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.

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

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

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

BACKGROUND:Cytoprotective effect of Na+/H+ exchanger type 1 (NHE1) inhibitors has been studied in ischemic/reperfusion (IR) injury. The aim of this study was to evaluate the renoprotective effect and the mechanism of NHE1 inhibitor (Cariporide(R)) on IR injury of rat kidney. METHODS:IR injury was produced by clamping both renal arteries and then rats were treated with intravenous (IV) Cariporide(R) (0.5 or 1.0 mg/kg) in Sprague-Dawley rats. The effects of Cariporide(R) treatment on subsequent IR injury were evaluated in terms of renal function, tubular injury, inflammatory cytokines (IL-1beta, TNF-alpha), apoptosis, and the expression of MAPKs. RESULTS:BUN and serum creatinine increased after IR injury compared with sham-operated controls. However, treatment with Cariporide(R) significantly reduced BUN and serum creatinine. IR injury caused severe destruction of renal tubular cells in the outer medulla, but treatment with Cariporide(R) decreased the tubular damage. Treatment with Cariporide(R) also significantly decreased the expression of IL-1beta and TNF-alpha mRNA compared with IR injury. Apoptotic cell death was increased with I/R injury, but was significantly decreased in kidneys treated with Cariporide(R). At molecular basis, caspase 3 protein decreased more in Cariporide(R)-treated group than in IR injury group. The expression of MAPKs significantly increased with IR injury compared with sham- operated controls. However, kidneys treated with Cariporide(R) showed further increase of ERK expression compared with IR injury, but showed a significant decrease of JNK expression. CONCLUSIONS:NHE1 inhibitors, Cariporide(R), partially prevented IR injury-induced acute renal failure by the mechanism involving apoptosis, inflammation and MAPKs.
Acute Kidney Injury ; Animals ; Apoptosis ; Caspase 3 ; Cell Death ; Constriction ; Creatinine ; Cytokines ; Inflammation ; Kidney* ; Rats* ; Rats, Sprague-Dawley ; Renal Artery ; RNA, Messenger ; Tumor Necrosis Factor-alpha

OBJECTIVE: To correlate existing evaluation tools with clinical information on Duchenne muscular dystrophy (DMD) patients following age and to investigate genetic mutation and its relationship with clinical function. METHOD: The medical records of 121 children with DMD who had visited the pediatric rehabilitation clinic from 2006 to 2009 were reviewed. The mean patient age was 9.9+/-3.4 years and all subjects were male. Collected data included Brooke scale, Vignos scale, bilateral shoulder abductor and knee extensor muscles power, passive range of motion (PROM) of ankle dorsi-flexion, angle of scoliosis, peak cough flow (PCF), fractional shortening (FS), genetic abnormalities, and use of steroid. RESULTS: The Brooke and Vignos scales were linearly increased with age (Brooke (y1), Vignos (y2), age (x), y1=0.345x-1.221, RBrooke2=0.435, y2=0.813x-3.079, RVignos2=0.558, p<0.001). In relation to the PROM of ankle dorsi-flexion, there was a linear decrease in both ankles (right and left R2=0.364, 0.372, p<0.001). Muscle power, Cobb angle, PCF, and FS showed diversity in their degrees, irrespective of age. The genetic test for dystrophin identified exon deletions in 58.0% (69/119), duplications in 9.2% (11/119), and no deletions or duplications in 32.8% (39/119). Statistically, the genetic abnormalities and use of steroid were not definitely associated with functional scale. CONCLUSION: The Brooke scale, Vignos scale and PROM of ankle dorsi-flexion were partially available to assess DMD patients. However, this study demonstrates the limitations of preexisting scales and clinical parameters incomprehensively reflecting functional changes of DMD patients.
Animals ; Ankle ; Child ; Cough ; Dystrophin ; Exons ; Gene Deletion ; Humans ; Knee ; Male ; Medical Records ; Muscles ; Muscular Dystrophy, Duchenne ; Range of Motion, Articular ; Scoliosis ; Shoulder ; Weights and Measures

OBJECTIVE: To evaluate the efficacy of GnRH antagonist multiple dose protocol (MDP) in controlled ovarian hyperstimulation (COH) for in vitro fertilization and embryo transfer (IVF-ET) comparing with the standard GnRH agonist long protocol (GnRH-a LP). METHODS: From January 2000 to September 2002, 57 infertile women with tubal factor alone who had undergone IVF-ET were enrolled in the present study. Study group consisted of 28 patients in 28 cycles in which GnRH antagonist Cetrorelix 0.25 mg was given daily when the leading follicle reached 14 mm in mean diameter until the human chorionic gonadotropin (hCG) injection. Control group consisted of 29 patients in 29 cycles in which COH was performed using standard GnRH-a luteal LP. RESULTS: Patient's characteristics were comparable in both groups. Premature luteinization was not developed in all patients in each group. The number of ampules and duration of exogenous gonadotropins required were significantly lower in the study group than those in the control group (p<0.05; p<0.001, respectively). There were no significant differences in the number of follicles >or=14 mm diameter on the day of hCG injection, the number of oocytes retrieved, fertilization rate, and the number of grade I, II embryos between the two groups, but the numbers of mature oocytes retrieved and fertilized oocytes were significantly lower in the study group than in the control group (p<0.01, p<0.01). The clinical pregnancy rate seemed to be lower in the study group, but the difference did not achieve significance (28.6% vs 34.5%). There were also no differences in the miscarriage rate and multiple pregnancy rate between the two groups. CONCLUSION: This study demonstrates that GnRH antagonist Cetrorelix MDP can result in the comparable pregnancy outcome as the GnRH-a LP and furthermore reduce the total dose of gonadotropins and duration of stimulation.
Abortion, Spontaneous ; Chorionic Gonadotropin ; Embryo Transfer* ; Embryonic Structures* ; Female ; Fertilization ; Fertilization in Vitro* ; Gonadotropin-Releasing Hormone* ; Gonadotropins ; Humans ; Lutein ; Luteinization ; Oocytes ; Pregnancy ; Pregnancy Outcome ; Pregnancy Rate ; Pregnancy, Multiple