• 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.

• Clinical and Experimental Pediatrics
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• v.49 no.11
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• pp.1186-1193
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• 2006

Purpose : Enterovirus infection is a type of viral infection that occurs relatively frequently in children during summer. It has clinical symptoms of non-specific fever, aseptic encephalomeningitis, gastrointestinal diseases, skin rash and, hand-foot-mouth disease. However, it can also occcaisionally, result in fatal symptoms like myocarditis, epicardial inflammation, transverse myelitis, quadriplegia and etc. There have been epidemic enterovirus studies, but not in the Chungnam area. Therefore, we undertook this study in order to comprehend the cause viruses in this area. Methods: We enlisted 157 children hospitalized with enteroviral infections at Soonchunhyang University hospital in Cheonan between May and August 2005. Cerebrospinal fluids or feces were collected during the acute phase after hospitalization, and observed the cytopathic effects caused by enterovirus and using reverse transcription polymerase chain reaction (RT-PCR). Results : The number of children hospitalized due to possible enteroviral infection during the period of study was 157. The number of children who tested positive with the reverse transcription polymerase chain reaction totalled 32 cases (20.4 percent). Among the children with entroviral diseases, 20 were male and 12 were female, thus the sex ratio of male to female was 1.67:1. Their clinical symptoms included fever most frequently (93.7 percent), was followed by headaches (90.0 percent), meningeal irritation signs (65.0 percent), and abdominal pain (30.0 percent). As for the type of isolated enterovirus, there were 17 cases of echovirus 18 and 6 cases of coxsackievirus B5. Furthermore, there were 2 cases of echovirus 9, 1 case of coxsackievirus A6 and coxsackievirus B3, respectively. But 5 cases were not determined by genotype. Conclusion : Echovirus 18 is circulating in Korea. We reported on identified enteroviruses, including echovirus 18, using RT-PCR in the Chungnam area during the summer of 2005.

• The Journal of Korean Society for Radiation Therapy
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• v.19 no.1
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• pp.51-54
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• 2007

Purpose: Total body irradiation is used to kill the total malignant cell and for immunosuppression component of preparatory regimens for bone-marrow restitution of patients. Beam spoiler is used to increase the dose to the superficial tissues. This paper finds the property of the distance between beam spoiler and patient. Materials and Methods: Set-up conditions are 6 MV-Xray, 300 MU, SAD = 400 cm, field size = $40{\times}40cm^2$. The parallel plate chamber located in surface, midpoint and exit of solid water phantom. The surface dose is measured while the distance between beam spoiler and patient is altered. Because it should be found proper distance. The solid water phantom is fixer and beam spoiler is moving. Results: Central dose of phantom is 10.7 cGy and exit dose is 6.7 cGy. In case of distance of 50 cm to 60 cm between beam spoiler and solid water phantom, incidence dose is $14.58{\sim}14.92cGy$. Therefore, The surface dose was measured $99.4{\sim}101%$ with got near most to the prescription dose. Conclusion: In clinical case, distance between beam spoiler and patient affect surface dose. If once $50{\sim}60cm$ of distance between beam spoiler and patient, surface dose of patient got near prescription dose. It would be taken distance between beam spoiler and patient into account in clinical therapy.

• Radiation Oncology Journal
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• v.21 no.1
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• pp.66-74
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• 2003

Purpose : The matrix metalloprotelnases (MMPs) are a family of enzymes whose main function is the degradation of the extracellular matrix. Several studies have revealed that MMPs and TIMPS are related to the wound heating process and in photoaging caused by ultraviolet Irradiation. However, the expressions of MMP and TIMP after irradiation have not, to the best of our knowledge, been studied. This study investigates the expressions of MMP-2 and TIMP-2 in rat Intestinal mucosa following irradiation. Materials and Methods : The entire abdomen of Sprague-Dawley rats was irradiated using a single dose method. The rats were sacrificed on day 1, 2, 3, 5, 7 and 14 following irradiation. Histopathological observations were made using hematoxilin & eosin staining. The expressions of MMP-2 and TIMP-2 were examined using immunohistochemistry, Irnrnunoblotting and ELISA. Results : Radiation induced damage associated with atrophic villi, and infiltration of inflammatory cell was observed from the first postirradiation day, and severe tissue damage was observed on the second and the third postirradiation days. An increase in mitosis and the number of regenerating crypts, as evidence of regeneration, were most noticeable on the fifth postirradiation day. From the immunohistochemlstry, the MMP-2 expression was observed from the first postirradiation day, but was most conspicuous on the third and the fifth postirradiation days. The TIMP-2 expression was most conspicuous on the fifth postirradiation day. From the irnrnunoblotting, the MMP-2 expression was strongly positive on the third postirradlatlon day, and that of TIMP-2 showed a strong positive response on the fifth postirradiation day. In ELISA tests, the expressions of MMP-2 and TIMP-2 were increased in the postirradiation groups compared to those of the normal controls, and showed a maximum increase on the fifth postirradiatlon day. These results were statistically significant. Conclusion : The expressions of MMP-2 and TIMP-2 were increased in the intestinal mucosa of the rats following irradiation, and these results correlated with the histopathological findings, such as tissue damage and regeneration. Therefore, this study suggests that MMP-2 and TIMP-2 play roles in the mechanisms of radiation-induced damage and regeneration of intestinal mucosa of rats.

• Tuberculosis and Respiratory Diseases
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• v.51 no.4
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• pp.346-353
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• 2001

Background : Pulmonary Langerhans cell histiocytosis forms part of a spectrum of diseases that are characterized by a monoclonal proliferation and infiltration of organs by Langerhans cells. Several organ systems may be involved in Langerhans cell histiocytosis, including the lungs, bone, skin, pituitary gland, liver, lymph nodes and thyroid. Pulmonary histiocytosis X represents 2.8% of interstitial lung disease. Here we present the clinical, radiological, therapeutic aspects of pulmonary histiocytosis X. Method : Fourteen cases of biopsy-proven pulmonary histiocytosis X patients who were diagnosed in Seoul National University Hospital during the period from January 1990 to December 1998 were analyzed retrospectively. Result : There were 12 men and 2 women in this study. The initial presenting symptoms were dyspnea, cough, chest pain, which was associated with the pneumothorax, and chest radiography abnormalities. Only 8 patients (57%) were smokers. There were 5 patients with extra-pulmonary histiocytosis (pituitary, bone, skin). Eight patients had received the chemotherapy. There were no mortalities and only one patient experienced an aggravation of symptom during the follow-up period. Conclusion : In contrast to previous reports from other countries, the patients with pulmonary histiocytosis X in this study presented with several different clinical characteristics, such as a male predominance, relatively low smoker's rate, and a better prognosis.

• Clinical and Experimental Pediatrics
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• v.45 no.8
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• pp.1007-1015
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• 2002

Purpose : The aim of this study is to find a method to diagnose and treat children who showed benign hemophagocytic histiocytes in bone marrow examination. Methods : We analyzed the clinical data of thirty patients retrospectively who showed benign hemophagocytic histiocytes in bone marrow examination from January 1995 to November 2001 at Keimyung University Dong-san Hospital. Bone marrow histiocytes were classified into a few, some, and many according to the number of histiocytes comparing with the white cells. Results : The age of thirty patients ranged from two months to 15 years. The median age was 5.6 years with male predominance(2.3 : 1). The most frequent clinical manifestation was fever; others were respiratory symptom, hepatosplenomegaly, lymphadenopathy and skin rash(in order of frequency). Common laboratory findings were leukopenia, thrombocytopenia, anemia and abnormal liver function test. Infection was present in 30 patients; causative organisms were documented in 15 patients, and bacterial infection was more common. Epstein Barr virus was the cause of infection in four patients. Bone marrow examination showed a few(20.0%), some(75.0%) and many(30.0%) hemophagocytic histiocytes. Combination immunochemotherapy including immunoglobulin, steroids and cyclosporine were helpful in 22 out of 30 patients. The mortality rate was high in young patients who showed some to many hemophagocytic histiocytes. Conclusion : Bone marrow examinations and early detection of histiocytes will be helpful in children who have fever, hepatosplenomegaly and abnormal liver function test. Immunochemotherapy were helpful and further investigation will be needed for the detection of the relationship between the bone marrow findings and prognosis.

• Korean Journal of Food Science and Technology
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• v.45 no.2
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• pp.221-226
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• 2013

This study aimed to examine the antioxidant, tyrosinase inhibitory, and anti-proliferative activities (A549, G361, HT-29, and MDA-MB-231) of fermented gochujang (made from sword bean cheonggukjang powder (SBC) for 90 days. Gochujang was prepared by adding 0 (SBC 0), 2 (SBC 2), 5 (SBC 5), 8 (SBC 8) and 10% (SBC 10) levels with SBC, and all experiments were measured at diluted levels of 20, 50 and 100 times. The antioxidant activity and tyrosinase inhibitory effect demonstrated that SBC 10 increased approximately 1.2 and 1.1 times compared with SBC 0, respectively, at diluted levels of 50 and 100 times. The anti-proliferative effects of A549, G361, and HT-29 presented that SBC 10 were 2.8, 1.1, and 8.9 times higher compared with SBC 0, respectively, at diluted levels of 50, 20, and 100 times. In the case of MDA-MB-231, SBC 10 was 3.7 times higher compared with SBC 5 at diluted level of 20 times. As a result, we confirmed that SBC gochujang was improved for physiological activities and anti-proliferative effects.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.1
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• pp.24-33
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• 2016

Mucopolysaccharidosis type VI (MPS VI) is a rare disease caused by the mutation of ARSB with prevalence range from 1/5,000 in northeast Brazil to 1/2,057,529 births in Czech Republic. In Asia, there is only one published figure in Taiwan of about 1/833,000 births. The exact prevalence in the Korean population is unknown, but we estimated the incidence of MPS VI is about 0.03/100,000 live births. Enzyme replacement therapy (ERT) with recombinant human Arylsulfatase B (rhASB) is a modality for the treatment of MPS VI that reduces the excretion of urine glycosaminoglycan (GAG) and improves joint motion, pulmonary function, and endurance. We presented the clinical features, molecular analysis and outcome of ERT in three Korean MPS VI patients. All patients had the typical characteristic clinical features of MPS IV. Short stature, dysostosis multiplex, corneal opacity and valvular heart disease were found at first presentation, while restrictive lung disease and carpal tunnel syndrome developed later in all patients. Molecular analysis demonstrated novel missense and nonsense mutation in the patients, including p.Ile 67Ser, p.Gly328Arg, $p.Arg191^*$, p.Asp352Asn, and p.Gly17Asp. After ERT, urine GAG was decreased in all patients. Skeletal involvement, corneal opacity, heart valve abnormalities and pulmonary function were not improved with ERT, but it had a better outcome on regarding joint motion and endurance. One patient underwent allogeneic bone marrow transplantation (BMT) prior to ERT, but their clinical response was not improved much after BMT. This study demonstrates clinical phenotypes and molecular analysis of the severe form of MPS VI in Korean patients.

• Journal of Korean Society of Transportation
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• v.25 no.4
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• pp.123-133
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• 2007

This study analyzed whether a transit network with hierarchy is efficient or not, and if transit network hierarchy has cost efficiency, then which condition guarantees the efficiency of the transit network hierarchy. The authors modeled the total cost of the transit network and suggested the conditions in which the transit network hierarchy has cost efficiency through comparing the cost of the transit network with and without hierarchy. The efficiency of transit network hierarchy is guaranteed when the travel cost savings induced by using a higher hierarchy transit network is larger than the increasing non-travel cost, which is the sum of access cost, waiting cost, and operating cost, induced by the introduction of a higher hierarchy transit network. This result is consistent with common sense and with the concept of cost and benefit analysis. If a passenger traveling within the area divided by a higher hierarchy transit network uses only a lower hierarchy transit network and the passenger traveling out of the area divided by the higher hierarchy transit network uses both lower and higher hierarchy transit networks, the travel demand using the higher hierarchy transit network is inversely proportional to the square of the line spacing. This means that the transit network becomes more efficient and small increases of travel demand guarantee the efficiency of the transit network hierarchy as the connectivity of the network becomes higher. This result shows that transit networks have economies of aggregation. This study is the first analytical research on transit network hierarchy and is expected to be a basis for numerical research. However, numerical research should complement this study, since analytical research has some limitations for considering a real network.

• Korean Journal of Food Science and Technology
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• v.5 no.3
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• pp.169-173
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• 1973

Secondary amines and nitrites in various daily foods have been known as the precursors of potent carcinogenic nitroso compound produced in the human stomach when they were ingested simultaneouly in high concentration. In this report, the amounts and distribution of secondary amines and nitrites in Korean daily foods, kim-chi, fishes, fish eggs, sausages, canned fish foods and fish sauces (salted fish) were studied.Nitrite contents were low in most subjected foods except in sausages. Secondary amines showed low contents in kim-chi, fishes, but high in fish sauces, fish eggs and canned fish foods. The result of this study suggested that the possible formation of carcinogenic nitrosamines during manufacturing, storage and cooking of all Korean foods should be studied.