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 evaluate nutrient intake and anthropometric parameters related to obesity in Korean female adolescents according to dietary diversity score. We analyzed data from the combined 2007-2009 Korean National Health and Nutrition Examination Survey (KNHANES). The subjects were 770 female adolescents. Nutrient intakes, Dietary Diversity Score (DDS) ) and Dietary Variety Score (DVS) were derived by using the data from the 24-recall method. The DDS was defined as the number of six food groups (cereals, meats, fruits, vegetables, dairy, fats and oils) consumed. The DVS was defined as the number of food items consumed. The average age of the subjects of the study was 15.02 years and the average height, weight, and BMI were 159.50 cm, 52.58 kg, 20.62 kg/m2, respectively. The energy and nutrients intakes, percent of the recommended intake for nutrients in DDS = 5~6 group were significantly higher than those of the other groups. Calcium and vitamin C INQ in DDS = 5~6 group were significantly higher than those of the other groups. The average DVS of the subjects was 29.33. The most frequent style of food pattern was CMDFVO (cereals, meats, dairy, fruits, vegetables, fats and oils) = 111011. In conclusion, in healthy Korean female adolescents, food diversity intake variety did beneficially affect the intakes of calcium and vitamin C. Further studies are needed to confirm these findings.
Adolescent ; Ascorbic Acid ; Calcium ; Fats ; Female ; Fruit ; Humans ; Meat ; Nutrition Surveys ; Obesity ; Vegetables

We would like to correct the affiliation for the first author.

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

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

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