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.

Objective: This study was performed to evaluate the efficacy and safety of lurasidone (160 mg/day) compared to quetiapine XR (QXR; 600 mg/day) in the treatment of acutely psychotic patients with schizophrenia. Methods: Patients were randomly assigned to 6 weeks of double-blind treatment with lurasidone 160 mg/day (n=105) or QXR 600 mg/day (n=105). Primary efficacy measure was the change from baseline to week 6 in Positive and Negative Syndrome Scale (PANSS) total score and Clinical Global Impressions severity (CGI-S) score. Adverse events, body measurements, and laboratory parameters were assessed. Results: Lurasidone demonstrated non-inferiority to QXR on the PANSS total score. Adjusted mean±standard error change at week 6 on the PANSS total score was -26.42±2.02 and -27.33±2.01 in the lurasidone and QXR group, respectively. The mean difference score was -0.91 (95% confidence interval -6.35–4.53). The lurasidone group showed a greater reduction in PANSS total and negative subscale on week 1 and a greater reduction in end-point CGI-S score compared to the QXR group. Body weight, body mass index, and waist circumference in the lurasidone group were reduced, with significantly lower mean change compared to QXR. Endpoint changes in glucose, cholesterol, triglycerides, and low-density lipoprotein levels were also significantly lower. The most common adverse drug reactions with lurasidone were akathisia and nausea. Conclusion Lurasidone 160 mg/day was found to be non-inferior to QXR 600 mg/day in the treatment of schizophrenia with comparable efficacy and tolerability. Adverse effects of lurasidone were generally tolerable, and beneficial effects on metabolic parameters can be expected.

Objective: Dysphagia is a common clinical condition characterized by difficulty in swallowing. It is sub-classified into oropharyngeal dysphagia, which refers to problems in the mouth and pharynx, and esophageal dysphagia, which refers to problems in the esophageal body and esophagogastric junction. Dysphagia can have a significant negative impact one’s physical health and quality of life as its severity increases. Therefore, proper assessment and management of dysphagia are critical for improving swallowing function and preventing complications. Thus a guideline was developed to provide evidence-based recommendations for assessment and management in patients with dysphagia. Methods: Nineteen key questions on dysphagia were developed. These questions dealt with various aspects of problems related to dysphagia, including assessment, management, and complications. A literature search for relevant articles was conducted using Pubmed, Embase, the Cochrane Library, and one domestic database of KoreaMed, until April 2021. The level of evidence and recommendation grade were established according to the Grading of Recommendation Assessment, Development and Evaluation methodology. Results: Early screening and assessment of videofluoroscopic swallowing were recommended for assessing the presence of dysphagia. Therapeutic methods, such as tongue and pharyngeal muscle strengthening exercises and neuromuscular electrical stimulation with swallowing therapy, were effective in improving swallowing function and quality of life in patients with dysphagia. Nutritional intervention and an oral care program were also recommended. Conclusion This guideline presents recommendations for the assessment and management of patients with oropharyngeal dysphagia, including rehabilitative strategies.

Varicella-zoster virus (VZV) is a causative agent for chickenpox in primary infection and shingles after reactivation from latency. Both varicella and zoster can be prevented by live attenuated vaccines, but the molecular mechanism of attenuation is not clearly understood. In this study, the genome sequences of three varicella vaccine strains were analyzed for the genetic diversity including single nucleotide polymorphism (SNP) and genetic polymorphism. A total of 38 SNPs were identified including 29 substitutions and 9 insertion/deletions. The number of genetically polymorphic sites (GPS) was highest in Varivax and lowest in Varilrix. GPS in the R region including R1, R2, and R3 appeared to be responsible for the genetic polymorphisms in the open reading frame (ORF) 11, 14, and 22 in all three vaccine strains. A relatively large number of GPS were observed in ORF31, 55, and 62, which are known to be essential for virus replication, suggesting that the attenuation of the vaccine strains may be attributed by the diversity of these genes.

OBJECTIVETo evaluate the efficacy of Bawu Decoction (, BWD, Palmul-tang in Korean) against benign prostatic hyperplasia (BPH).

METHODSTwenty-four male Wistar rats were divided into 4 groups, with 6 rats in each group. The 4 study groups included sham-operated group (CON), BPH model group, fifinasteride-treated group, and BWD-treated group. All the groups except CON group received a subcutaneous injection of 10 mg/kg of testosterone, while CON group received saline. Finasteride at a dose of 5 mg/kg was administered to the finasteride-treated group for a period of 4 weeks. BWD group received BWD at a dose of 200 mg/kg for 4 weeks. The prostatic weight, prostate weight to body weight ratio, relative prostate weight ratio, serum testosterone and dihydrotestosterone (DHT) level, and histological analysis of prostatic tissue were analyzed.

RESULTSCompared to BPH model group, BWD administration was associated with reductions in prostatic weight, prostate and relative prostate weight ratio weight to body weight ratio (P<0.05). The concentration of serum testosterone and DHT were higher in BPH group compared with CON group (P<0.05). Administration of finasteride and BWD suppressed the elevation of serum testosterone and DHT levels signifificantly (both P<0.05). In addition, BWD suppressed the growth of prostatic tissue (P<0.05).

CONCLUSIONBWD has suppressant effects on development of BPH through inhibition of serum testosterone and DHT.

A large aortic aneurysm invading the aortic arch can be catastrophic if rupture occurs. In the past, the standard treatment was an open thoracotomy followed by total aortic arch replacement. However, open surgery is difficult in patients at high operative risk. Consequently, thoracic endovascular aortic repair (TEVAR) is preferred in high-risk patients. In patients with a short proximal landing whose aortic aneurysm invades the aortic arch, TEVAR is not available because of innominate, left carotid, and left subclavian artery occlusion. We report two cases in which aortic aneurysms invaded the aortic arch, and who were treated with TEVAR after a supra-aortic artery bypass operation.
Aneurysm* ; Aorta ; Aorta, Thoracic* ; Aortic Aneurysm ; Arteries ; Endovascular Procedures ; Humans ; Rupture ; Stents ; Subclavian Artery ; Thoracotomy

A large aortic aneurysm invading the aortic arch can be catastrophic if rupture occurs. In the past, the standard treatment was an open thoracotomy followed by total aortic arch replacement. However, open surgery is difficult in patients at high operative risk. Consequently, thoracic endovascular aortic repair (TEVAR) is preferred in high-risk patients. In patients with a short proximal landing whose aortic aneurysm invades the aortic arch, TEVAR is not available because of innominate, left carotid, and left subclavian artery occlusion. We report two cases in which aortic aneurysms invaded the aortic arch, and who were treated with TEVAR after a supra-aortic artery bypass operation.
Aneurysm* ; Aorta ; Aorta, Thoracic* ; Aortic Aneurysm ; Arteries ; Endovascular Procedures ; Humans ; Rupture ; Stents ; Subclavian Artery ; Thoracotomy

Endomyocardial biopsy (EMB) is one of the reliable methods for the diagnosis of various cardiac diseases. However, EMB can cause various complications. The purpose of this study is to evaluate the complication of transfemoral EMB with both fluoroscopic and two-dimensional (2-D) echocardiographic guidance. A total of 228 patients (148 men; 46.0+/-14.6 yr-old) who underwent EMB at Kyungpook National University Hospital from January 2002 to June 2012 were included. EMB was performed via the right femoral approach with the guidance of both echocardiography and fluoroscopy. Overall, EMB-related complications occurred in 21 patients (9.2%) including one case (0.4%) with cardiac tamponade requiring emergent pericardiocentesis, four cases (1.8%) with small pericardial effusion without pericardiocentesis, two cases (0.9%) with hemodynamically unstable ventricular tachycardia (VT), one case (0.4%) with nonsustained VT, one case (0.4%) with tricuspid regurgitation, twelve cases (5.3%) with right bundle branch block. There was no occurrence of either EMB-related death or cardiac surgery. Left ventricular ejection fraction was significantly lower (32.0+/-18.7% vs 42.0+/-19.1%, P=0.023) and left ventricular end-diastolic dimension was larger (60.0+/-10.0 mm vs 54.2+/-10.2 mm, P=0.013) in patients with EMB related complications than in those without. It is concluded that transfemoral EMB with fluoroscopic and 2-D echocardiographic guidance is a safe procedure with low complication rate.
Adult ; Biopsy/*adverse effects ; Cardiac Tamponade/etiology ; Echocardiography/*adverse effects ; Endocardium/*ultrasonography ; Female ; Fluoroscopy/*adverse effects ; Heart Diseases/*pathology ; Heart Ventricles/metabolism ; Humans ; Male ; Middle Aged ; Pericardial Effusion/etiology ; Tachycardia, Ventricular/etiology ; Ventricular Function