Narcolepsy is chronic devastating disease that characterized by excessive daytime sleepiness, cataplexy, which often precipitated by intense emotion or excitement, hypnagogic, or hypnapompic hallucinations, sleep paralysis and nocturnal disrupted sleep. In child onset narcolepsy, the presentations of narcolepsy can be very variable, making misdiagnosis as seizure disorders or delaying diagnosis as much as several years after disease onset. For the diagnosis of narcolepsy, overnight polysomnography(PSG) and multiple sleep latency test(MSLT) should be evaluated. Test for Cerebrospinal fluid hypocretin(orexin) concentration and human leukocyte antigens(HLA) would be great helpful to confirm the narcolepsy with cataplexy even in early stage of disease in children. The mainstays of treatment are that reducing the excessive daytime sleepiness, preventing the intrusion of the REM related phenomena including cataplexy and consolidating the nighttime sleep. Central nervous system stimulators such as methylphenidate or amphetamine decrease excessive daytime sleepiness and tricyclic antidepressant(TCA) or selective serotonin reuptake inhibitors(SSRI) can prevent cataplexy. Recently, new therapeutic agents such as modafinil and sodium oxybate are emerging in clinical practice with much effectiveness. Counseling for poor school performance, social isolation and depression should be provided. Early diagnosis and treatment can greatly improve the quality of life. Awareness of excessive daytime sleepiness in children or adolescent will allow pediatricians to effectively identify hypersomnia such as narcolepsy.
Adolescent ; Amphetamine ; Benzhydryl Compounds ; Cataplexy ; Central Nervous System ; Child ; Counseling ; Depression ; Diagnostic Errors ; Disorders of Excessive Somnolence ; Early Diagnosis ; Epilepsy ; Hallucinations ; Humans ; Leukocytes ; Methylphenidate ; Narcolepsy ; Quality of Life ; Serotonin ; Sleep Paralysis ; Social Isolation ; Sodium Oxybate

BACKGROUND: Left ventricular hypertrophy is common in chronic renal failure patients and may contribute increased risk of cardiovascular morbidity and mortality. We evaluated the left ventricular morphology and function in renal transplant recipients to find the relationship between hemodynamic changes and morphologic and functional improvement after transplantation. METHODS: Serial echocardiographic evaluations were performed in 27 adults(20 men and 7 women) at the time of transplantaion and posttransplantation 1 month and 4 months. The average duration of hemodialysis was 16+/-24 months(mean+/-S.D.). RESULTS: At the time of transplantation, the hematocrit level was 21+/-6% and posttransplantation 1 month and 4 months, that was increased to 39+/-5% and 42+/-7%, respectively(p<0.001). Left ventricular mass index by echocardiography was decreased significantly from 246+/-87g/m2(pre-KT) to 169+/-38g/m2(post-KT 1 month) and 153+/-40g/m2(post-KT 4 months), respectively (p<0.001). Interventricular septal thickness and left ventricular posterior wall thickness were decreased significantly after 4 months of transplantation. Left ventricular systolic and diastolic dimensions were also decreased significantly after 1 month and 4 months of transplantation. Left ventricular volumes and cardiac output were also decreased significantly. But A/E ratio, ejection fraction and fractional shortening did not change significantly. CONCLUSION: These findings showed that pretransplant high output state was resolved radipidly(within 1 month) but the diastolic function did not improved after transplantation 1 month and 4 months.
Cardiac Output ; Echocardiography* ; Hematocrit ; Hemodynamics ; Humans ; Hypertrophy, Left Ventricular ; Kidney Failure, Chronic ; Kidney Transplantation ; Male ; Mortality ; Renal Dialysis ; Transplantation*

No abstract available.

As internet technology rapidly improves, clinical services are increasingly computerized in most hosptials. In order to effectively support patient care and clinical researches, information technologies are widely utilized to integrate clinical databases and disseminate information. However, most hospital information systems in Korea are still independently implemented and not fully integrated due to lack of standardization and security measure. In order to meet increasing demand for better patient care and upgrading clinical research, there is a need for a development and sharing of integrated clinical information system with a common database including clinical data collected from various hospitals. The purpose of this study was to develop a web-based clinical information system for management of Behcet's disease by constructing an integrated database with treatment data, test results, and patient demographic data in collaboration with several hospitals. Specifically, current treatment procedure for Behcet's disease was analyzed first and enhanced treatment procedure as well as a web-based clinical information system for management of Behcet's disease was proposed using a structured systems analysis and literature review on the shared clinical information systems. Expected benefits of the system include an improvement of consistency in patient management and reduction of duplicate prescriptions and test orders. In addition, this system can help improve communication among clinics and tertiary hospitals by sharing common clinical database. In a long run, the share system can also help reducing hospital expenditures by reducing duplicate investment on high cost equipments by sharing them among hospitals.
Cooperative Behavior ; Health Expenditures ; Hospital Information Systems ; Humans ; Information Systems* ; Internet ; Investments ; Korea ; Patient Care ; Prescriptions ; Security Measures ; Systems Analysis ; Tertiary Care Centers

BACKGROUND: The application of gel test to routine immunohematologic works brought on easier interpretation of results and better quality control over conventional tube method. Under the current Korean medical insurance system however, it is very difficult to apply gel test to all routine immunohematologic works because of high cost. We tried to assess its applicability to irregular antibody screening. MATERIALS AND METHODS: Antibody screenings of 2,005 sera from transfusion-scheduled patients were carried out using DiaMedTM LISS/Coombs gel card. Antibody identifications of screen-positive or screen-negative but incompatible cross matched sera were done by conventional tube method firstly, and then by DiaMedTM LISS/Coombs gel test or DiaMedTM NaCl/Enzyme gel test in the cases of negative results for conventional tube method. RESLUTS: Total 34 irregular antibodies (8 warm and 26 cold antibodies) were screened by gel test. For warm antibody screening, the reactivity of LISS/Coombs gel test was much higher than that of conventional tube method, and for cold antibodies, tube method or NaCl/Enzyme gel test revealed better reactivity. CONCLUSION: Antibody screening by LISS/Coombs gel test alone appears to be enough for detecting clinically significant warm antibodies.
Antibodies ; Humans ; Insurance ; Mass Screening* ; Quality Control

Bone is a complex tissue in which resorption and formation continue throughout life. The bone tissue contains various types of cells, of which the bone forming osteoblasts and bone resorbing osteoclasts are mainly responsible for bone remodeling. Periodontal disease represents example of abnormal bone remodeling. Osteoclasts are multinucleated cells present only in bone. It is believed that osteoclast progenitors are hematopoietic origin, and they are recruited from hematopoietic tissues such as bone marrow and circulating blood to bone. Cells present in the osteoclast microenvironment include marrow stromal cells, osteoblasts, macrophages, T-lymphocytes, and marrow cells. These cells produce cytokines that can affect osteoclast formation. In vitro model systems using bone marrow cultures have demonstrated that IL-1 beta, IL-3, TNF-alpha, bFGF can stimulate the formation of osteoclasts. In contrast, IL-4 inhibits osteoclast formation. Knowledge of cytokines and bFGF that affect osteoclast formation and their capacity to modulate the bone-resorbing process should provide critical insights into normal calcium homeostasis and disorders of bone turnover such as periodontal disease, osteoporosis and Paget's disease.
Bone and Bones ; Bone Marrow* ; Bone Remodeling ; Calcium ; Cytokines* ; Fibroblast Growth Factor 2 ; Homeostasis ; Interleukin-1beta ; Interleukin-3 ; Interleukin-4 ; Macrophages ; Osteoblasts ; Osteoclasts* ; Osteoporosis ; Periodontal Diseases ; Stromal Cells ; T-Lymphocytes ; Tumor Necrosis Factor-alpha

Chronic alcohol consumption causes alcoholic liver disease, which is associated with the initiation of dysregulated lipid metabolism. Recent evidences suggest that dysregulated cholesterol metabolism plays an important role in the pathogenesis of alcoholic fatty liver disease. Ecklonia stolonifera (ES), a perennial brown marine alga that belongs to the family Laminariaceae, is rich in phlorotannins. Many studies have indicated that ES has extensive pharmacological effects, such as antioxidative, hepatoprotective, and antiinflammatory effects. However, only a few studies have investigated the protective effect of ES in alcoholic fatty liver. Male Sprague-Dawley rats were randomly divided into normal diet (ND) (fed a normal diet for 10 weeks) and ethanol diet (ED) groups. Rats in the ED group were fed a Lieber-DeCarli liquid diet (containing 5% ethanol) for 10 weeks and administered ES extract (50, 100, or 200 mg/kg/day), silymarin (100 mg/kg/day), or no treatment for 4 weeks. Each treatment group comprised of eight rats. The supplementation with ES resulted in decreased serum levels of triglycerides (TGs), total cholesterol, alanine aminotransferase, and aspartate aminotransferase. In addition, there were decreases in hepatic lipid and malondialdehyde levels. Changes in liver histology, as analyzed by Oil Red O staining, showed that the ES treatment suppressed adipogenesis. In addition, the ES treatment increased the expression of fatty acid oxidation-related genes (e.g., PPAR-α and CPT-1) but decreased the expression of SREBP 1, which is a TG synthesis-related gene. These results suggest that ES extract may be useful in preventing fatty acid oxidation and reducing lipogenesis in ethanol-induced fatty liver.
Adipogenesis ; Alanine Transaminase ; Alcohol Drinking ; Animals ; Aspartate Aminotransferases ; Cholesterol ; Diet ; Ethanol ; Fatty Liver* ; Fatty Liver, Alcoholic ; Humans ; Lipid Metabolism ; Lipogenesis ; Liver ; Liver Diseases, Alcoholic ; Male ; Malondialdehyde ; Metabolism ; Rats* ; Rats, Sprague-Dawley ; Silymarin ; Triglycerides

OBJECTIVE: To investigate the influence of activin and follistatin on the expression of IGF (insulin-like growth factor)-I, II, IGFBP (insulin-like growth factor binding protein)-1, 2, and 3 mRNA in cultured mouse granulosa cells MATERIALS AND METHODS: The granulosa cells were obtained from the mouse and cultured for 6 days with 10 ng/ml of activin, 10 ng/ml of follistatin, and 10 ng/ml of activin with 10 ng/m of follistatin, respectively. The cells not treated with activin or follistatin served as control. Reverse transcription-polymerase chain reaction (RT-PCR) has been used to examine the expression of IGF-I, II, IGFBP-1, 2, and 3 mRNA. Results were analyzed with Kolmogorov-Smirnov test and analysis of variance (ANOVA) and statistical significance was defined as p<0.05. RESULTS: The expression of IGF-I and II mRNA were not different significantly. However, the expression of IGFBP-3 mRNA was significantly increased in the follistatin group compared to the control group (p<0.05) and significantly decreased in the activin with follistatin group compared to the control group (p<0.05). The expression of IGFBP-1 mRNA was seemed to be increased in the activin group and decreased in the follistatin group compared to the control group, respectively (p=0.07, p=0.07). The expression of IGFBP-2 and 3 mRNA were seemed to be decreased in the activin group compared to the control group (p=0.06, p=0.07, respectively). CONCLUSION: Activin and follistatin might play a role as regulators of mouse ovarian physiology by modulating the IGF system, especially IGFBPs.
Activins* ; Animals ; Carrier Proteins* ; Female ; Follistatin* ; Granulosa Cells* ; Insulin-Like Growth Factor Binding Protein 1 ; Insulin-Like Growth Factor Binding Protein 2 ; Insulin-Like Growth Factor Binding Protein 3 ; Insulin-Like Growth Factor Binding Proteins ; Insulin-Like Growth Factor I ; Mice* ; Physiology ; RNA, Messenger*

OBJECTIVE: To evaluate the clinical efficacy and safety of Lorelin(R), a depot form of leuprorelin acetate made in Korea, in the treatment of endometriosis. MATERIALS AND METHODS: Twenty patients with surgically proven endometriosis were recruited and followed during about 21 weeks of treatment. Lorelin(R) 3.75 mg was injected every 4 weeks after the first injection following initial operation and a total of six doses were injected to a patient. Symptom severity score, chemical battery, lipid battery, and serum levels of follicule stimulating hormone (FSH), luteinizing hormone (LH), estradiol (E2), and CA-l25 were assayed before Lorelin(R) treatment, after 3 doses, and after 6 doses. Statistical analysis was performed utilizing repeated analysis of variance (ANOVA) and results with P-value less than 0.05 were considered significant. RESULTS: The age range at initial operation was 25 to 48 and the mean age was 36.5+/-6.1 (mean+/-SD) years. Symptom severity score and serum levels of LH, FSH, E2, and CA-125 were significantly lower after 3 and 6 doses of Lorelin(R) than before treatment. Transient elevation of liver enzyme was observed in 2 patients after 3 doses of Lorelin(R). Side effects were mainly due to treatment-induced hypoestrogenism and the most frequent symptom was hot flush (55%), vaginal dryness (30%), transient nausea sense (25%), and arthralgia (25%). All patients were able to tolerate these symptoms and no one discontinued Lorelin(R) therapy. CONCLUSION: This study suggests that Lorelin(R) could be an effective and safe regimen in the treatment of endometriosis.
Arthralgia ; Endometriosis* ; Estradiol ; Female ; Humans ; Korea ; Leuprolide* ; Liver ; Luteinizing Hormone ; Nausea

BACKGROUND: Ultrafiltration failure resulting from increased peritoneal vascular permeability during peritonitis is a major problem in CAPD patients. However, the mechanism of increased peritoneal permeability during peritonitis has not been clearly determined. We studied changes in the peritoneal permeability and the expression of vascular endothelial growth factor(VEGF), which is known to increase vascular permeability, in the peritoneal tissues of rats with peritonitis. METHODS: After implanting peritoneal dialysis catheter to 20 Sprague-Dawley rats and performing peritoneal equilibration test(PET), rats were divided into control group(n=7) and peritonitis group(n=13). One ml of saline or Staphylococcus aureus(1X10(9) colony forming unit/mL) was injected intraperitoneally to control or peritonitis group for five days, and PET was repeated. Peritoneal transport rates of glucose and total protein were measured, and macrophages infiltration and VEGF expression in peritoneal tissues were examined by immunohistochemical stain. RESULTS: Peritoneal transport rates of glucose and total protein were significantly increased in peritonitis group compared with control group, suggesting that peritonitis increased peritoneal transport rates of both low and high molecular weight solutes. The peritoneal tissues of peritonitis rats showed profuse infiltration of macrophages in the submesothelial space, submesothelial widening and denudation of mesothelial cells. While VEGF was slightly expressed in peritoneal mesothelial cells in control rats, it was intensively stained not only in the peritoneal mesothelial cells but also in the infiltrated macrophages in the submesothelial space in peritonitis rats. CONCLUSION: Peritonitis increases peritoneal vascular permeability and VEGF expression in peritoneal mesothelial cells and infiltrated macrophages in the submesothelial space. These data suggest that VEGF may play a role in the increased peritoneal permeability during peritonitis.
Animals ; Capillary Permeability ; Catheters ; Glucose ; Humans ; Macrophages ; Molecular Weight ; Peritoneal Dialysis ; Peritoneal Dialysis, Continuous Ambulatory ; Peritonitis* ; Permeability ; Rats* ; Rats, Sprague-Dawley ; Staphylococcus ; Ultrafiltration ; Vascular Endothelial Growth Factor A*