• Journal of mucopolysaccharidosis and rare diseases
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• v.4 no.1
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• pp.14-20
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• 2018

Mucopolysaccharidoses (MPSs) are a group of rare inherited metabolic disorders caused by specific lysosomal enzyme deficiencies leading to the sequential degradation of glycosaminoglycans, causing substrate accumulation in various cells and tissues and progressive multiple organ dysfunction. The rare disease medical care team at Mackay Memorial Hospital in Taiwan has been dedicated to the study of MPSs for more than 20 years. Since 1999, more than 50 academic papers focusing on MPSs have been published in international medical journals. Topics of research include the following items regarding MPSs: incidence, natural history, clinical manifestations, gene mutation characteristics, cardiac function, bone mineral density, sleep studies, pulmonary function tests, hearing assessments, percutaneous endoscopic gastrostomy, anesthetic experience, imaging analysis, special biochemical tests, laboratory diagnostics, global expert consensus conferences, prenatal diagnosis, new drug clinical trials, newborn screening, and treatment outcomes. Of these published academic research papers, more than half were cross-domain, cross-industry, and international studies with results in cooperation with experts from European, American and other Asian countries. A cross-specialty collaboration platform was established based on high-risk population screening criteria with the acronym "BECARE" (Bone and joints, Eyes, Cardiac and central nervous system, Abdomen and appearance, Respiratory system, and Ear, nose, and throat involvement). Through this platform, orthopedic surgeons, rheumatologists, ophthalmologists, cardiologists, rehabilitation physicians, gastroenterologists, otorhinolaryngologists, and medical geneticists have been educated with regards to awareness of suspected cases of MPSs patients to allow for a further confirmative diagnosis of MPSs. Because of the progressive nature of the disease, an early diagnosis and early multidisciplinary therapeutic interventions including surgery, rehabilitation programs, symptom-based treatments, hematopoietic stem cell transplantation, and enzyme replacement therapy, are very important.

• The Korean Journal of Nuclear Medicine
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• v.24 no.2
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• pp.279-285
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• 1990

Simultaneous presence of ascites and pleural effusion has been documented in patients with cirrhosis of the liver, renal disease, Meigs' syndrome and in patients undergoing peritoneal dialysis. Mechanisms proposed in the formation of pleural effusion in most of the above diseases are lymphatic drainage and diaphragmatic defect. But sometimes, hepatic hydrothoraxes in the absence of clinical ascites and pleural effusion secondary to pulmonary or cardiac disease are noted. It is not always possible to differentiate between pleural effusion caused by transdiaphragmatic migration of ascites and by other causes based soly on biochemical analysis. Authors performed radionuclide scintigraphy after intraperitoneal administration of $^{99m}Tc-labeled$ colloid in 23 patients with both ascites and pleural effusion in order to discriminate causative mechanisms responsible for pleural effusion. Scintigraphy demonstrated the transdiaphragmatic flow of fluid from the peritoneum to pleural cavities in 13 patients correctly. In contrast, in 5 patients with pleural effusion secondary to pulmonary, pleural and cardiac diseases, radiotracers fail to traverse the diaphragm and localize in the pleural space. Ascites draining to mediastinal lymph nodes and blocked passage of lymphatic drainage were also clarified, additionaly. Conclusively, radionuclide peritoneal scintigraphy is an accurate, rapid and easy diagnostic tool in patients with both ascites and pleural effusion. It enables the causes of pleural effusion to be elucidated, as well as providing valuable information required when determining the appropriate therapy.

• The Korean Journal of Nuclear Medicine
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• v.6 no.2
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• pp.21-39
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• 1972

The employment of gamma-ray scintillation camera with the use of short-lived radioisotopic pharmaceuticals, sucu as $^{99m}Tc$ sodium pertechnetate, have enabled us to perform RI (radioisotopic) angiocardiography. Although conventional cardiac catheterization or angiocardiography using contrast media have been an important diagnostic tool, they may carry some risks or serious complications. The author investigated on RI angiocardiography in twelve normal and twenty five patients with cardiovascular diseases in an effort to evaluate its diagnostic value. The results obtained with this study are as follows; 1. In normal subjects, the scintillation camera transit time of arm-to-right heart was found to be $2.1{\pm}0.67$ seconds, right heart-to-lung $1.5{\pm}0.40$ seconds and lung-to-left heart $3.5{\pm}0.86$ seconds. 2. Transformation or displacement of the heart and the great vessel are easily discriminated by RI angiocardiography. Both in the cases with tricuspid atresia and tetralogy of Fallot, ventricular septal defects are well recongnized by sequential RI angiocardiography. 3. It is also helpful in determinining the site and extent of obstruction, and estimatiing the postoperative course both in the cases with superior vena cava syndrome and pulmonary stenosis. 4. Pericarditis with effusion is readilly diagnosed by RI angiocardiogram showing characteristic "dead space" between intracardiac and intrapulmonary radioactivity. 5. It was found that the diagnostic accuracy of this study was 78.4%. It is concluded that above results obtained are useful and accurate enough for the diagnostic screening methode for clinical practice.

• Tuberculosis and Respiratory Diseases
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• v.77 no.1
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• pp.28-33
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• 2014

We report a case of invasive pulmonary aspergillosis invading the mediastinum and the left atrium. A 70-year-old woman was hospitalized for dyspnea. She had been well controlled for her diabetes mellitus and hypertension. The chest X-ray disclosed mediastinal widening, and the computed tomography scan of the chest showed that there was a large mediastinal mass and this lesion extended into the left atrium and right bronchus. The cardiac echocardiography showed that a huge mediastinal cystic mass compressed in the right atrium and a hyperechoic polypoid lesion in the left. The pathology from the bronchoscopic biopsy observed abundant fungal hyphae which was stained with periodic acid-Schiff and Gomori's methenamine silver. Despite the treatment with antifungal agents, she died from cardiac tamponade after three months. Invasive pulmonary aspergillosis, which involves the mediastinum and the heart, is very rare in immunocompetent patients.

• Endocrinology and Metabolism
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• v.33 no.4
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• pp.485-492
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• 2018

Background: Increasing evidence supports interplay between aldosterone and parathyroid hormone (PTH), which may aggravate cardiovascular complications in various heart diseases. Negative structural cardiovascular remodeling by primary aldosteronism (PA) is also suspected to be associated with changes in calcium levels. However, to date, few clinical studies have examined how changes in calcium and PTH levels influence cardiovascular outcomes in PA patients. Therefore, we investigated the impact of altered calcium homeostasis caused by excessive aldosterone on cardiovascular parameters in patients with PA. Methods: Forty-two patients (mean age $48.8{\pm}10.9$ years; 1:1, male:female) whose plasma aldosterone concentration/plasma renin activity ratio was more than 30 were selected among those who had visited Severance Hospital from 2010 to 2014. All patients underwent adrenal venous sampling with complete access to both adrenal veins. Results: The prevalence of unilateral adrenal adenoma (54.8%) was similar to that of bilateral adrenal hyperplasia. Mean serum corrected calcium level was $8.9{\pm}0.3mg/dL$ (range, 8.3 to 9.9). The corrected calcium level had a negative linear correlation with left ventricular end-diastolic diameter (LVEDD, ${\rho}=-0.424$, P=0.031). Moreover, multivariable regression analysis showed that the corrected calcium level was marginally associated with the LVEDD and corrected QT (QTc) interval (${\beta}=-0.366$, P=0.068 and ${\beta}=-0.252$, P=0.070, respectively). Conclusion: Aldosterone-mediated hypercalciuria and subsequent hypocalcemia may be partly involved in the development of cardiac remodeling as well as a prolonged QTc interval, in subjects with PA, thereby triggering deleterious effects on target organs additively.

• Journal of Biomedical Engineering Research
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• v.45 no.3
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• pp.109-117
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• 2024

We developed a device that integrates digital otoscope and stethoscope for telemedicine. The integrated device was utilized for the collection of tympanic membrane images and cardiac auscultation data. Data accumulated on the platform server can support real-time diagnosis of heart and eardrum diseases using artificial intelligence. Public data from Kaggle were used for deep learning. After comparing with various deep learning models, the MobileNetV2 model showed superior performance in analyzing tympanic membrane data, and the VGG16 model excelled in analyzing cardiac data. The classification algorithm achieved an accuracy of 89.9% for eardrums data and 100% for heart sound data. These results demonstrate the possibility of diagnosing diseases without the limitations of time and space by using this platform.

• The Journal of Korean Medicine
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• v.28 no.4
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• pp.8-12
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• 2007

Objectives : This study aimed to investigate the circadian variation in stroke types, ischemic stroke subtypes, and Sasang constitutions. Methods : 295 patients with acute stroke within 14 days onset were included, who were admitted to Kyunghee Oriental Medical Center from October 2005 to May 2007. The stroke onset time was subdivided into four groups of 6-hour intervals in a day. Stroke types, ischemic stroke subtypes, and Sasang constitutions in four groups were examined. Results : Most ischemic stroke occurred between 6:01-12:00 hours (30.2%). For ischemic stroke subtypes, the peak period of small-vessel occlusion was between 6:01-12:00 hours (33.2%), large-artery atherosclerosis was most common between 12:01-18:00 hours (39.5%), and cardiac embolism was most frequent between 18:01-24:00 hours (50%). In terms of Sasang constitution, Soyeumin and Taeyeumin were most common between 6:01-12:00 hours (36.4% versus 41.5%, respectively), but the peak time of Soyangin was between 12:01-18:00 hours (35.2%). Conclusion :Most ischemic stroke events occurred in the mid-to-late morning hours in the present study and there was a circadian variation of onset in ischemic stroke subtypes and Sasang constitution.

• Journal of Preventive Medicine and Public Health
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• v.33 no.1
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• pp.36-44
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• 2000

Objective : The heart diseases are known as a major cause of sudden death, as well as a cause of poor life-quality of school-age children. But there have been few mass screening of heart diseases in these children in Korea. This study was done to estimate the prevalence of heart diseases of these population. Methods : We screened all elementary students(grade 1) in 12 cities and 16 counties(Gun) in Kyonggi province from 1992 to 1955. The first screening was done by auscultation of doctors and simultaneously by checking using 'auto-interpreter of EKG-cardiac sound' (Fukuda Densi ECP 50A). We conducted luther examinations to whom classified as being abnormal condition in first screening, by using EKG, chest x-ray, doppler echocardiograpy(if needed). Results : The total number of examined students was 161,308(92% of the population), the male were 83,238 and female were 78,070. The congenital heart diseases(CHD) patients were 290(18 per 10,000) - male 155(18.6 per 10,000) and female 135(17.3 per 10,000). The most frequent disease was ventricula septal defect(VSD, 45.5%), Atrial septal defect(ASD, 14.8%), Tetralogy of Follot(TOF, 11.7%), and Patent Dutus Arteriosis(PDA, 7.6%) in order. In female, the order was VSD(48.1%), ASD(13.3%), TOF(11.1%), and PDA(10.4%). The total number of EKG abnormality were 433(62.7 per 10,000) among 69,056 screened children in 1995. The complete right bundle branch block(CRBBB) and paroxymal ventricular contraction(PVC) were frequent(26.6%, 26.3% in each), and incomplete right bunddle branch block(IRBBB, 14.6%), paroxymal atrial contraction(PAC, 6.7%), abnormal Q(5.8%), Wolf-Pakinson-White syndrom (5.5%) in order. In female, the most frequent abnormality was PVC(29.8%), and CRBBB(19.9%) in order. Conclusion : We could present the stable prevalence of the rare heart disease. The prevalence of congenital heart diseases was 18.0 per 10,000 and of EKG abnormality was 62.7 per 10,000 among school children.

• Journal of Chest Surgery
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• v.19 no.4
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• pp.644-662
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• 1986

Seventy cases of open heart surgery were performed in the department of Thoracic and Cardiovascular Surgery, Pusan Paik Hospital, Inje College, from Oct. 1985 to Oct. 1986. And the results were summarized as follows. 1. Among the 70 cases, there were 48 cases of congenital heart anomalies and 22 cases of acquired rheumatic valvular heart diseases. Age range of the congenital patients was 7 months to 31 years with the mean age of 10 years, and the acquired patients was 18 to 62 years with the mean age of 40 years. 2. The heart-lung machine used for cardiopulmonary bypass was Sarns 7000, 5-head roller pump, and the number and type of oxygenators were 5 of membrane type and 65 of bubble type. For all cases GIK [glucose-insulin-potassium] solution was used as cardioplegic solution for myocardial protection during operation. 3. Among the 48 congenital anomalies, there were 12 cases of ASD group, 29 of VSD group, 3 of ECD, 3 of TOF and one of PDA + MR, and to all of which the appropriate radical operations were applied. 4. Among the 22 acquired valvular diseases, there were 11 cases of mitral valve diseases [MS; 4, MSr; 3, MRs; 4], 3 cases of aortic valve diseases [AR:1, ARs;1, ASr;1], 4 cases of double valve diseases [MRs+TR; 3, MRs+ARs; 1] and 4 cases of triple valve diseases [MSr+ASr+TR; 3, MSr+Ar+TR; 1]. To all the diseased mitral and aortic valves, artificial valve replacement was applied except one [As], in which valve plication was applied. And to all the diseased tricuspid valve, DeVega annuloplasty was applied. 5. The number of replaced artificial valves were 29 in 25 patients [congenital; 3, acquire; 22]. In MVR, 6 of mechanical valves [St. Jude Medical valve; 6] and 15 of tissue valves [Carpentier-Edward valve; 11, lonescu-Shiley valve; 4] were used. In AVR, 6 of mechanical valves [St. Jude Medical valve; 6] and 2 of tissue valves [Carpentier-Edward valve; 2] were used. 6. Postoperative complications were occurred in 12 cases. Among them 11 cases were recovered with intensive cares, but one patient [VSD + Fistula of Valsalva sinus] was expired with low cardiac out put syndrome.

• Journal of Korean Academy of Nursing
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• v.47 no.2
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• pp.178-187
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• 2017

Purpose: The purposes of this study were to identify the relationships among perceived parental bonding, illness perception, and anxiety and to determine the influences of perceived parental bonding and illness perception on anxiety in adult patients with congenital heart diseases. Methods: In this study a descriptive correlational design with survey method was utilized. The participants were 143 adult patients with congenital heart disease being cared for in the cardiology out-patient clinic of A medical center. Data were collected using the Parental Bonding Instrument, Illness Perception Questionnaire Revised Scale, and Cardiac Anxiety Questionnaire Scale. Data were analyzed using descriptive statistics, independent t-test, one-way ANOVA, Pearson correlation analysis, and hierarchial regression analyses. Results: There showed significant positive relationships of anxiety with maternal overprotection, consequences, and personal control respectively. Among predictors, maternal overprotection (${\beta}=.45$), consequence (${\beta}=.26$), and personal control (${\beta}=-.03$) had statistically significant influence on anxiety. Conclusion: Nursing interventions to decrease maternal overprotection and negative consequence, and to enhance personal control are essential to decrease the anxiety of adult patients with congenital heart diseases.