• Childhood Kidney Diseases
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• v.9 no.2
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• pp.137-142
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• 2005

Purpose : Acute poststreptococcal glomerulonephritis(APSGN) follows infection of group A$\beta$-hemolytic streptococci. The prognosis of APSGN has been reported as favorable. However, several studies have reported that some patients progress to chronic renal failure. In an attempt to clarify this, we analyzed the clinical course of patients with APSGN. Methods : Between January 2000 and December 2004, a total of 48 children who were diagnosed with APSGN according to the presence of hematuria, transient hypocomplementemia and evidence of group A $\beta$-hemolytic streptococcal infection were evaluated. Results : Six(12.5$\%$) patients showed elevation of serum creatinine level but there was no patient with Persistent renal dysfunction. Blood pressure was controlled with ease in all patients and there was no case of persistent hypertension. Renal biopsy was done in 5 patients who showed heavy proteinuria or renal insufficiency and the outcomes showed findings consistent with ordinary APSGN except one with findings of rapidly progressive glomerulonephritis(RPGN). Serum complement levels normalized within 8 weeks(92.9$\%$). Hematuria disappeared within 6 months(79$\%$) and proteinuria within 6 months(100$\%$) from the disease onset. Conclusion : Prolonged renal dysfunction or heavy proteinuria found in five patients(10.4$\%$) led to renal biopsy. All these problems resolved within 6 months. Our data support that the prognosis of childhood APSGN is favorable without any serious sequoia. (J Korean Soc Pediatr Nephrol 2005;9:137-142)

• Tuberculosis and Respiratory Diseases
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• v.52 no.4
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• pp.376-384
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• 2002

Background : The mortality from acute respiratory distress syndrome(ARDS) in the late stage, which is characterized by progressive pulmonary fibroproliferation, is ${\geq}80%$. Although previous prospective trials failed to show a survival benefit of steroid therapy in early ARDS, recently, a few of reports have described the survival benefit of the long-term use of steroid in patients with late ARDS. In this study, we analyzed the effect of steroid therapy on patients with late ARDS retrospectively in a single. Medral intensive care unit (MICU). Methods : Over a 3-year period, the medical records of 48 ARDS patients who had been on mechanical ventilation more than 8 days were reviewed. 14 patients were treated by the long-term use of methylprednisolone and another 34 patients served as a control. Both groups were comparable regarding clinical and physiologic data lung injury score(LIS), multiple organ failure score, APACHE III and SAPS II score. Because steroid was instituted after 8 days of advanced mechanical ventilatory support in average, we arbitrarily defined the $8^{th}$ day of ARDS as first day of the study. Results : Initially, the groups had similar PF($PaO_2/FiO_2$) ratio, LIS, APACHE III and SAPS II score. By 7th day after the start of steroid therapy, there were significant improvements in PF ratio, LIS, APACHE III and SAPS II score. The mortality in the steroid treated group was significantly lower(42.9% vs 73.5%, p<0.05). Conclusion : Although the data of this study was retrospective and was not randomized, in order to improve the patient's outcomes, steroid therapy should be considered in late ARDS patients. However, prospective trials are needed to define the indication and the effect of steroid therapy in late ARDS.

• Asia-Pacific Journal of Business Venturing and Entrepreneurship
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• v.10 no.1
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• pp.129-141
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• 2015

The mentoring program, conducted as part of an entrepreneurship education program by the Business Incubator, has been acknowledged to help entrepreneurs feel less anxious and fearful of embarking on new business endeavors and the possibility of failure and gain a positive view toward starting new businesses. For this reason, the investment into and the contents of the program have been reinforced. Accordingly, this study aims to examine the effects of mentoring functions on the improvement of the entrepreneurial mindset of young entrepreneurs and verify the moderating effect of the start-up preparation period between the mentoring functions and start-up competence as a means to analyze the interaction effect of the business start-up preparation period between the mentoring functions and entrepreneurship. For the purpose of this study, the SPSS 18.0 statistical program was used to perform validity, reliability and hierarchical factor analyses of the survey data obtained from 156 subjects, who took part in the entrepreneurship mentoring program. The results of the analyses showed that the mentoring functions had a positive impact on the progressiveness and risk-taking aspects of entrepreneurship and a negative impact on innovativeness. On the other hand, the business start-up preparation period had a significant moderating effect between the mentoring functions and the progressiveness and risk-taking aspects. Based on these results, it was deemed that while the mentoring functions efficiently improved the progressive and risk-taking characteristics of potential entrepreneurs, they were not efficient in improving their innovativeness. Also, the application of the entrepreneurship mentoring program according to the start-up preparation period had an interaction effect in which the progressiveness and risking-taking aspects of entrepreneurship were improved. Based on these findings, a proposal was made in regard to the development of a tailored mentoring program that reflects the various characteristics of the entrepreneurs and their start-up preparation periods as a means to improve their entrepreneurial mindset.

• Clinical and Experimental Pediatrics
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• v.49 no.3
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• pp.292-297
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• 2006

Purpose : In medulloblastoma, craniospinal radiation therapy combined with chemotherapy improves the prognosis of tumors but results in significant endocrine morbidities. We studied the endocrine morbidity, especially growth pattern changes. Methods : The medical records of 37 patients with medulloblastoma were reviewed retrospectively for evaluation of endocrine function and growth. We performed the growth hormone stimulation test in 16 patients whose growth velocity was lower than 4 cm/yr. Results : The height loss was progressive in most patients. The height standard deviation score (SDS) decreased from $-0.1{\pm}1.3$ initially to $-0.6{\pm}1.0$ after 1 year(P<0.01). Growth hormone deficiency(GHD) developed in 14 patients. During the 2 years of growth hormone(GH) treatment, the improvements of height gain or progressions of height loss were not observed. Twelve patients(32.4 percent) revealed primary hypothyroidism. One of six patients diagnosed with compensated hypothyroidism progressed to primary hypothyroidism. Primary and hypergonadotropic hypogonadism were observed in two and one patients respectively. There was no proven case of central adrenal insufficiency. Conclusion : Growth impairment developed frequently, irrespective of the presence of GHD in childhood survivors of medulloblastoma. GH treatment may prevent further loss of height. The impairment of the hypothalamic-pituitary-gonadal and hypothalamic-pituitary-thyroidal axis is less common, while central adrenal insufficiency was not observed.

• Tuberculosis and Respiratory Diseases
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• v.40 no.5
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• pp.519-531
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• 1993

Background: Lymphangioleiomyomatosis(LAM) is a very rare disease occurring in women of reproductive age and leading to progressive respiratory failure despite therapy. But the natural history of this disease is uncertain and although anti-estrogenic agents have been used for more than twenty years, it's efficacy is still in debate. This study was performed to enhance understanding of this fatal disease in Korea by examining clinical, radiological, and pathologic findings of all the previously reported cases of LAM on Korea along with four new cases of LAM whom we report in this paper. Method: Out of twlve cases of LAM previously unpublished and published in domestic papers, two cases whose diagnoses were considered doubtful after review of clinical, radiological, and pathologic findings at "Asian Congress on Lymphangioleiomyomatosis" at Kyoto, Japan in feburary of 1993 were excluded from this study. Six cases which were reported previously and four new cases of LAM whom we report in this paper were analysed for the clinical, radiological, and pathologic characteristics. Results: All ten patients were women with mean age of $33{\pm}7$. The most common symptom was exertional dyspnea and most patients had history of pneumothoraces. Pulmonary function tests showed decreased diffusing capacity. on high resolution computed tomography(HRCT), all the cases had characteristic cysts. Most of the patients did not respond to hormonal therapy. Conclusion: In women of reproductive age, presenting with dyspnea who has a history of pneumothorax, LAM should always be considered as one of the diagnostic possibilities. If suspected, HRCT should be done to look for characteristic cysts and if needed, open lung biopsy should be done to confirm the diagnosis.

• Journal of Chest Surgery
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• v.36 no.11
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• pp.812-819
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• 2003

Background: Left ventricular dysfunction is one of the important prognostic factors of early mortality and long-term survival after valve operation. We studied the intermediate term results of mitral valve reconstruction in patients with moderate to severe left ventricular dysfunction. Material and Method: Forty four patients who underwent mitral valve reconstruction with a left ventricular ejection fraction (EF) of <45% or less (20∼45%) from April 1995 through July 2001 were reviewed retrospectively. Ages ranged from 10 to 67 years (46∼14 years) and 32 patients were in NYHA class III-IV. The mitral valve diseases were regurgitation (MR) in 28 patients, stenosis(MS) in 10, and mixed lesion in 5. The etiologies of mitral valve disease were rheumatic in 20 patients, degenerative in 14, ischemic in 5, annular dilatation in 2, congenital in 2, and endocarditis in 1. Operatively, all patients had annuloplasty and/or various valvuloplasty techniques, and a total of 52 procedures were concomitantly performed. Total cardiopulmonary bypass and aortic crossclamp time were 160$\pm$57 minutes and 112$\pm$45 minutes respectively. Result: Two operative deaths occurred as a result of left ventricular failure (4.5%). After the mean follow-up of 39 months (range, 10∼83 months), there was no late death. Transthoracic echocardiography revealed no or grade I of MR in 29 patients (72.5%) and no or mild MS in 35 patients (87.5%). The actuarial survival at 5 years was 100%. Four patients required mitral valve replacement due to progressive mitral valvular disease. The actuarial freedom from valve-related reoperation at 5 years was 84$\pm$9%. Conclusion: This study suggests that mitral valve reconstruction in patients with moderate to severe left ventricular dysfunction offers good early and intermediate survival and acceptable freedom from valve-related reoperation, and it is the strategy for effective management for these patients.

• Tuberculosis and Respiratory Diseases
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• v.41 no.3
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• pp.222-230
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• 1994

Background: Paraquat, a widely used herbicide, is extremely toxic, causing multiple organ failure in humans. Paraquat especially leads to irreversible progressive pulmonary fibrosis, which is related to oxygen free radicals. However, its biochemical mechanism is not clear. Natural mechanisms that prevent damage from oxygen free radicals include changes in glutathione level, G6PDH, superoxide dismutase(SOD), catalase, and glutathione peroxidase. The authors think catalase is closely related to paraquat toxicity in the lungs Method: The effects of 3-amino-1,2,4-triazole(aminotriazole), a catalase inhibitor, on mice administered with paraquat were investigated. We studied the effects of aminotriazole on the survival of mice administered with paraquat, by comparing life spans between the group to which paraquat had been administered and the group to which a combination of paraquat and aminotriazole had been administered. We measured glutathion level, glucose 6-phosphate dehydrogenase(G6PDH), superoxide dismutase(SOD), catalase, and glutathione peroxidase(GPx) in the lung tissue of 4 groups of mice: the control group, group A(aminotriazole injected), group B(paraquat administered), group C(paraquat and aminotriazole administered). Results: The mortality of mice administered with paraquat which were treated with aminotriazole was significantly increased compared with those of mice not treated with aminotriazole. Glutathione level in group B was decreased by 20%, a significant decrease compared with the control group. However, this level was not changed by the administration of aminotriazole(group C). The activity of G6PDH in all groups was not significantly changed compared with the control group. The activities of SOD, catalase, and glutathione peroxidase(GPx) in the lung tissue were significantly decreased by paraquat administration(group B); catalase showed the largest decrease. Catalase and GPX were significantly decreased by aminotriazole treatment in mice administered with paraquat but change in SOD activity was not significant(group C). Conclusion: Decrease in catalase activity by paraquat suggests that paraquat toxicity in the lungs is closely related to catalase activity. Paraquat toxicity in mice is enhanced by aminotriazole administration, and its result is related to the decrease of catalase activity rather than glutathione level in the lungs. Production of hydroxyl radicals, the most reactive oxygen metabolite, is accelerated due to increased hydrogen peroxide by catalase inhibition and the lung damage probably results from nonspecific tissue injury of hydroxyl radicals.

• Childhood Kidney Diseases
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• v.14 no.1
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• pp.42-50
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• 2010

Purpose : The clinical characteristics and associated anomalies in children with solitary kidney (SK) were analyzed retrospectively. Methods : Total 38 children diagnosed to have SK at our hospital between December 1989 and December 2009 were recruited, and the clinical records including imaging studies were retrospectively reviewed. SK was defined as unilateral renal agenesis by imaging studies only, and patients with regression of unilateral dysplastic kidney were excluded. Results : Among total 38 patients, 12 were male. The median age at the diagnosis of SK was 6.5 months (at birth-13 years). SK was detected by prenatal ultrasonography in 14 patients and during work-up for renal or urinary tract diseases in 13 (including urinary tract infection in 7). In 10 patients, SK was detected incidentally. Anomalies in the SK were noted in 17 patients including vesicoureteral reflux in 11. Other anomalies in the genitourinary tract were present in 16 patients, and multi-organ-involving syndromes or chromosomal anomalies were detected in 9. The mean duration of follow-up was 9 years (9 months-20 years). Two patients developed chronic renal failure during follow-up, and the median serum creatinine concentration of the remaining 36 at their last follow-up was 0.6 mg/dL. Conclusion : SK may be isolated and clinically asymptomatic; it is frequently accompanied by other anomalies in genitourinary tract and other organs, some of which can induce progressive renal dysfunction. Early recognition of associated anomalies with SK and regular follow-up is recommended to reduce long-term risk.

• Journal of Genetic Medicine
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• v.4 no.1
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• pp.72-79
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• 2007

Purpose : Glycogen storage disease type III (GSD-III) is a rare autosomal recessive disorder of glycogen metabolism. The affected enzyme, amylo-1,6-glucosidase, 4-alpha-glucanotransferase (AGL, glycogen debranching enzyme), is responsible for the debranching of the glycogen molecule during catabolism. The disease shows clinical and biochemical heterogeneity, reflecting genotype-phenotype heterogeneity among different patients. In this study, we aim at analyzing mutations of the AGL gene in three unrelated Korean GSD-III patients, and characterizing their clinical and laboratory findings. Methods : We characterized the clinical features of three unrelated Korean GSD-III patients by biochemical, histological and imaging studies. The 35 exons and part of exon-intron boundaries of AGL were analyzed by direct sequencing using genomic DNA extracted from the peripheral leukocytes of patients. Results : Diverse clinical features were observed in these patients including hepatomegaly (all patients), seizures (patient 2), grow th failure (patients 1 and 2), hyperlipidemia (patients 1 and 3), raised transaminase and creatine kinase concentrations (all patients), and mild cardiomyopathy (patient 2). Liver transplantation w as performed in patient 2 due to progressive hepatic fibrosis. A dministration of uncooked corn starch maintained normoglycemia and improved biochemical and growth profiles. DNA sequence analysis revealed mutations in 5 out of 6 alleles. Patient 1 was a compound heterozygote of c.1282 G>A (p.R428K) and c.1306delA (p.S603PfsX6), patient 2 had c.1510_1511insT (p.Y 504L fsX 10), and patient 3 had c.3416 T >C (p.L 1139P) and c.1735+1 G>T (p.Y 538_R578delfsX 4) mutations. A part from the p.R428K mutation, the 4 other substitutions identified w ere nov el. Conclusion : GSD-III patients display variable phenotypic characteristics resembling those of GSD-Ia. Molecular defects in the AGL gene of Korean GSD-III patients are genetically heterogeneous.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.3
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• pp.87-94
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• 2018

We present the case of long-term observation of a patient with chronic kidney disease (CKD) caused by advanced focal segmental glomerulosclerosis (FSGS) resulting from underlying congenital chloride diarrhea (CLD). A 20-year-old woman was admitted for prolonged proteinuria despite conservative treatment for CLD. She was diagnosed with CLD and started taking KCl salt supplementation from the time of birth. Mild proteinuria was first found at 12 years of age, which progressed to moderate proteinuria at 16 years of age. At 16 years of age, CKD stage 2 with FSGS was diagnosed based on the initial assessment of the glomerular filtration rate (GFR) and kidney histology. On admission, we re-assessed her renal function, histology and genetic analysis. GFR had deteriorated to CKD stage 4 and renal histology revealed an advanced FSGS combined with tubulointerstitial fibrosis. A homozygous mutation in the SLC26A3 gene (c.2063-1G>T) was found by diagnostic exome sequencing and may have been inherited from both parents. CLD patients can be more vulnerable to renal injury, which may also cause progression of renal failure. Therefore, even if there is an early diagnosis and adequate salt supplementation, close monitoring of renal function and tailored treatment should be emphasized for renal protection and favorable CLD prognosis.