial mapping and uncertainty analysis, respectively. The proposed approach is illustrated through a case of time-series Particulate Matter 10 ($PM_{10}$) concentration mapping in Incheon Metropolitan city using monthly $PM_{10}$ concentrations at 13 stations for 3 years. It is shown that the proposed approach would generate reliable time-series $PM_{10}$ concentration maps with less mean bias and better prediction capability, compared to conventional spatial-only ordinary kriging. It is also demonstrated that the conditional variances and the probability exceeding a certain thresholding value would be useful information sources for interpretation.

Implementation about measurement of the head SAR and variable parameter according to operation control mode in brain MR study with 1.5Tesia (1.57 BRAIN MRI검사에서의 작동제어모드를 통한 두부 SAR측정과 변화인자에 관한 고찰)

  • Lee, Kyu-Su;Sim, Hyun;Moon, Ji-Hoon;Oh, Jae-Cheol
    • Proceedings of the KIEE Conference
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    • 2007.04a
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    • pp.58-60
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    • 2007
  • Magnetic Resonance Imaging(MRI) has become a very widely used medical procedur e. Clo.sed and open systems are typically used with static magnetic fields at or below 2 Tesla. BWhole body SAR(specific absorbsion rate) is the value of SAR averaged over the entire body of the patient over any period of 15 minutes. Head SAR is the value of SAR averaged over the head of the patient for any period of 10 minutes. SAR is a measure of the absorption of electromagnetic energy in the body' (typically in watts per kilogram (W/kg)). The normal operating mode comprises values of head SAR not higher than 3 W/kg. The second level controlled operating mode comprises values higher than 3 W/kg. Current FDA guidance limits the SAR in the whole body. including the head to a range of 1.5 to 4.0 W/kg, depending on the patient's clinical condition. SAR, limit restrictions are incorporated in all MRI systems. and domestic' s guidance limits the SAR in a part body. including the head to 3.2w/kg and less. The purpose of this study is to evaluate on change of head SAR in using MRI pulse sequence and to check if exceed 3.2(w/kg) level in domestic a part exposure through measured head SAR. 23 patient's the average head SAR of pulse sequence is that T2WI sagittal is 0.5375. T2WI axial(FSE) is 0.4817, T1WI axial(SE) is, 0.8179. FLAIR axial is 0.4580. GRE axial is 0.0077, Diffusion is 0.0824w/kg. The head SAR exposed per patient was proved 2.3845w/kg less than the international standard. Coefficient of correlation for the relations body weight and SAR or for the relations ETL(echo train length) and SAR is 1 value. Coefficient of correlation for the relations between TR(time to repeat) and SAR is -0.602 value. so SAR increased relative to weight body and ETL. But the relations between TR and SAR is negative definite.

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AN EXPERIMENTAL STUDY ON THE TISSUE RESPONSE OF THE TEMPOROMANDIBULAR JOINT IN UNILATERAL MANDIBULAR EDENTULISM (하악편측치아의 결손에 따른 악관절의 조직반응에 대한 연구)

  • Paik, Hyee-Seon;Kim, Yung-Soo
    • The Journal of Korean Academy of Prosthodontics
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    • v.29 no.2
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    • pp.285-294
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    • 1991
  • The human temporomandibular joint as a ginglymoarthrodial one has much in common with the other synovial joints of the body, but it does possess an unique charachteristic in that it must accomodate the various occlusal relations of dentition during an end point of closure. For that reason, the movement of the condyle in the temporomandibular joint is susceptible to influences from the nature of occlusion. Undue loading to the temporomandibular joint can be applied on the occasion of premature contacts in centric relation, balancing side interferences, change of occlusal surfaces due to excessive attrition, loss of tooth. Such occlusal disharmonies in association with the systemic and emotional factors may give rise to the temporomandibular disorder. On the other hand, the changes of occlusal patterns in the growing body can also have an effect on the growth of the temporomandibular joint through the alterations of functional stresses. The purpose of this study was to observe histopathologic response of the temporomandibular joint in unilateral chewing on one side exclusively for 10 months. Three dogs showing normal masticatory function were chosen. One dog aged about 12 months was for control, two dogs for experimental specimens were about 12 and 18 months old respectively. For chewing on the left side only, unilateral lower right premolar and molar were extracted in two experimental specimens. And then three dogs were sacrificed 10 months later. Frontal histologic sectioning of joints were done for the observation of the effects of one-side chewing. 24 specimens from three dogs were obtained and fixed in 10% formalin and routinly processed with H-E staining for histologic examination. The light microscopic findings were interpretated as follows: 1. Experimental specimen 1 aged about 22 months: In comparison with control and right non-chewing side, the proliferative and hypertrophic zone were increased at the mesial and lateral part of left chewing side condyle. There was no change of the articular tissue of temporal bone. 2. Experimental specimen 2 aged about 28 months: The articular tissues of adult joint were observed. The differences between the chewing and non-chewing side were not seen in the articular tissues of condyle and temporal bone.

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Effects of Debris Barrier on Community Structure and Functional Feeding Groups of the Benthic Macroinvertebrate (사방공작물의 시공이 저서성대형무척추동물의 군집구조 및 섭식기능군에 미치는 영향)

  • Seo, Jun-Pyo;Lee, Heon-Ho
    • Journal of Korean Society of Forest Science
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    • v.101 no.3
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    • pp.480-487
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    • 2012
  • This study was conducted to search the effects of debris barrier on the benthic macroinvertebrate. Gimcheon was selected as the survey site as it has relatively stable ecosystem with constantly running water. The survey was conducted 6 times before and after the construction of debris barrier from February in 2009 to October in 2010. In the first survey before construction, the identified species were 36 species belonged to 22 families, 9 order, 4 class, and 4 phylum. The figure slightly decreased to 30 species belonged to 18 families, 7 order, 2 class, and 2 phylum in the sixth survey after construction. Before construction, occupation ratio of EPT taxa was showed in the following order: Ephemeroptera (50.0%, 85.0%), Trichoptera (35.3%, 10.0%), and Plecopteran (14.8%, 5.0%). After construction, it was showed in the following order: Trichoptera (50.3%, 68.0%), Ephemeroptera (42.1%, 29.4%), and Plecopteran (7.5%, 2.7%). Ephemeroptera was the highest before construction. Trichoptera increased rapidly after construction. The Diversity, Richness, Evenness, and Dominance indices were all turned low in the second survey right after the construction. However, each index tended to increase with the course of time. In Functional Feeding Groups, GC type was the highest of 60.7% before construction. After construction, SC(53.1%) and FC(35.4%) increased rapidly and they became stabilized since the third survey. The result of this study reveals that debris barrier greatly affects the Aquatic Ecosystem right after its construction, but the system becomes stable and returns to normal with the course of time (about 18 months). Therefore, the study considering various influence factors such as time is required to recover completely through further long-term monitoring.