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Semantic variant primary progressive aphasia (svPPA), a well-known subtype of the frontotemporal dementia often shows peculiar clinical features and structural neuroimage findings. To strengthen the accuracy of a clinical diagnosis, amyloid positron emission tomography-computed tomography (PET-CT) might be helpful. However, in patients with late-onset svPPA, an admixture of the various neuropathology would interfere with diagnostic approach. Herein, we report a case of late-onset bilateral svPPA showing a regional amyloid deposition on PET-CT scan.
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Purpose@#Specialty choice in residency training has a significant impact on an individual’s career and satisfaction, as well as the supply-demand imbalance in the healthcare system. The current study aimed to investigate the quality of life (QOL), stress, self-confidence, and job satisfaction of residents, and to explore factors associated with such variables, including postgraduate year, sex, and especially specialty, through a cross-sectional survey. @*Methods@#An online survey was administered to residents at 2 affiliated teaching hospitals. The survey had a total of 46 items encompassing overall residency life such as workload, QOL, stress, confidence, relationship, harassment, and satisfaction. Related survey items were then reconstructed into 4 key categories through exploratory factor analysis for comparison according to group classification. @*Results@#The weekly work hours of residents in vital and other specialties were similar, but residents in vital specialties had significantly more on-call days per month. Residents in vital specialties had significantly lower scores for QOL and satisfaction. Specifically, vital-surgical residents had significantly lower QOL scores and higher stress scores than the other specialty groups. Satisfaction scores were also lowest among vital-surgical residents, with a marginal difference from vital-medical, and a significant difference from other-surgical residents. Female residents had significantly lower satisfaction scores than their male counterparts. @*Conclusion@#Residents in vital specialties, particularly vital-surgical specialties, experience significantly worse working conditions across multiple dimensions. It is necessary to improve not only the quantity but also the quality of the system in terms of resource allocation and prioritization.
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Schwannoma is a slowly-growing, benign tumor that originates from the Schwann cell and is rarely found in the auricle. In this article, we describe a 20-year-old male patient with an approximately 2 cm auricular mass located in the antitragus of the left ear. The mass was slowlygrowing following an auricular trauma. The mass was completely removed by local excision and the histopathologic review confirmed the diagnosis as an auricular schwannoma. Characteristic immunohistochemical findings may be helpful in differential diagnosis of other tumors. Herein, we present with a literature review a rare case of auricular schwannoma following a trauma.
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Objective@#Fiberoptic endoscopic evaluation of swallowing (FEES) is a standard diagnostic tool for swallowing disorders. However, it has not been used frequently in Korea because of the long test time, low cost, and the absence of a standard evaluation system. The purpose of this study was to suggest a standard fill-out form for the FEES result. @*Methods@#From February 2019 to June 2020, a total of 98 FEES tests were performed by an otolaryngologist (JYJ) at the Wonkwang University Hospital. After the exclusion of 68 cases, 30 cases were analyzed twice by 4 raters with over 5 years of experience as otolaryngologists working in various hospitals. The results were measured for the rater’s test-retest reliability and inter-rater consistency. @*Results@#Cohen’s kappa values for measuring the intra-rater consistency of the four raters were 0.984, 0.887, 0.848, and 0.930, respectively, meaning very good alignment of 0.8 or more, respectively. The Fleiss Kappa value for measuring inter-rater consistency was 0.276, meaning ‘fair’ for values of 0.2 or more. To examine consistency, an intraclass correlation coefficient (ICC) analysis conducted by assuming the grading score to be a constant continuous variable gave an ICC value of 0.729 (P＜0.001), showing a very reliable tendency. @*Conclusion@#In this study, all the items of the fill-out form were rated using a three-step grading scale, so the degree of agreement was high when performed twice by the same rater, but the degree of agreement among raters was relatively low. Therefore, our fill-out form for FEES will be useful in evaluating the improvement of a patient over the course of clinical treatment.
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Purpose@#Osteoporosis is a common calcium and metabolic skeletal disease which is characterized by decreased bone mass, microarchitectural deterioration of bone tissue and impaired bone strength, thereby leading to enhanced risk of bone fragility.In this study, we aimed to identify novel genes for susceptibility to osteoporosis and/or bone density. @*Materials and Methods@#To identify differentially expressed genes (DEGs) between control and osteoporosis-induced cells, annealing control primer-based differential display reverse-transcription polymerase chain reaction (RT-PCR) was carried out in pre-osteoblast MC3T3-E1 cells. Expression levels of the identified DEGs were evaluated by quantitative RT-PCR. Association studies for the quantitative bone density analysis and osteoporosis case-control analysis of single nucleotide polymorphism (SNPs) were performed in Korean women (3,570 subjects) from the Korean Association REsource (KARE) study cohort. @*Results@#Comparison analysis of expression levels of the identified DEGs by quantitative RT-PCR found seven genes, Anxa6, Col5a1, Col6a2, Eno1, Myof, Nfib, and Scara5, that showed significantly different expression between the dexamethasontreated and untreated MC3T3-E1 cells and between the ovariectomized osteoporosis-induced mice and sham mice. Association studies revealed that there was a significant association between the SNPs in the five genes, ANXA6, COL5A1, ENO1, MYOF, and SCARA5, and bone density and/or osteoporosis. @*Conclusion@#Using a whole-genome comparative expression analysis, gene expression evaluation analysis, and association analysis, we found five genes that were significantly associated with bone density and/or osteoporosis. Notably, the association P-values of the SNPs in the ANXA6 and COL5A1 genes were below the Bonferroni-corrected significance level.
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27-Hydroxycholesterol (27OHChol) exhibits agonistic activity for liver X receptors (LXRs). To determine roles of the LXR agonistic activity in macrophage gene expression, we investigated the effects of LXR inhibition on the 27OHChol-induced genes. Treatment of human THP-1 cells with GSK 2033, a potent cell-active LXR antagonist, results in complete inhibition in the transcription of LXR target genes (such as LXRα and ABCA1) induced by 27OHChol or a synthetic LXR ligand TO 901317. Whereas expression of CCL2 and CCL4 remains unaffected by GSK 2033, TNF-α expression is further induced and 27OHChol-induced CCL3 and CXCL8 genes are suppressed at both the transcriptional and protein translation levels in the presence of GSK 2033. This LXR antagonist downregulates transcript levels and surface expression of CD163 and CD206 and suppresses the transcription of CD14, CD80, and CD86 genes without downregulating their surface levels. GSK 2033 alone had no effect on the basal expression levels of the aforementioned genes. Collectively, these results indicate that LXR inhibition leads to differential regulation of 27-hydroxycholesterolinduced genes in macrophages. We propose that 27OHChol induces gene expression and modulates macrophage functions via LXR-dependent and -independent mechanisms.
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Anti-hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) reductase (anti-HMGCR) myopathy is a subtype of immune-mediated necrotizing myopathy regardless of exposure to statins. Necrotizing myopathy is distinguished by necrotic muscle fibers with a relative lack of inflammation. It is frequently associated with a high risk of permanent muscle damage and disability. Recent studies have suggested a slightly increased risk of cancer in patients with anti-HMGCR myopathy. This report describes a case of statin-naïve paraneoplastic anti-HMGCR myopathy, with rapidly progressive muscle weakness leading to respiratory failure.
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Objective@#High disease activity of ankylosing spondylitis (AS) is associated with poor sleep quality. The purpose of this study was to identify which of the representative tools for evaluating the disease activity of AS best reflect the quality of sleep. @*Methods@#A total of 107 AS patients were enrolled in the study and the sleep quality was assessed using the Pittsburgh Sleep Quality Index (PSQI). Age, sex, concomitant medication, erythrocyte sedimentation rate (ESR), serum C-reactive protein (CRP) level, Beck Depression Inventory second edition (BDI-II), Bath ankylosing spondylitis disease activity index (BASDAI), ankylosing spondylitis disease activity score (ASDAS)-ESR, ASDAS-CRP, pain visual analog scale, Insomnia Severity Index (ISI), and Epworth Sleepiness Scale (ESS) were analyzed as covariates. @*Results@#Overall, 65% (70/107) of subjects reported poor sleep quality (PSQI＞5). There was a positive correlation between the sleep quality and disease activity as measured by the BASDAI, ASDAS-ESR, and ASDAS-CRP. In addition, the BASDAI demonstrated good correlations with ISI, ESS, and BDI-II, respectively. However, only BASDAI showed reliable correlation with PSQI among the disease activity parameters of AS (adjusted odd ratio 5.36, p=0.023). @*Conclusion@#BASDAI is the most reliable parameter of disease activity associated with the sleep quality in patients with AS.
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27-Hydroxycholesterol (27OHChol) exhibits agonistic activity for liver X receptors (LXRs). To determine roles of the LXR agonistic activity in macrophage gene expression, we investigated the effects of LXR inhibition on the 27OHChol-induced genes. Treatment of human THP-1 cells with GSK 2033, a potent cell-active LXR antagonist, results in complete inhibition in the transcription of LXR target genes (such as LXRα and ABCA1) induced by 27OHChol or a synthetic LXR ligand TO 901317. Whereas expression of CCL2 and CCL4 remains unaffected by GSK 2033, TNF-α expression is further induced and 27OHChol-induced CCL3 and CXCL8 genes are suppressed at both the transcriptional and protein translation levels in the presence of GSK 2033. This LXR antagonist downregulates transcript levels and surface expression of CD163 and CD206 and suppresses the transcription of CD14, CD80, and CD86 genes without downregulating their surface levels. GSK 2033 alone had no effect on the basal expression levels of the aforementioned genes. Collectively, these results indicate that LXR inhibition leads to differential regulation of 27-hydroxycholesterolinduced genes in macrophages. We propose that 27OHChol induces gene expression and modulates macrophage functions via LXR-dependent and -independent mechanisms.
ABSTRACT
Anti-hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) reductase (anti-HMGCR) myopathy is a subtype of immune-mediated necrotizing myopathy regardless of exposure to statins. Necrotizing myopathy is distinguished by necrotic muscle fibers with a relative lack of inflammation. It is frequently associated with a high risk of permanent muscle damage and disability. Recent studies have suggested a slightly increased risk of cancer in patients with anti-HMGCR myopathy. This report describes a case of statin-naïve paraneoplastic anti-HMGCR myopathy, with rapidly progressive muscle weakness leading to respiratory failure.
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Objective@#High disease activity of ankylosing spondylitis (AS) is associated with poor sleep quality. The purpose of this study was to identify which of the representative tools for evaluating the disease activity of AS best reflect the quality of sleep. @*Methods@#A total of 107 AS patients were enrolled in the study and the sleep quality was assessed using the Pittsburgh Sleep Quality Index (PSQI). Age, sex, concomitant medication, erythrocyte sedimentation rate (ESR), serum C-reactive protein (CRP) level, Beck Depression Inventory second edition (BDI-II), Bath ankylosing spondylitis disease activity index (BASDAI), ankylosing spondylitis disease activity score (ASDAS)-ESR, ASDAS-CRP, pain visual analog scale, Insomnia Severity Index (ISI), and Epworth Sleepiness Scale (ESS) were analyzed as covariates. @*Results@#Overall, 65% (70/107) of subjects reported poor sleep quality (PSQI＞5). There was a positive correlation between the sleep quality and disease activity as measured by the BASDAI, ASDAS-ESR, and ASDAS-CRP. In addition, the BASDAI demonstrated good correlations with ISI, ESS, and BDI-II, respectively. However, only BASDAI showed reliable correlation with PSQI among the disease activity parameters of AS (adjusted odd ratio 5.36, p=0.023). @*Conclusion@#BASDAI is the most reliable parameter of disease activity associated with the sleep quality in patients with AS.
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Objectives@#This study aimed to investigate the prevalence of unhealthy dietary habits by maternal educational level and how the effect of maternal education changed between 2009 and 2019. @*Methods@#Using data from the Korea Youth Risk Behavior Survey conducted between 2009 and 2019, we assessed the prevalence of each unhealthy dietary habit (insufficient consumption of milk and fruit/vegetable, breakfast-skipping, and frequent consumption of fast food, soft drinks, and instant noodles) and the prevalence difference between maternal middle/high school and college graduate groups in four periods. The models included maternal educational level, four periods and the interaction between them, sex, and grade. In addition, we estimated the ORs of unhealthy dietary habits between the two maternal educational groups at each period. @*Results@#Throughout the study period, unhealthy dietary habits were consistently more prevalent among mothers with lower education levels. Between 2009–2010 and 2017– 2019, the prevalence of unhealthy dietary habits increased, with a particular increase in that of frequent consumption of fast food and soft drink. The prevalence differentials between the middle school and college graduate group decreased or did not differ, while those between the high school and college graduate groups increased over time.A similar trend was observed in the relative scale. @*Conclusion@#Considering the increase in the prevalence of unhealthy dietary habits and the prevalence differentials by maternal educational level, targeted efforts are needed not only for all adolescents and their parents but also for those with low socioeconomic status to improve the dietary habits of adolescents.
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Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by the production of autoantibodies that result in the inflammation of multiple organs, including the skin and musculoskeletal, renal, nervous, cardiovascular, respiratory, and gastrointestinal systems. Treatment includes the use of immunomodulatory and immunosuppressive agents to target specific organ manifestations. The treatment goal in SLE is to reduce disease activity and prevent organ damage and death. Optimal long-term outcomes require not only treatment of the disease, but also the management of comorbidities. This paper reviews treatments of SLE with the aim of improving outcomes.
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We investigated effects of reblastatins on phenotypic changes in monocytes/macrophages induced by 27-hydroxycholesterol (27OHChol). Treatment of THP-1 monocytic cells with reblastatin derivatives, such as 17-demethoxy-reblastatin (17-DR), 18-dehydroxyl-17-demethoxyreblastatin (WK88-1), 18-hydroxyl-17-demethoxyreblastatin (WK88-2), and 18-hydroxyl-17-demethoxy-4,5-dehydroreblastatin (WK88-3), resulted in blockage of CCL2, CCL3, and CCL4 expression at the transcription and protein levels, which, in turn, impaired migration of monocytes/macrophages and Jurkat T cells expressing CCR5, and almost complete inhibition of transcription of M1 marker cytokines, like CXCL10, CXCL11, and TNF-α. Reblastatins also downregulated surface CD14 as well as soluble CD14 along with inhibition of LPS response and matrix metalloprotease-9 expression. Surface levels of mature dendritic cell (mDC)-specific markers, including CD80, CD83, CD88, CD197, and MHC class I and II molecules, were remarkably down-regulated, and 27OHChol-induced decrease of endocytic activity was recovered following treatment with 17-DR, WK88-1, WK88-2, and WK88-3. However, 15-hydroxyl-17-demethoxyreblastatin (DHQ3) did not affect the molecular or functional changes in monocytic cells induced by 27OHChol. Furthermore, surface levels of CD105, CD137, and CD166 were also down-regulated by 17-DR, WK88-1, WK88-2, and WK88-3, but not by DHQ3. Collectively, results of the current study indicate that, except DHQ3, reblastatins regulate the conversion and differentiation of monocytic cells to an immunostimulatory phenotype and mDCs, respectively, which suggests possible applications of reblastatins for immunomodulation in a milieu rich in oxygenated cholesterol molecules.
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Neuromyelitis optica spectrum disorder (NMOSD) is a rare inflammatory demyelinating disease. Anti-aquaporin-4 antibodies serve as a specific biomarker, while other factors including antecedent infection may also play a role in the development of NMOSD. Abnormal cerebrospinal fluid (CSF) findings such as leukocytosis with concentration >50/mm3 are one of the characteristics of NMOSD, but these were not specific for identifying other infective neurological diseases. Here we describe a rare case of NMOSD with CSF findings suggestive of bacterial meningomyelitis.
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Angiofibroma is benign hypervascular tumor that usually occurs in the nasopharynx of adolescent male patient and rarely originates from outside of the nasopharynx, called extranasopharyngeal angiofibroma. The clinical and radiologic findings of extranasopharyngeal angiofibroma are similar to other hypervascular tumors, which may delay diagnosis and treatment. We present a case of extranasopharyngeal angiofibroma arising from nasal septum of a 43-year-old male who complained of right nasal obstruction. Contrast enhancement CT revealed a hypervascular tumor of the nasal septum and the tumor was removed by endoscopic resection. Histopathologic examination confirmed the diagnosis of angiofibroma.
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Erythema ab igne (EAI) is a persistent chronic skin condition that is characterized by reticulated macular hyperpigmented lesions caused by repeated exposure to infrared radiation. EAI has reemerged in recent years because of the increasing infrared exposure due to the use of new technologies, such as portable computers and automobile seat heaters. We report a case of a 54-year-old female patient presenting with asymptomatic mottled brownish plaque on both sides of her neck for 1 month prior to the visit. Patient history revealed that she had used a home skincare device for skin rejuvenation and relaxation. The clinicopathological features suggested a diagnosis of EAI. To our knowledge, there has only been one reported case of EAI on the neck related to a thermal pillow, and this was the first case of EAI related to the self-skincare devices. Herein, we report a rare case of EAI presenting on both sides of the neck due to the use of a modern home skincare device.
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Female , Humans , Middle Aged , Automobiles , Diagnosis , Erythema , Neck , Rejuvenation , Relaxation , SkinABSTRACT
PURPOSE@#Nurses working at rehabilitation hospitals play an important role in interdisciplinary teams. However, conflicts with others within the teams are common. This study (1) explored the frequency and seriousness of role conflicts and determined the relationship between turnover intention and type of role conflict among nurses in rehabilitation hospitals and (2) investigated differences in turnover intention by the type of role conflict.@*METHODS@#Data were collected from 110 nurses in two rehabilitation hospitals. A survey was performed to measure role conflicts including conflicts regarding nursing practice, and conflicts with staff nurses, patients/guardians, staffs in other departments, and doctors. Also, nurses' turnover intentions were evaluated. Data were analyzed using descriptive statistics and cluster analysis.@*RESULTS@#The most common and serious conflicts were developed with patients/guardians, followed by conflicts related to nursing practice. Conflicts in relation to nurses' roles were classified into four categories: ‘High conflict type’, ‘High frequency of conflict type’, ‘High seriousness of conflict type’, and ‘Low conflict type’. The level of turnover intention was increased in this order.@*CONCLUSION@#To decrease rehabilitation nurses' intentions to leave a hospital, it is important to reduce role conflicts by providing tailored interventions for each type of conflict and to improve the organizational interrelationship process.
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BACKGROUND/AIMS@#To define standard reference values for musculoskeletal ultrasonography (MSUS) in Korea.@*METHODS@#A total of 251 healthy adults were recruited for this study. Ultrasonography was performed by experienced rheumatologists who had undergone four appropriate training programs in Korea. A General Electric LOGIQ electronic ultrasound device fitted with a 12 MHz linear transducer was employed. Mean values ± standard deviations (SDs) were defined as standard reference values. Intraclass correlation coefficients was employed to evaluate the extent of inter- and intraobserver agreement when MSUS measurements were made.@*RESULTS@#The 251 study participants included 122 males. Mean subject age was 28.6 years. The average bone-to-capsule distance of the right-side second and third metacarpophalangeal (MCP) joints were 0.68 and 0.72 mm respectively, and those of the left-side joints 0.62 and 0.68 mm. The cartilage thicknesses of the right-side second and third MCP joints were 0.55 and 0.55 mm, and those of the left-side joints were 0.55 and 0.56 mm, respectively. The bone-to-capsule distances of the right and left wrists were 0.80 and 0.82 mm. In 12.4% of participants (31/251), the erosion score of the humeral head was 1.71. In the right-side knee joint, mean cartilage thicknesses of the medial and lateral condyles were 1.86 and 2.03 mm in longitudinal scans. High overall interobserver agreement was evident after appropriate training that included instruction on standard MSUS methodology.@*CONCLUSIONS@#We defined standard reference values for MSUS in healthy Korean adults. The reliabilities of interobserver agreements were high after appropriate training program.