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.

Background: Atherogenic dyslipidemia, which is frequently associated with type 2 diabetes (T2D) and insulin resistance, contributes to the development of vascular complications. Statin therapy is the primary approach to dyslipidemia management in T2D, however, the role of non-statin therapy remains unclear. Ezetimibe reduces cholesterol burden by inhibiting intestinal cholesterol absorption. Fibrates lower triglyceride levels and increase high-density lipoprotein cholesterol (HDL-C) levels via peroxisome proliferator- activated receptor alpha agonism. Therefore, when combined, these drugs effectively lower non-HDL-C levels. Despite this, few clinical trials have specifically targeted non-HDL-C, and the efficacy of triple combination therapies, including statins, ezetimibe, and fibrates, has yet to be determined. Methods: This is a multicenter, prospective, randomized, open-label, active-comparator controlled trial involving 3,958 eligible participants with T2D, cardiovascular risk factors, and elevated non-HDL-C (≥100 mg/dL). Participants, already on moderate-intensity statins, will be randomly assigned to either Ezefeno (ezetimibe/fenofibrate) addition or statin dose-escalation. The primary end point is the development of a composite of major adverse cardiovascular and diabetic microvascular events over 48 months. Conclusion This trial aims to assess whether combining statins, ezetimibe, and fenofibrate is as effective as, or possibly superior to, statin monotherapy intensification in lowering cardiovascular and microvascular disease risk for patients with T2D. This could propose a novel therapeutic approach for managing dyslipidemia in T2D.

Background: Primary cicatricial alopecia (PCA) is a rare disease that causes irreversible destruction of hair follicles and affects the quality of life (QOL). Objective: We aimed to investigate the disease awareness, medical use behavior, QOL, and real-world diagnosis and treatment status of patients with PCA. Methods: A self-administered questionnaire was administered to patients with PCA and their dermatologists. Patients aged between 19 and 75 years who visited one of 27 dermatology departments between September 2021 and September 2022 were included. Results: In total, 274 patients were included. The male-to-female ratio was 1:1.47, with a mean age of 45.7 years. Patients with neutrophilic and mixed PCA were predominantly male and younger than those with lymphocytic PCA. Among patients with lymphocytic PCA, lichen planopilaris was the most common type, and among those with neutrophilic PCA, folliculitis decalvans was the most common type. Among the total patients, 28.8% were previously diagnosed with PCA, 47.0% were diagnosed with PCA at least 6 months after their first hospital visit, 20.0% received early treatment within 3 months of disease onset, and 54.4% received steady treatment. More than half of the patients had a moderate to severe impairment in QOL. Topical/intralesional steroid injections were the most common treatment. Systemic immunosuppressants were frequently prescribed to patients with lymphocytic PCA, and antibiotics were mostly prescribed to patients with neutrophilic PCA. Conclusion This study provides information on the disease awareness, medical use behavior, QOL, diagnosis, and treatment status of Korean patients with PCA. This can help dermatologists educate patients with PCA to understand the necessity for early diagnosis and steady treatment.

Interleukin-1 receptor-associated kinase 4 (IRAK4) is a pivotal enzyme in the Toll-like receptor (TLR)/MYD88 dependent signaling pathway, which is highly activated in rheumatoid arthritis tissues and activated B cell-like diffuse large B-cell lymphoma (ABC-DLBCL). Inflammatory responses followed by IRAK4 activation promote B-cell proliferation and aggressiveness of lymphoma. Moreover, proviral integration site for Moloney murine leukemia virus 1 (PIM1) functions as an anti-apoptotic kinase in propagation of ABC-DLBCL with ibrutinib resistance. We developed a dual IRAK4/PIM1 inhibitor KIC-0101 that potently suppresses the NF-κB pathway and proinflammatory cytokine induction in vitro and in vivo. In rheumatoid arthritis mouse models, treatment with KIC-0101 significantly ameliorated cartilage damage and inflammation. KIC-0101 inhibited the nuclear translocation of NF-κB and activation of JAK/STAT pathway in ABC-DLBCLs. In addition, KIC-0101 exhibited an anti-tumor effect on ibrutinib-resistant cells by synergistic dual suppression of TLR/MYD88-mediated NF-κB pathway and PIM1 kinase. Our results suggest that KIC-0101 is a promising drug candidate for autoimmune diseases and ibrutinib-resistant B-cell lymphomas.

Background: This study aimed to 1) assess the effect of total hip arthroplasty (THA) on coronal limb alignment, namely, the hip–knee–ankle angle (HKA), 2) identify factors that determine changes in the HKA, and 3) determine whether alignment changes influence the knee joint space width. Methods: We retrospectively evaluated 266 limbs of patients who underwent THA. Three types of prostheses with neck shaft angles (NSAs) of 132°, 135°, and 138° were used. Several radiographic parameters were measured in the preoperative and final radiographs (at least 5 years after THA). A paired t-test was used to confirm the effect of THA on HKA change.Multiple regression analysis was performed to identify radiographic parameters related to HKA changes following THA and changes in knee joint space width. Subgroup analyses were performed to reveal the effect of NSA change on the HKA change, and the proportion of total knee arthroplasty usage and changes in radiographic parameters between maintained joint space and narrowed joint space groups were compared. Results: The preoperative mean HKA was 1.4° varus and increased to 2.7° varus after THA.This change was related to changes in the NSA, lateral distal femoral angle, and femoral bowing angle. In particular, in the group with a decrease in NSA of > 5°, the preoperative mean HKA was largely changed from 1.4° varus to 4.6° varus after THA. The prostheses with NSA of 132° and 135° also led to greater varus HKA changes than those with an NSA of 138°. Narrowing of the medial knee joint space was related to changes in the varus direction of the HKA, decrease in NSA, increase in femoral offset. Conclusion A large reduction in NSA can lead to considerable varus limb alignment after THA, which can have adverse effects on the medial compartment of the ipsilateral knee.

Sarcomatoid carcinoma is rarely diagnosed as gallbladder cancer. Its aggressive nature, due to the characteristics of both sarcoma and carcinoma, results in a poor prognosis. We report a case of gallbladder sarcomatoid carcinoma in an 82-year-old male who was referred to our hospital for evaluation of gallbladder cancer observed on abdominopelvic computed tomography. The characteristics of the cancer were not confirmed after several imaging modalities. The surgically resected tumor was positive for both cytokeratin and vimentin as revealed via immunohistochemical staining, and a sarcomatoid carcinoma was finally diagnosed. The role of chemotherapy has not yet been identified. Therefore, radiation therapy is planned to reduce the risk of recurrence.

Anaplastic large cell lymphoma (ALCL) is a subtype of non-Hodgkin lymphoma, that was first described by Stein et al. in 1985. In most cases, ALCL invades the lymph nodes, but primary extranodal lymphoma can occur in the gastrointestinal tract, skin, bone, central nervous system, and lungs. Moreover, the occurrence of primary ALCL in the skeletal muscle is extremely rare. This paper reports a case of primary ALCL involving the iliopsoas, adductor, pectineus, and obturator muscles around the left hip in a nine-year-old girl with a review of the relevant literature.

Purpose: Although adjuvant chemotherapy (CTx) is still recommended for high-risk patients with hormone receptorpositive and human epidermal receptor (HER)-2-negative breast cancer, recent studies found that selected patients with low disease burden may be spared from CTx and receive hormonal treatment (HT) alone. This study aims to evaluate the trends of treatment (CTx + HT vs. HT alone) in Korea and to assess the impact on overall survival (OS) according to treatment pattern. Methods: The Korean Breast Cancer Society Registry was queried (2000 to 2018) for women with pT1-2N0-1 hormone receptor-positive and HER2-negative disease who underwent surgery and adjuvant systemic treatment (CTx and HT). Clinicopathologic factors, change in pattern of treatment over time, and OS for each treatment option were analyzed. Results: A total of 40,938 women were included in the study; 20,880 (51.0%) received CTx + HT, while 20,058 (49.0%) received HT only. In recent years, there has been a steady increase in the use of HT alone, from 21.0% (2000) to 64.6% (2018). In Cox regression analysis, age, type of breast and axillary operations, T and N stages, body mass index, histologic grade,and presence of lymphovascular invasion were prognostic indicators for OS. There was no significant difference between CTx + HT and HT alone in terms of OS (P = 0.126). Conclusion Over the years, there has been a shift from CTx + HT to HT alone without a significant difference in OS. Therefore, HT alone could be a safe treatment option in selected patients, even those with T2N1 disease.

Background: Pediatric alopecia areata (AA) can affect the quality of life (QoL) of patients and their family members. Research on the QoL and burden on family members in pediatric AA is limited. Objective: This nationwide multicenter questionnaire study described the QoL and burden of the family members of patients with pediatric AA. Methods: This nationwide multicenter questionnaire study enrolled AA patients between the ages of 5 and 18 years from March 1, 2017 to February 28, 2018. Enrolled patients and their parents completed the modified Children’s Dermatology Life Quality Index (CDLQI) and the modified Dermatitis Family Impact (mDFI). The disease severity was measured using the Severity of Alopecia Tool (SALT) survey scores. Results: A total of 268 patients with AA from 22 hospitals participated in this study. Our study found that the efficacy and satisfaction of previous treatments of AA decreased as the severity of the disease increased. The use of home-based therapies and traditional medicines increased with the increasing severity of the disease, but the efficacy felt by patients was limited. CDLQI and mDFI scores were higher in patients with extensive AA than those with mild to moderate AA. The economic and time burden of the family members also increased as the severity of the disease increased. Conclusion The severity of the AA is indirectly proportional to the QoL of patients and their family members and directly proportional to the burden. Physicians need to understand these characteristics of pediatric AA and provide appropriate intervention to patients and their family members.

Background: The purpose of this study was to analyze the epidemiology of T-score discordance between the spine and femur in the South Korean population and compare the prevalence of T-score discordance between the Korean osteoporosis population and atypical femoral fracture (AFF) patients. Methods: A total of 12,422 subjects from the Korea National Health and Nutrition Examination Survey were reviewed retrospectively. T-score discordance was defined as a difference of ≥ 1 standard deviation between the lumbar spine (LS) and femoral neck (FN) bone mineral density (BMD). The prevalence of T-score discordance (low LS [LS BMD < FN BMD], low FN [LS BMD > FN BMD], and total [low LS + low FN]) was investigated in the osteoporosis and non-osteoporosis groups and stratified by sex and age. Tscore discordance of 63 patients with AFFs diagnosed at a single institution was compared with that of the Korean osteoporosis population using propensity score matching. Results: T-score discordance was prevalent in the Korean osteoporosis population (44.8%), and low LS discordance (37.5%) was more frequently seen than low FN discordance (7.2%) (p < 0.001). The prevalence of total and low LS discordance was significantly higher in AFF patients than in the Korean osteoporosis population (total discordance: 69.8% and 42.5%, respectively; low LS discordance: 63.5% and 31.7%, respectively; p < 0.001). Conclusions T-score discordance was highly prevalent in the Korean osteoporosis population, and low LS discordance was more common than low FN discordance. Nevertheless, the prevalence of low LS discordance was significantly higher in AFF patients than in the Korean osteoporosis population.