• Clinical and Experimental Pediatrics
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• v.58 no.10
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• pp.392-397
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• 2015

Purpose: Alagille syndrome is a complex hereditary disorder that is associated with cardiac, hepatic, skeletal, ocular, and facial abnormalities. Mutations in the Notch signaling pathway, such as in JAG1 and NOTCH2, play a key role in embryonic development. A cardiac or hepatic presentation is a critical factor for determining the prognosis. Methods: We conducted a retrospective study of 41 patients with Alagille syndrome or a JAG1 mutation between 1983 and 2013. Results: The first presentations were jaundice, murmur, cyanosis, and small bowel obstruction at a median age of 1.0 months (range, 0-24 months). The JAG1 mutation was found in 27 of the 28 genetically-tested patients. Cardiovascular anomalies were identified in 36 patients, chronic cholestasis was identified in 34, and liver transplantation was performed in 9. There was no significant correlation between the severity of the liver and cardiac diseases. The most common cardiovascular anomaly was peripheral pulmonary stenosis (83.3%), with 13 patients having significant hemodynamic derangement and 12 undergoing surgical repair. A total bilirubin level of >15 mg/dL with a complex surgical procedure increased the surgical mortality (P=0.022). Eight patients died after a median period of 2.67 years (range, 0.33-15 years). The groups with fetal presentation and with combined severe liver and heart disease had the poorest survival (P<0.001). Conclusion: The group with combined severe liver and heart disease had the poorest survival, and a multidisciplinary approach is necessary to improve the outcome.

• Journal of Community Nutrition
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• v.2 no.2
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• pp.119-128
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• 2000

Lifestyle and dietary behavior intervention as the primary prevention of lipid disorder seems safe and compatible with other treatments of cardiovascular diseases. Cross-sectional associations between lifestyle factors and dietary behavioral factors with plasma lipid and lipoprotein levels were analyzed in 189 middle-aged men in Suwon, Korea. Overnight fasting plasma levels of total cholesterol, high-density lipoprotein(HDL)-cholesterol, triacylglycerol and glucose were analyzed. Blood pressure and anthropometric data were also measured. Lifestyle factors such as smoking status, alcohol consumption and frequency of physical exercise were evaluated by a self-administered questionnaire. Questions regarding dietary behavior were also asked. The subjects were 43.8%${\pm}$7.9 years old, and 23.8%${\pm}$2.6kg/m$^2$. From stepwise regression analyses, significant correlates with total cholesterol level were body mass index(BMI), alcohol intake(negative), age and coffee drinking(model R$^2$=14.3%). BMI, breakfast-skipping, age, and sleeping hours were significant for triacylglycerol level(model R$^2$=15.8%). BMI, alcohol drinking(negative), age, and coffee drinking were significant for low-density lipoprotein(LDL)(model R$^2$=11.7%). Age(negative), BMI(negative), alcohol drinking, stress level(negative), physical exercise, and cigarette smoking(negative) were significant for high-density lipoprotein(HDL)(model R$^2$=12.1%). From stepwise regression analyses, excluding BMI and age as factors in the model, alcohol intake(negative) and coffee drinking were significantly correlated with total cholesterol level(model R$^2$=4.4%) : breakfast-skipping with triacylglycerol(model R$^2$=3.2%) : alcohol intake (negative) with LDL level(model R$^2$=3.4%) : alcohol intake, physical exercise and stress level(negative) with HDL level(model R$^2$=6.3%). The findings suggest that a healthy daily lifestyle and dietary behavior may have an anti-atherogenic effect by altering plasma lipid and lipoprotein levels in middle-aged Korean men. (J Community Nutrition 2(2) : 119∼128, 2000)

• Journal of Biomedical Engineering Research
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• v.18 no.3
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• pp.285-290
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• 1997

In this paper, the power spectral analysis and the fractal analysis of heart rate variability(HRV) were performed to evaluate the effects of brain lesion on cardiovascular system and autonomic function for 24 normal subjects and 22 hemiplegic patients. The ECG and respiration signals were recorded at tilt angles of $0^{\circ}$ and $70^{\circ}$ for 5 and 6 minutes successively under the condition of frequency controlled respiration (0.25Hz). For normal subjects, HR, LF component, HF component and fractral dimension of HRV were distinctly changed after orthostatic stress, whereas, for hemiplegic patients, those were little changed. Complexity and variability of heart rate of patients were smaller than those of normal subjects. Sympathetic tone of patients was higher than that in normal subjects. All of these results support that autonomic disorder and cardiovascular disturbance accompanied by brain lesion could be assessed by the power spectral analysis and fractal analysis of HRV.

• Journal of Korean Physical Therapy Science
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• v.4 no.1
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• pp.243-253
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• 1997

The purpose of this study was to determine the effect of systolic blood pressure, heart rate and myocardial oxygen consumption on 3-point nonweight bearing ambulation with axillary wooden crutch according to age. The subjects were fifty healty volunteers(22 male, 28 female), ages from 20 to 69(mean age of 43.8 years) with no history of cardiovascular or respiratory disease, diabetes mellitus, arthritis and orthopedic disorder within the past three years. This study was carried out from May 10 to August 10, 1996. The data were analyzed by percentage, mean and standard deviation and ANOVA. The results were as follows : 1. In systolic blood pressure(SBP), there was no significantly change between before walk and after 20 m in crutch walking with age, however, there did differ significantly after 40 m and 60 m in crutch walking(p<0.01, p<0.001). 2. In heat rate(HR), there was no significantly change between before walk and after 20 m, 40 m in crutch walking with age, however, there did differ significantly after 60 m in crutch walking(p<0.05). 3. In myocardial oxygen consumption($MVO_{2}$), there was no significantly change between before walk and after 20m in crutch walking with age, however, there did differ significantly after 40 m and 60 m in crutch walking(p<0.01, p<0.001). These result showed that cardiovascular system had a great effect on the olderly when 3-point nonweight bearing ambulation with axillary wooden crutch. Therefore, when train for axillary crutch in the olderly, we needed suitably basis of walking distance.

• Clinical Psychopharmacology and Neuroscience
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• v.16 no.4
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• pp.505-507
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• 2018

Clozapine may be associated with cardiovascular adverse effects including QTc prolongation and, more rarely, with myocarditis and pericarditis. Although rare, these latter cardiovascular adverse effects may be life-threatening and must be immediately recognized and treated. Several cases of clozapine related-pericarditis have been described and often it has a subtle and insidious onset with symptoms that may be often misdiagnosed with psychiatric manifestations (e.g. anxiety, panic or somatization) leading to a delayed correct diagnosis with potential fatal consequences. In the present report we describe the case of a 27-year-old girl with schizoaffective disorder taking long acting aripiprazole and valproate who developed a sudden onset clozapine-related pericarditis during titration phase that resolved with immediate clozapine discontinuation and indomethacin administration. We underline the importance of an early diagnosis of clozapine-related pericarditis and the need to have monitoring protocols to prevent this potentially fatal adverse effect especially when polypharmacy is administered to patients taking clozapine.

• Journal of Korean Academy of Oral Health
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• v.42 no.4
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• pp.238-241
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• 2018

Williams syndrome is a rare congenital disorder with various physical abnormalities and characterized by facial, oral, and dental features. Individuals with Williams syndrome typically have eating disorders in the early childhood, which lead to prolonged night feeding. Prolonged night feeding is a risk factor for rampant dental caries. Williams syndrome is caused by the microdeletion of chromosome 7, resulting in elastin deficiency. Elastin is integral to cardiovascular health. Many patients with Williams syndrome have complex cardiovascular abnormalities that must be considered a part of dental management. Complications related to cardiovascular diseases may induce adverse effects such as dangerously elevated blood pressure. This may occur in patients during stressful dental treatment. In addition, characteristics of auditory hyperalgesia and anxiety disorders among patients with William syndrome, complicate receiving routine dental management. Therefore, dental treatment under sedation or general anesthesia may be preferable for patients with Williams syndrome; in particular, patients who are very uncooperative and/or needs extensive dental treatment. A thorough evaluation of each patient's physical condition is required before making decisions regarding dental treatment. Careful monitoring and preparation for emergencies are very important during and shortly after dental treatment under general anesthesia or sedation. Monitoring is critical until vital signs have stabilized and return to normal. A 28-month-old man diagnosed as having Williams syndrome, visited the Dental Hospital of ${\bigcirc}{\bigcirc}$ University for the management of rampant dental caries. We reported on the management of this patient who had peripheral pulmonic stenosis, and received dental treatment under general anesthesia. We also reviewed the characteristics of Williams syndrome and discussed considerations for dental treatment under general anesthesia.

• Journal of Preventive Medicine and Public Health
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• v.57 no.1
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• pp.55-64
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• 2024

Objectives: This study investigated the prevalence and characteristics of comorbid conditions in patients exposed to ionizing radiation and those who were involved in the Soviet-Afghan war. Methods: This study analyzed the frequency and spectrum of morbidity and comorbidity in patients over a long-term period (30-35 years) following exposure to ionizing radiation at the Semipalatinsk nuclear test site or the Chornobyl nuclear power plant, and among participants of the Soviet-Afghan war. A cohort study, both prospective and retrospective, was conducted on 675 patients who underwent comprehensive examinations. Results: Numerical data were analyzed using the Statistica 6 program. The results are presented as the mean±standard deviation, median, and interquartile range (25-75th percentiles). The statistical significance of between-group differences was assessed using the Student t-test and Pearson chi-square test. A p-value of less than 0.05 was considered statistically significant. We found a high prevalence of cardiovascular diseases, including hypertension (55.0%) and cardiac ischemia (32.9%); these rates exceeded the average for this age group in the general population. Conclusions: The cumulative impact of causal occupational, environmental, and ultra-high stress factors in the combat zone in participants of the Soviet-Afghan war, along with common conventional factors, contributed to the formation of a specific comorbidity structure. This necessitates a rational approach to identifying early predictors of cardiovascular events and central nervous system disorders, as well as pathognomonic clinical symptoms in this patient cohort. It also underscores the importance of selecting suitable methods and strategies for implementing treatment and prevention measures.

• Journal of Chest Surgery
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• v.13 no.3
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• pp.301-305
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• 1980

Myasthenia gravis is a disorder that affects neuromuscular transmission in a way that is still poorly understood. some think that myasthenia gravis results from a reduction of available acetylcholine receptors in neuromuscular junctions, consequent to some form of autoimmune injury. Surgical interest in this disease was first aroused in 1939 when Blalock observed that some patients with thymic tumors and myasthenia gravis improved following thymectomy. This report represents two cases of myasthenia gravis. The 14-year-old girl was admitted to Korea Universtiy Hospital with chief complaintment of bilateral ptosis, diplopia, swallowing difficulty, and mastication difficulty, which were relieved by administration of edrophonium (Tensilon) chloride, given intravenously. Myasthenica gravis was confirmed and thymectomy was given. After thymectomy, symptoms were relieved but the administration of neostigmine was contijued to be needed till following 3 months. After that period, she was free from this symptoms without anticholinesterase drugs. Second case is 57 year old male who has the symptoms of diplopia, bilatreal ptosis, walking disturbance, and speech difficulty. He had thymectomy too but in thymic tissue, malignant thymoma was included. He has subjective improvement only, with no major reduction of medication requirements after thymectomy.

• Journal of Chest Surgery
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• v.13 no.4
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• pp.470-474
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• 1980

Achalasia is a neurogenic esophageal disorder, characterized by incomplete relaxation of the gastroesophageal sphincter in response to deglutition and absence of peristalsis from the body of the esophaugs. Because there is no known method by which esophageal peristalsis can be restored, therapy is directed toward the relief of dital esophageal obstruction. During the period of June 1965 to September 1980, 13 cases of achalasia were operated at the Department of Thoracic SUrgery, Seoul Natonal University Hospital. 1. Among 13 cases, 5 were male and 8 were female. 2. Esophagomyotomy was performed in 12 cases, and 1 case was treated with transverse suture of lower esophagus after longitudinal incision. 3. There was no operative mortality, but 2 cases subsequently underwent esophagogastrostomy after esophagomyotomy. 4. One of 13 cases was combined with mongolism.

• Journal of Chest Surgery
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• v.13 no.4
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• pp.490-494
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• 1980

Myasthenia gravis is a neuromuscular disorder manifested by fatigability and weakness of voluntary muscles. The basic defect in the myasthenia is reduction of available acetylcholine receptors at neuromuscuiar junctions by an autoimmune attack. Removal of the thymus gland now play an Important role in the management of this disease. We have two experiences of thymectomy for myasthenic patients. The 31-year-old housewife(J.H) was admitted to the Taegu Presbyterian Medical Center because of bilateral ptosis, mastication and swallowing difficulties. The presence of thymoma was strongly suggested by roentgenographic studies. A 99.8 gm thymoma was removed completely by total thymectomy. On the 2nd postoperative day respiratory failure developed. Tracheostomy was performed and ventilatory assist was given for 3 days. The patient was completely recovered with antichollestrase drug only. C.N. was 17-year-old girl who has the symptoms of bilateral ptosis and diplopia for 3 years. Tensilon test was positive and antichollnestrase was given for several months, but the patient showed no improvement. After thymectomy she was free from myasthenic symptoms with out anticholinestrase drug.