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 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.