• Childhood Kidney Diseases
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• v.2 no.1
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• pp.77-81
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• 1998

The author exprienced a case of glycogen storage disease type Ia(GSD-I) in an 18-year-old male patient who was admitted to our hospital due to proteinuria and hypertension. he was suspected to have GSD when 12 years old because of his family history of short stature and hepatomegaly. On admission, physical examination revealed short stature, heparomegaly, and The diagnosis of GSD-I was confirmed by compatible liver biopsy finding and enzyme assay which erealeddeficiency of glcose-6-phosphatase if hepatocyte. Sympromatic treatment was done using antihypertensive drugs and allopurinol with diet control. The authors report a case of glycogen storage disease type Ia completely confirmed by typical clinical manifestation, pathologic findings of the liver and the kidney, and the result of enzyme assay which revealed deficiency of glucose-6-phosphatase in hepatocytes with brief review fo related literatures.

• Biomedical Science Letters
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• v.2 no.2
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• pp.167-174
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• 1996

Alcoholics increased susceptibility to microbial infection that is associated with decreased immunity. but there has been little experimental evidence to support alcoholics-induced increase of microbial infection directly in non-specific immunity. Therefore, we were used the method of phagocytic-plaque including all the stimulating factors for the phagocytosis, subtypes of lymphocytes and T-lymphocyte proliferation. The experimental groups were divided into 3 groups: (1) alcoholics who were hospitalized less than 1 week (newly hospitalized alcoholics), (2) alcoholics who were hospitalized more than 2 weeks (old hospitalized alcoholics), (3) healthy blood donors. We have studied 98 alcoholics and 35 healthy blood donors and control groups. A physician has checked the biological markers and diagnosed the body-condition alcoholics. The immunity and non-specific immunity on the alcoholics were analyzed by using the simultest kit and flow cytometry. Proliferation of the lymphocytes was analyzed by the phytohemmagglutinine mitogen. Phagocytosis and migration properties of leukocytes were identified on the layer formed by Staphylococcus aureus Cowan I strain. Biological markers of alcoholics and control groups, by such as blood glucose, ${\gamma}$-glutamyl transpeptidase and mean corpuscular volumes of red blood cells, were determined by biochemical and hematological methods. Compared with control groups, cluster of differentiation (CD)3+, CD8+ and CD19+ in alcoholic were more decreased except CD4+/CD8+ ratio. Proliferation of the T-lymphocytes, phagocytosis and migration properties of the leukocytes in alcoholics were decreased compared with those of control groups. According to the results observed in our experiment, they can be summerized as follows: 1, Cellular, humoral and non-specific immunities, are markedly decreased in alcoholics than those in control groups. 2. It is inferred that Phagocytic plaque formation is a very useful method to evaluate phagocytosis and migration properties of the alcoholic leukocytes 3. It is thought that the subtypes of lymphocytes, especially CD4+/CD8+ ratio, are essential methods to analyzed the alcoholic immunity. 4. Specific and non-specific immunity on the old hospitalized alcoholics was slightly increased, which depends upon the alcoholic medication.

• Journal of Life Science
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• v.19 no.9
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• pp.1226-1231
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• 2009

Enzyme substitution with recombinant phenylalanine ammonia-lyase (PAL, EC 4.3.1.5) is currently being explored for treatment of phenylketonuria (PKU), an autosomal recessive genetic disorder with mutations of the gene encoding phenylalanine-4-hydroxylase (EC 1.14.16.1). However, oral administration of PAL is limited because of proteolytic digestion in the gastrointestinal tract. The aim of this study was to determine the biochemical properties of PAL and delinate the susceptibility of wild-type PAL to pancreatic proteolysis by exploring several mutants, and to develop therapeutic drugs with PAL for PKU. The specific activity of PAL was assayed and its optimal pH, temperature stability, and intestinal protease susceptibility were investigated. Its $V_{max}$ values for phenylalanine and tyrosine were 1.77 and $0.47{\mu}mol$/ min/mg protein, respectively, and its $K_m$ values were $4.77{\times}10^{-4}$ and $4.37{\times}10^{-4}\;M$, respectively. PAL showed an optimal pH at 8.5, corresponding to the average pH range of the small intestine. It showed no loss of activity at $-80^{\circ}C$ for 5 months and possessed 93.4% of its activity under $4^{\circ}C$ for 4 wks. PAL was susceptible to chymotrypsin digestion and, to a lesser extent, to trypsin, elastase, carboxypeptidase A, and B. The trypsin and chymotrypsin cleaving sites were mutated to investigate protection from pancreatic digestion and the specific activities of these mutants were evaluated. The six mutants displayed low specific activities compared to the wild-type, suggesting that the primary trypsin and chymotrypsin cleaving sites may be essential for catalytic reaction. The PAL mutants could therefore be applied as a pretreatment modality without susceptibility to proteolytic attack, however, additional modification for enhancing enzymatic activity is needed to reduce the Phe levels effectively.

• Science of Emotion and Sensibility
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• v.13 no.1
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• pp.47-60
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• 2010

Most of the researches about classification usually have used kNN(k-Nearest Neighbor), SVM(Support Vector Machine), which are known as learn-based model, and Bayesian classifier, NNA(Neural Network Algorithm), which are known as statistics-based methods. However, there are some limitations of space and time when classifying so many web pages in recent internet. Moreover, most studies of classification are using uni-gram feature representation which is not good to represent real meaning of words. In case of Korean web page classification, there are some problems because of korean words property that the words have multiple meanings(polysemy). For these reasons, LSA(Latent Semantic Analysis) is proposed to classify well in these environment(large data set and words' polysemy). LSA uses SVD(Singular Value Decomposition) which decomposes the original term-document matrix to three different matrices and reduces their dimension. From this SVD's work, it is possible to create new low-level semantic space for representing vectors, which can make classification efficient and analyze latent meaning of words or document(or web pages). Although LSA is good at classification, it has some drawbacks in classification. As SVD reduces dimensions of matrix and creates new semantic space, it doesn't consider which dimensions discriminate vectors well but it does consider which dimensions represent vectors well. It is a reason why LSA doesn't improve performance of classification as expectation. In this paper, we propose new LSA which selects optimal dimensions to discriminate and represent vectors well as minimizing drawbacks and improving performance. This method that we propose shows better and more stable performance than other LSAs' in low-dimension space. In addition, we derive more improvement in classification as creating and selecting features by reducing stopwords and weighting specific values to them statistically.

• 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.

• Journal of Chest Surgery
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• v.30 no.8
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• pp.780-785
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• 1997

Mediastinal tumor had been fascinated by its location on heart, great vessels, esophagus, and nervous tissue, its convenience of surgical treatment and superiority of its operative result. Between January 1989 and June 1995, eighty-seven patients with mediastinal tumor which were treated surgically in the Department of Thoracic and Cardiovascular Surgery, Asan Medical Center, School of Medicine, University of Ulsan. To provide the appropriate surgical management of mediastinal tumor, the demographic data, diagnostic evaluation, clinical presentation, location, size, operative finding and histopathologic distribution were reviewed and we analyzed relativity between invasiveness in chest computed tomographic finding or invasiveness on operative finding and histopathologic invasiveness. The anterosuperior mediastinum was the most commonly involved site of a mediastinal tumor(57%), followed by the posterior mediastinum(35%) and middle mediastinum(8%). The most frequently encountered tumors were thymic neoplasia(31%), followed by primary cyst(22%), neurogenic tumor(22%) and teratoma(10%) in decreasing order of frequency. Histopathologically invasive tumors were present in 17 patients(20%) and its site included anterosuperior mediastinum(16%) and posterior mediastinum(4%). All patients in this study underwent chest CT. In chest CT's finding, 15 patients(17%) showed invasiveness. A total excision of the tumor was performed 80 patients(92%), subtotal excision 6 patients(7%) and biopsy only 1 patient(2%). In operative finding, 14 patients(16%) were suspected invasiveness. The mean size of the tumor was 6.0$\pm$ 3.2cm. In anterosuperior mediastinum, the mean size was 6.2$\pm$3.1cm, in middle mediastinum, it was 3.9$\pm$1.1cm, in posterior mediastinum, it was 5.8$\pm$2.6cm. In malignant tumors, the mean size was 7.3$\pm$4.6cm, in benign tumor, it was 5.5$\pm$2.6cm(P<0.05). Relativity between histopathological invasiveness(17 patients) and invasiveness in chest CT's finding(15 patients) included sensitivity 35%, specificity 87% and predictability 35%, relativity between histopathological invasiveness(17 patients) and invasiveness on operative finding included sensitivity 52%, specificity 93% and predictability 64%. In conclusion, since it was proved that the compatibility of preoperative chest CT findings or operative findings and histopathological invasiveness is quite low, it is considered that wide excision of the mediastinal tumor except cystic lesion including adjacent tissues would yield better postoperative results.

• The Korean Journal of Nuclear Medicine
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• v.28 no.1
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• pp.75-84
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• 1994

The need for assessment of ureteric function in the patient with an obviousely dilated ureter has increased particularly with the added spectrum of asymptomatic patients presenting with hydrone-phrosis and hydroureter on antenatal and perinatal ultrasound. To assess the influence of ureteral status on kidney washout during $^{99m}Tc$-DTPA diuretic renography, ureteral images were reviewed in 80 children referred for hydronephrosis. A scintigraphically abnormal ureter was defined as an intense and continuous image of > 10 min during diuretic renography. Out of them, a total of 16 nephroureteral systems in 12 children with scintigraphically abnormal ureter were analyzed. A diuretic washout index using response half time (t1/2) by linear fitting after lasix injection, was determined on renal (Kt1/2) and ureteral (Ut1/2) curves (diuretic renogram vs. diuretic ureterogram). Diuretic ureterogram curve patterns corresponding to normal (type I), obstructive (II) and non-obstructive (III) cases were described. Compared with X-ray data, diuretic renography was highly sensitive (88%) and specific (99%) for detecting any ureteral abnormality. Despite an obstructive Kt1/2 (>20 min), no patient with an abnormal ureter underwent therapy at the ureteropelvic junction because the hydronephrosis regressed after surgery at the lower level. Our data indicate that the abnormal ureter findings during diuretic renography have to be recognized before therapy for children with hydeonephrosis.

• Radiation Oncology Journal
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• v.24 no.1
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• pp.51-57
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• 2006

Puroose: We examined whether intratumoral (i.t.) administration of dendritic cells (DCs) into a treated tumor could induce local and systemic antitumor effects in a mouse tumor model. Methods and Materials: C57BL/6 mice were inoculated s.c. in the right and left thighs with MCA-102 fibrosarcoma cells on day 0 and on day 7, respectively. On day 7, the tumors (usually 6 mm in diameter) on the right thigh were heated by immersing the tumor-bearing leg in a circulating water bath at $43^{\circ}C$ for 30 min; thereafter, the immature DCs were i.t administered to the right thigh tumors. This immunization procedure was repeated on days 7, 14 and 21. The tumors in both the right and left thighs were measured every 7 days and the average sizes were determined by applying the following formula, tumor $size=0.5{\times}(length+width)$. Cytotoxicity assay was done to determine tumor-specific cytotoxic T-lymphocyte activity. Results: Hyperthermia induced apoptosis and heat shock proteins (HSPs) in tumor occurred maximally after 6 hr. For the local treated tumor, hyperthermia (HT) alone inhibited tumor growth compared with the untreated tumors (p<0.05), and furthermore, the i.t. administered DCs combined with hyperthermia (HT + DCs) additively inhibited tumor growth compared with HT alone (p<0.05). On the distant untreated tumor, HT alone significantly inhibited tumor growth (p<0.05), and also HT + DCs potently inhibited tumor growth (p<0.001); however, compared with HT alone, the difference was not statistically significant. In addition, HT + DCs induced strong cytotoxicity of the splenocytes against tumor cells compared to DCs or HT alone. Conclusion: HT + DCs induced apoptosis and increased the expression of HSPs, and so this induced a potent local and systemic antitumor response in tumor-bearing mice. This regimen may be beneficial for the treatment of human cancers.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.9 no.1
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• pp.23-30
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• 2006

Purpose: The estimation of fluid deficit is crucial to the proper management of dehydrated children. Without well-documented serial weights on the same scale, the estimation of any given child's fluid deficit is imprecise and dependent largely on subjective clinical criteria. Despite the abundance of literature on clinical and laboratory evaluation of dehydration, few studies have focused on serum uric acid. So, we examined the usefulness of scrum uric acid in gastroenteritis patients with dehydration. Methods: Medical records of 90 gastroenteritis patients were retrospectively reviewed. By the body weight loss, we classified patients with mild, moderate, and severe dehydration groups. We studied the relevance of laboratory data (BUN, creatinine, serum bicarbonate, glucose, urine specific gravity, and uric acid) with dehydration. Results: 54 children (60%) were dehydrated mildly, 24 (26%) dehydrated and moderately, and 12 (14%) dehydrated severely. Statistically significant differences in BUN, creatinine, serum bicarbonate, glucose, and urine specific gravity could not be observed. But there was significant relationship between uric acid and the degree of dehydration. Data analysis suggested that the level of 7.0 mg/dL is the best cut-off value for predicting the development of moderate or severe dehydration. At this cut-off value, the sensitivity and specificity were 66.6% and 87.1%. Conclusion: Our study supports that the measurement of serum uric acid with traditional scale is useful for predicting the development of dehydration. But, in order 10 be used as the indicator for proper treatment at an earlier stage, further validation about serum uric acid is necessary.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.2
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• pp.43-49
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• 2018

Maple syrup urine disease (MSUD, OMIM#248600) is a rare and autosomal recessively-inherited metabolic disorder that is caused by mutations in the branched-chain ${\alpha}$-ketoacid dehydrogenase (BCKDH) genes. It prevents the normal breakdown of branched-chain amino acids (BCAAs), such as leucine, isoleucine, and valine, and leads to poor feeding, lethargy, abnormal movements, seizure, and death if untreated. Here, we report the case of a Korean newborn of biochemically- and genetically-confirmed MSUD manifesting lethargy and central apnea, the acute state of which was successfully treated. The molecular genetic investigation revealed two novel heterozygous mutations (p.Ala32Phefs*48 and p.Val 130Phe) in BCKDHB, and both parents were confirmed as carriers. We emphasize the importance of early diagnosis and prompt introduction of specific treatment for MSUD in life saving and prognosis.