• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.3
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• pp.92-95
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• 2017

Fabry disease (FD) is an X-linked lysosomal storage disorder caused by an ${\alpha}$-galactosidase A (GLA, MIM 300644) enzyme deficiency due to pathogenic variants in the ${\alpha}$-galactosidase A gene (GLA). The disease leads to accumulation of globotriaosylceramide (Gb3) and related glycophospholipids affecting nearly all major organ systems, with the primary sites damaged by Gb3 including renal glomeruli, myocardium, neurons of the dorsal ganglion and autonomic nervous system, and vascular endothelial and smooth muscle. Progressive deposition in these organ systems present with various clinical manifestations including acroparesthesia, renal failure and heart failure. Here, we report a Chinese male diagnosed with Fabry disease in his late $4^{th}$ decades showing improvement of acroparesthesia during enzyme replacement therapy (ERT). A 48-year-old Chinese man who presented with chronic recurrent severe burning pain in his fingers and toes since the age of 10, with worse involvement of the former visited to our clinic for further evaluation. His medical history included a transient ischemic attack aged 40 and diagnosed with stage 4-5 chronic kidney disease aged 47. In the family history, the patient's brother was found to be have Fabry disease 1 month before his visit. Except for his brother, all other members of the family are healthy. Based on his medical history and family history, he was strongly suspicious for Fabry disease. He was found to have a galactose-alpha-1,3-galactose level 4.96 (Reference range, 42.5-67.9) suggestive of Fabry disease. The followed sequencing of GLA coding region in our patient revealed hemizyosity for the mutation c.988C>T (Q330X) in Exon 7. Since ERT start, he showed significant improvement in his symptoms of burning sensation of fingers and toes. On the contrary, due to deteriorating kidney function even with ERT, he is considered for kidney transplantation. Despite of diagnostic delay until late 4th decades, ERT showed a potential improvement of acroparesthesia in our patient. However, late start of ERT can lead to poor outcome in multiorgan function. Therefore, early diagnosis with high index of suspicion followed by continuous ERT with regular monitoring have an impact on quality of life in Fabry disease.

• The Korean Journal of Nuclear Medicine Technology
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• v.16 no.2
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• pp.149-155
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• 2012

Purpose : Serum insulin levels are useful indicator which of reflecting the function of insulin secretion in pancreatic ${\beta}$ cell and diagnosis of diabetes, differentiating the cause of impaired glucose tolerance. Insulin measurement kits have shown some differences in many ways such as test methods as well as quality control. The purpose of this study was to evaluate the diagnostic performance of seven manufacturing companies commercial kits. Materials and Methods : The values of insulin measured by three manufacturing companies (Biosource, Siemens, TFB) with 59 samples in August 2009 were compared with those measured by four manufacturing companies (Immunotech, Izotope, BNIBT, Cisbio) with 68 samples in December 2011. We evaluated precision, recovery rate, dilution test and correlation of serum insulin measurement using seven manufacturing company kits. Statistical program SPSS 12.0 was used for the verification of results. Results : The coefficients variation of the precision on all seven different kits were showed within 5.0%. Recovery rate of Biosource, Siemens, TFB kits on three different levels showed 94.2~103.7%, 99.0~104.6%, 99.7~107.6% respectively. Immunotech, Izotope, BNIBT, Cisbio were 93.5~99.1%, 91.4~99.1%, 99.2~131.0%, 84.8~102.3% respectively. There was strong correlation between the measurement of insulin by Biosource kit and that by two commercial kits, Siemens (R2=0.96), TFB (R2=0.99). There was good correlation between the measurement of insulin by TFB kit and that by three commercial kits, Immunotech (R2=0.97), Izotope (R2=0.96), Cisbio (R2=0.97). In the dilution test performed with more than 200 ${\mu}IU/ml$ high concentration samples, samples with diabetes correctly was measured in all seven manufacturing kits. However, as measured with insulinoma samples TFB, Siemens, Izotope, Cisbio kits were correctly measured, but Biosource and Immunotech kits were measured 47.4 ${\mu}IU/ml$, 72.3 ${\mu}IU/ml$, respectively. Conclusion : Serum Insulin radioimmunoassay kits were showed excellent precision, correlation and good recovery rate. However, some kits were not measured correctly in the high concentration insulin values. when selecting a kit should be considered many factors that cost effectiveness, compatible for automation equipment, high performance kit, the environment for each laboratory such as reaction time and reporting time.

• Korean Journal of Adult Nursing
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• v.12 no.2
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• pp.184-195
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• 2000

It has been believed that cancer is an omnious factor threatening the future and life itself. Patients having the disease experience anxiety, fear, feeling of weakness, depression and feelings of uncertainty and hopelessness. Most cancer patients, however, have expectations of possible recovery and a better future, very different from the patients who feel hopeless. Therefore. hope allows people to respond effectively to the fatal disease they have and prevents them from detoriorating physically and spiritually, positively influencing their survival, response to treatment and sense of security. Studies previously performed showed that hope is positively correlated with social and family supports, self-esteem, spiritual well-being, responsive action, health promotion behavior and quality of life. Thus, the study attempted to provide basic information on nursing cancer patients by investigating their levels of hope and determining predictive factors which influence hope. For the study 200 cancer patients in two university hospitals located in Pusan were sampled as subjects. Data were collected for twenty nine days from Feburary 1, 1999 to March 1. Instrumets for the study included 10 items from the self-esteem scale by Rosenberg (1965), 39 hope measurements by Kim and Lee(1965), 16 of the social support scale by Tae(1986) and 16 of the general characteristics scale, all of which totaled 81 items. The data were analyzed using the SPSS program. General characteristics of the investigated based on numbers and percentage. Hope, self-esteem and social support were analyzed using means, minimum, maximum and standard deviation. Relations among the foregoing three factors were analyzed using Pearson' correlation coefficient. Levels of hope in cancer patients were determined using t-test, ANOVA and Scheffe test. Predictive factors influencing hope were investigated using multiple stepwise regression analysis. Results of the study are summarized as follows: 1. An average level of hope was $185.55{\pm}23.39$ points(96 min. and 234 max.) 2. Levels of hope showed a significant difference among them according to sex (t=-3.69, P=.000), age(F=4.714, P=.000), job(F=3.247, P=.008), monthly income (F=6.113, P=.003), treatment charge (F=3.796, P=.011), supportive resources (F=10.554, P=.000), diagnosis(F=2.287, P=.029), perceived health status(F=22.184, P=.000), level of pain(F=3.334, P=.021), religion (F=4.911, P=.001) and religion's effect in life (F=11.706, P=.000), 3. For the subjects, self-esteem and social support were $38.32{\pm}7.21$(13 min, and 50 max.) and $52.97{\pm}8.49$points(28 min, 80 max.). Concerning social support, average levels of family support and medical support were found $35.95{\pm}6.05$(18 min, and 40 max) and $27.02{\pm}4.99$ points(20 min and 40 max). The hope the cancer patients showed significant correlations with self-esteem (r=.588, P=.000), family support(r=.224, p=.001) and medical support(r=.221, P=.002). 4. The five variables related to hope (self-esteem, religion's effect in life, perceived health status, social support and age) accounted for 54.2 percent of the hope level; especially, self-esteem was the highest at 34.6%. As shown in the above results, predictive factors which most influence hope in cancer patients were self-esteem and religion's effect of life. Therefore, nursing interventions to increase self-esteem should be developed. Regarding religion's effects, studies on spiritual aspects should be carried out in a way that contributes to promotion of hope.

• Journal of Korean Ophthalmic Optics Society
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• v.12 no.4
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• pp.55-69
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• 2007

The purpose of this study was to perform a clinical test using ez NANOsence II RGP contact lenses for the effects of visual acuity and cornea on subjects with abnormal refraction status. One hundred twenty one adults (twenty nine males, ninety two females, range = 17 to 43, mean = 22.86) were recorded. The subject's history including, the symptoms from previous lens wear, were studied. The subjects were observed, both pre and post lens wear, for any symptoms and signs of change of the eye. The Visual acuity and the binocular status were tested at the far distance using the contact lens. The refraction test was performed on the naked eye using the objective method (Topcon KR-8100, Japan). The Stereopsis test was performed at the near distance after contact lens wear by the Titmus fly (Stereo Optical Co., U.S.A) and TNO (TECH, The netherlands). The contrast sensitivity diagnosis was performed at 1m distance after contact lens wear by the contrast sensitivity chart (pelli-Robertson, USA). The corneal topography was analyzed on the naked eye after lens wear by ORB scan (Bausch Lomb, U.S.A.). The ultra structure of surface on the contact lens was observed using SEM (JMS-5800, Japan). The chemical component's of the contact lens was analyzed by EDS program. The results of this study were as follows: 1. The longer of contact lens wear period, The subjects have acquired the most improved visual function. 2. Subjects had experienced few side effects wearing the contact lenses. 3. The lenses were easy to use by the subjects. 4. The longer of contact lens wear period, the power of cornea had more decrease. but the base curve of cornea had more increase and corneal astigmatism was decrease (p<0.01). Also, the thickness of corneal center was few decrease. 5. Longer periods of using the contact lens showed stereopsis and contrast sensitivity at more normal values. 6 The corneal topographical after lens wear showed most subjects with similar morphology for different wear periods. 7. The surface ultrastructure of the new and used contact lens was the similar fine shape. 8. The chemical component's of in the new and used contact appeared to have similar results. In conclusion, this study showed that the surface ultrastructure and chemical component's of the new and used contact lens are similar. In addition, the subjects had improved the quality of vision and few experienced any side effects during long periods of contact lens wear. Also they have decrease of corneal astigmatism during the long period of lens wear. Our test has showed that the chemical composition and fine structure of contact lens have related to the visual function on contact lens wearer. In this paper, we suggested that ez NANOsence II RGP contact lenses had a moderate effect for correcting vision of abnormal refraction eye.

• The Korean Society of Law and Medicine
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• v.17 no.2
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• pp.85-124
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• 2016

The Human gene testing act (GenDG) in Germany starts from the characteristic features of gene testing, i.e. dualisting structure consisted of anlaysis on the one side and the interpretation on the other side. The linguistic distincion of 'testing', 'anlaysis' and 'judgment' in the act is a fine example. Another important basis of the regulation is the ideological purpose of the law, that is information autonomy. The normative texts as such and the founding principle are the basis of the classification of testing types. Especially in the case of gene testing for medical purpose is classified into testing for diagnostic purpose and predictive purpose. However, those two types are not always clearly differentiated because the predictive value of testing is common in both types. In the legal regulation of gene testing it is therefore important to manage the uncertainty and subjectivity which are inherent in the gene-analysis and the judgment. In GenDG the system ensuring the quality of analysis is set up and GEKO(Commity for gene tisting) based on the section 23 of GenDG concretes the criterium of validity through guidelines. It is also very important in the case of gene testing for medical purpose to set up the system for ensurement of procedural rationality of the interpretation. The interpretation of the results of analysis has a wide spectrum because of the consistent development of technology on the one side and different understandings of different subjects who performs gene testings. Therefore the process should include the communication process for patients in oder that he or she could understand the meaning of gene testing and make plans of life. In GenDG the process of genetic counselling and GEKO concretes the regulation very precisely. The regulation as such in GenDG seems to be very suggestive to Korean legal polic concerning the gene testing.

• Journal of the Korean Society of Radiology
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• v.6 no.6
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• pp.447-454
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• 2012

To examine the performance of a diagnostic X-ray system, we tested a linearity, reproducibility, and Half Value Layer(HVL). The linearity was examined 4 times of irradiation with a given condition, and we recorded a level of radiation. We then calculated the mR/mAs. And the measured value should not be more than 0.1. If the measured value was more than 0.1, we could know that the linearity was decreased. The reproducibility was analyzed 10 times of irradiations at 80kVp, 200mA, 20mAs and 120kVp, 300mA, 8mAs. The values from these analyses were integrated into CV equation, and we could get outputs. The reproducibility was good if the output was lower than 0.05. HVL was measured 3 times of irradiation without a filter, and we inserted additional HLV filters with 0, 1, 2, 4 mm of thickness. We tested the values until we get the measured value less than a half of the value measured without additional filter. We tested the linearity, the reproducibility, and HVL of 5 diagnostic X-ray generators in this facilities. The linearity of No. 1 and No. 5 generator didn't satisfy the standard for radiation safety around 300mA~400mA and 100mA~200mA, respectively. HVL of No.1 generator was not satisfied at 80kVp. The outputs were higher in the three-phase equipment than the single-phase equipment. The old generators need to maintain and exchange of components based on the these results. Then, we could contribute to getting more exact diagnosis increasing a quality of the image and decreasing an expose dose of radiation.

• The Korean Journal of Nuclear Medicine Technology
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• v.18 no.1
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• pp.115-121
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• 2014

Purpose: In addition to improving the quality of the PET image, through much research, the development of various programs are performed. Astonish TF reconstruction techniques by Philips can confirm the improved contrast of the lesion. Also, It's image reconstruction of 2 mm is possible with rapid reconstruction rate than conventional. In this study, we compared and evaluated Standardized Uptake Value (SUV) in accordance with the 2 mm reconstruction techniques and traditional 4 mm from the $^{18}F-FDG$ PET whole body image. Materials and Methods: In the phantom experiment, NEMA IEC body phantom (sphere: 10, 13, 17, 22, 28, 37 mm) was used to obtain images by using GEMINI TF 64 PET/CT (Philips, Cleveland, USA). Also, In the clinical images, we performed $^{18}F-FDG$ PET/CT examination to 30 women (age: $55.1{\pm}11.3$, BMI: $24.1{\pm}2.9$) with a diagnosis of breast cancer. After that, we reconstructed images in 2 mm and 4 mm respectively. The region of interest was drawn to acquired images. Since then, we measured SUV and statistically analyzed with SPSS ver.18 by using EBW (Extended Brilliance Workstation) NM ver.1.0. Results: After analyzing the result of the phantom study, there was a tendency that the bigger hot sphere size, the higher SUV. If you compared the 2 mm reconstruction techniques to 4 mm, it increased 95.78% in 10 mm, 50.60% in 13 mm, 25.00% in 17 mm, 30.04% in 22 mm, 31.81% in 28 mm, and 27.84% in 37 mm. Through the result of the analysis of the 2 mm reconstruction techniques and 4 mm in clinical images, it appeared that SUV of 2 mm was higher than that of 4 mm. Also the smaller the volume was, the more the change rate increased. Conclusion: After analyzing the result of the clinical picture and phantom experiments applied by Astonish TF reconstruction techniques, as the size of the volume was small, the change rate of the SUV increased. Therefore, it was necessary to further research about the SUV correction for accurate and active utilization of 2 mm reconstruction techniques which had excellent lesion discrimination ability and contrast in clinic.

• Progress in Medical Physics
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• v.12 no.2
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• pp.113-124
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• 2001

For veterinary imaging diagnosis, we obtained MR images of the canine brain, spine, kidney and pelvis from 3T MRI system which was equipped with the world first 3T active shield magnet. Spin echo (SE) and fast Spin Echo (FSE) images were obtained from the canine brain, spine, kidney and pelvis of normal and sick dogs using a homemade birdcage and transverse electromagnetic (TEM) resonators operating in quadrature and tuned to 128 MHz. In addition, we employed a homemade saddle shaped RF coil. Typical common acquisition parameters were as follows: matrix=512$\times$512, field of view (FOV)=20cm, slice thickness=3 w, number of excitations (NEX)=1. For T1-weighted MR images, we used TR=500 ms, TE=10 or 17.4 ms. For T2-weighted MR images, we used TR=4000 ms, TE=108 ms. Signal to noise ratio (SNR) of 3T system was measured 2.7 times greater than that of prevalent 1.57 system. The high resolution images acquired in this study represent more than a 4-fold increase in in-plane resolution relative to conventional images obtained with a 20 cm field of view and a 5 mm slice thickness. MR images obtained from 3T system revealed numerous small venous structures throughout the image plane and provided reasonable delineation between gray and white matter The present results demonstrate that the MR images from 3T system could provide better diagnostic quality of resolution and sensitivity than those of 1.5T system. The elevated SNR observed in the 3T high field magnetic resonance imaging can be utilized to acquire images with a level of resolution approaching the microscopic structural level under in vivo conditions. These images represent a significant advance in our ability to examine small anatomical features with noninvasive imaging methods. Moreover, MRI technique could begin to apply for veterinary medicine in Korea.

• Progress in Medical Physics
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• v.14 no.2
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• pp.131-140
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• 2003

As the importance of accuracy in measurings of 3-D anatomical structures continues to be stressed, an objective and quantitative of assessing image quality and accuracy of 3-D volume-rendered images is required. The purpose of this study was to evaluate the quantitative accuracy of 3-D rendered images obtained with MDCT, scanned at various scanning parameters (scan modes, slice thicknesses and reconstruction slice thickness). Twelve clinically significant points that play an important role for the craniofacial bone in plastic surgery and dentistry were marked on the surface of a dry human skull. The direct distances between the reference points were defined as gold standards to assess the measuring errors of 3-D images. Then, we scanned the specimen with acquisition parameters of 300 mA, In kVp, and 1.0 sec scan time in axial and helical scan modes (pitch 3:1 and 6:1) at 1,25 mm, 2.50 mm, 3.75 mm and 5.00 mm slice thicknesses. We performed 3-D visualizations and distance measurements with volumetric analysis software and statistically evaluated the quantitative accuracy of distance measurements. The accuracy of distance measurements on the 3-D images acquired with 1.25, 2.50, 3,75 and 5.00 mm slice thickness were 48%, 33%, 23%, 14%, respectively, and those of the reconstructed 1.25 mm were 53%, 41%, 43%, 36% respectively. Meanwhile, there were insignificant statistical differences (P-value<0.05) in the accuracy of the distance measurements of 3-D images reconstructed with 1.25 mm thickness. In conclusion, slice thickness, rather than scan mode, influenced the quantitative accuracy of distance measurements in 3-D rendered images with MDCT. The quantitative analysis of distance measurements may be a useful tool for evaluating the accuracy of 3-D rendered images used in diagnosis, surgical planning, and radiotherapeutic treatment.

• Journal of the Korean Institute of Landscape Architecture
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• v.38 no.1
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• pp.25-38
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• 2010

This study aims to, first, classify the approach system of utilizing national and local amenities into three groups: evaluation of the level of the amenity, evaluation of the value of amenity resources, and utilization of amenity resources. Secondly, the study aims to derive an evaluation index which is required according to the approach system. As for a strategy of utilizing amenities, the self-diagnosis based on the evaluation of the level of local amenities is required. In this study, three sectors, ten items and 43 detailed items have been derived though specialists, FGIs(Focus Group Interview), and the cases within and outside of the country. In the survey with specialists, a unit-space key index which can be used in accordance with the unit-space characteristics(such as urban and rural area, fishing villages, mountain villages, and littoral areas), environmental area, important index and optional index have been derived. As for the evaluation index which is needed for the development of typical local amenities resources, 14 detailed evaluation indexes from three sectors have been derived. These have been classified again into six key indexes, six important indexes and two optional indexes. In the evaluation index of amenity resources, natural and environmental value-such as ecological value of preservation, uniqueness of aesthetic landscape and ecological value of restoration-historic and cultural value of preservation and individuality of the resources, and aesthetic and cultural values are derived as being important. As for utilization of amenity resources, 15 items from three sectors-such as reservation, restoration, intentional use, and industrial use-have been derived. Also, through a survey with specialists, key indexes and important indexes were derived by unit-space. As a result, in urban areas, seven items-including creative development of space, aesthetic landscape control and development of cultural contents-have been derived as key indexes; in rural areas, mountain villages, fishing villages and littoral areas, eight items-including brand of region, brand of the products, and brand of tourism resources-have been derived. In environmental areas, six items-including reservation of natural resources, historic and cultural heritage, and restoration of ecosystem-have been derived. Indexes derived from this study can be utilized in order to establish a strategy for amenity plans and an implementation strategy, but can be readjusted, allowing for various regional characteristics. It is, thus, advisable that local governments selectively utilize indexes according to regional characteristics or newly develop them for the maximum utilization of regional amenities.