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.

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.

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: The Fleischner Society established consensus guidelines for imaging in patients with coronavirus disease 2019 (COVID-19). We investigated the prevalence of pneumonia and the adverse outcomes by dividing groups according to the symptoms and risk factors of patients and assessed the suitability of the Fleischner society imaging guidelines in evaluating chest radiographs of COVID-19 patients. Methods: From February 2020 to May 2020, 685 patients (204 males, mean 58 ± 17.9 years) who were diagnosed with COVID-19 and hospitalized were included. We divided patients into four groups according to the severity of symptoms and presence of risk factors (age > 65 years and presence of comorbidities). The patient groups were defined as follows: group 1 (asymptomatic patients), group 2 (patients with mild symptoms without risk factors), group 3 (patients with mild symptoms and risk factors), and group 4 (patients with moderate to severe symptoms). According to the Fleischner society, chest imaging is not indicated for groups 1–2 but is indicated for groups 3–4. We compared the prevalence and score of pneumonia on chest radiographs and compare the adverse outcomes (progress to severe pneumonia, intensive care unit admission, and death) between groups. Results: Among the 685 COVID-19 patients, 138 (20.1%), 396 (57.8%), 102 (14.9%), and 49 (7.1%) patients corresponded to groups 1 to 4, respectively. Patients in groups 3–4 were significantly older and showed significantly higher prevalence rates of pneumonia (group 1–4: 37.7%, 51.3%, 71.6%, and 98%, respectively, P < 0.001) than those in groups 1-2. Adverse outcomes were also higher in groups 3–4 than in groups 1–2 (group 1–4: 8.0%, 3.5%, 6.9%, and 51%, respectively, P < 0.001). Patients with adverse outcomes in group 1 were initially asymptomatic but symptoms developed during follow-up. They were older (mean age, 80 years) and most of them had comorbidities (81.8%). Consistently asymptomatic patients had no adverse events. Conclusion The prevalence of pneumonia and adverse outcomes were different according to the symptoms and risk factors in COVID-19 patients. Therefore, as the Fleischner Society recommended, evaluation and monitoring of COVID-19 pneumonia using chest radiographs is necessary for old symptomatic patients with comorbidities.

Purpose: In delayed myelopathy (DM) from osteoporotic vertebral compression fractures (OVCF), the mechanisms of spinal cord impingement differ according to the stability of the fractured vertebra. This study examined the availability of a posterior surgery comprised of pedicle screw augmentation with polymethylmethacrylate (PMMA) and selective anterior reinforcement (PS-PMMA+SAR) according to the stability. Materials and Methods: This was a retrospective single-center study. The consecutive patients who had a PS-PMMA+SAR for a DM from OVCF and were followed up more than two years were reviewed. Thirty patients were eligible for this study. All patients used fenestrated screws for PMMA augmentation. Anterior reinforcement was selected according to the stability. The unstable type was done by filling the vacuum cleft with bone chips or PMMA, and the stable type was done by vertebral body anterior translation with/without an interbody bone graft. The radiological and functional outcomes were evaluated. Results: There were 20 unstable and 10 stable cases. The regional kyphotic angle was improved significantly from 31.3°±10.8° to 10.4°±8.3° (p<0.001). The anterior vertebral height ratio was improved significantly from 39.4%±17.1% to 86.4%±9.2% (p<0.001). The spinal canal invasion ratio was improved significantly from 39.2%±14.8% to 19.1%±10.8% (p<0.001). The walking ability was improved mostly by two Nurick’s grades (p<0.001). The Oswestry disability index was improved from 72% to 33% (p<0.001). Conclusion Posterior surgery with PMMA-augmented pedicle screws, and selective anterior reinforcement for DM from OVCF was available to achieve a good functional and radiological outcome.

Background and objectives: Athlete’s heart is characterized by structural cardiac changes, including enlargement and hypertrophy. However, exercise-induced cardiac electrical remodeling is not well known in Asian athletes. We sought to evaluate the association between vigorous exercise and the development of abnormal late potential on signal-averaged electrocardiogram (SAECG).Method: We analyzed 48 Korean professional soccer players and 71 healthy sedentary controls who underwent SAECG and transthoracic echocardiography at Kyung Hee University Hospital. An SAECG was considered abnormal (positive for ventricular late potential) when any one of the three following criteria was met: filtered QRS dura‑ tion > 114 ms, root-mean-square voltage in the terminal 40 ms < 20 uV, or a voltage < 40 uV for more than 38 ms. Results: Fragmented QRS was more commonly found in athletes (1.4% vs. 10.4%). Athletes demonstrated signifi‑ cantly higher proportion of filtered QRS duration > 114 ms (7.0% vs. 22.9%, P = 0.013) and lower terminal QRS rootmean-square voltage < 20 uV (5.6% vs. 20.8%, P = 0.012). Ventricular late potential on SAECG was significantly more frequent in athletes (15.5% vs. 35.4%, P = 0.012). Regarding echocardiographic parameters, the athletes had larger cardiac chamber size; however, these differences became non-significant after adjustment for body surface area, except left ventricular mass index (65.7 ± 12.7 g/m2 vs. 84.7 ± 17.7 g/m2 , P < 0.001). Conclusion Abnormal SAECG findings were significantly more common in athletes than in controls. Further study is needed to determine the clinical impact of these abnormal SAECGs in athletes and cardiac outcomes in the long term.

Background/Aims: The clinical characteristics of patients with masked uncontrolled hypertension (MUCH) have been poorly defined, and few studies have investigated the clinical predictors of MUCH. We investigated the demographic, clinical, and blood pressure (BP) characteristics of patients with MUCH and proposed a prediction model for MUCH in patients with hypertension. Methods: We analyzed 1,986 subjects who were enrolled in the Korean Ambulatory Blood Pressure Monitoring (Kor-ABP) Registry and taking antihypertensive drugs, and classified them into the controlled hypertension (n = 465) and MUCH (n = 389) groups. MUCH was defined as the presence of a 24-hour ambulatory mean systolic BP ≥ 130 mmHg and/or diastolic BP ≥ 80 mmHg in patients treated with antihypertensive drugs, having normal office BP. Results: Patients in the MUCH group had significantly worse metabolic profiles and higher office BP, and took significantly fewer antihypertensive drugs compared to those in the controlled hypertension group. Multivariate logistic regression analyses identified high office systolic BP and diastolic BP, prior stroke, dyslipidemia, left ventricular hypertrophy (LVH, ≥ 116 g/m2 for men, and ≥ 96 g/m2 for women), high heart rate (≥ 75 beats/min), and single antihypertensive drug use as independent predictors of MUCH. A prediction model using these predictors showed a high diagnostic accuracy (C-index of 0.839) and goodness-of-fit for the presence of MUCH. Conclusions MUCH is associated with a high-normal increase in office BP and underuse of antihypertensive drugs, as well as dyslipidemia, prior stroke, and LVH, which could underscore achieving optimal BP control. The proposed model accurately predicts MUCH in patients with controlled office BP.

Background and objectives: Athlete’s heart is characterized by structural cardiac changes, including enlargement and hypertrophy. However, exercise-induced cardiac electrical remodeling is not well known in Asian athletes. We sought to evaluate the association between vigorous exercise and the development of abnormal late potential on signal-averaged electrocardiogram (SAECG).Method: We analyzed 48 Korean professional soccer players and 71 healthy sedentary controls who underwent SAECG and transthoracic echocardiography at Kyung Hee University Hospital. An SAECG was considered abnormal (positive for ventricular late potential) when any one of the three following criteria was met: filtered QRS dura‑ tion > 114 ms, root-mean-square voltage in the terminal 40 ms < 20 uV, or a voltage < 40 uV for more than 38 ms. Results: Fragmented QRS was more commonly found in athletes (1.4% vs. 10.4%). Athletes demonstrated signifi‑ cantly higher proportion of filtered QRS duration > 114 ms (7.0% vs. 22.9%, P = 0.013) and lower terminal QRS rootmean-square voltage < 20 uV (5.6% vs. 20.8%, P = 0.012). Ventricular late potential on SAECG was significantly more frequent in athletes (15.5% vs. 35.4%, P = 0.012). Regarding echocardiographic parameters, the athletes had larger cardiac chamber size; however, these differences became non-significant after adjustment for body surface area, except left ventricular mass index (65.7 ± 12.7 g/m2 vs. 84.7 ± 17.7 g/m2 , P < 0.001). Conclusion Abnormal SAECG findings were significantly more common in athletes than in controls. Further study is needed to determine the clinical impact of these abnormal SAECGs in athletes and cardiac outcomes in the long term.

Background/Aims: The clinical characteristics of patients with masked uncontrolled hypertension (MUCH) have been poorly defined, and few studies have investigated the clinical predictors of MUCH. We investigated the demographic, clinical, and blood pressure (BP) characteristics of patients with MUCH and proposed a prediction model for MUCH in patients with hypertension. Methods: We analyzed 1,986 subjects who were enrolled in the Korean Ambulatory Blood Pressure Monitoring (Kor-ABP) Registry and taking antihypertensive drugs, and classified them into the controlled hypertension (n = 465) and MUCH (n = 389) groups. MUCH was defined as the presence of a 24-hour ambulatory mean systolic BP ≥ 130 mmHg and/or diastolic BP ≥ 80 mmHg in patients treated with antihypertensive drugs, having normal office BP. Results: Patients in the MUCH group had significantly worse metabolic profiles and higher office BP, and took significantly fewer antihypertensive drugs compared to those in the controlled hypertension group. Multivariate logistic regression analyses identified high office systolic BP and diastolic BP, prior stroke, dyslipidemia, left ventricular hypertrophy (LVH, ≥ 116 g/m2 for men, and ≥ 96 g/m2 for women), high heart rate (≥ 75 beats/min), and single antihypertensive drug use as independent predictors of MUCH. A prediction model using these predictors showed a high diagnostic accuracy (C-index of 0.839) and goodness-of-fit for the presence of MUCH. Conclusions MUCH is associated with a high-normal increase in office BP and underuse of antihypertensive drugs, as well as dyslipidemia, prior stroke, and LVH, which could underscore achieving optimal BP control. The proposed model accurately predicts MUCH in patients with controlled office BP.

We retrospectively reviewed patients with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infections who wereadmitted to an intensive care unit in Daegu, South Korea. The outcomes of patients who did (cases) or did not (controls) receivedarunavir-cobicistat (800–150 mg) therapy were compared. Fourteen patients received darunavir-cobicistat treatment, and 96 receivedother antiviral therapy (controls). Overall, the darunavir-cobicistat group comprised patients with milder illness, and thecrude mortality rate of all patients in the darunavir-cobicistat group was lower than that in the controls [odds ratio (OR) 0.20, 95%confidence interval (CI) 0.04–0.89, p=0.035]. After 1:2 propensity-score matching, there were 14 patients in the darunavir-cobicistatgroup, and 28 patients in the controls. In propensity score-matched analysis, the darunavir-cobicistat group had lower mortalitythan the controls (OR 0.07, 95% CI 0.01–0.52, p=0.009). In conclusion, darunavir-cobicistat therapy was found to be associatedwith a significant survival benefit in critically ill patients with SARS-CoV-2 infection.