Cholesteatoma of the upper urinary tract is a rare nonmalignant condition histologically characterized by keratinizing desquamative squamous metaplasia (KDSM). Until now about 80 cases including one case from Korea have been reported. Cholesteatoma shows microscopic features of squamous metaplasia of the transitional epithelium and keratinization. These changes with subsequent desquamation of the superficial epithelial layers explain clinical manifestations of the disease. Flank pain, passage of cornified material in the urine and a filling defect on the IVP constitute the characteristic triad. Most cases were managed by extensive ablative surgery for fear of malignant potential. We report a case of renal cholesteatoma which was misdiagnosed as renal stone in 58 year-old female patient who had been suffered from intermittent left flank pain.
Cholesteatoma* ; Epithelium ; Female ; Flank Pain ; Humans ; Korea ; Metaplasia ; Middle Aged ; Urinary Tract

PURPOSE: This study was aimed to investigate the relationship between the mandibular asymmetry and the internal derangement of temporomandibular joint. MATERIALS AND METHODS: One hundred and sixty eight patients had been assessed through clinical examinations, panoramic radi-ographs and magnetic resonance imagings (MRIs), were selected. The samples were classified into three subgroups according to the severity of the mandibular asymmetries in the panoramic radiographs and the status of TMJ discs on the MRI were compared among each groups. RESULTS: In an apparent asymmetry group, there was a significant difference in the number of temporomandibular disk displacement without reduction between the long and short side (66.7%, 18/27 joints on the short side) when the ratio of condylar process and coro-noid process was used (P<0.05), but there was no statistically significant difference when the ratio of condyle and ramus was used. CONCLUSION: The probability of the disc displacement without reduction was higher at the side with relatively shorter condylar process on the panoramic radiograph, and also it might be more effective to use ratio of condylar process and coronoid process in the assessment of mandibular asymmetry. Therefore, a careful assessment on the temporomandibular disorders is necessary to diagnose and establish the treatment plans for the patients with a mandibular asymmetry and the panoramic radiograph can be used effectively on that way.
Humans ; Joints ; Magnetic Resonance Imaging* ; Temporomandibular Joint Disc* ; Temporomandibular Joint Disorders ; Temporomandibular Joint*

BACKGROUND AND PURPOSE: The level of 14-3-3 protein in the cerebrospinal fluid (CSF) is increased in Creutzfeldt-Jakob disease (CJD) patients, which has led to it being used as a clinical biomarker for the ante-mortem diagnosis of human prion diseases. However, the specificity of the 14-3-3 protein is less reliable for CJD diagnosis. Newly developed assays including real-time quaking-induced conversion (RT-QuIC) have made it possible to detect the PrPSc-like abnormal prion isoform with a high sensitivity in animal and human specimens that might contain a minute amount of PrP(Sc) due to in vitro prion replication. METHODS: This study applied a highly sensitive RT-QuIC assay using recombinant human PrP to detect PrP(Sc) in the CSF of 81 patients with sporadic CJD (sCJD) in Korea. RESULTS: RT-QuIC analysis of the CSF samples based on the expression levels of 14-3-3 and total tau proteins revealed positivity in 62 of 81 sCJD patients (sensitivity of 76.5%) but no positive results in the 100 non-CJD patients. CONCLUSIONS: The sensitivity of the RT-QuIC in this study was similar to that in some previous reports, and the specificity of RT-QuIC was higher than that of 14-3-3 in CSF, suggesting that RT-QuIC analysis can complement the weakness of the specificity of 14-3-3 for the diagnosis of sCJD. These results indicate that RT-QuIC might be very useful for the rapid and specific diagnosis of sCJD and provide a practical novel method for the ante-mortem diagnosis of human prion diseases.
14-3-3 Proteins ; Animals ; Cerebrospinal Fluid ; Complement System Proteins ; Creutzfeldt-Jakob Syndrome* ; Diagnosis* ; Humans ; Korea* ; Prion Diseases ; Sensitivity and Specificity ; tau Proteins

Scrapie is a mammalian transmissible spongiform encephalopathy or prion disease that predominantly affects sheep and goats. Scrapie has been shown to overcome the species barrier via experimental infection of other rodents. To confirm the re-transmissibility of the mouse-adapted ME7 scrapie strain to ovine prion protein (PrP) transgenic mice, mice of an ovinized transgenic mouse line carrying the Suffolk sheep PrP gene that contained the A₁₃₆ R₁₅₄ Q₁₇₁/ARQ allele were intracerebrally inoculated with brain homogenates obtained from terminally ill ME7-infected C57BL/6J mice. Herein, we report that the mouse-adapted ME7 scrapie strain was successfully re-transmitted to the transgenic mice expressing ovine PrP. In addition, we observed changes in the incubation period, glycoform profile, and pattern of scrapie PrP (PrP(Sc)) deposition in the affected brains. PrP(Sc) deposition in the hippocampal region of the brain of 2nd-passaged ovine PrP transgenic mice was accompanied by plaque formation. These results reveal that the mouse-adapted ME7 scrapie strain has the capacity to act as a template for the conversion of ovine normal monomeric precursors into a pathogenic form in ovine PrP transgenic mice. The change in glycoform pattern and the deposition of plaques in the hippocampal region of the brain of the 2nd-passaged PrP transgenic mice are most likely cellular PrP species dependent rather than being ME7 scrapie strain encoded.
Alleles ; Animals ; Brain ; Gliosis ; Goats ; Humans ; Mice ; Mice, Transgenic ; Plaque, Amyloid ; Prion Diseases ; PrPSc Proteins ; Rodentia ; Scrapie ; Sheep ; Terminally Ill

PURPOSE: Coiling or infusion of embolic materials into a wide necked aneurysm can be performed with stenting. The purpose of our study is to assess the technical feasibility of aneurysm treatment with glue embolization after stenting. MATERIALS AND METHODS: We used four Wallstents for surgically repairing eight canine carotid aneurysms. After confirmation of the aneurysms on the angiogram, we introduced a 6-7 F guiding catheter in order to deploy the stents. After stenting, we passed a microcatheter into the aneurysm lumen through the stent mesh. 28% glue was slowly injected until the glue cast completely filled the lumen. We evaluated the passage of a microcatheter through the stent meshwork, formation of the glue cast and the stents' ability to protection for any leakage of glue. The follow-up angiogram was obtained for two dogs, one to three times until 8 weeks, and then we sacrificed the dogs and performed pathologic examinations. RESULTS: Stenting was successful in all cases except one in which the vessel was occluded because the stent was not completely expanded within the lumen. The microcatheter could not pass through the stent mesh in one aneurysm. The two week follow-up angiogram showed complete occlusion of the aneurysm and a patent carotid lumen in a case after successful stenting and glue embolization without distal migration of glue. Tungsten in the glue was noted to migrate out of aneurysm into the soft tissue of the neck. Histopathologic examination showed successful obliteration and stable organization of the aneurysmal lumen with ingrowth of fibroblasts and a foreign body reaction. In contrast, the aneurysms without the glue embolization being performed showed partially thrombosed aneurysmal lumens that became smaller and indistinct on the 8 week follow-up angiograms. Histopathologic examination showed a disorganized thrombus with numerous recanalizations. CONCLUSION: Glue embolization after stenting could be performed for aneurysm without distal migration of the glue or gluing of the catheter. This concept appears to be useful for applications to the further research and the treatment of aneurysm.
Adhesives* ; Aneurysm* ; Animals ; Carotid Arteries* ; Catheters ; Dogs ; Fibroblasts ; Follow-Up Studies ; Foreign-Body Reaction ; Intracranial Aneurysm ; Neck ; Stents* ; Thrombosis ; Tungsten

Gigantomastia is a rare condition characterized by excessive breast growth. It has been reported that the majority of gigantomastia cases occur during either pregnancy or puberty. We were presented with a rare case of gigantomastia associated with neither pregnancy nor puberty, and successfully treated it with reduction mammaplasty and free nipple graft. This idiopathic gigantomastia is the very first case in Korea, and adds to the worldwide total of 9 reported cases.
Adolescent ; Breast ; Female ; Humans ; Korea ; Mammaplasty ; Nipples ; Pregnancy ; Puberty ; Transplants

Infliximab (IFX) is an anti-tumor necrosis factor (TNF) monoclonal antibody used to treat rheumatoid arthritis, ankylosing spondylitis, and Crohn's disease. Rarely, anti-TNF-induced lupus (ATIL) may occur. ATIL differs from classical drug-induced lupus. We report a 49-year-old woman who developed polyarthralgia after 2 years of IFX treatment for Crohn's disease. Based on the autoantibody profiles, ATIL was diagnosed and low-dose glucocorticoid, hydroxychloroquine, and celecoxib were prescribed. However, arthralgia and hemolytic anemia developed. Because the anti-dsDNA titers waxed and waned, she was switched to vedolizumab, a monoclonal antibody to the human lymphocyte α4β7 integrin. Six months after switching treatment, the arthralgia had improved and the anti-dsDNA antibody normalized. Here, we report a case of ATIL that resolved after switching from infliximab to vedolizumab.
Anemia, Hemolytic ; Arthralgia ; Arthritis, Rheumatoid ; Celecoxib ; Crohn Disease ; Female ; Humans ; Hydroxychloroquine ; Infliximab ; Lupus Erythematosus, Systemic ; Lymphocytes ; Middle Aged ; Necrosis ; Spondylitis, Ankylosing

Infliximab (IFX) is an anti-tumor necrosis factor (TNF) monoclonal antibody used to treat rheumatoid arthritis, ankylosing spondylitis, and Crohn's disease. Rarely, anti-TNF-induced lupus (ATIL) may occur. ATIL differs from classical drug-induced lupus. We report a 49-year-old woman who developed polyarthralgia after 2 years of IFX treatment for Crohn's disease. Based on the autoantibody profiles, ATIL was diagnosed and low-dose glucocorticoid, hydroxychloroquine, and celecoxib were prescribed. However, arthralgia and hemolytic anemia developed. Because the anti-dsDNA titers waxed and waned, she was switched to vedolizumab, a monoclonal antibody to the human lymphocyte α4β7 integrin. Six months after switching treatment, the arthralgia had improved and the anti-dsDNA antibody normalized. Here, we report a case of ATIL that resolved after switching from infliximab to vedolizumab.

BACKGROUND AND OBJECTIVES: Increased plasma homocysteine(tHcy) has been implicated as an independent risk factor for coronary artery diseas(CAD), but the relationship has not been firmly established. Present study aimed to determine the difference of plasma homocysteine between patients with CAD and normal control, and to identify the relation between plasma homocysteine and genotype variation of its metabolic enzymes, and serological characteristics. METHODS: Plasma homocysteine, fasting and post-methionin loading, folate and vitamin B12 were measured among 149 patients and 80 control subjects. Both group consisted of those younger than 65 years. Frequencies of prevalent mutations of enzymes involved in homocysteine metabolism, cytosine to thymidine transition (C(677)T) of methylentetrahydrofolate reductase (MTHFR) was determined by polymerase chain reaction (PCR) in 85 patients and 47 control. RESULT: There was no significant difference in homocysteine level between patients and control group (fasting tHcy; 10.4 +/- 3.6 vs 11.4 +/- 8.4 ng/ml, post-methionine loading tHcy; 18.8 +/- 4.9 vs 17.2 +/- 9.5 ng/ml, p> 0.05 respectively). Genotype frequency of MTHFR C(677)T was similar between two groups. Plasma homocysteine level did not appear to vary with genotypes of MTHFR both in patients and control subjects. Multiple linear regression analysis identified smoking as the most significant factor affecting plasma homocysteine level, followed by age, MTHFR genotype, obesity, and folate level. CONCLUSION: Homocysteine concentration was not different between controls and patients with CAD. Significant variation of homocysteine level according to genetypic polymorphism of metabolism enzymes was not observed. On multiple linear regression, several factors were identified to be related to homocysteine level, including MTHFR genotype. Further study is warranted to clarify the significance of homocysteine in the development of CAD.
Coronary Artery Disease* ; Coronary Vessels* ; Cystathionine beta-Synthase ; Cytosine ; Fasting ; Folic Acid ; Genotype* ; Homocysteine* ; Humans ; Linear Models ; Metabolism ; Methylenetetrahydrofolate Reductase (NADPH2) ; Obesity ; Oxidoreductases ; Plasma* ; Polymerase Chain Reaction ; Risk Factors* ; Smoke ; Smoking ; Thymidine ; Vitamin B 12

Glycoproteins isolated from fruit bodies and mycelial cultures of mushrooms exhibit anti-carcinogenic actions in human cancer cells and animal tumor cells by induction of apoptosis. Here, we report that isoflavone-conjugated glycoproteins (designate Gluvone), exhibit strong anti-carcinogenic effects on human breast cancer MCF-7 cells by induction of apoptosis. Gluvone with 9.4 kDa of molecular weight was isolated from submerged-liquid culture of Agaricus blazei mycelia (ABM) in soy flake-containing liquid medium. MCF-7 cells were incubated with various amounts of Gluvone (0~250 microM) for a period of 6 days. Gluvone exhibited anti-proliferative actions in a dose-dependent manner and 62% growth inhibition at 200 microM for 4 days relative to control. Hoechst 33258 staining analysis revealed that Gluvone induced formation of apoptotic bodies. Gluvone was associated with down-regulation of anti-apoptotic Bcl-2 protein expression as well as up-regulation of pro-apoptotic Bax protein expression. Gluvone treatment induced proteolytic activation of caspase-9 and caspase-3 through cytochrome c release from mitochondria to cytosol as well as concomitant degradation of poly (ADP-ribose) polymerase (PARP). In addition, Gluvone induced activation of caspase-8. Taken all together, these results indicate that the anti-proliferative effect of Gluvone is associated with induction of apoptotic cell death through the mitochondrial dysfunction pathway mediated by enhancement of Bax protein expression and suppression of Bcl-2 protein expression.
Agaricales ; Agaricus* ; Animals ; Anticarcinogenic Agents ; Apoptosis ; bcl-2-Associated X Protein* ; Bisbenzimidazole ; Breast Neoplasms ; Caspase 3 ; Caspase 8 ; Caspase 9 ; Cell Death ; Cytochromes c ; Cytosol ; Down-Regulation ; Fruit ; Glycoproteins* ; Humans ; Isoflavones* ; MCF-7 Cells ; Mitochondria ; Molecular Weight ; Up-Regulation