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.

Objective: Non-Suicidal Self-Injury (NSSI) scars are common in individuals with NSSI experiences. However, little is known about NSSI scars because related tools are limited. This study aimed to develop and validate the Korean version of the multidimensional Non-Suicidal Self-Injury Scar Scale (K-NSSI-ScarS), consisting of three components: NSSI scar measurement, NSSI scar cognition, and NSSI scar concealment. Methods: A total of 333 Korean adults with at least one NSSI scar and history of NSSI within the last 5 years (age: 18 to 39 years) completed the online survey. We conducted exploratory (n=133) and confirmatory (n=200) factor analyses of NSSI scar cognition. To measure the internal consistency of each subfactor of the scar cognition and scar concealment components, we used Cronbach’s α. Kappa and intraclass correlation coefficients were used to measure the test-retest reliability of the entire scale. We also assessed the convergent and construct validity of the K-NSSI-ScarS. Results: Factor analyses showed a 5-factor structure consisting of 23 items. Internal consistencies and test-retest reliability were excellent. The moderate correlation between the five subfactors of NSSI scar cognition and related concepts (e.g., acquired capability of suicide) confirmed the convergent validity. Lastly, moderate correlations were found between NSSI scar concealment, self-concealment, NSSI scar measurement information, and the five subfactors of NSSI scar cognition. Conclusion The results verify the psychometric properties and support the necessity of a multidimensional NSSI scar scale.

Objective: This study examined the effect of social situations on risky decision-making in individuals with a borderline personality tendency (BT). Methods: Fifty-eight participants with either high or low levels of BT were included in this study. Those who met the screening criteria were assigned to one of the two social situations (exclusion vs. inclusion) and they played the Cyberball game. Next, the participants were required to play the “Game of Dice” task to measure their decision-making pattern. Results: The results revealed that individuals with high BT (n=28) showed significantly higher risky decision-making, compared with low BT (n=30) in the exclusion condition. However, no significant difference was found in the social inclusion condition. Conclusion In the social exclusion condition, individuals with high BT made risky decision in response to negative feedback, regardless of the previous decision. These findings can be applied to develop appropriate interventions in psychotherapy for individuals with borderline personality disorder/tendency.

Purpose: Renal tumors account for approximately 7% of all childhood cancers. These include Wilms tumor (WT), clear cell sarcoma of the kidney (CCSK), malignant rhabdoid tumor of the kidney (MRTK), renal cell carcinoma (RCC), congenital mesoblastic nephroma (CMN) and other rare tumors. We investigated the epidemiology of pediatric renal tumors in Korea. Materials and Methods: From January 2001 to December 2015, data of pediatric patients (0–18 years) newly-diagnosed with renal tumors at 26 hospitals were retrospectively analyzed. Results: Among 439 patients (male, 240), the most common tumor was WT (n=342, 77.9%), followed by RCC (n=36, 8.2%), CCSK (n=24, 5.5%), MRTK (n=16, 3.6%), CMN (n=12, 2.7%), and others (n=9, 2.1%). Median age at diagnosis was 27.1 months (range 0-225.5) and median follow-up duration was 88.5 months (range 0-211.6). Overall, 32 patients died, of whom 17, 11, 1, and 3 died of relapse, progressive disease, second malignant neoplasm, and treatment-related mortality. Five-year overall survival and event free survival were 97.2% and 84.8% in WT, 90.6% and 82.1% in RCC, 81.1% and 63.6% in CCSK, 60.3% and 56.2% in MRTK, and 100% and 91.7% in CMN, respectively (p < 0.001). Conclusion The pediatric renal tumor types in Korea are similar to those previously reported in other countries. WT accounted for a large proportion and survival was excellent. Non-Wilms renal tumors included a variety of tumors and showed inferior outcome, especially MRTK. Further efforts are necessary to optimize the treatment and analyze the genetic characteristics of pediatric renal tumors in Korea.

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.

Gastric adenocarcinoma is among the top causes of cancer-related death and is one of the most commonly diagnosed carcinomas worldwide. Benzyl isothiocyanate (BITC) has been reported to inhibit the gastric cancer metastasis. In our previous study, BITC induced apoptosis in AGS cells. The purpose of the present study was to investigate the effect of BITC on autophagy mechanism in AGS cells. First, the AGS cells were treated with 5, 10, or 15 μM BITC for 24 h, followed by an analysis of the autophagy mechanism. The expression level of autophagy proteins involved in different steps of autophagy, such as LC3B, p62/SQSTM1, Atg5-Atg12, Beclin1, p-mTOR/mTOR ratio, and class III PI3K was measured in the BITC-treated cells. Lysosomal function was investigated using cathepsin activity and Bafilomycin A1, an autophagy degradation stage inhibitor. Methods including qPCR, western blotting, and immunocytochemistry were employed to detect the protein expression levels. Acridine orange staining and omnicathepsin assay were conducted to analyze the lysosomal function. siRNA transfection was performed to knock down the LC3B gene. BITC reduced the level of autophagy protein such as Beclin 1, class III PI3K, and Atg5-Atg12. BITC also induced lysosomal dysfunction which was shown as reducing cathepsin activity, protein level of cathepsin, and enlargement of acidic vesicle. Overall, the results showed that the BITC-induced AGS cell death mechanism also comprises the inhibition of the cytoprotective autophagy at both initiation and degradation steps.

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.

Background and Objectives: Whether beta blockers favorably impact the clinical outcome in patients with acute myocardial infarction (AMI) remains in debate. We investigated the impact of beta blocker on major clinical outcomes during 2 years after percutaneous coronary intervention (PCI) in patients with AMI. Methods: All patients with the first AMI treated with PCI for the period of 2005 to 2014 from the Korean National Health Insurance Service claims database were enrolled. We defined the regular user as medication possession ratio (MPR) ≥80% and non-user as MPR=0%. We compared the occurrence of all cause death, myocardial infarction (MI) and stroke according to adherence of beta-blockers. A 1:1 propensity score-matching was conducted to adjust for between-group differences. Results: We identified a total 81,752 patients with met eligible criteria. At discharge, 63,885 (78%) patients were prescribed beta blockers. For 2 years follow up period, regular users were 53,991 (66%) patients, non-users were 10,991 (13%). In the propensity score matched population, regular use of beta blocker was associated with a 36% reduced risk of composite adverse events (all death, MI or stroke) (hazard ratio [HR], 0.636; 95% confidence interval [CI], 0.555–0.728; p<0.001). Compared to no use of beta blocker, regular use significantly reduced all death (HR, 0.736; 95% CI, 0.668–0.812; p<0.001), MI (HR, 0.729; 95% CI, 0.611–0.803; p<0.001) and stroke (HR, 0.717; 95% CI, 0.650–0.791; p<0.001). Conclusions Prescription of beta blocker in patients with AMI after PCI was sequentially increased. Continuous regular use of beta blocker for 2 years after AMI reduced major adverse events compared to no use of beta blocker.

Papillary fibroelastomas are the second most common primary cardiac tumor in adults. Over 80% of fibroelastomas occur on the cardiac valves, usually on the left side of the heart, while the remaining lesions are typically scattered throughout the atria and ventricles. Although the optimal timing for surgery is controversial and depends on tumor size and location, prompt surgical resection is warranted in patients at high risk of embolism. A tumor on the cardiac valve can be removed using the slicing excision technique without leaflet injury. Here we present two cases of papillary fibroelastomas occurring on the ventricular surface of the aortic valve and in the right ventricle.