BACKGROUND AND OBJECTIVES: Idiopathic restrictive cardiomyopathy is a very rare and poorly recognized disease in children. This study is performed to describe the clinical course and to define potential predictors of outcome. MATERIAL AND METHOD: We reviewed the medical records and diagnostic studies of 11 consecutive patients during the period from Jan.1991 to Aug. 2000. RESULTS: The age at diagnosis was 1.2-13 years (median 7 years) and the duration of follow up was 3-90 months (median 3.6 years). All except one were symptomatic (dyspnea in ten, chest pain in four). The chest pain was associated with significant ST depression on both resting and exercise ECG, suggesting myocardial ischemia. Two had complete heart block as either initial or terminal event. Cardiac catheterization was done in nine ( mean pulmonary arterial wedge pressure 23+/-6mmHg, systolic pulmonary arterial pressure 47+/-14mmHg, mean right atrial pressure 11+/-9mmHg). Echocardiographic dimensional ratio of left atrium and aorta (LA/Ao) was 2.41+/-0.58. Mitral E/A inflow ratio was 2.72+/-1.42, E wave deceleration time was 93.6+/-44.2ms. During follow up, six died. The 2 year and 5 year cumulative survival rates were 54.5% and 18.8% respectively. The predictor for nonsurvivor were pulmonary venous congestion and LA/Ao >2.5(p<0.05). Verapamil was tried in 6 cases without favorable effect in all. CONCLUSION: Considerable numbers of restrictive cardiomyopathy have myocardial ischemia associated with ST depression and chest pain. The patients with pulmonary venous congestion and severe left atrial enlargement (LA/Ao>2.5) were at risk for death, requiring prompt definitive treatment such as cardiac transplantation.
Aorta ; Arterial Pressure ; Atrial Pressure ; Cardiac Catheterization ; Cardiac Catheters ; Cardiomyopathy, Restrictive* ; Chest Pain ; Child* ; Deceleration ; Depression ; Diagnosis ; Echocardiography ; Electrocardiography ; Follow-Up Studies ; Heart Atria ; Heart Block ; Heart Transplantation ; Humans ; Hyperemia ; Medical Records ; Myocardial Ischemia ; Pulmonary Wedge Pressure ; Survival Rate ; Verapamil

We reviewed 26 cases of cardiac rhabdomyoma in children which were diagnosed between 1986 and 1991 in Seoul National University Children's Hospital. Twenty-two cases were associated with tuberous sclerosis. Total tumor count was 58 and mean tumor count per patient was "daehakgyo".23. Their cardiac rhabdomyomas were diagnosed from fetal period through 12 years after birth. Median age was 9."uiwon" months. Fifteen patients were males and rest 11 patients were females. Cardiac symtoms and signs at initial diagnosis were present in 12 cases. They consisted of cardiac murmur in "hakgyo", arrhythmia in 8 and congestive heart failure in "daehakgyo" cases (one with murmur and the other with arrhythmia). Cardiomegaly was seen in 9 cases on chest X-ray and other "byeongwon" case showed abnormal left cardiac border. Electrocardiography and 24 hour Holter monitorings showed arrhythmia in 15 cases. Clinically significant arrhythmias were found in 7 cases on 24 hour Holter monitorings. They consisted of 1with frequent premature ventricular contractions, "daehakgyo uigwadaehak" with frequent premature atrial contractions, "daehakgyo" with atrial tachycardia and "daehakgyo uigwadaehak" with atrio-ventricular block ("daehakgyo" cases of atrio-ventricular block had other arrhythmias also). The locations of tumors were left ventricle in 21, interventricular septum in 21, right ventricle in 13 and atrium in "daehakgyo uigwadaehak" tumors. Mean tumor count per patient in fetal period and infancy was "daehakgyo".86 and it was statistically much more than that after infancy which was "byeongwon".5. Mean tumor area which was calculated on two dimensional echocardiography was larger in fetal period and infancy(128.9 +/- 38.2mm2)than that after infancy(47.8 +/- 11.2mm2). Mean relative tumor area to aortic root dimension was also significantly larger in fetal period and infancy than that after infancy ("daehakgyo".07vs 0.30). In tuberous sclerosis, mean mass area and mean relartive tumor area to aortic root dimension in fetal period and infancy was significantly larger than those in patients after infancy. Of 26 cases, tumorectomy was done in "daehakgyo uigwadaehak" cases due to hemodynamic obstruction and arrhythmia. One case died immediately after operation and the other "daehakgyo" cases are still alive with the follow up period of 30 and 44 months each. Of the 7 patients with significant arrhythmia on 24 hour Holter monitorings, pacemaker insertion was done in "byeongwon" case who received tumorectomy and other 1 case was treated with anti-arrhythmic drugs. All the other patients did not need anti-arrhythmic drugs or other management.
Arrhythmias, Cardiac ; Atrial Premature Complexes ; Cardiomegaly ; Child* ; Diagnosis ; Echocardiography ; Electrocardiography ; Female ; Follow-Up Studies ; Heart Failure ; Heart Murmurs ; Heart Ventricles ; Hemodynamics ; Humans ; Male ; Parturition ; Rhabdomyoma* ; Seoul ; Tachycardia ; Thorax ; Tuberous Sclerosis ; Ventricular Premature Complexes

No abstract available.
Child* ; Heart Neoplasms* ; Humans

Infantile hemangioendothelioma of the liver is a common vascular tumor in infancy. The tumor is usually multinodular or diffuse and classified into two types. We present a case of infantile hemangioendothelioma of the liver, which predominantly consists of type 2. A 4-month-old female was admitted for an evaulation of an abdominal distension. A CT scan of the liver showed a multinodular mass. The right lobectomy was done. Grossly, the mass consisted of round nodules ranging from 2cm to 5cm in diameter. Microscopically, the tumor revealed proliferation of small vascular channels lined by endothelial cells. Bizarre cells and mitotic cells were frequently noted. Vesicular nuclei and multilayering of the endothelial cells were also noted.
Endothelial Cells ; Female ; Hemangioendothelioma* ; Humans ; Infant ; Liver* ; Tomography, X-Ray Computed

This study was conducted to investigate the effects of smoking on nutrition intake, bone mineral density and blood mineral status. The subjects were composed of two groups: 100 smokers and 100 non-smokers was used along with a questionnaire. Anthropometric measurements, dietary intakes using 24-hours recall method were compared. At twenty subjects from each group were selected, bone mineral density were measured by quantitative ultrasound and plasma mineral levels were analyzed by ICP spectrometer. The average ages of smokers and non-smokers were 23.9 and 22.8 years old, respectively. The height, weight and BMI of the smokers were no significant difference. The average numbers of smoked cigarettes were 11.8/d and the average packyear was 3.1 in the smokers. About 97% of the smokers drank alcoholic beverages, while 85% of the non-smokers did. The smokers tended to eat less meals and dinner meal, but drink coffee more often compared to the non-smokers. The mean daily energy intake and CPF energy intake ratio were 2184. 9 kcal and 57.2 : 13.7 : 26.4 in the smokers and 2262.6 kcal and 55.8 : 13.1 : 28.6 in the non-smokers. The smokers consumed significantly lower intake of beta-carotene. There were no significant differences in bone mineral density of BUA, SOS and QUI. There were no significant differences in plasma levels of Ca, Mg, Fe and Zn. However, plasma Cu level of the smokers was significantly higher than that of the non-smokers. In conclusion, the smokers of this study showed a more undesirable dietary intake in the light of their low beta-carotene and high alcoholic beverages, and coffee. The plasma Cu level of the smokers was higher than that of the non-smokers, showing that Cu is involved in smoking. Therefore, it could be suggested that more systematic research be conducted with respect to Cu and smoking and that increased nutrition education and guidelines for smokers are required.
Adult* ; Alcoholic Beverages ; beta Carotene ; Bone Density* ; Coffee ; Education ; Energy Intake ; Humans ; Male* ; Meals ; Nutritional Status* ; Plasma* ; Smoke* ; Smoking* ; Tobacco Products ; Ultrasonography

Epstein-Barr virus (EBV) has been known to be associated with a wide variety of neoplastic conditions including nasopharyngeal carcinoma, Hodgkin's disease, and non-Hodgkin's lymphoma. Recent studies reveal the presence of EBV in certain subtypes of gastric carcinoma in which EBV appears to be pathogenetically related. To evaluate the relationship between EBV and gastric adenocarcinoma, we examined EBV DNA using direct in situ polymerase chain reaction, and expression of p53 protein and bcl-2 protein using immunohistochemical staining method on paraffin embedded tissues. The materials consisted of one hundred twenty-eight gastric adenocarcinomas and twenty benign peptic ulcers. EBV DNA was detected in 14 of 128 gastric adenocarcinomas (10.9%). p53 protein was positive in 10 of 14 EBV positive adenocarcinomas (71.4%) and in 61 of 114 EBV negative adenocarcinomas (53.5%). Bcl-2 protein was positive in 2 of 14 EBV positive adenocarcinomas (14.3%) and in 19 of 114 EBV negative adenocarcinomas (16.7%). The above results indicate that EBV is associated with gastric adenocarcinoma, and p53 protein may play a role in carcinogenesis of EBV in gastric adenocarcinoma.
Adenocarcinoma* ; Carcinogenesis ; DNA ; Herpesvirus 4, Human* ; Hodgkin Disease ; Lymphoma, Non-Hodgkin ; Paraffin ; Peptic Ulcer ; Polymerase Chain Reaction

BACKGROUND: Diabetic ketoacidosis(DKA) is serious acute metabolic complication and the most important cause of high morbidity and mortality of diabetes. The object of this study is to examine the clinical characteristics of patients with DKA who had a prior history of diabetes or not. METHOD: Authors reviewed retrospectively the medical records of 49 cases adimitted to Kyungpook National University Hospital from January 1991 to June 1997 with a diagnosis of DKA and classified cases as type I, type II and newly diagnosed diabetics according to prior history of diabetes. RESULTS: 1. Of 49 cases of DKA, 24(49%) were classified as type I, 17(35%) as type II from data available in the medical records, and 8(16%) had DKA as the initial manifestation of the disease. 2. The male to female ratio was 0.5 : 1 in type I, 1.1 : 1 in type II and 1.7 : 1 in newly diagnosed diabetics, and the mean age was 24.4 in type I, 57.9 in type II and 23.9 years old in newly diagnosed diabetics. 3. The mean duration between initial diagnosis of diabetes and the occurrence of DKA was 2.6 in type I and 6.9 years in type II diabetes. The occurrence of DKA within 2 years of initial diagnosis of diabetes was 54% in type I and 18% in type II diabetes, but the occurrence of DKA after 5 years of initial diagnosis of diabetes was 17% in type I and 47% in type II diabetes. 4. The precipitating factors of DKA were identified in 88% in type I, 76% in type II and 38% in newly diagnosed diabetics, and the most common precipitating factor was omission of treatment in both type I and type II(type I: 56%, type II: 35%). 5. The altered mental status was correlated with increased osmolality (p<0.05), but not with other laboratory values such as pH, bicabonate, glucose, anion gap and dehydration status(p>0.05). CONCLUSION: It is necessary to conduct early aggressive evaluation for early diagnosis and proper treatment of DKA, because DKA occurs in patients with prior history of type II diabetes and without prior diabetic history as well as patients with prior history of type I diabetes.
Acid-Base Equilibrium ; Dehydration ; Diagnosis ; Early Diagnosis ; Female ; Glucose ; Gyeongsangbuk-do ; Humans ; Hydrogen-Ion Concentration ; Male ; Medical Records ; Mortality ; Osmolar Concentration ; Precipitating Factors ; Retrospective Studies

Maligant melanomas of the oral or nasal cavity, and the vulvovaginal area are relatively common among the melanomas of non-ocular mucosa. But, primary malignant melanoma arising in the mucosa of the palatine tonsil is rare. We present a case of primary malignant melanoma arising in the mucosa of the palatine tonsil. A 36-year-old male was admitted for evaluation of a recurrent sore throat. Tonsillectomy was performed on the basis of clinical suspicion of chronic tonsillitis. Grossly, the left tonsil was focally dark. Microscopically, the tonsillar mucosa was diffusely infiltrated with tumor cells. Tumor cells revealed numerous melanin pigments. Intraepithelial nests of tumor cells were noted, but pagetoid spread of tumor cells was not found. Tumor cells were positive for S-100 protein and HMB45 stain. There was no evidence of melanoma in the skin or eye.
Male ; Humans

PURPOSE: The aim of this study was to investigate the relationship between instant noodle intake and metabolic factors in Korean adults. METHODS: Study subjects were 5,894 (male 2,293, female 3,601) aged 19~64 years who participated in the 2013~2014 KNHANES. Information on frequency and consumption of instant noodles was obtained by the food frequency questionnaires method in KNHANES (Korean National Health and Nutrition Examination Survey), and subjects were classified according to age, sex, and instant noodle consumption (INC). RESULTS: The frequency and consumption of instant noodles was 1.2 times/week and 1.2 servings in subjects. High INC group (≥ 1 serving/week) was significantly younger in age compared with the low INC group (< 1 serving/week). However, the high INC group had significantly higher waist circumference, metabolic factors (triglyceride, cholesterol, and HDL-cholesterol), and dietary intake (energy intake, fat, and sodium density) compared with the low INC group. Hyperglycemia showed association with higher risk of highest quartile of INC after adjustments for multiple confounding factors, including age, gender, household income, education, smoking, and alcohol compared with the lowest quartile (OR: 1.4, 95% CI: 1.1-1.8). In female, abdominal obesity showed association with higher risk of highest quartile of INC after adjustments for multiple confounding factors compared with the lowest quartile (OR: 1.6, 95% CI: 1.2-2.2). CONCLUSION: Consumption of instant noodles was associated with increased prevalence of abdominal obesity and hyperglycemia in women. These findings suggest an association of instant noodle consumption status with metabolic risk
Adult ; Cholesterol ; Education ; Family Characteristics ; Female ; Humans ; Hyperglycemia ; Korea* ; Methods ; Nutrition Surveys* ; Obesity, Abdominal ; Prevalence ; Smoke ; Smoking ; Sodium ; Waist Circumference

Recent studies have suggested that natural agents such as isoflavones, resveratrol, and anthocyanin have beneficial effects on metabolic syndrome-related disorders. The objective of this study was to evaluate dietary isoflavone intake, urinary isoflavone level, and their relationship with the risk of metabolic syndrome (MetS) among Korean postmenopausal women. The subjects included 46 MetS and 60 controls. The MetS risk score was determined by adding the number of risk factors such as waist circumference, blood pressure (BP) and levels of triglyceride (TG), HDL-cholesterol, and glucose. Dietary isoflavone intake was not significantly different between the MetS and control groups; however, the urinary daidzein level was significantly higher in the MetS subjects compared to that of the controls. Subjects with high TG had higher urinary daidzein and isoflavone (daidzein + genistein) levels than those without such abnormalities. But, the MetS risk score showed no significant correlation with urinary daidzein, genistein, and isoflavone excretions.
Anthocyanins ; Blood Pressure ; Female ; Genistein ; Glucose ; Humans ; Isoflavones ; Risk Factors ; Triglycerides ; Waist Circumference