Atopic dermatitis (AD) is the most prevalent inflammatory skin condition, with approximately 80% of cases originating in childhood and some emerging in adulthood. In South Korea, the estimated prevalence of AD ranges between 10% and 20% in children and 1% and 3% in adults. Severe/recalcitrant AD manifests as a chronic, relapsing skin disorder, persisting with uncontrolled symptoms even after topical steroid treatment. Corticosteroids and systemic immunosuppression, conventionally the standard care for difficult-to-treat diseases, cause numerous undesirable side effects. When AD persists despite topical steroid application, systemic therapies like cyclosporine or systemic steroids become the second treatment strategy. The desire for targeted treatments, along with an enhanced understanding of AD’s pathophysiology, has spurred novel therapeutic development. Recent advances introduce novel systemic options, such as biological agents and small-molecule therapy, tailored to treat severe or recalcitrant AD. Notably, dupilumab, a monoclonal antibody inhibiting interleukin 4 and 13, marked a transformative breakthrough upon gaining approval from the U.S. Food and Drug Administration (FDA) in 2017, leading to a paradigm shift in the systemic treatment of AD. Furthermore, both dupilumab and Janus kinase inhibitors, including baricitinib, abrocitinib, and tofacitinib, now approved by the Korean FDA, have established their applicability in clinical practice. These innovative therapeutic agents have demonstrated favorable clinical outcomes, effectively addressing moderate to severe AD with fewer side reactions than those associated with previous systemic immunosuppressants. This review summarizes the latest advancements and evidence regarding systemic treatments for AD, including newly approved drugs in Korea.

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.

With increasing demands for medical care by society, the medical system, and general citizens and rapid changes in doctor’s awareness, the competencies required of doctors are also changing. The goal of this study was to develop a doctor’s competency framework from the patient’s perspective, and to make it the basis for the development of milestones and entrustable professional activities for each period of medical student education and resident training. To this end, a big data analysis using topic modeling was performed on domestic and international research papers (2011–2020), domestic newspaper articles (2016–2020), and domestic social networking service data (2016–2020) related to doctor’s competencies. Delphi surveys were conducted twice with 28 medical education experts. In addition, a survey was conducted on doctor’s competencies among 1,000 citizens, 407 nurses, 237 medical students, 361 majors, and 200 specialists. Through the above process, six core competencies, 16 sub-competencies, and 47 competencies were derived as subject-oriented doctor’s competencies. The core competencies were: (1) competency related to disease and health as an expert; (2) competency related to patients as a communicator; (3) competency related to colleagues as a collaborator; (4) competency related to society as a health care leader (5) competency related to oneself as a professional, and (6) competency related to academics as a scholar who contributes to the development of medicine.

Defining a competent doctor is important for educating and training doctors. However, competency frameworks have rarely been validated during the process of their development in Korea. The purpose of this study was to validate the patient-centered doctor’s competency framework, which had been developed by our expert working group (EWG). Two rounds of Delphi questionnaire surveys were conducted among a panel of experts on medicine and medical education. The panel members were provided with six core competencies, 17 sub-competencies, and 53 enabling competencies, and were asked to rate the importance of these competencies on a 5-point Likert scale. Between April and July 2021, a total of 28 experts completed both rounds. The data of the Delphi study were analyzed for the mean, standard deviation, median, inter-rater agreement (IRA), and content validity ratio (CVR). A CVR >0.36 and IRA ≥0.75 were deemed to indicate validity and agreement. This study found that five enabling competencies were not valid, and agreement was not reached for three sub-competencies and two enabling competencies. In consideration of CVR and the individual opinions of panel members at each session, the final competencies were extracted through consensus meetings of the EWG. The competencies were modified into six core competencies, 16 sub-competencies, and 47 enabling competencies. This study is meaningful in that it proposes patient-centered doctor’s competencies enabling the development of residents’ milestone competencies, an assessment system, and educational programs.

Defining a competent doctor is important for educating and training doctors. However, competency frameworks have rarely been validated during the process of their development in Korea. The purpose of this study was to validate the patient-centered doctor’s competency framework, which had been developed by our expert working group (EWG). Two rounds of Delphi questionnaire surveys were conducted among a panel of experts on medicine and medical education. The panel members were provided with six core competencies, 17 sub-competencies, and 53 enabling competencies, and were asked to rate the importance of these competencies on a 5-point Likert scale. Between April and July 2021, a total of 28 experts completed both rounds. The data of the Delphi study were analyzed for the mean, standard deviation, median, inter-rater agreement (IRA), and content validity ratio (CVR). A CVR >0.36 and IRA ≥0.75 were deemed to indicate validity and agreement. This study found that five enabling competencies were not valid, and agreement was not reached for three sub-competencies and two enabling competencies. In consideration of CVR and the individual opinions of panel members at each session, the final competencies were extracted through consensus meetings of the EWG. The competencies were modified into six core competencies, 16 sub-competencies, and 47 enabling competencies. This study is meaningful in that it proposes patient-centered doctor’s competencies enabling the development of residents’ milestone competencies, an assessment system, and educational programs.

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.

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.

With increasing demands for medical care by society, the medical system, and general citizens and rapid changes in doctor’s awareness, the competencies required of doctors are also changing. The goal of this study was to develop a doctor’s competency framework from the patient’s perspective, and to make it the basis for the development of milestones and entrustable professional activities for each period of medical student education and resident training. To this end, a big data analysis using topic modeling was performed on domestic and international research papers (2011–2020), domestic newspaper articles (2016–2020), and domestic social networking service data (2016–2020) related to doctor’s competencies. Delphi surveys were conducted twice with 28 medical education experts. In addition, a survey was conducted on doctor’s competencies among 1,000 citizens, 407 nurses, 237 medical students, 361 majors, and 200 specialists.Through the above process, six core competencies, 16 sub-competencies, and 47 competencies were derived as subject-oriented doctor’s competencies. The core competencies were: (1) competency related to disease and health as an expert; (2) competency related to patients as a communicator; (3) competency related to colleagues as a collaborator; (4) competency related to society as a health care leader (5) competency related to oneself as a professional, and (6) competency related to academics as a scholar who contributes to the development of medicine.

Purpose: Atypical teratoid/rhabdoid tumor (ATRT) is a highly aggressive malignancy with peak incidence in children aged less than 3 years. Standard treatment for central nervous system ATRT in children under the age of 3 years have not been established yet. The objective of this study was to analyze characteristics and clinical outcomes of ATRT in children aged less than 3 years. Materials and Methods: A search of medical records from seven centers was performed between January 2005 and December 2016. Results: Forty-three patients were enrolled. With a median follow-up of 90 months, 27 patients (64.3%) showed at least one episode of disease progression (PD). The first date of PD was at 160 days after diagnosis. The 1- and 3-year progression-free survivals (PFS) were 51.2% and 28.5%, respectively. The 1- and 3-year overall survivals were 61.9% and 38.1%, respectively. The 3-year PFS was improved from 0% in pre-2011 to 47.4% in post-2011. Excluding one patient who did not receive any further therapy after surgery, 27 patients died due to PD (n=21), treatment-related toxicity (n=5), or unknown cause (n=1). In univariate analysis, factors associated with higher 3-year PFS were no metastases, diagnosis after 2011, early adjuvant radiotherapy, and high-dose chemotherapy (HDCT). In multivariate analysis, the use of HDCT and adjuvant radiotherapy remained significant prognostic factors for PFS (both p < 0.01). Conclusion Aggressive therapy including early adjuvant radiotherapy and HDCT could be considered to improve outcomes of ATRT in children under the age of 3 years.