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

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

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

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

The purpose of this study was to compare the nutrient intake and diet quality of postmenopausal osteoporotic women to those of control subjects, and to investigate the relationship among diet quality and bone mineral density in postmenopausal women. In this study, we classified the subjects into the postmenopausal osteoporotic women (n = 38) and control (n = 43) according to their lumbar spine bone mineral density and age. Dietary intakes, anthropometric measurements and dietary quality indices were measured and evaluated. The average age of osteoporotic and control group were 60.4 yrs and 58.3 yrs, respectively and there was no significant difference. Body weight, body mass index of osteoporotic group were significantly lower than those of control group. The average energy intake of osteoporotic and control group were 1243.3 kcal and 1475.8 kcal, respectively and there was a significant difference. The osteoporotic group consumed significantly lower quantities of protein, plant protein, vitamin A, vitamin B1, vitamin B2, folate, vitamin C, calcium, animal calcium, plant calcium, iron and zinc compared to the control group. The osteoporotic group consumed significantly lower of food, vegetables, mushrooms, fruits intakes compared to the control group. In the diet quality, protein, vitamin A, vitamin B1, vitamin B2, folate, vitamin C, calcium, iron, Zinc nutrient adequacy ratio (NAR) of osteoporotic group were significantly lower than that of control group. Mean adequacy ratio (MAR) of osteoporotic and control group were 0.63 and 0.78, respectively and there was significant difference. To evaluate nutrient density, Index of nutritional quality (INQ) was calculated by dividing nutrient content per 1,000 kcal of diet with RDA per 1,000 kcal. The average dietary variety score (DVS) of osteoporotic and control group were 22.4 and 33.2, respectively and there was significant difference. DVSs of pulses (p < 0.01), seeds (p < 0.01) and vegetables (p < 0.05) in osteoporotic group were significantly lower than those of the control. In conclusion, postmenopausal osteoporotic women had lower protein, vitamin A, folate, vitamin C, calcium, iron zinc intake quality and vegetables, mushrooms, fruits DVSs than those of the control. Therefore, to promote skeletal health enough energy and food should be consumed, and the maintenance of vitamin and mineral balance by increasing of vitamin A, folate, vitamin C, calcium, iron of intakes are very important.
Agaricales ; Animals ; Ascorbic Acid ; Body Mass Index ; Body Weight ; Bone Density ; Calcium ; Diet* ; Energy Intake ; Female ; Folic Acid ; Fruit ; Humans ; Iron ; Nutritive Value ; Plants ; Riboflavin ; Spine ; Thiamine ; Vegetables ; Vitamin A ; Vitamins ; Zinc

The purpose of this study was to estimate the daily intake of vitamin A in Korean female university students. Vitamin A intake was estimated using an inconsecutive 3-day dietary intake survey from 481 young women. Vitamin A intake values were calculated based on the data in USDA database. Average age, height, and weight of the subjects were 20.05 years, 162.13 cm and 54.38 kg, respectively. The subject's average intake of energy was 1645.67 kcal. The mean vitamin A, retinol and beta carotene intakes were 908.35 +/- 863.18 ug retinol equivalent/day, 199.19 +/- 166.00 ug/day and 3872.59 +/- 4972.17 ug/day, respectively. The 21.83% of the subjects consumed less than the Estimated Average Requirement (EAR) for vitamin A. And subjects consumed 141.69% of the Recommended Nutrient Intake (RNI) for vitamin A. Food groups consumed with high vitamin A content in our subjects included vegetables (423.96 ug RE/day), potato and starches (213.64 ug RE/day), cereals (62.60 ug RE/day), eggs (55.17 ug RE/day) and milks (53.45 ug RE/day). The major food sources of vitamin A were sweet potato, carrot, spinach, egg, and cereal, and the top 30 foods provided 89.57% of total vitamin A. Also animal-derived food provided 9.65% of the vitamin A intake from the top 30 foods. In conclusion, judging from RNI, the vitamin A intake of the Korean female university students in this study was generally adequate. The result of our study may be used as a basis for follow-up studies of vitamin A intake like assessment of vitamin A nutritional status or evaluation of carotenoid food sources in Korean young women.
beta Carotene ; Carotenoids ; Edible Grain ; Daucus carota ; Eggs ; Female ; Humans ; Ipomoea batatas ; Milk ; Nutritional Status ; Ovum ; Solanum tuberosum ; Spinacia oleracea ; Starch ; United States Department of Agriculture ; Vegetables ; Vitamin A ; Vitamins

Malignant struma ovarii is a rare tumor of the ovary. This can be diagnosed by histologically when carcinomas of the thyroid gland are excluded. We present a case of malignant struma ovarii arising from cystic teratoma. A 42-year-old female was admitted for evaluation of lower abdominal mass. Hysterectomy and both adnexectomy were performed on the basis of a clinical impression of uterine leiomyoma and an ovarian mass. The uterus revealed intramural leiomyoma, measuring 8 cm in diameter. Ovarian mass was predominantly cystic and partly solid. Microscopically, thyroid tissue with papillary structures were found in the solid area Papillae were complex, branching and randomly oriented. These papillae were infiltrated in the adjacent fibrous capsule, Clinical evidence of a primary thyroid carcinoma was not found.
Female ; Humans

Malignant struma ovarii is a rare tumor of the ovary. This can be diagnosed by histologically when carcinomas of the thyroid gland are excluded. We present a case of malignant struma ovarii arising from cystic teratoma. A 42-year-old female was admitted for evaluation of lower abdominal mass. Hysterectomy and both adnexectomy were performed on the basis of a clinical impression of uterine leiomyoma and an ovarian mass. The uterus revealed intramural leiomyoma, measuring 8 cm in diameter. Ovarian mass was predominantly cystic and partly solid. Microscopically, thyroid tissue with papillary structures were found in the solid area Papillae were complex, branching and randomly oriented. These papillae were infiltrated in the adjacent fibrous capsule, Clinical evidence of a primary thyroid carcinoma was not found.
Female ; Humans

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

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