Primary aldosteronism (PA) is the most common cause of secondary hypertension and increases the morbidity and mortality associated with cardiovascular diseases. When PA coexists with autonomous cortisol secretion (ACS), the cardiovascular risk increases significantly, especially in cases of bilateral adrenal adenomas with asymmetric hormone secretion, which poses diagnostic and therapeutic challenges. A 50-year-old female presented with hypertension and hypokalemia. PA was diagnosed based on elevated aldosterone levels, suppressed plasma renin activity, and the results of various dynamic endocrine tests. Imaging revealed bilateral adrenal adenomas, and adrenal venous sampling (AVS) confirmed aldosterone hypersecretion from the left adrenal gland and cortisol hypersecretion from both adrenal glands. The patient subsequently underwent left adrenalectomy, which resolved the aldosterone hypersecretion and normalized blood pressure and potassium levels. However, the cortisol hypersecretion persisted. This case highlights the importance of AVS in identifying the sources of hormone secretion and enabling targeted surgical treatment while avoiding bilateral adrenalectomy, which can lead to lifelong adrenal insufficiency. Comprehensive endocrine evaluation, including ACS assessment, in patients with PA is essential to help reduce the cardiovascular risks associated with PA and ACS and thus improve treatment outcomes.

Primary aldosteronism (PA) is the most common cause of secondary hypertension and increases the morbidity and mortality associated with cardiovascular diseases. When PA coexists with autonomous cortisol secretion (ACS), the cardiovascular risk increases significantly, especially in cases of bilateral adrenal adenomas with asymmetric hormone secretion, which poses diagnostic and therapeutic challenges. A 50-year-old female presented with hypertension and hypokalemia. PA was diagnosed based on elevated aldosterone levels, suppressed plasma renin activity, and the results of various dynamic endocrine tests. Imaging revealed bilateral adrenal adenomas, and adrenal venous sampling (AVS) confirmed aldosterone hypersecretion from the left adrenal gland and cortisol hypersecretion from both adrenal glands. The patient subsequently underwent left adrenalectomy, which resolved the aldosterone hypersecretion and normalized blood pressure and potassium levels. However, the cortisol hypersecretion persisted. This case highlights the importance of AVS in identifying the sources of hormone secretion and enabling targeted surgical treatment while avoiding bilateral adrenalectomy, which can lead to lifelong adrenal insufficiency. Comprehensive endocrine evaluation, including ACS assessment, in patients with PA is essential to help reduce the cardiovascular risks associated with PA and ACS and thus improve treatment outcomes.

Primary aldosteronism (PA) is the most common cause of secondary hypertension and increases the morbidity and mortality associated with cardiovascular diseases. When PA coexists with autonomous cortisol secretion (ACS), the cardiovascular risk increases significantly, especially in cases of bilateral adrenal adenomas with asymmetric hormone secretion, which poses diagnostic and therapeutic challenges. A 50-year-old female presented with hypertension and hypokalemia. PA was diagnosed based on elevated aldosterone levels, suppressed plasma renin activity, and the results of various dynamic endocrine tests. Imaging revealed bilateral adrenal adenomas, and adrenal venous sampling (AVS) confirmed aldosterone hypersecretion from the left adrenal gland and cortisol hypersecretion from both adrenal glands. The patient subsequently underwent left adrenalectomy, which resolved the aldosterone hypersecretion and normalized blood pressure and potassium levels. However, the cortisol hypersecretion persisted. This case highlights the importance of AVS in identifying the sources of hormone secretion and enabling targeted surgical treatment while avoiding bilateral adrenalectomy, which can lead to lifelong adrenal insufficiency. Comprehensive endocrine evaluation, including ACS assessment, in patients with PA is essential to help reduce the cardiovascular risks associated with PA and ACS and thus improve treatment outcomes.

Primary aldosteronism (PA) is the most common cause of secondary hypertension and increases the morbidity and mortality associated with cardiovascular diseases. When PA coexists with autonomous cortisol secretion (ACS), the cardiovascular risk increases significantly, especially in cases of bilateral adrenal adenomas with asymmetric hormone secretion, which poses diagnostic and therapeutic challenges. A 50-year-old female presented with hypertension and hypokalemia. PA was diagnosed based on elevated aldosterone levels, suppressed plasma renin activity, and the results of various dynamic endocrine tests. Imaging revealed bilateral adrenal adenomas, and adrenal venous sampling (AVS) confirmed aldosterone hypersecretion from the left adrenal gland and cortisol hypersecretion from both adrenal glands. The patient subsequently underwent left adrenalectomy, which resolved the aldosterone hypersecretion and normalized blood pressure and potassium levels. However, the cortisol hypersecretion persisted. This case highlights the importance of AVS in identifying the sources of hormone secretion and enabling targeted surgical treatment while avoiding bilateral adrenalectomy, which can lead to lifelong adrenal insufficiency. Comprehensive endocrine evaluation, including ACS assessment, in patients with PA is essential to help reduce the cardiovascular risks associated with PA and ACS and thus improve treatment outcomes.

Purpose: A “Smart Cancer Care” platform that integrates patient-reported outcomes (PROs) with management has been established in Korea. This study focused on improving health behaviors and connecting patients to welfare services by introducing and assessing the feasibility of “Smart Cancer Care 2.0,” an enhanced version designed for monitoring complications post-cancer treatment. Materials and Methods: Smart Cancer Care 2.0 was developed by conducting a literature review and consulting with expert panels to identify symptoms or variables requiring monitoring and management guidelines based on the treatment type. Qualitative and quantitative surveys were conducted to assess the feasibility of the app and web system based on the experiences of patients with cancer and healthcare workers. Results: A total of 81 symptoms or variables (chemotherapy-, surgery-, radiotherapy-, rehabilitation-, and health management-related) were selected for management in Smart Cancer Care 2.0. PROs for these symptoms were basically categorized into three severity grades: preventive management, self-treatment, and consultation with a healthcare worker or visit to a healthcare institution. The overall mean scores in the feasibility evaluation by patients and healthcare workers were 3.83 and 3.90 points, respectively, indicating high usefulness. Conclusion Smart Cancer Care 2.0 leverages the existing information and communication technologies–based platform, Smart Cancer Care, and further includes health behaviors and welfare services. Smart Cancer Care 2.0 may play a crucial role in establishing a comprehensive post-discharge management system for patients with cancer as it provides suitable interventions based on patients’ responses and allows the regularly collected PROs to be easily viewed for streamlined care.

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.

Objective: This is the first prospective cohort study of Huntington’s disease (HD) in Korea. This study aimed to investigate the caregiver burden in relation to the characteristics of patients and caregivers. Methods: From August 2020 to February 2022, we enrolled patients with HD from 13 university hospitals in Korea. We used the 12-item Zarit Burden Interview (ZBI-12) to evaluate the caregiver burden. We evaluated the clinical associations of the ZBI-12 scores by linear regression analysis and investigated the differences between the low- and high-burden groups. Results: Sixty-five patients with HD and 45 caregivers were enrolled in this cohort study. The average age at onset of motor symptoms was 49.3 ± 12.3 years, with an average cytosine-adenine-guanine (CAG)n of 42.9 ± 4.0 (38–65). The median ZBI-12 score among our caregivers was 17.6 ± 14.2. A higher caregiver burden was associated with a more severe Shoulson–Fahn stage (p = 0.038) of the patients. A higher ZBI-12 score was also associated with lower independence scale (B = -0.154, p = 0.006) and functional capacity (B = -1.082, p = 0.002) scores of patients. The caregiving duration was longer in the high- than in the low-burden group. Caregivers’ demographics, blood relation, and marital and social status did not affect the burden significantly. Conclusion HD patients’ neurological status exerts an enormous impact on the caregiver burden regardless of the demographic or social status of the caregiver. This study emphasizes the need to establish an optimal support system for families dealing with HD in Korea. A future longitudinal analysis could help us understand how disease progression aggravates the caregiver burden throughout the entire disease course.

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.

Background: This study investigates the long-term efficacy and safety of evogliptin add-on therapy in patients with inadequately controlled type 2 diabetes mellitus (T2DM) previously received dapagliflozin and metformin (DAPA/MET) combination. Methods: In this multicenter randomized placebo-controlled phase 3 trial, patients with glycosylated hemoglobin (HbA1c) levels 7.0% to 10.5% (n=283) previously used DAPA 10 mg plus MET (≥1,000 mg) were randomly assigned to the evogliptin 5 mg once daily or placebo group (1:1). The primary endpoint was the difference in the HbA1c level from baseline at week 24, and exploratory endpoints included the efficacy and safety of evogliptin over 52 weeks (trial registration: ClinicalTrials.gov NCT04170998). Results: Evogliptin add-on to DAPA/MET therapy was superior in HbA1c reduction compared to placebo at weeks 24 and 52 (least square [LS] mean difference, –0.65% and –0.55%; 95% confidence interval [CI], –0.79 to –0.51 and –0.71 to –0.39; P<0.0001). The proportion of patients achieving HbA1c <7% was higher in the triple combination group at week 52 (32.14% vs. 8.51% in placebo; odds ratio, 5.62; P<0.0001). Evogliptin significantly reduced the fasting glucose levels and mean daily glucose levels with improvement in homeostatic model assessment of β-cell function (LS mean difference, 9.04; 95% CI, 1.86 to 16.21; P=0.0138). Adverse events were similar between the groups, and no serious adverse drug reactions were reported in the evogliptin group. Conclusion Long-term triple combination with evogliptin added to DAPA/MET showed superior HbA1c reduction and glycemic control compared to placebo at 52 weeks and was well tolerated.

Objective: : Endothelial colony-forming cells (ECFCs) have been reported to play an important role in the pathogenesis of moyamoya disease (MMD). We have previously observed stagnant growth in MMD ECFCs with functional impairment of tubule formation. We aimed to verify the key regulators and related signaling pathways involved in the functional defects of MMD ECFCs. Methods: : ECFCs were cultured from peripheral blood mononuclear cells of healthy volunteers (normal) and MMD patients. Low-density lipoproteins uptake, flow cytometry, high content screening, senescence-associated β-galactosidase, immunofluorescence, cell cycle, tubule formation, microarray, real-time quantitative polymerase chain reaction, small interfering RNA transfection, and western blot analyses were performed. Results: : The acquisition of cells that can be cultured for a long time with the characteristics of late ECFCs was significantly lower in the MMD patients than the normal. Importantly, the MMD ECFCs showed decreased cellular proliferation with G1 cell cycle arrest and cellular senescence compared to the normal ECFCs. A pathway enrichment analysis demonstrated that the cell cycle pathway was the major enriched pathway, which is consistent with the results of the functional analysis of ECFCs. Among the genes associated with the cell cycle, cyclin-dependent kinase inhibitor 2A (CDKN2A) showed the highest expression in MMD ECFCs. Knockdown of CDKN2A in MMD ECFCs enhanced proliferation by reducing G1 cell cycle arrest and inhibiting senescence through the regulation of CDK4 and phospho retinoblastoma protein. Conclusion : Our study suggests that CDKN2A plays an important role in the growth retardation of MMD ECFCs by inducing cell cycle arrest and senescence.