• Journal of Genetic Medicine
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• v.18 no.2
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• pp.142-146
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• 2021

Alagille syndrome (AGS) is a rare autosomal dominant inherited disorder, with major clinical manifestations of bile duct paucity, cholestasis, cardiovascular anomaly, ophthalmic abnormalities, butterfly vertebrae, and dysmorphic facial appearance. It is caused by heterozygous mutations in JAG1 or NOTCH of the Notch signaling pathway presenting with variable phenotypic penetrance and involving multiple organ systems. The following case report describes a unique case of a 16-year-old female with AGS who presented with the primary complaint of renovascular hypertension. She had a medical history of ventricular septal defect and polycystic ovary syndrome. The patient had a dysmorphic facial appearance including frontal bossing, bulbous tip of the nose, a pointed chin with prognathism, and deeply set eyes with mild hypertelorism. Stenoocclusive changes of both renal arteries, celiac artery, lower part of the abdominal aorta, and left intracranial artery, along with absence of the left internal carotid artery were found on examination. Whole exome sequencing was performed and revealed a pathologic mutation of JAG1, leading to the diagnosis of AGS. Reverse phenotyping detected butterfly vertebrae and normal structure and function of the liver and gallbladder. While the representative symptom of AGS in most scenarios is a hepatic problem, in this case, the presenting clinical features were the vascular anomalies. Clinical manifestations of AGS are diverse, and this case demonstrates that renovascular hypertension might be in some cases a presenting symptom of AGS.

• Journal of Information Technology Applications and Management
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• v.29 no.1
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• pp.47-57
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• 2022

The purpose of this study is to analyze the current status of augmented reality picture books, which have been steadily developed since 2010, as a genre of electronic picture books, and to reveal how children's immersion in augmented reality picture books differs from paper picture books. To this end, 30 augmented reality picture books on the market were analyzed according to genre, life theme, implementation method, and augmented reality scene ratio. As a result of the study, it was found that the genre of picture books was in the order of information fairy tales, daily fairy tales, and historical fairy tales, and there were no traditional or fantasy fairy tales. Animals and plants accounted for about half of the life topics, and in some cases, there were only a few or no other life topics. In the augmented reality implementation scene, it consisted of only one page in the early days, but all pages are now possible to implement augmented reality due to technology development, production cost reduction, and improved hardware performance of smartphones. It was found that the augmented reality implementation method used CD-ROM in the early days, but gradually became possible to implement using only mobile phones and tablets that were easy for readers to access. In addition, after presenting four picture books to eight 5-year-old infants, the immersion time was measured and the immersion behavior was observed. As a result, augmented reality picture books showed higher immersion[immersion time, immersion behavior] than paper picture books, but compared by literature genre, life fairy tales were higher in paper picture books and natural fairy tales in augmented reality picture books. It was higher when presenting augmented reality picture books after presenting paper picture books according to the order of presentation of picture book types. The results of this study suggest that more diverse life topics and augmented reality picture books in the genre of children's literature should be developed to increase the utilization of augmented reality picture books. In addition, considering that there are differences in immersion between types, literary genres, and reading experience [presentation order], it is expected to increase the educational effect by using picture books complementarily.

• Journal of the Korean Society of Radiology
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• v.81 no.3
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• pp.707-713
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• 2020

Tumor thrombus in the portal vein without any liver parenchymal abnormality is extremely rare. In the liver, the primary tumor most frequently presenting with intravascular tumor thrombi is hepatocellular carcinoma and lymphoma is rarely considered. Even though thrombosis occurs quite often in lymphoma, cases of tumor thrombus are rare and cases of tumor thrombus in the portal vein are even rarer. Only four cases of lymphoma with portal vein tumor thrombosis have been reported to date and all cases were the result of direct extensions of a dominant nodal or extra-nodal mass. To our knowledge, there has been no report on diffuse large B-cell lymphoma (DLBCL) presenting only within the lumen of the portal vein and not intravascular B-cell lymphoma. We present the first case of DLBCL presenting only within the lumen of the portal vein in an immunocompetent patient.

• The Journal of the Institute of Internet, Broadcasting and Communication
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• v.13 no.3
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• pp.79-86
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• 2013

Departments of family medicine at some general hospitals find it difficult to hold regular conferences because they have a small number of faculty members. The recent technological development of videoconferencing has made it possible for these small departments to regularly participate in the conferences held by departments with a larger number of faculty members. The objective of this study was to show the level of satisfaction with and acceptance of our videoconferencing system. One presenting site and four receiving sites participated in the videoconferences, which were held every Monday for three weeks. There were no significant differences in the levels of satisfaction with the videoconferences between faculty at the presenting site and faculty at the receiving sites. In contrast, residents at the receiving sites rated most items related to satisfaction with the videoconferences lower than residents at the presenting site. There were no changes between the pre-test and post-test scores of residents both at the presenting site and at the receiving sites. Despite its limitations, the advantages of joint-videoconferencing among several departments of family medicine were comfort level, time-saving, increasing the number of participants, promoting discussion, and provoking thought.

• Clinical and Experimental Pediatrics
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• v.52 no.6
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• pp.713-716
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• 2009

Facial palsy as the presenting symptom of leukemia is very rare, especially in acute myeloid leukemia. A review of the medical literature identified reports on 8 children with AML who had facial paralysis as the presenting sign. Whole brain irradiation (WBI) has been applied in most cases. We present the cases of 3 such children. Achieving a remission without WBI, the patients underwent stem cell transplantations (SCTs). Two patients remain event-free 52 months and 62 months after allotransplants. Facial palsy was the harbinger of leukemic relapse in one case after autotransplant. This patient is disease-free 59 months after unrelated SCT rescue. Facial palsy persisted in 2 cases. Allogeneic SCT without WBI may be an effective therapy in patients presenting with facial palsy. A brief review of the literature is presented here.

• Science of Emotion and Sensibility
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• v.10 no.1
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• pp.127-133
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• 2007

The present study investigated whether the emotional states reading ability through facial expression changes by age(3-, 5-year-old and university student groups), sex(male, female), facial expression's presenting areas(face, eyes) and the type of emotions(basic emotions, complex emotions). 32 types of emotional state's facial expressions which are linked relatively strong with the emotional vocabularies were used as stimuli. Stimuli were collected by taking photographs of professional actors facial expression performance. Each individuals were presented with stories which set off certain emotions, and then were asked to choose a facial expression that the principal character would have made for the occasion presented in stories. The result showed that the ability of facial expression reading improves as the age get higher. Also, they performed better with the condition of face than eyes, and basic emotions than complex emotions. While female doesn't show any performance difference with the presenting areas, male shows better performance in case of facial condition compared with eye condition. The results demonstrate that age, facial expression's presenting areas and the type of emotions effect on estimation of other people's emotion through facial expressions.

• Korean Journal of Radiology
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• v.19 no.6
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• pp.1089-1098
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• 2018

Objective: To investigate the diagnostic yield of contrast-enhanced computed tomography (CT) in Crohn's disease (CD) patients presenting with acute severe lower gastrointestinal bleeding (LGIB), and the role of CT in predicting the risk of rebleeding. Materials and Methods: A consecutive series of 110 CD patients presenting with acute severe LGIB between 2005 and 2016 were analyzed. Among them, 86 patients who had undergone contrast-enhanced CT constituted the study cohort. The diagnostic yield of CT for detecting contrast extravasation was obtained for the entire cohort and compared between different CT techniques. In a subgroup of 62 patients who had undergone CT enterography (CTE) and showed a negative result for extravasation on CTE, the association between various clinical and CTE parameters and the risk of rebleeding during subsequent follow-up was investigated using Cox regression analysis. Results: The diagnostic yield of CT was 10.5% (9 of 86 patients). The yield did not significantly differ between single-phase and multiphase examinations (p > 0.999), or between non-enterographic CT and CTE (p = 0.388). Extensive CD (adjusted hazard ratio [HR], 3.27; 95% confidence interval [CI], 1.09-9.80; p = 0.034) and bowel wall-to-artery enhancement ratio (adjusted HR, 2.81; 95% CI, 1.21-6.54; p = 0.016) were significantly independently associated with increased rebleeding risks, whereas anti-tumor necrosis factor-${\alpha}$ therapy after the bleeding independently decreased the risk of rebleeding (adjusted HR, 0.26; 95% CI, 0.07-0.95; p = 0.041). Conclusion: The diagnostic yield of contrast-enhanced CT was not high in CD patients presenting with acute severe LGIB. Nevertheless, even a negative CTE may be beneficial as it can help predict the risk of later rebleeding.

• 대한관절경학회:학술대회논문집
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• 2007.10a
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• pp.99-100
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• 2007

• Proceedings of the IEEK Conference
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• 2000.06c
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• pp.43-46
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• 2000

In this paper, we propose the method to express various factors for software architecture modeling effectively using UML extension mechanism. Furthermore, we show usefulness of proposed method by presenting several modeling techniques and their examples.

• Proceedings of the Korean Nuclear Society Conference
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• 2004.10a
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• pp.1200-1201
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• 2004