Fabry nephropathy is characterized by a deficiency of lysosomal alpha-galactosidase A, which results in proteinuria and kidney disease. The ineffectiveness of enzyme replacement therapy (ERT) for severe kidney failure highlights the need for early detection and meaningful markers. However, because the diagnosis and treatment of Fabry disease can vary according to the expertise of physicians, we evaluated the opinions of Korean specialists. Methods: A questionnaire regarding the management of Fabry nephropathy was emailed to healthcare providers with the experience or ability to treat individuals with Fabry nephropathy. Results: Of the 70 experts who responded to the survey, 43 were nephrologists, and 64.3% of the respondents reported having treated patients with Fabry disease. Pediatricians are treating primarily patients with classic types of the disease, while nephrologists and cardiologists are treating more patients with variant types. Only 40.7% of non-nephrologists agreed that a kidney biopsy was required at the time of diagnosis, compared with 81.4% of nephrologists. Thirty-eight of 70 respondents (54.3%) reported measuring globotriaosylsphingosine (lyso-Gb3) as a biomarker. The most common period to measure lyso-Gb3 was at the time of diagnosis, followed by after ERT, before ERT, and at screening. For the stage at which ERT should begin, microalbuminuria and proteinuria were chosen by 51.8% and 28.6% of respondents, respectively. Conclusion: Nephrologists are more likely to treat variant Fabry disease rather than classic cases, and they agree that ERT should be initiated early in Fabry nephropathy, using lyso-Gb3 as a biomarker.

Purpose: Acute promyelocytic leukemia (APL) is a rare disease in children and there are some different characteristics between children and adult. We aimed to evaluate incidence, clinical characteristics and treatment outcomes of pediatric APL in Korea. Materials and Methods: Seventy-nine pediatric APL patients diagnosed from January 2009 to December 2016 in 16 tertiary medical centers in Korea were reviewed retrospectively. Results: Of 801 acute myeloid leukemia children, 79 (9.9%) were diagnosed with APL. The median age at diagnosis was 10.6 years (range, 1.3 to 18.0). Male and female ratio was 1:0.93. Thirty patients (38.0%) had white blood cell (WBC) count greater than 10×109/L at diagnosis. All patients received induction therapy consisting of all-trans retinoic acid and chemotherapy. Five patients (6.6%) died during induction chemotherapy and 66 patients (86.8%) achieved complete remission (CR) after induction chemotherapy. The causes of death were three intracranial hemorrhage, one cerebral infarction, and one sepsis. Five patients (7.1%) suffered a relapse during or after maintenance chemotherapy. The estimated 4-year event-free survival and overall survival (OS) rates were 82.1%±4.4%, 89.7%±5.1%, respectively. The 4-year OS was significantly higher in patients with initial WBC < 10×109/L than in those with initial WBC ≥ 10×109/L (p=0.020). Conclusion This study showed that the CR rates and survival outcomes in Korean pediatric APL patients were relatively good. The initial WBC count was the most important prognostic factor and most causes of death were related to serious bleeding in the early stage of treatment.

In November 2021, 14 international travel-related severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) B.1.1.529 (omicron) variant of concern (VOC) patients were detected in South Korea. Epidemiologic investigation revealed community transmission of the omicron VOC. A total of 80 SARS-CoV-2 omicron VOC-positive patients were identified until December 10, 2021 and 66 of them reported no relation to the international travel.There may be more transmissions with this VOC in Korea than reported.

Many previous studies have shown reduced glucose uptake in the ischemic brain. In contrast, in a permanent unilateral common carotid artery occlusion (UCCAO) mouse model, our pilot experiments using 18F-fluorodeoxyglucose positron emission tomography (FDG PET) revealed that a subset of mice exhibited conspicuously high uptake of glucose in the ipsilateral hemisphere at 1 week post-occlusion (asymmetric group), whereas other mice showed symmetric uptake in both hemispheres (symmetric group). Thus, we aimed to understand the discrepancy between the two groups. Cerebral blood flow and histological/metabolic changes were analyzed using laser Doppler flowmetry and immunohistochemistry/Western blotting, respectively. Contrary to the increased glucose uptake observed in the ischemic cerebral hemisphere on FDG PET (p<0.001), cerebral blood flow tended to be lower in the asymmetric group than in the symmetric group (right to left ratio [%], 36.4±21.8 vs. 58.0±24.8, p=0.059). Neuronal death was observed only in the ischemic hemisphere of the asymmetric group. In contrast, astrocytes were more activated in the asymmetric group than in the symmetric group (p<0.05). Glucose transporter-1, and monocarboxylate transporter-1 were also upregulated in the asymmetric group, compared with the symmetric group (p<0.05, respectively). These results suggest that the increased FDG uptake was associated with relatively severe ischemia, and glucose transporter-1 upregulation and astrocyte activation. Glucose metabolism may thus be a compensatory mechanism in the moderately severe ischemic brain.

Background: Hodgkin's lymphoma (HL) constitutes 10%–20% of all malignant lymphomas and has a high cure rate (5-year survival, around 90%). Recently, interest has increased concerning preventing secondary complications (secondary cancer, endocrine disorders) in long-term survivors. We aimed to study the epidemiologic features and therapeutic outcomes of HL in children, adolescents, and young adults in Korea. Methods: We performed a multicenter, retrospective study of 224 patients aged < 25 years diagnosed with HL at 22 participating institutes in Korea from January 2007 to August 2016. Results: A higher percentage of males was diagnosed at a younger age. Nodular sclerosis histopathological HL subtype was most common, followed by mixed cellularity subtype.Eighty-one (36.2%), 101 (45.1%), and 42 (18.8%) patients were classified into low, intermediate, and high-risk groups, respectively. Doxorubicin, bleomycin, vinblastine, dacarbazine was the most common protocol (n = 102, 45.5%). Event-free survival rate was 86.0% ± 2.4%, while five-year overall survival (OS) rate was 96.1% ± 1.4%: 98.7% ± 1.3%, 97.7% ± 1.6%, and 86.5% ± 5.6% in the low, intermediate, and high-risk groups, respectively (P = 0.021). Five-year OS was worse in patients with B-symptoms, stage IV disease, highrisk, splenic involvement, extra-nodal lymphoma, and elevated lactate dehydrogenase level.In multivariate analysis, B-symptoms and extra-nodal involvement were prognostic factors for poor OS. Late complications of endocrine disorders and secondary malignancy were observed in 17 and 6 patients, respectively. Conclusion This is the first study on the epidemiology and treatment outcomes of HL in children, adolescents, and young adults in Korea. Future prospective studies are indicated to develop therapies that minimize treatment toxicity while maximizing cure rates in children, adolescents, and young adults with HL.

BACKGROUND: This study investigated characteristics according to demographic, occupational factors of Maslach Burnout Inventory-General Survey (MBI-GS) and related scales to MBI-GS. METHODS: The subjects of the study were 3,331 workers in 3 different workplaces of one electronics company. They filled in demographic factors surveys, occupational factors surveys, MBI-GS, Korean Occupational Stress Scale-Short Form (KOSS-SF), Patient Health Questionnaire-9 (PHQ-9), and World Health Organization Quality Of Life-Abbreviated version (WHOQOL-BREF). The correlations between sub-scales of MBI-GS and KOSS-SF, PHQ-9, WHOQOL-BREF were analyzed respectively. And KOSS-SF, PHQ-9, and WHOQOL-BREF were categorized; mean scores of sub-scales of MBI-GS were compared; and the quartiles of sub-scales of MBI-GS were presented. RESULTS: A comparison of mean scores of MBI-GS according to demographic and occupational factors showed a significant difference according to age, problem drinking behavior, working time, and working duration in exhaustion regardless of sex. In professional efficacy, a significant difference was observed in age, marital status, working type, and working duration. And as a result of correlation analysis, the correlation coefficient between exhaustion and PHQ-9 was the highest regardless of sex. In addition, regardless of sex, exhaustion and cynicism scores tended to increase and professional efficacy score tended to decrease as the work stress level rose. Same tendency is shown in case of the more severe the symptom of depression and the lower quality of life. When the quartile for sub-scales' score of MBI-GS were investigated, the burnout was more pronounced in female than in male. CONCLUSIONS: Many demographic and occupational factors affect burnout were identified in one electronics company, and we investigated which sub-scales of MBI-GS were affected. Through this study, burnout characteristics were identified in a few population group of Korea, and the results are expected to be useful for burnout risk group identification, counseling, etc.
Counseling ; Demography ; Depression ; Drinking Behavior ; Female ; Humans ; Korea ; Male ; Marital Status ; Population Groups ; Quality of Life ; Social Identification ; Weights and Measures ; World Health Organization

PURPOSE: The purpose of this study was to identify levels of health-related quality of life (EQ-5D) and its related factors among merchants in traditional markets.METHODS: A cross-sectional design was used. Data were collected through questionnaires and physical examination on blood pressure, blood glucose, total cholesterol and BMI between February 12 and April 13, 2018. The participants were 175 merchants from two traditional markets in U. Metropolitan city. The data were analyzed with descriptive statistics, t-test, ANOVA and multiple regression analysis using the SPSS/WIN 22.0 program.RESULTS: Levels of health-related EQ-5D were significantly different according to demographic characteristics, smoking status, exercise, total cholesterol, BMI, subjective health status, stress, and depression. The EQ-5D related factors were variables that had significant influence on subjective health status (β=.30, p < .001), gender (β=.27, p < .001), BMI (β=−.16, p=.012), depression (β=−.17, p=.010), and working period (β=−.15, p=.021). They explained 27.2% of the total variance of health-related quality of life (F=13.91, p < .001).CONCLUSION: The findings of this study indicated that disease prevention and health management is essential for merchants who work in traditional markets to promote their health-related quality of life. For better merchants outcome, the intervention program should consider factors that are subjective health status, gender, BMI, depression, and working period in order to improve levels of EQ-5D.

PURPOSE: Art program has been found to enhance mood such as increasing motivation, self-expression and decreasing depression in older adults. This study was conducted to investigate the effects of clay art and drawing on depression and self-expression among elderly people at a long-term care center. METHODS: The research was a pre-test and post-test non-equivalent design. Sixty older patients (clay art program=28. Drawing art program=32) over the age of 65 were recruited from 2 long-term care hospitals to participate in the 5 weeks program. Each group was scheduled with 10,120-minute evaluation sessions, twice per week. Test measures were completed before and after the 5 week intervention period for all participants. The data was collected from April 27 to May 29, 2015. It was analyzed with SPSS 22.0 using chi-square, t-test and paired t-test. RESULTS: There were no significant difference in the levels of depression (t=0.21, p=.830) and total score of self-expression (t=-0.10, p=.919) between the two groups. However, the pre-post scores of self-expression in the clay art program (t=0.22, p=.826) were improved compared to the drawing art program (t=0.80, p=.430). CONCLUSION: Further studies are needed to evaluate the effectiveness of clay art program for extended senior care applications.
Adult ; Aged* ; Art Therapy ; Depression* ; Humans ; Long-Term Care* ; Motivation

OBJECTIVE: To assess the outcome of the treatment of primary vaginal cancer using definitive radiotherapy (RT) and to evaluate the prognostic factors of survival. METHODS: The medical records of nine institutions were retrospectively reviewed to find the patients with vaginal cancer treated with definitive RT with or without chemotherapy. A total of 138 patients met the inclusion criteria. None had undergone curative excision. RESULTS: The median follow-up time of the survivors was 77.6 months and the median survival time was 46.9 months. The 5-year overall survival, cancer-specific survival (CSS), and progression-free survival (PFS) rates were 68%, 80%, and 68.7%, respectively. In the survival analysis, the multivariate analysis showed that a lower the International Federation of Gynecology and Obstetrics (FIGO) stage and prior hysterectomy were favorable prognostic factors of CSS, and a lower FIGO stage and diagnosed prior to year 2000 were favorable prognostic factors of PFS. In the subgroup analysis of the patients with available human papillomavirus (HPV) results (n=27), no statistically significant relationship between the HPV status and recurrence or survival was found. Grade 3 or 4 acute and late toxicity were present in 16 and 9 patients, respectively. The FIGO stage and the tumor size were predictors of severe late toxicity. CONCLUSION: The data clearly showed that a higher FIGO stage was correlated with a worse survival outcome and higher severe late toxicity. Therefore, precise RT and careful observation are crucial in advanced vaginal cancer. In this study, the HPV status was not related to the survival outcome, but its further investigation is needed.
Adult ; Aged ; Aged, 80 and over ; Brachytherapy ; Carcinoma, Squamous Cell/mortality/*radiotherapy/secondary/virology ; Chemoradiotherapy ; Disease-Free Survival ; Female ; Follow-Up Studies ; Humans ; Hysterectomy ; Middle Aged ; Neoplasm Staging ; Papillomavirus Infections/diagnosis ; Radiotherapy/adverse effects ; Republic of Korea ; Retrospective Studies ; Survival Rate ; Treatment Outcome ; Tumor Burden ; Vaginal Neoplasms/mortality/pathology/*radiotherapy/virology