• Childhood Kidney Diseases
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• v.8 no.2
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• pp.176-185
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• 2004

Purpose: Rapidly progressive glomerulonephritis (RPGN) is a clinicopathologic entity characterized by extensive crescent formation and rapid deterioration of renal function within few months. For better understanding of its clinical course and designing better treatment strategies, a clinicopathological study of childhood RPGN was performed. Methods: The clinical manifestations and pathological findings were reviewed retrospectively in 12 children who were diagnosed as having RPGN by clinical manifestations and renal biopsy during a period from 1991 to 2003. Several clinicopathological parameters were analyzed as prognostic factors. Results: Among a total of 12 patients, 4 were male and 8 were female. The median onset age was 11.5 years(range 5.5-14.6 years), and the median period of follow-up was 25 months(range 7 months-6.6 years). According to the pathological classification, 10 patients (83%) were type II RPGN(immune-complex mediated glomerulonephritis), 2 patients were type III RPGN(pauci-immune glomerulonephritis), and none was type I RPGN(anti-glomerular basement membrane nephritis). All patients were treated with oral steroid in various combinations with methylprednisolone pulse therapy(10 patients, 83%), cyclophosphamide(8 patients, 67%), or plasmapheresis(4 patients, 33%). Clinical outcomes of 12 patients were complete remission in 1(8%), end-stage renal disease in 2(17%), chronic renal insufficiency with persistent proteinuria in 2(17%), and normal renal function with persistent proteinuria in 7(58%) at the last follow-up. Poor prognosis is associated with increased serum creatinine level, severe anemia and younger age at the time of diagnosis. Conclusion: Immune-complex mediated glomerulonephritis is the major cause RPGN in children and most cases showed improvement of renal function with aggressive management. For better understanding of this rare disease, a prospective multicenter study should be done.

• Clinical and Experimental Pediatrics
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• v.45 no.12
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• pp.1528-1533
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• 2002

Purpose : Isolated noncompaction of the ventricular myocardium(INVM) is one of the unclassified cardiomyopathies that is characterized by numerous, excessively prominent trabeculations, and deep intertrabecular recesses. We performed this study to evaluate the clinical features of INVM in children. Methods : The medical records of 10 patients with INVM were reviewed. We analyzed the clinical manifestations, hemodynamics, pattern of inheritance, and long-term prognosis of INVM in children. Results : Age at diagnosis was $45{\pm}53months$(1 day-14 years) with follow-up lasting as long as 78 months. Most INVM was asymptomatic on diagnosis. Associated cardiac anomalies were noted in six patients(ventricualr or atrial septal defect, patent ductus arteriosus with mitral valve prolapse, or mitral valve cleft). Depressed or flat changes of T wave in lead II, III and aVF were observed on electrocardiography. Various arrhythmia including WPW syndrome with paroxysmal supraventricular tachycardia, third-degree atrioventricular block, and familial sick sinus node dysfuction were observed. The degree of trabeculation in INVM was significantly prominent from level of mitral valve to apex compared to age-matched control. Familial recurrences were noted in two patients. The systolic function of the left ventricle was decreased in 20% of patients during the follow-up period, but systemic embolism or ventricular tachycardia was not observed. Conclusion : INVM is not a rare disorder. The cardiac function may be deteriorated in children as well as adults during long-term follow up. Thus early diagnosis and long-term follow-up must be done. So, the nation-wide multicenter clinical study would be mandatory to evaluate the incidence, long-term prognosis, and establishment of objective diagnostic criteria of INVM.

• Management & Information Systems Review
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• v.38 no.1
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• pp.23-41
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• 2019

Smart Factory is different from existing factory automation in that it aims to produce personalized products with minimum time and cost through ICT. However, previous researches, not from consumers but from product suppliers, have focused on technology trends and technology application methods. In order for Smart Factory to be successful, it must go beyond supplier-focus to meet the needs of consumers. In this study, we surveyed the purchase intention of the personalized product manufactured by smart factory. Influencing factors of purchase intention were drawn as consumers' need for uniqueness, innovativeness, need for touch, and privacy concern, based on previous research. As results of data analysis, it was confirmed that respondents were willing to purchase personalized products, and that consumers' need for uniqueness, innovativeness, and need for touch had a significant impact on purchase intention of personalized products. Our findings can be summarized as follows. First, Consumers' need for uniqueness was found to have positive effects(${\beta}=0.168$) on purchase intention of personalized products. The desire to differentiate themselves from others will be reflected in their personalized products. Therefore, consumers with a higher desire for uniqueness tend to be more willing to purchase personalized products. Second, consumer innovativeness was found to have positive effects(${\beta}=0.233$) on purchase intention of personalized products. Personalized shoes suggested in this study is a new type of personalized product that is manufactured by the latest information and communication technologies such as multi-function robots and 3D printing. Therefore, consumers seeking innovative new experiences are more willing to purchase personalized products. Third, need for touch was found to have positive effects(${\beta}=0.299$) on purchase intention of personalized products. In a smart factory environment, prosuming participation is given to consumers. If consumers participate in the product development process and reflect their requirements on the product, they are expected to increase their purchase intention by virtually satisfying the need for touch. Fourth, privacy concern was found to have no significantly related to purchase intention of personalized products. This is interpreted as a willingness to tolerate the risk of exposing personal information such as home address, telephone number, body size, and preference for consumers who feel highly useful in personalized products.