Background: s/Aims: Reported incidence of extrahepatic bile duct cancer is higher in Asians than in Western populations. Korea, in particular, is one of the countries with the highest incidence rates of extrahepatic bile duct cancer in the world. Although research and innovative therapeutic modalities for extrahepatic bile duct cancer are emerging, clinical guidelines are currently unavailable in Korea. The Korean Society of Hepato-Biliary-Pancreatic Surgery in collaboration with related societies (Korean Pancreatic and Biliary Surgery Society, Korean Society of Abdominal Radiology, Korean Society of Medical Oncology, Korean Society of Radiation Oncology, Korean Society of Pathologists, and Korean Society of Nuclear Medicine) decided to establish clinical guideline for extrahepatic bile duct cancer in June 2021. Methods: Contents of the guidelines were developed through subgroup meetings for each key question and a preliminary draft was finalized through a Clinical Guidelines Committee workshop. Results: In November 2021, the finalized draft was presented for public scrutiny during a formal hearing. Conclusions The extrahepatic guideline committee believed that this guideline could be helpful in the treatment of patients.

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.

Objective: We aimed to present the study design and baseline cross-sectional participant characteristics of biobank innovations for chronic cerebrovascular disease with Alzheimer’s disease study (BICWALZS) participants. Methods: A total of 1,013 participants were enrolled in BICWALZS from October 2016 to December 2020. All participants underwent clinical assessments, basic blood tests, and standardized neuropsychological tests (n=1,013). We performed brain magnetic resonance imaging (MRI, n=817), brain amyloid positron emission tomography (PET, n=713), single nucleotide polymorphism microarray chip (K-Chip, n=949), locomotor activity assessment (actigraphy, n=200), and patient-derived dermal fibroblast sampling (n=175) on a subset of participants. Results: The mean age was 72.8 years, and 658 (65.0%) were females. Based on clinical assessments, total of 168, 534, 211, 80, and 20 had subjective cognitive decline, mild cognitive impairment (MCI), Alzheimer’s dementia, vascular dementia, and other types of dementia or not otherwise specified, respectively. Based on neuroimaging biomarkers and cognition, 199, 159, 78, and 204 were cognitively normal (CN), Alzheimer’s disease (AD)-related cognitive impairment, vascular cognitive impairment, and not otherwise specified due to mixed pathology (NOS). Each group exhibited many differences in various clinical, neuropsychological, and neuroimaging results at baseline. Baseline characteristics of BICWALZS participants in the MCI, AD, and vascular dementia groups were generally acceptable and consistent with 26 worldwide dementia cohorts and another independent AD cohort in Korea. Conclusion The BICWALZS is a prospective and longitudinal study assessing various clinical and biomarker characteristics in older adults with cognitive complaints. Details of the recruitment process, methodology, and baseline assessment results are described in this paper.

Background: The temporal changes in the Staphylococcus aureus genotypes causing S. aureusbacteremia (SAB) and the corresponding clinical changes over the last decade in South Korea are rarely investigated. Methods: A longitudinal study of adult SAB patients was conducted in a large referral hospital in Seoul, South Korea. Adult monomicrobial SAB patients were enrolled between August 2008 and December 2018. Genotyping was performed by multilocus sequence typing (MLST) and staphylococcal protein A (spa) typing. Trends in changes were identified by linear regression analysis. Results: Of 1782 adult SAB patients, the blood isolates of 1,778 (99.8%) and 1,634 (91.7%) were determined to be MLST and spa type, respectively. ST5 (–2.626%/year) and ST239 (–0.354%/year) decreased during the study period (P < 0.001 for both), but ST72 (2.009%/ yr)-and ST8 (0.567%/yr) increased (P < 0.001 for both). The most common genotype was changed from ST5 in 2008 (44.9%) to ST72 in 2018 (36.3%). Panton-Valentine leukocidinpositive spa-t008-MRSA (USA300) was found in 28.6%. Central venous catheter (CVC)-related SAB (–2.440%/yr) and persistent SAB (–1.016%/yr) decreased, but mortality and recurrence rates were unchanged. Conclusion Over the last decade, the hospital clones ST5 and ST239 have been replaced by community genotype ST72. This was associated with decreased CVC-related and persistent SAB. Increased USA300 was observed in community and hospital settings. Further research is required to identify the reasons for the ST72 epidemic and predict the impending epidemic of ST8 strains, including USA300.

Background: The temporal changes in the Staphylococcus aureus genotypes causing S. aureusbacteremia (SAB) and the corresponding clinical changes over the last decade in South Korea are rarely investigated. Methods: A longitudinal study of adult SAB patients was conducted in a large referral hospital in Seoul, South Korea. Adult monomicrobial SAB patients were enrolled between August 2008 and December 2018. Genotyping was performed by multilocus sequence typing (MLST) and staphylococcal protein A (spa) typing. Trends in changes were identified by linear regression analysis. Results: Of 1782 adult SAB patients, the blood isolates of 1,778 (99.8%) and 1,634 (91.7%) were determined to be MLST and spa type, respectively. ST5 (–2.626%/year) and ST239 (–0.354%/year) decreased during the study period (P < 0.001 for both), but ST72 (2.009%/ yr)-and ST8 (0.567%/yr) increased (P < 0.001 for both). The most common genotype was changed from ST5 in 2008 (44.9%) to ST72 in 2018 (36.3%). Panton-Valentine leukocidinpositive spa-t008-MRSA (USA300) was found in 28.6%. Central venous catheter (CVC)-related SAB (–2.440%/yr) and persistent SAB (–1.016%/yr) decreased, but mortality and recurrence rates were unchanged. Conclusion Over the last decade, the hospital clones ST5 and ST239 have been replaced by community genotype ST72. This was associated with decreased CVC-related and persistent SAB. Increased USA300 was observed in community and hospital settings. Further research is required to identify the reasons for the ST72 epidemic and predict the impending epidemic of ST8 strains, including USA300.

Background: Hereditary hemolytic anemia (HHA) is a rare disease characterized by premature red blood cell (RBC) destruction due to intrinsic RBC defects. The RBC Disorder Working Party of the Korean Society of Hematology established and updated the standard operating procedure for making an accurate diagnosis of HHA since 2007. The aim of this study was to investigate a nationwide epidemiology of Korean HHA. Methods: We collected the data of a newly diagnosed pediatric HHA cohort (2007–2016) and compared this cohort's characteristics with those of a previously surveyed pediatric HHA cohort (1997–2006) in Korea. Each participant's information was retrospectively collected by a questionnaire survey. Results: A total of 369 children with HHA from 38 hospitals distributed in 16 of 17 districts of Korea were investigated. RBC membranopathies, hemoglobinopathies, RBC enzymopathies, and unknown etiologies accounted for 263 (71.3%), 59 (16.0%), 23 (6.2%), and 24 (6.5%) of the cases, respectively. Compared to the cohort from the previous decade, the proportions of hemoglobinopathies and RBC enzymopathies significantly increased (P < 0.001 and P = 0.008, respectively). Twenty-three of the 59 hemoglobinopathy patients had immigrant mothers, mostly from South-East Asia. Conclusion In Korea, thalassemia traits have increased over the past 10 years, reflecting both increased awareness of this disease and increased international marriages. The enhanced recognition of RBC enzymopathies is due to advances in diagnostic technique; however, 6.5% of HHA patients still do not have a clear diagnosis. It is necessary to improve accessibility of diagnosing HHA.

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

Papillary renal cell carcinomas (RCCs) can be classified into 2 subtypes (types 1 and 2), depending on their characteristic cytogenetics, immunostaining profiles, and gene-expression profiles. Compared with type 1 papillary RCCs, type 2 papillary RCCs are relatively rare and show more aggressive features. For those reasons, they are associated with a worse prognosis. A 13-year-old patient was admitted to the hospital with right kidney mass. A laparoscopic radical nephrectomy was performed, and results of the histopathologic examination confirmed it to be type 2 papillary RCC. Type 2 papillary RCCs are rarely found in unilateral and multifocal forms, especially in adolescence. Here, we report the unique case of papillary RCC at a young age.
Adolescent ; Carcinoma, Renal Cell ; Cytogenetics ; Humans ; Kidney ; Nephrectomy ; Prognosis

BACKGROUND: Lead and cadmium have been identified as risk factors for hearing loss in animal studies, but large-scale studies targeting the general human population are rare. This study was conducted to investigate the link between heavy metal concentrations in blood and hearing impairment, using a national population-based survey. METHODS: The study participants comprised 6409 Koreans aged 20 or older, who were included in the Fifth and Sixth Korea National Health and Nutrition Examination Surveys (KNHANES 2010–2013). Hearing impairment was categorized into two types, low- and high-frequency hearing impairment, using pure tone audiometry. Low-frequency hearing impairment was defined as having a binaural average of hearing thresholds for 0.5, 1, and 2 kHz exceeding 25 dB, and high-frequency hearing impairment was defined as having a binaural average of hearing thresholds for 3, 4, and 6 kHz exceeding 25 dB. The blood levels of heavy metals (lead and cadmium) were classified into quartiles. Cross-sectional association between hearing impairment and the level of heavy metals (lead and cadmium) was examined in both sexes. Multivariate logistic regression was used to obtain adjusted odds ratios (ORs) and 95% confidence intervals (CIs). RESULTS: Among men, the prevalence of low- and high- frequency hearing impairment was 13.9% and 46.7%, respectively, which was higher than the prevalence among women (11.8% and 27.0%, respectively). Regarding lead, the adjusted OR of high-frequency hearing impairment for the highest blood level group versus the lowest group was significant in both men (OR = 1.629, 95% CI = 1.161–2.287) and women (OR = 1.502, 95% CI = 1.027–2.196), after adjusting for age, body mass index, education, smoking, alcohol consumption, exercise, diagnosis of diabetes mellitus, hypertension, and noise exposure (occupational, loud, firearm noises). No links were found between blood lead levels and low-frequency hearing impairment, or between blood cadmium levels and low- or high-frequency hearing impairment in either sex. CONCLUSIONS: The present study findings suggest that even exposure to low-level lead is a risk factor for high-frequency hearing loss. A prospective epidemiologic study should be conducted to identify the causal relationship between human health and exposure to heavy metals, and efforts to reduce heavy metal exposure in the general population should continue.
Alcohol Drinking ; Animals ; Audiometry ; Body Mass Index ; Cadmium ; Diabetes Mellitus ; Diagnosis ; Education ; Environmental Exposure ; Epidemiologic Studies ; Female ; Hearing Loss ; Hearing Loss, High-Frequency ; Hearing ; Humans ; Hypertension ; Korea ; Logistic Models ; Male ; Metals, Heavy ; Noise ; Nutrition Surveys ; Odds Ratio ; Prevalence ; Prospective Studies ; Risk Factors ; Smoke ; Smoking

BACKGROUND: Shift work disturbs workers' biological clocks and this condition can cause various health problems including cardiovascular disease. The elevated albuminuria level has been significantly associated with the risk of the cardiovascular disease even within a normal reference range. Therefore, this study aimed to investigate the association between shift work and microalbuminuria. METHODS: Workers aged over 20 years from the fifth and sixth Korea National Health and Nutrition Examination Survey(KNHANES 2012–2014; n = 3000) were included in this analysis. The multiple logistic regression analysis was performed to determine the association between shift work and microalbuminuria stratified by gender. RESULTS: The prevalence of microalbuminuria in male subjects was higher among day workers, but the difference was not significant. However, the prevalence of microalbuminuria among females was higher in shift workers with statistical significance. For female, the Odds ratio of microalbuminuria in shift workers was significantly higher with 1.86 (95% CI 1.02–3.39) compared with day workers. After dividing into 5 subgroups of the shift work pattern, the odds ratio of microalbuminuria for fixed night shift was significantly higher at 4.68 (95% CI 1.29–17.00) compared with day workers. CONCLUSIONS: This study showed that shift work was associated with microalbuminuria in female workers. Especially we found out the association between fixed night shift and microalbuminuria in female workers.
Albuminuria ; Biological Clocks ; Cardiovascular Diseases ; Female ; Humans ; Korea ; Logistic Models ; Male ; Odds Ratio ; Prevalence ; Reference Values