• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.3
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• pp.92-95
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• 2017

Fabry disease (FD) is an X-linked lysosomal storage disorder caused by an ${\alpha}$-galactosidase A (GLA, MIM 300644) enzyme deficiency due to pathogenic variants in the ${\alpha}$-galactosidase A gene (GLA). The disease leads to accumulation of globotriaosylceramide (Gb3) and related glycophospholipids affecting nearly all major organ systems, with the primary sites damaged by Gb3 including renal glomeruli, myocardium, neurons of the dorsal ganglion and autonomic nervous system, and vascular endothelial and smooth muscle. Progressive deposition in these organ systems present with various clinical manifestations including acroparesthesia, renal failure and heart failure. Here, we report a Chinese male diagnosed with Fabry disease in his late $4^{th}$ decades showing improvement of acroparesthesia during enzyme replacement therapy (ERT). A 48-year-old Chinese man who presented with chronic recurrent severe burning pain in his fingers and toes since the age of 10, with worse involvement of the former visited to our clinic for further evaluation. His medical history included a transient ischemic attack aged 40 and diagnosed with stage 4-5 chronic kidney disease aged 47. In the family history, the patient's brother was found to be have Fabry disease 1 month before his visit. Except for his brother, all other members of the family are healthy. Based on his medical history and family history, he was strongly suspicious for Fabry disease. He was found to have a galactose-alpha-1,3-galactose level 4.96 (Reference range, 42.5-67.9) suggestive of Fabry disease. The followed sequencing of GLA coding region in our patient revealed hemizyosity for the mutation c.988C>T (Q330X) in Exon 7. Since ERT start, he showed significant improvement in his symptoms of burning sensation of fingers and toes. On the contrary, due to deteriorating kidney function even with ERT, he is considered for kidney transplantation. Despite of diagnostic delay until late 4th decades, ERT showed a potential improvement of acroparesthesia in our patient. However, late start of ERT can lead to poor outcome in multiorgan function. Therefore, early diagnosis with high index of suspicion followed by continuous ERT with regular monitoring have an impact on quality of life in Fabry disease.

• Journal of the Korean Arthroscopy Society
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• v.10 no.2
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• pp.165-172
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• 2006

Purpose: The purpose of this study is to evaluate the effects of intratunnel fixation in the tibial side on the arthroscopic ACL reconstruction with quadruple hamstring tendon at the second look arthroscopy. Materials and Method: From Dec 1999 to May 2005, we arthroscopically reexamined 32 cases who had been done arthroscopic ACL reconstruction with quadruple hamstring tendons. Hamstring tendons of all cases were fixed at femoral side with RigidfixTM. At the tibial side hamstring tendons were fixed only Post-tie (Group I) or Post-tie combined with IntrafixTM (Group II). At the time of second look arthroscopy mean age of cases was 30 years and mean duration for second look arthroscopy was 21.3 months. We analyzed the results with IKDC score, KT-1000 arthrometer under anesthesia, Telos stress radiography, tibial tunnel widening on the radiography and second look arthroscopic findings. Results: Group II had more superior than group I at side to side differences with KT-1000 and Telos stress radiograph, IKDC score, but the differences were insignificant. At arthroscopic evaluation, Group ll also had more superior than group I at graft tension and graft appearance, graft synovialization, but the differences were insignificant. Tibial tunnel widening in the knee AP radiograph was 2.3 mm in Group I and 1.7 mm in Group II and the difference was significant. (P=0.042) Conclusions: Additional procedure of tibial intratunnel fixation in arthroscopic ACL reconstruction with autogenous hamstring tendon significantly prohibited from tibial tunnel widening but clinical results, radiologic joint stability, findings in second look arthroscopy were insignificantly different. We concluded that Post-tie itself induced satisfactory clinical results, joint stability and graft maturation and that tibial tunnel widening did not affect the results.

• Clinical and Experimental Pediatrics
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• v.52 no.3
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• pp.303-309
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• 2009

Purpose : To identify the short-term clinical outcomes of late preterm infants and to test the hypothesis that late preterm infants have more clinical problems during the early postnatal period than term infants. Methods : One hundred late preterm infants [gestational age (GA) $34^{+0}$-$36^{+6}$ weeks] and the same number of term infants (GA $37^{+0}$-$41^{+6}$ weeks) were randomly selected from 289 late preterm infants and 825 term infants born in the Seoul National University Bundang Hospital between January 2007 and December 2007, and their electronic medical records were reviewed and analyzed. Results : Compared to term infants, late preterm infants had significantly more medical problems such as temperature instability (odds ratio [OR] 8.7), hypoglycemia (OR 17.5), intravenous fluid infusion (OR 10.2), evaluation for sepsis (OR 9.4), respiratory problems (OR 7.5), apnea and bradycardia (OR 8.6), phototherapy for jaundice (OR 3.6), and feeding intolerance (OR 10.0). Hospital stay was also significantly longer in late preterm infants. Conclusion : Late preterm infants had significantly more medical problems and increased length of hospital stay compared to term infants. More attention should be given to caring for these late preterm infants in newborn nursery during the early postnatal period.

• Journal of Gastric Cancer
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• v.5 no.3 s.19
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• pp.169-173
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• 2005

Background: Gastric polyps encompass a wide variety of lesions that most commonly arise from the gastric epithelium. However, coincidental gastric carcinomas have rarely been reported, being found in $1.5{\sim}2.1%$ of patients with hyperplastic polyps. The sizes and the pathologies of polyps seem to be important in the application of treatment. Therefore, it is necessary to classily gastric polypoid lesions after a gastrectomy. Materials and Methods: During a follow-up endoscopy study, 23 patients were found to have developed gastric polyps after a gastrectomy. Most of those polyps were removed by using an endoscopic polypectomy. We performed clinical and pathologic evaluations of the gastric polyps in the remainding in the stomach after a gastrectomy, Results: The mean age of the patients was 64.5 years old with the incidence of polyps remainding in the stomach after a gastrectomy increasing after the first year following the gastrectomy. The sizes of the polyps ranged from 0.3cm to 3.5cm in diameter and the numbers of polyps below 1.0cm were 19 (82.6%). The anastomotic site was the most prevalent place 10 (43.2%), followed by the cardia 6 (26.0%) and the body 4 (17.3%). Among 23 gastric polypoid lesions Yamada types of gastric polyps in the remainding in the stomach were as follows: 1 case in type I, 12 cases in type II, 9 cases in type III, 1 case in type IV. The pathologic diagnoses of the polyps were hyperplastic polyps in 6 cases, tubular adenomas in 2 cases and inflammatory polyps in 15 cases. Conclusion: Endoscopic polypectomy is believed to be important in assessing the precise diagnosis of gastric polyps remainding in the stomach. In this study, hyperplastic polyps were found to have no malignant potential, despite their sizes. As a result aggressive biopsy with a polypectomy of gastric polyp afier gastrectomy is recommended and frequent follow-up be performed.

• Journal of Chest Surgery
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• v.32 no.9
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• pp.790-798
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• 1999

Background: As the early outcome after coronary artery bypass grafting(CABG) has been stabilized, neurologic complication has now become one of the most important morbidity. The aim of this study was to find out the risk factors associated with the neurologic complications after CABG. Material and Method: In 351 patients who underwent CABG, the incidence and features of neurologic complications, with associated perioperative risk factors, were retrospectively reviewed. Neurologic complication was defined as a new cerebral infarction confirmed by postoperative neurologic examination and radiologic studies, or delayed recovery of consciousness and orientation for more than 24 hours after the operation. Result: Neurologic complications occurred in 18 patients(5.1%), of these nine(2.6%) were diagnosed as having new cerebral infarctions(stroke). Stroke was manifested as motor paralysis in four patients, mental retardation or orientation abnormality in four, and brain death in one. Statistical analysis revealed the following variables as significant risk factors for neurologic complications by both univariate and multivariate analyses: cardiopulmonary bypass longer than 180 minutes, atheroma of the ascending aorta, carotid artery stenosis detected by Duplex sonography, and past history of cerebrovascular accident or transient ischemic attack. Age over 65 years, aortic calcification detected by simple X-ray, and intraoperative myocardial infarction were significant risk factors by univariate analysis only. Neither the severity of carotid artery stenosis nor technical modifications such as cannulation of the aortic arch or single clamp technique, which were expected to affect the inciden e of neurologic complications, had significant relationship with the incidence. Conclusion: This study confirmed the strong association between neurologic complications after CABG and atherosclerosis of the arterial system. Therefore, to minimize the incidence of neurologic complications, systematic evaluation focused on atherosclerotic lesions of the arterial system followed by adequate alteration of operative strategy is needed.

• Journal of the Korean Institute of Landscape Architecture
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• v.49 no.4
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• pp.41-54
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• 2021

This study targets the urban park corresponding to the core areas (Hubs) of Green Infrastructure and estimates their value utilizing the Contingent Valuation Method (CVM) and determines the planning factors which affect them. The research aims to provide basic data for supporting the value improvement in the planning stage for urban parks representing green infrastructure. The primary purpose of this research is to derive variables that affect economic value and planning factors to improve the use-value of urban parks, one of the Hubs of the green infrastructure. In this study, Sejong Lake Park, located in Sejong City, is the target site. This study collected the responses of 105 people by conducting a survey on the intention to pay for the use-value and the planning factors that affect it, targeting visitors to Sejong Lake Park. The study conducts Contingent Valuation Method (CVM) on this survey responses. The results are as follows: first, as a result of analyzing the variables which affect willingness to pay for use-value, residence and age influence the willingness to pay significantly among socioeconomic characteristics. Next, the survey responses of Double-bounded dichotomous choices (DB-DC) CVM are converted into variables through statistic techniques. Furthermore, the variables are used for a Logit model to draw coefficients. The average willingness to pay per person for the use-value of Sejong Lake Park using the derived coefficients was approximately found to be 8,597 won. Therefore, as of 2019, Sejong Lake Park, with a total of 430,000 visitors, is estimated to have an annual economic value of 3.7 billion won. Third, the average Likert scale of the planning factor affecting the decision to pay for the economic value of Sejong Lake Park was the highest along the waterfront landscape, and the convenience facilities and waterfront landscape showed the highest willingness to pay, 10,000 won. In the range between 2,500 won and 5,000 won, the waterfront area ranks highest. Therefore, it can be said that visitors to Sejong Lake Park take account of the economic value of using the waterfront landscape the most. This study is meaningful as a thesis on use-value and the planning factors that affected value evaluation results of urban parks, and the analysis of the correlation between the planning factors of urban parks as hubs located in urban areas.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.11 no.1
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• pp.28-35
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• 2008

Purpose: The aim of this study is to report the efficacy of infliximab, a monoclonal antibody directed against tumor necrosis factor alpha which is used for both treatment of refractory pediatric Crohn disease (CD) and induction of remission. Methods: Among pediatric patients who were diagnosed with CD at Samsung Medical Center between March 2001 and August 2007, a total of 16 patients were given infliximab to treat conventional therapyresistant refractory CD and severe active CD for induction of remission. Patients needing maintenance therapy were treated with an infliximab infusion every 8 weeks, and fistulizing CD patients occasionally received the infusion upon the condition that a fistula developed. The efficacy of treatment was assessed by comparing the Pediatric Crohn Disease Activity Index (PCDAI), Hct, ESR, CRP, and serum albumin levels using paired t-test. Results: The male/female ratio was 13:3, and the median age was 13 years (range, 21 months~15 years). The patients included 7 cases of therapy-resistant refractory CD, 7 cases of severe active CD, and 2 cases of fistulizing CD. Mean PCDAI before infliximab therapy was 34.19${\pm}$14.96, and mean follow-up PCDAI within 2 to 4 weeks after the last infusion was significantly lower, at 6.88${\pm}$10.31 (p=0.000). Hematological markers such as ESR (p=0.000), serum albumin (p=0.016), and CRP (p=0.009) also improved significantly after infusion. Remission was achieved in 2 of 4 patients refractory to conventional therapy. Among 3 steroid-dependent patients, 2 were able to discontinue steroid therapy, and dose reduction was possible in 1 patient. Remission after top-down therapy without prior use of other immunomodulators was achieved in 6 weeks in all 7 of the patients who had severe CD. Nine of ten refractory fistulizing CD patients also showed improvement after infliximab therapy. Conclusion: Infliximab was effective in pediatric refractory CD for induction of remission and maintenance therapy, as well as in severe CD for top-down induction therapy. Furthermore, infliximab has contributed to steroid cessation and dose reduction. Long-term follow-up evaluation is needed to determine safety and efficacy of infliximab in the future.

• Journal of Nutrition and Health
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• v.53 no.1
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• pp.68-82
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• 2020

Purpose: This study evaluated the dietary quality and nutritional status of elderly people using the Nutrition Quotient for Elderly (NQ-E). Methods: The participants were 204 elderly people over 65 years of age (38 men and, 166 women) in Seoul. The dietary information was analyzed using a questionnaire of NQ-E, which consisted of 19 checklist items, and 24-recall test data. The NQ-E scores and its four factors, including 'balance', 'moderation', 'diversity', and 'dietary behavior' factors, were calculated according to general characteristics of the subjects. The subjects were divided into the 'monitoring needed group' (62 > NQ-E score) and the 'good group' (62 ≤ NQ-E score) according to their NQ-E score. Results: The mean NQ-E score of the total subjects was 61.9, which was within the mediumhigh grade. The scores of balance, moderation, and dietary behavior factors were within the medium-high grade, while the score of the diversity factor was within the medium-low grade. The NQ-E score was 54.8 in the monitoring needed group and 69.3 in the good group. For the score of the diversity factor, the elderly living alone had a significantly lower score than the score for the elderly living with a spouse. The female subjects showed significantly higher scores of moderation and dietary behavior factors than did the male subjects. The daily intakes of vitamin A, thiamin, riboflavin, vitamin C and calcium in the monitoring needed group were significantly lower than those in the good group. The nutrient adequacy ratios (NAR) of vitamin A, thiamin, riboflavin, vitamin C and calcium in the monitoring needed group were significantly lower compared to the scores of the good group. The indexes of nutritional quality (INQ) of vitamin A, thiamin, riboflavin, vitamin C, calcium and potassium were less than 1 for all the subjects. The monitoring needed group had a significantly lower consumption of total foods, vegetables and mushrooms than the good group. As a result, the nutritional status of the monitoring needed group was significantly lower than that of the good group. Conclusion: The results of this study show that NQ-E would be a useful tool for assessing the dietary quality of the elderly. In conclusion, a focused-nutrition education program and a useful guideline are needed for promoting the health and nutritional status in elderly people.

• Tuberculosis and Respiratory Diseases
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• v.40 no.3
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• pp.274-282
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• 1993

Background: Pulmonary paragonimiasis is caused by consumption of raw or improperly cooked crabs infected by a laval stage (metacercaris) of the parasite. In our country it had been a highly prevalent endemic disease until th late 1960s, and after then it's prevalence has been markedly decreased. But because some people have continued to ingest undercooked crabs, this disease have yet occured sporadically. Methods: We reviewed the clinical and radiological findings retrospectively in seventy-four patients of pulmonary paragonimiasis including familial infestation in 7 familes (20 cases) who were confirmed by food history, clinical and radiological findings, and labaratory data. Results: The male: female ratio was 2.2:1 and most prevalent age was 40-49 years old. Twen6ty nine patients (39%) had ova-positive infection. The detection sites were sputum (48%), pleural fluid (17%), fine needle aspiration biopsy of nodular or cystic lesion (17%), pleural biopsy (7%), skin nodule biopsy (7%), and stool (3%). The patients had pulmonary symptoms in 63 cases (85%) but 9 cases did not have any symptoms. The 53 cases (72%) had abnormal radiological findings in lung parenchyme (75%) and pleura (63%). However 21 cases (28%) showed no specific findings in their chest X-ray. Serum titers (ELISA) of specific IgG for paragonimiasis in 13 cases were followed for average 9.8 months after treatment, which showed slow decreasement. In the evaluation of family member (7 family, 20 cases), all members having the common dietary history together with a proven patients were confirmed this disease by serological test, regardless of the presence or the abscence of clinical or radilogical symptoms. Conclusion: We evaluated the clinical and radiological findings in 74 cases of pulmonary paragonimiasis including 7 family members who had a history of ingestion of improperly cooked crabs together with patients. The paients of pulmonary paragonimiasis have various findings in clinical and radiological findings. Common diet exposure history and laboratory findings including specific IgG were important in earlier diagnosing and treating in family members of patients.

• The Korean Journal of Nuclear Medicine Technology
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• v.17 no.1
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• pp.18-24
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• 2013

Purpose: Myocardial perfusion SPECT using $^{201}Tl$ is an important method for viability of left ventricle and quantitative evaluation of cardiac function and now various reconstruction methods are used to improve the image quality. But in case of small sized heart, you should always be careful because of the Partial Volume Effect which may cause errors of quantitative indices at the reconstruction step. So, In this study, we compared those quantitative indices of left ventricle according to the reconstruction method of myocardial perfusion SPECT with the Echocardiography and verified the degree of the differences between them. Materials and Methods: Based on ESV 30 mL of Echocardiography, we divided 278 patients (male;98, female;188, Mean age;$65.5{\pm}11.1$) who visited the Asan medical center from February to September, 2012 into two categories; below the criteria to small sized heart, otherwise, normal or large sized heart. Filtered and output each case, we applied the method of FBP and OSEM to each of them, and calculated EDV, ESV and LVEF, and we conducted statistical processing through Repeated Measures ANOVA with indices that measured in Echocardiography. Results: In case of men and women, there were no significant difference in EDV between FBP and OSEM (p=0.053, p=0.098), but in case of Echocardiography, there were meaningful differences (p<0.001). The change of ESV especially women in small sized heard, significant differences has occurred among FBP, OSEM and Echocardiography. Also, in LVEF, there were no difference in men and women who have normal sized heart among FBP, OSEM and Echocardiography (p=0.375, p=0.969), but the women with small sized heart have showed significant differences (p<0.001). Conclusion: The change in quantitative indices of left ventricle between Nuclear cardiology image reconstruction, no difference has occurred in the patients with normal sized heart but based on ESV, under 30 mL of small sized heart, especially in female, there were significant differences in FBP, OSEM and Echocardiography. We found out that overestimated LVEF caused by PVE can be reduced in average by applying OSEM to all kinds of gamma camera, which are used in analyzing the differences.