• Clinical and Experimental Pediatrics
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• v.48 no.10
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• pp.1132-1138
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• 2005

Purpose : The mucopolysaccharidoses (MPSs) are a heterogeneous group of lysosomal storage disorders. They are caused by a deficiency of the enzymes involved in the degradation of glycosaminoglycans. Early recognition is important because recombinant enzyme replacement therapy is now available for MPS. We studied the clinical characteristics of 80 MPS children with the object of determining the epidemiological, clinical and radiological features in Korean MPS children. Methods : Diagnosis of MPS was confirmed by skin fibroblast enzyme analysis in 80 patients between February 1995 and December 2004. Charts were retrospectively reviewed for clinical and radiological findings, as well as for intelligence and speech evaluations. Results : Hunter syndrome (MPS type II) was the most prevalent type, appearing in 51/80 cases (64 %), followed by Sanfilippo syndrome (MPS III-18%), Hurler syndrome (MPS I-15%), and Morquio syndrome (MPS IV-4%). The average age at diagnosis was 5.5 years (range 1 to 20), and the male-to-female ratio was 4.7 : 1. Typical radiographic changes were observed in 45/54 cases (83%). Mitral regurgitation was the most common cardiac defect. Moderate to profound mental retardation and hearing loss were present in 14/35 cases (56%) and 33/38 cases (82%), respectively. Four MPS II patients had bone marrow transplantation, with mixed outcomes. Five MPS I patients are currently on enzyme replacement therapy. Conclusion : Our study showed a high proportion of MPS II cases (64%), which may represent population variability. By studying the clinical features of these patients, we hope to alert pediatricians of the warning signs of MPS.

• Journal of rehabilitation welfare engineering & assistive technology
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• v.6 no.1
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• pp.51-58
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• 2012

In this study, the active exercise load control system was developed that monitors patient's muscular condition based on EMG signals and controls the load by biofeedback in real-time. In order to select appropriate muscular parameter for the system, the pre-exercise test was performed to obtain EMG signals from healthy 12 males. Subsequently, the main exercise test was performed to evaluate the active exercise load control system based on IEMG: a selected muscular parameter, on healthy 10 males without musculoskeletal disorder. The accuracy and availability of developed system were confirmed through observing changes between exercise load and IEMG. A correlation was analyzed between the fatigue of muscles and RPE indicating the individual subjective fatigue. As a result, the active exercise load control system that was suggested in this study could be useful to control the initial load-balancing and the amount of exercise appropriate for individual in rehabilitation exercise therapy.

• The Journal of the Korean bone and joint tumor society
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• v.14 no.1
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• pp.10-16
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• 2008

With the advent of effective chemotherapy and the realization of high economic cost associated with amputation, limb salvage surgery has become the standard of treatment in children with primary malignant bone tumors. Reconstruction after resection of malignant bone tumors of children has to address the leg length inequality and also has to be durable to cope with high functional demands of young patients. Expandable endoprostheses have been used in children for achieving limb length equality with substantial risk of complications. Recently, significant advances in prosthetic designs have reduced the morbidities associated with these prostheses. The purpose of this study was to review the indications, characteristics, complications and recent developments of expandable endoprostheses used for malignant bone tumors in children.

• Journal of Chest Surgery
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• v.34 no.7
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• pp.552-555
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• 2001

Noonan syndrome is characterized by a Turner-like phenotype and a normal karyotype associated with congenital abnormalities, such as short stature, variable mental retardation, hypertelorism, webbed neck, low posterior hair line, skeletal malformation and congenital cardiovascular defect. Two third of Noonan syndrome have cardiac anormalies, half with pulmonary stenosis. We have experienced two cases of pulmonary stenosis associated with other cardiac anomalies in Noonan syndrome. The first 31-year-old male patient had characteristic appearance of Noonan syndrome with severe infundibular pulmonic stenosis and patent foramen ovate. The second 28-year-old male patient had valvular and subvalvular Pulmonary stenosis with typical Noonan\`s face and stature. Pulmonary valvotomy and hypertrophied muscle bundles in the right ventricular cavity were resected in both cases. Patent foramen ovate was closed directly in the first case. Postoperative follow-up examinations revealed no symptoms and successful outcome.

• Journal of the Society of Naval Architects of Korea
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• v.32 no.4
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• pp.9-18
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• 1995

The use of CAD/CAM systems is growing fast in the shipbuilding industry. To develope a geometric modeler, the existing CAD/CAM systems have been analysed. Because existing systems have closed architectures, it is not easy to investigate the internal structures. However, new trends in the software engineering, open architectured systems, pose some possibility to develope the geometric modeler. Several geometric modelers are analysed to extract component functions and modules. ACIS of the Spatial Technology, AIS of the CAM-I consortium, the STEP part for the geometry and topology, CAD*I of the ESPRIT project, and domestic modelers are investigated. Based on this analysis, a reference model which shows the framework of the modeler is proposed. With the data structure supporting non-manifold topologies, the reference model can be used to encourage a cooperative development program.

• Journal of the Korea Institute of Information and Communication Engineering
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• v.26 no.3
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• pp.403-411
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• 2022

A motion-based sports game uses a motion sensor or a camera to exercise based on body movements, and it is possible to obtain exercise effects such as improving physical strength while enjoying the game. In prior works, various studies such as usability evaluation has been conducted on motion-based sports games. However, there has been no discussion about how the exercise effect is exerted on users when experiencing motion-based sports games as individual or team play. This study compared the user's exercise effects by analyzing the user's ECG (Electrocardiogram) sensor and the Kinect sensor's skeletal information using Nintendo Switch game that is played individually and as a team. In this paper, the experimental design and method, the quantitative measurement results based on ECG and Kinect, and the results of the post-test subjective measurement are discussed.

• Journal of Digital Contents Society
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• v.9 no.1
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• pp.53-60
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• 2008

Based on similarities between fuzzy set theory and mathematical morphology, Grabish proposed a fuzzy morphology based on the Sugeno fuzzy integral. This paper proposes a fuzzy mathematical morphology based on the defuzzification of the fuzzy measure which corresponds to fuzzy integral. Its process makes a fuzzy set used as a measure of the inclusion of each fuzzy measure for subsets. To calculate such an integral a $\lambda$-fuzzy measure is defined which gives every subsets associated with the universe of discourse, a definite non-negative weight. Fast implementable definitions for erosion and dilation based on the fuzzy measure was given. An application for robust skeletonization of two-dimensional objects was presented. Simulation examples showed that the object reconstruction from their skeletal subsets that can be achieved by using the proposed was better than by using the binary mathematical morphology in most cases.

• The korean journal of orthodontics
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• v.24 no.1 s.44
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• pp.161-180
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• 1994

This study was performed to investigate the midline having the least difference between the right and left structure among the lines that had been used in the submentovertex analysis and secondarily to know the distribution of asymmetry and it's degree existed normal persons and asymmetric patients. The subjects consisted of 40 normal adult patients and 40 asymmetric adult patients. The computerized analyses from submentovertex cepholometric radiograph were carried out. The results were as follows : 1. The right and left difference of the perpendicular bisecting line between right and left foramen spinosum was larger than the other midlines in the anterior area and it was decreased gradually as it progressed posteriorly. Specially the difference of this line was the smallest in the area where there was foramen spinosum. 2. The right and left difference of the perpendicular line through crista gali to the line between right and left foramen spinosum was smaller than the other midlines in the anterior area and it was increased gradually as it progressed posteriorly. 3. The right and left difference of the line between crista gali and anterior process of atlas was constant and smaller than the other midlines. 4. Asymmetry was a common finding in both normal and asymmetry group and left or right dominance of asymmetry was not statistically singnificant. 5. When the analyses were undertaken after submentovertex radiogram was divided into cranial base, upper face and lower face, the more inferior part showed relative asymmetry than the more superior part.

• Journal of the Korean Chemical Society
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• v.34 no.2
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• pp.130-135
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• 1990

The title compound (C13H17NO2S) is monoclinic, space group P21/a, with a = 13.756 (3), b = 9.310(4), c = 21.305(3) $\AA$, $\beta$ = 95.0。, Z = 8, V = 2718.11 $\AA$3, Dc = 1.23$g·{\cdot}cm-3$, (Mo k$\alpha$) = 0.71069$\AA$,$\mu$ = 2.18 cm-1, F(000) = 1071.86, T = 298, R = 0.085 for 2935 unique observed reflections with I >2.0$\sigma$(I). The structure was solved by direct methods. The C-H bond lengths and the methyl groups are fixed and refined as their ideal geometry by allowing to ride on the parent atoms. Both molecules A and B have almost same structures except for two terminal ethyl groups. The ethylene-like skeleton including the nitro group in one molecule is nearly perpendicular to the plane of the methylbenzene group and two ethyl groups form a cis-type structure which has the dfferent orientations between two molecules; in the molecule A, two terminal methyl groups being the opposite directional arrangement against the plane of its skeleton, while in the B, with the same directional structure from its plane. The molecules in the crystal are packed together by non-bonded van der Waals forces.

• The korean journal of orthodontics
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• v.14 no.1
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• pp.15-24
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• 1984

The purpose of this study was to evaluate the effect of O.M.A on Angle's class III malocclusion. The author analysed the data obtained from pre- and post-treatment lateral cephalograms. The results obtained were as follows : 1. Forward growth of maxillary complex was accomplished. 2. The mandible showed few morphological changes in the presence of downward and backward swing. 3. It was assumed that the hooks attached to the intraoral appliance should be placed as far frontally as possibie. 4. Relations of the upper and lower jaws were improved resulting in better facial profiles.