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: We investigated the prevalence of pneumonia in novel coronavirus disease 2019 (COVID-19) patients using chest radiographs to identify the characteristics of those with initially negative chest radiographs, who were positive for pneumonia on follow-up. Materials and Methods: Retrospective cohort data of 236 COVID-19 patients were reviewed. Chest radiography was performed on admission, with serial radiographs obtained until discharge. The ‘positive conversion group’ was defined as patients whose initial chest radiographs were negative but were positive for pneumonia during follow-up. Patients with initially positive chest radiographs were defined as the ‘initial pneumonia group.’ Patients with negative initial and follow-up chest radiographs were defined as the ‘non-pneumonia group.’ Clinical and laboratory findings were compared between groups, and predictors of positive conversion were investigated. Results: Among 236 patients, 108 (45.8%) were in the non-pneumonia group, 69 (29.2%) were in the initial pneumonia group, and 59 (25%) were in the positive conversion group. The patients in the ‘initial pneumonia group’ and ‘positive conversion group’ were older, had higher C-reactive protein (CRP) and lactate dehydrogenase levels, and lower absolute lymphocyte counts than those in the ‘non-pneumonia group’ (all p < 0.001). Among patients with negative initial chest radiographs, age ≥ 45 years (odds ratio [OR]: 3.93, 95% confidence interval [CI]: 1.76–8.75, p = 0.001), absolute lymphocyte count < 1500 cells/µL (OR: 2.25, 95% CI: 1.03–4.89,p = 0.041), and CRP > 0.5 mg/dL (OR: 3.91, 95% CI: 1.54–9.91, p = 0.004) were independent predictors for future development of pneumonia. Conclusion More than a half of COVID-19 patients initially had normal chest radiographs; however, elderly patients (≥ 45 years of age) with abnormal laboratory findings (elevated CRP and low absolute lymphocyte counts) developed pneumonia on follow-up radiographs.

Guillain-Barré syndrome (GBS) is the most common immune-mediated polyradiculoneuropathy and it is also the most commonly reported severe adverse event following immunization in adults. To evaluate the results of clinical and laboratory features of GBS after vaccination in Korea, we analyzed the claims-based data from 2002 to 2014 using materials collected for the Advisory Committee Vaccination Injury Compensation (ACVIC) meeting including, clinical features, nerve conduction studies (NCSs), cerebrospinal fluid (CSF) profiles, treatment, and outcomes. Forty-eight compensated GBS cases (median age, 15 years; interquartile range [IQR], 13–51; male:female ratio, 1:1) of 68 suspected GBS were found following immunization and all of them with influenza immunizations with either monovalent (n = 35) or trivalent (n = 13). Among them, 30 cases fulfilled the Brighton criteria level 1–3 (62.5%). The median duration between the onset of symptoms to nadir, duration of the nadir, and total admission period were 3 (IQR, 2–7 days), 2 (IQR, 1–5 days), and 14 (IQR, 6–33 days) days, respectively. The most frequently reported symptom was quadriparesis which was present in 36 cases (75%) at nadir. CSF examination revealed albuminocytologic dissociation in 25.0% and NCS was abnormal in 61.8%. After treatment, most of them showed improvement. Clinical features were similar to typical post-infectious GBS and there were both demyelinating and axonal forms suggesting heterogeneous pathogenic mechanism. In order to improve the diagnostic certainty of post-vaccination GBS, careful documentation of clinical features and timely diagnostic work-up with follow-up studies are needed.
Adult ; Advisory Committees ; Axons ; Cerebrospinal Fluid ; Compensation and Redress ; Follow-Up Studies ; Guillain-Barre Syndrome* ; Humans ; Immunization ; Influenza, Human ; Korea* ; Neural Conduction ; Polyradiculoneuropathy ; Quadriplegia ; Vaccination

Aggression and aggressive behaviors, often explained as harmful social interaction with the intention of hurting or inflicting damage upon another, have been considered as an adaptive mechanism from the evolutionary psychological point of view. However, various studies on aggression and aggressive behaviors have been done with psychopathological approach as the extreme aggressive behaviors may harm themselves and others at the same time. Recently, researchers have attempted to explain aggression in terms of neurobiological substrates rather than based on traditional psychopathological and/or behavioral concept. In this regard, there have been findings of differences in neurotransmitters and their receptors, and genetic polymorphisms. In this review article, we provide a brief overview of the literature about seven most frequently reported neurotransmitters including neurohormones (serotonin, norepinephrine, dopamine, gamma-aminobutyric acid, nitric oxide, oxytocin and vasopressin) and an associated enzyme (monoamine oxidase A), which are known to be related with aggression and aggressive behaviors.
Aggression* ; Dopamine ; gamma-Aminobutyric Acid ; Intention ; Interpersonal Relations ; Neurobiology ; Neurotransmitter Agents* ; Nitric Oxide ; Norepinephrine ; Oxidoreductases ; Oxytocin ; Polymorphism, Genetic

We determined the genetic diversity and distance between Jeju and Thoroughbred horses by genotyping for 20 microsatellite loci consisting of (TG)n repetitive sequence. The expected heterozygosity ranged from 0.1 to 0.789 in the Jeju horses and from 0.505 to 0.824 in the Thoroughbred horses. Polymorphic information content (PIC) values ranged from 0.09 to 0.709 in the Jeju horses and 0.365 to 0.730 in Thoroughbred horses. There were no significant differences in heterozygosity and PIC values between Jeju and Thoroughbred horsesHowever, LEX035 was estimated relatively high heterozygosity (0.789) and PIC value 0.709) in Jeju horses and LEX050 was respectively 0.824, and 0.730 in the Thoroughbred horses. We may conclude that the genetic differentiation was low between Jeju and Thoroughbred horses. LEX 050, LEX055, LEX059 and LEX 063 could be used as geneticmarkers for differentiating Jeju from Thoroughbred horses.
DNA ; Genetic Markers ; Genetic Variation ; Horses ; Korea ; Microsatellite Repeats ; Polymorphism, Genetic ; Repetitive Sequences, Nucleic Acid

In order to study the treatment of aneurysms, the technique of making experimental aneurysms in laboratory animals must be established. In our study, to examine the feasibility of making experimental aneurysm and selective angiography on the common carotid artery in rabbits and to determine the size of experimental aneurysm after surgery, saccular aneurysms were fashioned on the right common carotid artery in 17 rabbits using a vein pouch technique. Selective angiography of the common carotid artery was performed immediately after surgery, and at 1 week, 4 weeks, and 8 weeks after surgery. Also, histological changes in the aneurysms were observed. In 16 rabbits with established successful experimental aneurysm, no differences were found in diet intake and behavior before and after surgery. The patency of the carotid artery was confirmed by selective angiography. The average size of the aneurysm immediately after surgery was similar to that of 1 week postoperatively in selective angiography, however it increased with time at 4weeks and 8 weeks. Histologically, infiltration of inflammatory cells and hemorrhage were found at the junction of the carotid artery and the vein pouch at 1 week, which disappeared at 4 weeks and 8 weeks. This study suggests experimental saccular aneurysm using the vein pouch technique might form aneurysms similar to that of the human in its properties such as increment of size, and selective angiography might be suitable for assessment of experimental aneurysm. Therefore, this animal model may be suitable for investigating new treatment methodologies for human aneurysms.
Aneurysm ; Angiography ; Animals, Laboratory ; Carotid Arteries ; Carotid Artery, Common ; Diet ; Hemorrhage ; Humans ; Models, Animal ; Rabbits ; Veins

Kaposi sarcoma is a rare tumor originated from skin in patients with immune suppressive illness like as acquired immunodeficiency syndrome (AIDS) or organ transplantation. It may be widely disseminated internally such as digestive or respiratory organ. In Korean patients with AIDS, Kaposi sarcoma is rarely seen rather than western countries. The reason is unknown. Although few cases of Kaposi sarcoma in patients with AIDS had been described in the Korean literatures, multi-organ involved cases were extremely rare. We describe a case of old AIDS patient in whom Kaposi sarcoma had affected multi-organs including esophagus, stomach, duodenum, colon, bronchi and skin.
Acquired Immunodeficiency Syndrome ; Bronchi* ; Colon ; Duodenum ; Esophagus ; Gastrointestinal Tract* ; Humans ; Organ Transplantation ; Sarcoma, Kaposi* ; Skin ; Stomach ; Transplants

BACKGROUND: This study was to evaluate a nationwide nosocomial infection rate and antimicrobial resistance in intensive care units(ICUs) in Korea. METHODS: The study was carried out at 16 university-affiliated teaching hospitals from July through October 2004. We performed a prospective multicenter study to investigate nosocomial infection rates, device-associated infection rated, and causative pathogens and their antimicrobial resistance. RESULTS: The urinary tract was the most commonly involved site. Nosocomial infection rate was 12.48 in medical. ICU (MICU), 9.59 in medical surgical ICU (MSICU), 14.76 in surgical ICU (MSICU), and 11.60 in other lCU. Device-associated infection rates were as follow: 1) rates of urinary catheter-associated urinary tract infection were 4.26 in MICU, 3.17 in SICU, 4.88 in MSICU, and 5.87 in other ICU; 2) rates of central line-associated bloodstream infection were 3.24 in MICU, 1.56 in SlCU, 2.36 in MSICU, and 1.78 in other ICU; 3) rates of ventilator-associated pneumonia were 3.61 in MlCU, 13.05 in SICU, 1.68 in MSICU, and 4.84 in other lCU. Staphylococcus aureus was the most frequently identified microorganism in this study; 93% of S. aurues were resistant to methicillin; 17% of Pseudomonas aeruginosa isolated were resistant to imipenem; 11% of Enterococcus faecium and 18% of Enterococcus faecalis showed resistance to vancomycin. Over a half of Acinetobacter spp, Stenotrophomonas maltophilia, Klebsiella pneumoniae, and Escherichia coli showed resistant to fluoroquinolone. Conclusion: This study shows the seriousness of antimicrobial resistance and the importance of infection control in the lCU in Korea. This study should provide a theoretical strategy to enforce the infection control.
Acinetobacter ; Cross Infection* ; Enterococcus faecalis ; Enterococcus faecium ; Escherichia coli ; Hospitals, Teaching ; Hospitals, University* ; Imipenem ; Infection Control ; Intensive Care Units* ; Critical Care* ; Klebsiella pneumoniae ; Korea* ; Methicillin ; Pneumonia, Ventilator-Associated ; Prospective Studies ; Pseudomonas aeruginosa ; Staphylococcus aureus ; Stenotrophomonas maltophilia ; Urinary Tract ; Urinary Tract Infections ; Vancomycin