Neurilemmoma (Schwannoma) is a extremely rare benign tumor which origin is thought to be of nerve sheath of peripheral, cranial and sympathetic nerve. Here we report a case of orbital neurilemmoma which has grown more than two years in the female patient of thirty years old. She visited to our hospital because of decreased visual acuity and protrusion of the right eyeball. Surgery of the orbit revealed a wall-nut sized, well encapsulated, gray colored, egg-shaped tumor which was confirmed by biopsy.
Biopsy ; Female ; Humans ; Neurilemmoma* ; Orbit* ; Visual Acuity

No abstract available.
Syringomyelia*

The purpose of the study is to investigate the changes of the blood aqueous barrier, the flow rate and IOP after cyclocryotherapy in the white rabbits. After cyclocryotherapy we injected intravenously a dose of 25mg/kg of fluorescein sodium and sampled the aqueous humor at 1 and 2 hour and measured the concentration of flouorescein sodium by means of HPLC(High Performance Liquid Chromatography). By the comparision study among the control group, 4 times cyclocryotherapy application group and 8 times cyclocryotherapy applications group during experimental period, the results were summarized as follows. 1. In the control group, the mean aqueous amount was 0.297+0.020ml, the flow rate was 3.282+0.426 ul/min. 2. In the cyclocryotherapy group, IOP was significantly decreased during experimental period. It was found that the IOP in the 8 times cyclocryotherapy application group was more decreased than that in the times cyclocryotherapy application group after 2 weeks. 3. There was marked increase of the concentration of fluorescein sodium in aqueous humour after acute cyclocryotherapy, but it was decreased to normal level at 4 weeks. The difference between the two groups was not found. 4. The flow rate was markedly decreased after acute cycrocryotherapy and was almost back to normal 4 weeks in two groups. There was difference between the two groups. 5. In Electron microscopic study, many wide intercellular gaps between the nonpigmented and pigmented epithelium are observed but the nucleus of the nonpigmented and pigmented epithelium are well preserved on the day of experiment. From 7 days the nucleus, and both nonpigmented and pigmented epithelium are markedly decreased in number. By 28 days the cytoplasm is shrunken, nucleus has disappeared, and a capillary near pig-ment epithelium obstructed but the basement membrane of thest cells is well pres-erved. 6. After cyclocryotherapy, The IOP decreased progressively during experimental period, but the flow rate was almost back to normal at 4 weeks. From the result we can deduce the outflow facility was not influenced by cyclocryotherapy. And so it was suggested from electron microscopic study that IOP lowering effect of cyclocryotherapy was due to quantitiative destruction of aqueous production.
Aqueous Humor ; Basement Membrane ; Blood-Aqueous Barrier* ; Capillaries ; Cytoplasm ; Epithelium ; Fluorescein ; Rabbits* ; Sodium

BACKGROUND: Previous studies have shown that 17beta-estradiol activates AMP-activated protein kinase (AMPK) in rodent muscle and C2C12 myotubes and that acute 17beta-estradiol treatment rapidly increases AMPK phosphorylation possibly through non-genomic effects but does not stimulate glucose uptake. Here, we investigated whether 24-hour 17beta-estradiol treatment stimulated glucose uptake and regulated the expression of genes associated with glucose and energy metabolism through the genomic effects of estrogen receptor (ER) in C2C12 myotubes. METHODS: C2C12 myotubes were treated with 17beta-estradiol for 24 hours, and activation of AMPK, uptake of glucose, and expression of genes encoding peroxisome proliferator-activated receptor γ coactivator 1α, carnitine palmitoyltransferase 1β, uncoupling protein 2, and glucose transporter 4 were examined. Furthermore, we investigated whether AMPK inhibitor (compound C) or estrogen receptor antagonist (ICI182.780) treatment reversed 17beta-estradiol-induced changes. RESULTS: We found that 24-hour treatment of C2C12 myotubes with 17beta-estradiol stimulated AMPK activation and glucose uptake and regulated the expression of genes associated with glucose and energy metabolism. Treatment of C2C12 myotubes with the estrogen receptor antagonist (ICI182.780) reversed 17beta-estradiol-induced AMPK activation, glucose uptake, and changes in the expression of target genes. Furthermore, treatment with the AMPK inhibitor (compound C) reversed 17beta-estradiol-induced glucose uptake and changes in the expression of target genes. CONCLUSION: Our results suggest that 17beta-estradiol stimulates AMPK activation and glucose uptake and regulates the expression of genes associated with glucose and energy metabolism in C2C12 myotubes through the genomic effects of ER.
AMP-Activated Protein Kinases ; Carnitine O-Palmitoyltransferase ; Energy Metabolism ; Estrogens ; Glucose Transport Proteins, Facilitative ; Glucose ; Muscle Fibers, Skeletal ; Peroxisomes ; Phosphorylation ; Rodentia

Gastrointestinal stromal tumor (GIST) is the most common non-epithelial neoplasm arising in the gastrointestinal tract, but this tumor is rarely seen in association with type 1 neurofibromatosis (NF-1). We report here on two cases of multiple GISTs of the small intestine that occurred in NF-1 patients. We also analyzed the mutations of c-kit exons 9, 11, 13 and 17 and the plateletderived growth factor receptor-alpha (PDGFRA) exons 12 and 18 in two GIST patients. Histologically, the NF-1-associated GISTs were similar to those of non-the NF-1 GISTs, but they characteristically revealed hyperplastic interstitial cells of Cajal around the GISTs. Immunohistochemically, these tumors showed strong co-expressions of CD117 and CD34. The molecular genetic analysis of the GISTs showed that all of the c-kit and PDGFRA exons that were analyzed in the GISTs of the two patients were the wild-type, suggesting a limited role for the c-kit and PDGFRA mutations in the tumorigenesis of NF-1-associated GISTs.
Carcinogenesis ; Exons ; Gastrointestinal Stromal Tumors* ; Gastrointestinal Tract ; Humans ; Interstitial Cells of Cajal ; Intestine, Small ; Molecular Biology ; Neurofibromatoses* ; Neurofibromatosis 1*

The upstream stimulatory factor 1 (USF1) gene has been shown to play an essential role as the cause of familial combined hyperlipidemia, and there are several association studies on the relationship between USF1 and metabolic disorders. In this study, we analyzed two single nucleotide polymorphisms in USF1 rs2073653 (306A>G) and rs2516840 (1748C>T) between the case (dyslipidemia or obesity) group and the control group in premenopausal females, postmenopausal females, and males among 275 Korean subjects. We observed a statistically significant difference in the GC haplotype between body mass index (BMI) > or =25 kg/m(2) and BMI <25 kg/m(2) groups in premenopausal females ( chi-square=4.23, p=0.04). It seems that the USF1 GC haplotype is associated with BMI in premenopausal Korean females.
Adult ; Aged ; *Body Mass Index ; Cholesterol, HDL/blood ; Female ; Genotype ; *Haplotypes ; Humans ; Linkage Disequilibrium ; Male ; Middle Aged ; *Polymorphism, Single Nucleotide ; Premenopause ; Upstream Stimulatory Factors/*genetics

BACKGROUND/AIMS: In chronic inflammatory conditions such as ulcerative colitis (UC), the migration of granulocytes and monocytes/macrophages from the circulation into the colonic mucosa is especially important in maintaining inflammation. The aim of this trial was to assess safety and efficacy of granulocyte and monocyte adsorption apheresis in patients with moderate-to-severe UC refractory to conventional drug therapies. METHODS: Twenty-seven patients with moderate (55.6%) to severe (44.4%) active UC refractory to conventional drug therapies who had no changes in their conventional therapy regimen in the past two weeks before the recruitment were enrolled in an open-label trial. Concomitant medications were allowed, and steroids were tapered down according to the clinical activity during the course. We used an adsorptive type extracorporeal column (Adacolumn(R); JIMRO, Takasaki, Japan), which selectively adsorb granulocytes and monocytes. Patients took five apheresis sessions, each with 60 minutes duration for 5 consecutive weeks. The primary efficacy variables were clinical disease activity, short inflammatory bowel disease questionnaire (SIBDQ), C-reactive protein (CRP), and endoscopic scores. These variables were scored at regular intervals, and analyzed at week 7 on an intention-to-treat (ITT) principles. RESULTS: At 7 weeks, 70.4% of patients showed overall improvement. Clinical disease activity (p<0.0001), endoscopic score (p<0.001), and the quality of life as assessed by SIBDQ (p<0.0001) were significantly improved after the therapy. In 56.3% of concomitant steroid users, tapering down or discontinuation of steroids was possible. Treatment was well tolerated, and no severe adverse events were observed. CONCLUSIONS: Adacolumn was very efficacious in patients with moderate-to-severe active UC refractory to conventional drug therapy, but further assessment is needed.
Adult ; Colitis, Ulcerative/*therapy ; English Abstract ; Female ; *Granulocytes ; Humans ; *Leukapheresis ; Male ; *Monocytes

BACKGROUND: The characteristic feature of pancreatic beta cells is highly developed endoplasmic reticulum (ER) due to a heavy engagement in insulin secretion. The ER serves several important function, including post-translational modification, folding, and assembly of newly synthesized secretory proteins, and its proper function is essential to cell survival. Various stress conditions can interfere with ER function. Pancreatic beta cells may be particularly vulnerable to ER stress that causes to impair insulin biosynthesis and beta cell survival through apoptosis. Glucagon like peptide-1 (GLP-1) is a new drug for treatment of type 2 diabetes and has effects on stimulation of insulin secretion and beta cell preservation. Also, it may have an antiapoptotic effect on beta cells, but detailed mechanisms are not proven. Therefore, we investigated the protective mechanism of GLP-1 in beta cells through ER stress response induced by 2-deoxy-D-glucose (2DG). METHODS: For induction of the ER stress, HIT-T15 cells (hamster beta cell line) were treated with 2DG (10 mM). Apoptosis was evaluated with MTT assay, hoechst 33342 staining and Annexin/PI flow cytometry. Expression of ER stress-related molecules was determined by real-time PCR or western blot. For blocking ER stress, we pretreated HIT-T15 cells with exendin-4 (Ex-4; GLP-1 receptor agonist) for 1 hour before stress induction. RESULTS: After induction with ER stress (2DG), beta cells were lost by apoptosis. We found that Ex-4 had a protective effect through ER stress related molecules (GRP78, GRP94, XBP-1, eIF2alpha, CHOP) modulation. Also, Ex-4 recovered the expression of insulin2 mRNA in beta cells. CONCLUSION: These results suggest that GLP-1 may protect beta cells apoptosis through ER stress modulation.
Apoptosis ; Benzimidazoles ; Blotting, Western ; Cell Survival ; Deoxyglucose ; Endoplasmic Reticulum ; Flow Cytometry ; Glucagon ; Glucagon-Like Peptide 1 ; Glucagon-Like Peptide-1 Receptor ; HSP70 Heat-Shock Proteins ; Insulin ; Insulin-Secreting Cells ; Membrane Proteins ; Peptides ; Protein Processing, Post-Translational ; Proteins ; Real-Time Polymerase Chain Reaction ; Receptors, Glucagon ; RNA, Messenger ; Venoms

A 67-year-old man presented with a 3-month history for intermittent epigastric pain and postprandial discomfort. Upper gastrointestinal endoscopy and contrast-enhanced axial computed tomographic scan demonstrated two separate tumors of the gastric antrum and the duodenal second portion, suggestive of the preoperative diagnosis of a double primary cancer. Pancreaticoduodenectomy with lymph node dissection was performed, and the microscopic features and immunohistochemical profiles of the resected specimen confirmed the diagnosis of the composite neuroendocrine carcinoma with adenocarcinoma of the stomach (mixed exocrine-endocrine carcinoma).
Adenocarcinoma* ; Aged ; Carcinoma, Neuroendocrine* ; Diagnosis ; Endoscopy, Gastrointestinal ; Humans ; Lymph Node Excision ; Neuroendocrine Tumors ; Pancreaticoduodenectomy ; Pyloric Antrum ; Stomach*

BACKGROUND/AIMS: This study compared the prognostic values of the Model for End-stage Liver Disease (MELD) and the hepatic venous pressure gradient (HVPG) in the prediction of death within 3 and 12 months in patients with decompensated liver cirrhosis. METHODS: We used data from 136 consecutive patients with decompensated cirrhosis who underwent HVPG between January 2006 and June 2008. Cox regression analysis was used to investigate the independent relationships with death of MELD and HVPG. The prognostic accuracies of MELD and HVPG were analyzed by calculating the area under the receiver operating characteristic curve (AUROC) for the occurrence of death within 3 and 12 months. RESULTS: Both MELD and HVPG were independent predictors of death [hazard ratio (HR)=1.11 and 1.12, respectively; 95% confidence interval (CI)=1.04~1.20 and 1.08-1.16]. Analysis of the AUROC demonstrated that the prognostic power did not differ between MELD and HVPG for predicting the 3-month survival (HR=0.76 and 0.68, respectively; 95% CI=0.62~0.89 and 0.52~0.84; P=0.22) or the 12-month survival (HR=0.72 and 0.73, 95% CI=0.61~0.83 and CI=0.61~0.84). CONCLUSIONS: Both MELD and HVPG are independent prognostic factors of death within 3 and 12 months in patients with decompensated liver cirrhosis, and their accuracies are similar. However, HVPG has a limited role in the prediction of death in decompensated cirrhosis due to its invasiveness and limited use.
Adult ; Aged ; Area Under Curve ; Cohort Studies ; Female ; Hepatic Veins/*physiopathology ; Humans ; Liver Cirrhosis/diagnosis/*mortality/physiopathology ; Liver Failure/diagnosis/*mortality/physiopathology ; Male ; Middle Aged ; Models, Biological ; Predictive Value of Tests ; Prognosis ; Proportional Hazards Models ; Regression Analysis ; Retrospective Studies ; Severity of Illness Index ; Survival Analysis ; Venous Pressure