No abstract available.
Rubinstein-Taybi Syndrome*

No abstract available.
Humans ; Infant, Newborn*

No abstract available.
Arthritis*

PURPOSE: To evaluate myocardial conductivity to understand cardiac involvement in patients with mitochondrial disease. METHODS: We performed retrospective study on fifty-seven nonspecific mitochondrial encephalopathy patients with no clinical cardiac manifestations. The patients were diagnosed with mitochondrial respiratory chain complex defects through biochemical enzyme assays of muscle tissue. We performed standard 12-lead electrocardiography (ECG) on all patients. RESULTS: ECG abnormalities were observed in 30 patients (52.6%). Prolongation of the QTc interval (>440 ms) was seen in 19 patients (33.3%), widening of the corrected QRS interval in 15 (26.3%), and bundle branch block in four (7.0%). Atrioventricular block, premature atrial contraction and premature ventricular contraction were seen in two patients each (3.5%) and Wolff-Parkinson-White syndrome in one patient (1.8%). CONCLUSION: Given this finding, we recommend active screening with ECG in patients with mitochondrial disease even in patients without obvious cardiac manifestation.
Atrial Premature Complexes ; Atrioventricular Block ; Bundle-Branch Block ; Child ; Electrocardiography ; Electron Transport ; Enzyme Assays ; Heart ; Humans ; Mass Screening ; Mitochondria ; Mitochondrial Diseases ; Mitochondrial Encephalomyopathies ; Muscles ; Retrospective Studies ; Ventricular Premature Complexes ; Wolff-Parkinson-White Syndrome

Neuregulin-1 (NRG1) signaling participates in the synaptic plasticity, maintenance or regulation of adult brain. Although ErbB4, a key NRG1 receptor, is expressed in multiple regions in the adult animal brain, little is known about its localization in Alzheimer's disease (AD) brains. We previously reported that ErbB4 immunoreactivity showed regional difference in the hippocampus of age-matched control. In the present paper, immunohistochemical characterization of the distribution of ErbB4 receptor in the hippocampus relative to pathology staging were performed in age-matched control (Braak stage 0, n=6) and AD (Braak stage I/V, n=10). Here, we found that ErbB4 immunoreactivity was significantly increased in apoptotic hippocampal pyramidal neurons in the brains of AD patients, compared to those of age-matched control subjects. In AD brains, ErbB4 immunoreactivity was demonstrated to colocalize with the apoptotic signal Bax in apoptotic hippocampal pyramidal neurons. These results suggest that up-regulation of ErbB4 immunoreactivity in apoptotic neuron may involve in the progression of pathology of AD.
Adult ; Alzheimer Disease ; Animals ; Apoptosis ; Brain ; Hippocampus ; Humans ; Neuregulin-1 ; Neurons ; Plastics ; Up-Regulation

For many years, the histogenesis of the granular cell tumor was controversial and this resulted in the tumor being given more than 20 synonyms. Abrikossoff, reporting on the fiist granular cell tomor, favored a muscle origin, but other authora have postulated a fibroblastic or a histiocytic origin. Recently, the concept of Schwann cell origin, supported by electron microscopic studies and immunohistochemistry of S-100 protein, has gained wider recognition. Approximately 90 cases of esophageal granular cell tumor have been reported in the literature, representing about 2% incidence of all reported granular cell tumor, In Korea, only four cases of granular cell tumors were reported in the literatures including the cases occuring in the cecum, colon, and anus until now. Recently we experienced 46-years old women with granular cell tumor of the esophagus, which might be the second case in Korean literature.
Anal Canal ; Cecum ; Colon ; Esophagus* ; Female ; Fibroblasts ; Granular Cell Tumor* ; Humans ; Immunohistochemistry ; Incidence ; Korea ; Middle Aged ; S100 Proteins

For over a half centry, physicians have suspected that patients with duodenal ulcer are offered an insurance policy against gastric carcinoma. This may be used clinically so that gastric lesion in the presence of duodenal ulcer, are considered benign. However, the duodenal ulcer may coexit with gastric cancer, Although rare in incidence of coexistence of duodenal ulcer and gastric cancer, physician always should be alert in diagnosing of gastric lesion in the presence of the duodenal ulcer. Here, we present 60 years old woman with coexitence of duodenal ulcer and gastric cancer, diagnosed by endoscopy and treated surgically.
Duodenal Ulcer* ; Endoscopy ; Female ; Humans ; Incidence ; Insurance ; Middle Aged ; Stomach Neoplasms*

No abstract available.
Brain* ; Choriocarcinoma* ; Female ; Neoplasm Metastasis* ; Pregnancy

BACKGROUND: The melanocortin 4 receptor (MC4R) is involved in the regulation of homeostatic energy balance by the hypothalamus. Recent reports showed that MC4R can also control the motivation for food in association with a brain reward system, such as dopamine. We investigated the expression levels of MC4R and the dopamine D2 receptor (D2R), which is known to be related to food rewards, in both the hypothalamus and brain regions involved in food rewards. METHODS: We examined the expression levels of D2R and MC4R by dual immunofluorescence histochemistry in hypothalamic regions and in the bed nucleus of the stria terminalis (BNST), the central amygdala, and the ventral tegmental area of transgenic mice expressing enhanced green fluorescent protein under the control of the D2R gene. RESULTS: In the hypothalamic area, significant coexpression of MC4R and D2R was observed in the arcuate nucleus. We observed a significant coexpression of D2R and MC4R in the BNST, which has been suggested to be an important site for food reward. CONCLUSION: We suggest that MC4R and D2R function in the hypothalamus for control of energy homeostasis and that within the brain regions related with rewards, such as the BNST, the melanocortin system works synergistically with dopamine for the integration of food motivation in the control of feeding behaviors.
Amygdala ; Animals ; Arcuate Nucleus ; Brain* ; Dopamine* ; Eating* ; Feeding Behavior ; Fluorescent Antibody Technique ; Homeostasis ; Hypothalamus ; Mice ; Mice, Transgenic ; Motivation ; Obesity ; Receptor, Melanocortin, Type 4* ; Receptors, Dopamine D2* ; Reward ; Ventral Tegmental Area

PURPOSE: Acute lower respiratory tract infections (LRI) are important causes of pediatric morbidity and mortality. Recently among the common pathogens causing acute LRI in children respiratory viruses are apparently increasing rather than bacteria and mycoplasma in Korea. This study was aimed to define the distribution of age, seasonal variation and clinical manifestation of respiratory virus in children. METHODS: All 328 children in Seoul, who had hospitalized at the Pediatric ward of Hanyang University (138 children), Hallym University (61 children) and Sungkunkwan University (129 children) for the treatment of respiratory diseases were studied from March, 1997 to February, 1998. In nasopharyngeal aspirates obtained from these patients viral agents were detected by virus isolation and/or antigen detection by indirect immunofluorescent staining. But the subjects who was not found respiratory virus were excluded, although respiratory symptoms were present. RESULTS: 1) One or more agents were identified in 328 subjects. 2) The pathogens identified were Respiratory syncytial virus (RSV : 44.7%), influenza A virus (25.6 %), parainfluenza virus (14.6%), influenza B virus (14.3%), adenovirus (4.3%) and two or more viruses (3%). 3) Infections with RSV, parainfluenza virus and influenza A and B virus occured in epidemics, while adenovirus was isolated sporadically throughout the study period. 4) Clinical patterns of viral LRI were pneumonia (39%), bronchiolitis (34%), croup (18%), acute pharyngitis (7%) and asthma (2%). CONCLUSIONS: RSV was the most important in viral respiratory tract infection in children. Clinical manifestation and epidemic characterics were variable according to the agent. Accordingly, we should acknowledge the importance of respiratory virus to cause the repiratory tract diseases in children.
Adenoviridae ; Asthma ; Bacteria ; Bronchiolitis ; Child ; Croup ; Epidemiology* ; Herpesvirus 1, Cercopithecine ; Humans ; Influenza A virus ; Influenza B virus ; Influenza, Human ; Korea ; Mortality ; Mycoplasma ; Paramyxoviridae Infections ; Pharyngitis ; Pneumonia ; Respiratory Syncytial Viruses ; Respiratory System* ; Respiratory Tract Infections* ; Seasons ; Seoul*