• Journal of Yeungnam Medical Science
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• v.32 no.2
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• pp.102-105
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• 2015

Secondary systemic (AA) amyloidosis is a severe complication of progressed Crohn disease (CD) characterized by the deposition of amyloid A in body organs and tissues. Various therapeutic approaches have been recommended, however there is still no effective treatment. Recently, several case reports have demonstrated the effects of anti-tumor necrosis factor-${\alpha}$ therapy in patients with AA amyloidosis associated with CD. We report on a 35-year-old female patient with CD complicated by AA amyloidosis in the gastrointestinal tract and renal involvement, who was treated with infliximab. The infliximab therapy improved the gastrointestinal symptoms and decreased the serum creatinine.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.19 no.1
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• pp.71-75
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• 2016

Esophageal candidiasis is commonly seen in immunocompromised patients; however, candida esophagitis induced stricture is a very rare complication. We report the first case of esophageal stricture secondary to candidiasis in a glycogen storage disease (GSD) 1b child. The patient was diagnosed with GSD type 1b by liver biopsy. No mutation was found in the G6PC gene, but SLC37A4 gene sequencing revealed a compound heterozygous mutation (p.R28H and p.W107X, which was a novel mutation). The patient's absolute neutrophil count was continuously under $1,000/{\mu}L$ when he was over 6 years of age. He was admitted frequently for recurrent fever and infection, and frequently received intravenous antibiotics, antifungal agents. He complained of persistent dysphagia beginning at age 7 years. Esophageal stricture and multiple whitish patches were observed by endoscopy and endoscopic biopsy revealed numerous fungal hyphae consistent with candida esophagitis. He received esophageal balloon dilatation four times, and his symptoms improved.

• Journal of Preventive Medicine and Public Health
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• v.51 no.2
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• pp.71-82
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• 2018

Objectives: Areca nut is widely consumed in many parts of the world, especially in South and Southeast Asia, where cardiovascular disease (CVD) is also a huge burden. Among the forms of CVD, acute coronary syndrome (ACS) is a major cause of mortality and morbidity. Research has shown areca nut chewing to be associated with diabetes, hypertension, oropharyngeal and esophageal cancers, and CVD, but little is known about mortality and re-hospitalization secondary to ACS among areca nut users and non-users. Methods: A prospective cohort was studied to quantify the effect of areca nut chewing on patients with newly diagnosed ACS by categorizing the study population into exposed and non-exposed groups according to baseline chewing status. Cox proportional hazards models were used to examine the associations of areca nut chewing with the risk of re-hospitalization and 30-day mortality secondary to ACS. Results: Of the 384 ACS patients, 49.5% (n=190) were areca users. During 1-month of follow-up, 20.3% (n=78) deaths and 25.1% (n=96) re-hospitalizations occurred. A higher risk of re-hospitalization was found (adjusted hazard ratio [aHR], 2.05; 95% confidence interval [CI], 1.29 to 3.27; p=0.002) in areca users than in non-users. Moreover, patients with severe disease were at a significantly higher risk of 30-day mortality (aHR, 2.77; 95% CI, 1.67 to 4.59; p<0.001) and re-hospitalization (aHR, 2.72; 95% CI, 1.73 to 4.26; p<0.001). Conclusions: The 30-day re-hospitalization rate among ACS patients was found to be significantly higher in areca users and individuals with severe disease. These findings suggest that screening for a history of areca nut chewing may help to identify patients at a high risk for re-hospitalization due to secondary events.

• Journal of Plant Biotechnology
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• v.30 no.4
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• pp.387-397
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• 2003

During the first half of twentieth century, even though the importance of non-calorie essential micronutrients of 13 vitamins and 17 minerals has been known to alleviate nutritional disorder; the primary objective of agriculture and plant breeding programs has been to increase the productivity and seed yields, and macronutrients of proteins, fats, and carbohydrates made up the bulk of foodstuff which were used primarily as an energy source. In the last decade it has been found that non-essential micronutrients encompass a vast group of phytochemicals including antioxidants that are not strictly required in the diet but when present at sufficient levels work as health-promoting chemicals. Nowadays agricultural crops are grown for health rather than for food or fiber, and modifying the nutritional compositions of plant foods has become an urgent health issue. To ensure an adequate intake of essential vitamins and minerals, and to increase the consumption of health-promoting phytochemicals, the researches on plant secondary metabolism have been made. The attempt to improve nutritional quality of crops has been blocked by a lack of basic knowledge of plant metabolism. The advent of genomics era enabled new approaches to make crossing regardless of species, family, or phylum barriers, and the accumulation in our basic knowledge on plant secondary metabolism during the coming decade would be tremendous. As the major staple crops contain insufficient amount of many micronutrients, fortification strategy will be a necessary practice. Elevated intake of specific vitamins, C, E, and $\beta$-carotene, mineral selenium, antioxidants, and phytochemicals significantly reduces the risk of chronic disease such as cancer, cardiovascular disorder, diabetis, and other degenerative disease associated with aging. As the attempt to improve the nutritional quality of crops requires the basic knowledges on plant metabolism, plant biochemistry, human physiology, and food chemistry, strong interdisplinary collaboration among plant biotechnologists, human nutritionists, and food scientists will be needed. Inhibition of cancer, cardiovascular disease, and other degenerative disorder may be the biggest goal facing nutritional plant breeders. But the assumption that simply increasing dietary level of any compound will necessarily improve human health is a dangerous idea because many plant secondary products and dietary contaminants have paradoxical (hermetic) effects. Before biotechnical manipulation is undertaken to elevate or reduce any individual constituent of crops, the contribution of the micronutrient to human health must first be investigated.

• Journal of Chest Surgery
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• v.16 no.4
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• pp.498-505
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• 1983

It has been over 20 years since successful operations of Cardiac valves at the Department of Thoracic and Cardiovascular surgery, college of medicine, Yonsei University. About six hundreds of patients with severely symptomatic valvular heart disease have had valve operations with complete loss or sharp decrease in their cardiac symptoms since 1956. As the number of cardiac patient increases, reoperation on valves assumes greater importance. To define the group of patients undergoing reoperations on valves and the factors influencing their survival, we have reviewed our experiences of the reoperation on valves at the Yonsei University, Severance Hospital. This is a report of 29 cases which was undergone secondary or more surgery for valvular heart disease from 1966 to 1983. The primary operations includes 159 cases of open heart surgery from 1966 to 1975 and 476 cases from 1976 to march, 1983. The secondary operations are classified into groups of secondary valvuloplasty or valvotomy [8 cases], prosthetic valve replacement following valvuloplasty or valvotomy [14 cases] and prosthetic valve rereplacement [2 case] for such as calcification, degeneration and perforation of the cusps and paravalvular leakage, of the bioprosthetic valves. The leading indication for reoperation of mitral valve was restenosis or stenoinsufficiency, The indications of aortic valve replacement was active bacterial endocarditis, medically uncontrollable prosthetic endocarditis or paravalvular leakage. Overall death rate of the reoperation was 17.4% [5 death among the 29 patients] and the leading causes of death were myocardial failure, arrhythmia, cerebral embolism, acute renal failure due to low output syndrome. And it was followed by sepsis associated with active prosthetic endocarditis. The death rate of reoperation was 4.3% in the elective cases except urgent cases and the death rate of overall cardiac valve except reoperation cases was 4.1% in the last two years. Although the general mortality of reoperation was high, both mortality rates were comparable except emergency cases due to urgent preoperative patient’s condition.

• Clinical and Experimental Pediatrics
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• v.61 no.5
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• pp.167-173
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• 2018

Purpose: Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome with many causes, including Kawasaki disease (KD). The purpose of this study was to identify the laboratory tests needed to easily differentiate KD with HLH from incomplete KD alone. Methods: We performed a retrospective study on patients diagnosed with incomplete KD and incomplete KD with HLH (HLH-KD) between January 2012 and March 2015. We compared 8 secondary HLH patients who were first diagnosed with incomplete KD with all 247 incomplete KD diagnosed patients during the study period. The complete blood count, erythrocyte sedimentation rate, platelet count, and serum total protein, albumin, triglyceride, C-reactive protein, N-terminal pro-brain natriuretic peptide (NT-proBNP), and ferritin levels were compared. Clinical characteristics and echocardiography findings were also compared between the 2 groups. Results: The total duration of fever was longer in the HLH-KD group than in the KD group. White blood cell and platelet counts were higher in the KD group. Alanine aminotransferase, ferritin, and coronary artery diameter were increased in the HLH-KD group compared with those in the KD group. The median of NT-proBNP was significantly higher in the HLH-KD group than in the KD group at 889.0 (interquartile range [IQR], 384.5-1792.0) pg/mL vs. 233.0 (IQR, 107.0-544.0) pg/mL. Conclusion: The NT-proBNP level may be helpful in distinguishing incomplete KD from KD with HLH. The NT-proBNP level should be determined in KD patients with prolonged fever, in addition to the white blood cell count, platelet count, and ferritin level, to evaluate secondary HLH.

• Journal of Chest Surgery
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• v.56 no.4
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• pp.264-271
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• 2023

Background: The optimal management strategy for aortoiliac occlusive disease (AIOD) remains debatable. This study compared early and late outcomes between direct surgical bypass and kissing stents for AIOD treatment. Methods: We retrospectively reviewed data, including age, sex, risk factors, comorbidities, symptoms, TransAtlantic Inter-Society Consensus (TASC) II classification, operation time, perioperative complications, in-hospital mortality, and length of hospital stay, from a cohort of 46 patients treated for AIOD (24 with kissing stents and 22 with direct surgical bypass) at Pusan National University Hostpital from January 2007 to December 2016. The primary, assisted primary, and secondary patency rates in both groups were compared. Results: The hospital stay (direct surgical bypass vs. kissing stents: 16.36±5.19 days vs. 9.08±10.88 days, p=0.007) and operation time (direct surgical bypass vs. kissing stents: 316.09±141.78 minutes vs. 99.54±37.95 minutes, p<0.001) were significantly shorter for kissing stents. Kaplan-Meier analysis revealed that the primary, assisted primary, and secondary patency rates in the direct surgical bypass group were 95.5%, 95.5%, and 95.5%, respectively, at 1 year; 86.4%, 86.4%, and 95.5% at 3 years; and 77.3%, 77.3%, and 95.5% at 5 years. The primary, assisted primary, and secondary patency rates in the kissing stent group were 100.0%, 100.0%, and 100.0%, respectively, at 1 year; 95.8%, 95.8%, and 100.0% at 3 years; and 95.8%, 95.8%, and 100.0% at 5 years. Conclusion: Except for special cases wherein endovascular revascularization is difficult, kissing stents are more advantageous for TASC II C and D lesions.

• Journal of Preventive Medicine and Public Health
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• v.34 no.4
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• pp.427-436
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• 2001

Objective : To estimate the prevalence of congenital heart disease from the 1990 student heart disease screening program. Methods : The heart disease screening program for elementary students was conducted in Kyonggi-do, in 1998. The subjects of the present study comprised the 40,402 students who attended the schools in the catchment area of a collaborative university hospital and who participated in the primary examination. The congenital heart disease (CHD) patients were initially identified through a questionnaire about prior medical history, and further through diagnostic tests & medical examinations in the secondary & the tertiary examinations. Certain assumptions were used in the estimation of the number of CHD cases among non-participants of the secondary & tertiary examinations. The overall prevalence of CHD was estimated by adding the CHD detection rates of the participants and the estimated prevalence of the non-participants. Results : Among the 40,402 primary participants, 1,655 were referred further, of whom 79.1% (1,309) participated in the secondary examination. Of these, 121 were referred to the tertiary examination, with a participation rate at this last stage of 80.2%. The positive predictive value (PPV) of the screening tools was the highest when the results of both EKG and the questionnaire were positive. Because 85.9% of the detected cases had a past history of CHD, PPV was higher when the selection criteria in the questionnaire included past CHD history than when it didnt. The CHD defection rate among the participants was 1.76 cases/1,000 and the presumed number of cases among the non-participants was 31; giving an estimated final CHD prevalence of 2.52 cases/1,000 (95% CI : 2.06-3.06). Among the identified cases of CHD, VSD (52.8%) was the most common, followed by PDA (9.7%), TOF (9.7%) & PS (9.7%). Conclusion : Because the characteristics of the non-participants differed from those of the participants, the estimation of prevalence was influenced by the participation rate. Of the detected cases, 85.9% had a past history of diagnosis or operation for CMD. These findings suggested that the prevalence estimated in this study may be an underestimation of the actual condition. Therefore, a birth cohort study is required in order to more accurately estimate the prevalence and the effects of the program.

• Clinical and Experimental Pediatrics
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• v.55 no.11
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• pp.438-444
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• 2012

Mucolipidosis II (ML II) or inclusion cell disease (I-cell disease) is a rarely occurring autosomal recessive lysosomal enzyme-targeting disease. This disease is usually found to occur in individuals aged between 6 and 12 months, with a clinical phenotype resembling that of Hurler syndrome and radiological findings resembling those of dysostosis multiplex. However, we encountered a rare case of an infant with ML II who presented with prenatal skeletal dysplasia and typical clinical features of severe secondary hyperparathyroidism at birth. A female infant was born at $37^{+1}$ weeks of gestation with a birth weight of 1,690 g (<3rd percentile). Prenatal ultrasonographic findings revealed intrauterine growth retardation and skeletal dysplasia. At birth, the patient had characteristic features of ML II, and skeletal radiographs revealed dysostosis multiplex, similar to rickets. In addition, the patient had high levels of alkaline phosphatase and parathyroid hormone, consistent with severe secondary neonatal hyperparathyroidism. The activities of ${\beta}$-D-hexosaminidase and ${\alpha}$-N-acetylglucosaminidase were moderately decreased in the leukocytes but were 5- to 10-fold higher in the plasma. Examination of a placental biopsy specimen showed foamy vacuolar changes in trophoblasts and syncytiotrophoblasts. The diagnosis of ML II was confirmed via GNPTAB genetic testing, which revealed compound heterozygosity of c.3091C>T (p.Arg1031X) and c.3456_3459dupCAAC (p.Ile1154GlnfsX3), the latter being a novel mutation. The infant was treated with vitamin D supplements but expired because of asphyxia at the age of 2 months.

• Journal of Chest Surgery
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• v.28 no.4
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• pp.398-400
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• 1995

Kawasaki s disease is a multisystemic disorder that is an important cause of cardiovascular disease in children. We experienced a case of coronary artery aneurysm secondary to Kawasaki s disease. The patient was 12-year-old female presented as exertional dyspnea & chest pain[NYHA classII . Her coronary angiogram showed saccular aneurysms at proximal right coronary artery and proximal left anterior descending artery. CABG was performed by use of double internal mammary artery.Postoperative E.K.G. showed a normal pattern.She discharged at postoperative 10th day without problem. 4 months after discharge, she had no dyspnea & chest pain in exertion[NYHA class I .