Background and Objectives: This study aimed to analyze the outcomes of Fontan surgery in the Republic of Korea, as there were only a few studies from Asian countries. Methods: The medical records of 1,732 patients who underwent Fontan surgery in 10 cardiac centers were reviewed. Results: Among them, 1,040 (58.8%) were men. The mean age at Fontan surgery was 4.3±4.2 years, and 395 (22.8%) patients presented with heterotaxy syndrome. According to the types of Fontan surgery, 157 patients underwent atriopulmonary (AP) type; 303, lateral tunnel (LT) type; and 1,266, extracardiac conduit (ECC) type. The overall survival rates were 91.7%, 87.1%, and 74.4% at 10, 20, and 30 years, respectively. The risk factors of early mortality were male, heterotaxy syndrome, AP-type Fontan surgery, high mean pulmonary artery pressure (mPAP) in pre-Fontan cardiac catheterization, and early Fontan surgery year. The risk factors of late mortality were heterotaxy syndrome, genetic disorder, significant atrioventricular valve regurgitation (AVVR) before Fontan surgery, high mPAP in pre-Fontan cardiac catheterization, and no fenestration. Conclusions In Asian population with a high incidence of heterotaxy syndrome, the heterotaxy syndrome was identified as the poor prognostic factors for Fontan surgery. The preoperative low mPAP and less AVVR are associated with better early and long-term outcomes of Fontan surgery.

OBJECTIVES: This study compared the current smoking prevalence among adults with human immunodeficiency virus (HIV) infection to that of the general Korean population and analyzed changes in smoking prevalence and cessation rates from 2009 to 2020. METHODS: The study included a total of 10,980 adults with HIV infection who underwent a health screening examination (National Health Insurance Service-National Health Information Database; NHIS-NHID), 1,230 individuals with HIV infection who participated in the Korea HIV/AIDS Cohort (KoCosHIV), and 76,783 participants from the Korea National Health and Nutrition Examination Survey (KNHANES). We estimated the current smoking prevalence and the quit ratio, defined as the ratio of former smokers to ever-smokers. RESULTS: In the NHIS-NHID and KoCosHIV studies, the prevalence of current and former smoking among adults with HIV was 44.2% (95% confidence interval [CI], 43.2 to 45.1) and 15.6% (95% CI, 14.9 to 16.3), and 47.7% (95% CI, 43.7 to 51.8) and 16.9% (95% CI, 11.8 to 22.0), respectively. In the KNHANES, these rates were 22.5% and 18.1%, respectively. The standardized prevalence ratio of current smoking among adults with HIV was 1.76 in the NHIS-NHID and 1.97 in the KoCosHIV. Furthermore, the likelihood of quitting smoking was lower among adults with HIV than in the general population (NHIS-NHID: 26.1%; 95% CI, 25.0 to 27.1; KoCosHIV: 26.2%; 95% CI, 20.2 to 32.1; KNHANES: 44.6%; 95% CI, 44.5 to 44.6). Among HIV-positive adults, there was a 1.53% decline in the current smoking rate and a 2.86% increase in the quit ratio. CONCLUSIONS Adults with HIV were more likely to smoke and less likely to quit smoking than the general adult population. Tobacco screening and cessation strategies should specifically target this population.

Rare endocrine diseases are complex conditions that require lifelong specialized care due to their chronic nature and associated long-term complications. In Korea, a lack of nationwide data on clinical practice and outcomes has limited progress in patient care. Therefore, the Multicenter Networks for Ideal Outcomes of Pediatric Rare Endocrine and Metabolic Disease (OUTSPREAD) study was initiated. This study involves 30 centers across Korea. The study aims to improve the long-term prognosis of Korean patients with rare endocrine diseases by collecting comprehensive clinical data, biospecimens, and patient-reported outcomes to identify complications and unmet needs in patient care. Patients with childhood-onset pituitary, adrenal, or gonadal disorders, such as craniopharyngioma, congenital adrenal hyperplasia (CAH), and Turner syndrome were prioritized. The planned enrollment is 1,300 patients during the first study phase (2022–2024). Clinical, biochemical, and imaging data from diagnosis, treatment, and follow-up during 1980–2023 were retrospectively reviewed. For patients who agreed to participate in the prospective cohort, clinical data and biospecimens will be prospectively collected to discover ideal biomarkers that predict the effectiveness of disease control measures and prognosis. Patient-reported outcomes, including quality of life and depression scales, will be evaluated to assess psychosocial outcomes. Additionally, a substudy on CAH patients will develop a steroid hormone profiling method using liquid chromatography-tandem mass spectrometry to improve diagnosis and monitoring of treatment outcomes. This study will address unmet clinical needs by discovering ideal biomarkers, introducing evidence-based treatment guidelines, and ultimately improving long-term outcomes in the areas of rare endocrine and metabolic diseases.

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.

OBJECTIVES: This study compared the current smoking prevalence among adults with human immunodeficiency virus (HIV) infection to that of the general Korean population and analyzed changes in smoking prevalence and cessation rates from 2009 to 2020. METHODS: The study included a total of 10,980 adults with HIV infection who underwent a health screening examination (National Health Insurance Service-National Health Information Database; NHIS-NHID), 1,230 individuals with HIV infection who participated in the Korea HIV/AIDS Cohort (KoCosHIV), and 76,783 participants from the Korea National Health and Nutrition Examination Survey (KNHANES). We estimated the current smoking prevalence and the quit ratio, defined as the ratio of former smokers to ever-smokers. RESULTS: In the NHIS-NHID and KoCosHIV studies, the prevalence of current and former smoking among adults with HIV was 44.2% (95% confidence interval [CI], 43.2 to 45.1) and 15.6% (95% CI, 14.9 to 16.3), and 47.7% (95% CI, 43.7 to 51.8) and 16.9% (95% CI, 11.8 to 22.0), respectively. In the KNHANES, these rates were 22.5% and 18.1%, respectively. The standardized prevalence ratio of current smoking among adults with HIV was 1.76 in the NHIS-NHID and 1.97 in the KoCosHIV. Furthermore, the likelihood of quitting smoking was lower among adults with HIV than in the general population (NHIS-NHID: 26.1%; 95% CI, 25.0 to 27.1; KoCosHIV: 26.2%; 95% CI, 20.2 to 32.1; KNHANES: 44.6%; 95% CI, 44.5 to 44.6). Among HIV-positive adults, there was a 1.53% decline in the current smoking rate and a 2.86% increase in the quit ratio. CONCLUSIONS Adults with HIV were more likely to smoke and less likely to quit smoking than the general adult population. Tobacco screening and cessation strategies should specifically target this population.

Background/Aims: The Gout Impact Scale (GIS), a part of the Gout Assessment Questionnaire 2.0, is used to measure gout-specific health-related quality of life (HRQOL). Although several studies have been conducted on the factors affecting the HRQOL of patients with gout, few have focused on lifestyle factors. This study aimed to investigate the correlation between lifestyle habits and HRQOL using the GIS in patients with gout. Methods: We used data from the Urate-Lowering TheRApy in Gout (ULTRA) registry, a prospective cohort of Korean patients with gout treated at multiple centers nationwide. The patients were aged ≥18 years and met the 2015 American College of Rheumatology/European League Against Rheumatism gout classification criteria. They were asked to complete a GIS and questions regarding their lifestyle habits at enrollment. Results: The study included 232 patients. ‘Gout concern overall’ scores in the GIS were significantly lower in patients who exercised more frequently and consumed soft drinks and meat less, and ‘well-being during attack’ scores were significantly lower in patients who consumed vegetables and exercised more frequently. The frequency of vegetable consumption had a negative linear relationship with the ‘well-being during attack’ and ‘gout concern during attack’ scores (p = 0.01, p = 0.001, respectively). The frequency of exercise had a negative linear relationship with the ‘gout concern overall’ and ‘gout concern during attack’ scores (p = 0.04 and p = 0.002, respectively). Conclusions Patients with gout who frequently consumed vegetables and exercised regularly experienced less impact of gout, exhibiting a better GIS that represented HRQOL.