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.

OBJECTIVES: This study developed an information and communication technology (ICT)-based comprehensive health and social-needs assessment (CHSNA) system based on the International Classification of Functioning, Disability, and Health (ICF) with the aim of enhancing person-centered community care for community residents and supporting healthcare professionals and social workers who provide healthcare and social services in the community. METHODS: Items related to a CHSNA tool were developed and mapped with ICF codes. Experts validated the CHSNA system design and process using the Delphi method, and a pilot test of the initial version of the system was conducted. RESULTS: The following three steps of CHSNA were embedded in the system, which had a user-friendly screen and images: basic health assessment, life and activity assessment, and in-depth health assessment. The assessment results for the community residents were presented with visualized health profiles, including images, graphs, and an ICF model. CONCLUSIONS: The developed CHSNA system can be used by healthcare professionals, social workers, and community residents to evaluate the reasoning underlying health and social needs, to facilitate the identification of more appropriate healthcare plans, and to guide community residents to receive the best healthcare services. A CHSNA system can improve the implementation of standardized terminology utilizing the ICF and the accuracy of needs assessments of community residents.
Classification ; Community Health Services ; Decision Support Techniques ; Delivery of Health Care ; Methods ; Needs Assessment ; Patient-Centered Care ; Social Work ; Social Workers

Dark circles refer to visible darkness of the infra-orbital areas. Dark circles are a cosmetic concern for many individuals, although not a medical concern. Moreover, clear definitions and possible causes of dark circles have not been elucidated. This study reviews the possible causes and treatment options for dark circles based on a review of the medical literature and the authors' clinical experience. Potential factors that contribute to dark circles include excessive pigmentation, shadowing due to tear troughs and infra-orbital fat herniation, shadowing due to infraorbital laxity and wrinkles, and thin, translucent skin overlying the orbicularis oculi muscle. Given the different possible causes for dark circles, therapeutic modalities must be individualized for each patient. Because various factors cause dark circles, it is useful to identify the underlying causes in order to select the appropriate treatment.
Darkness ; Humans ; Pigmentation ; Shadowing (Histology) ; Skin ; Tears

BACKGROUND: From May to July 2015, the Republic of Korea experienced the largest outbreak of Middle East respiratory syndrome (MERS) outside the Arabian Peninsula. A total of 186 patients, including 36 deaths, had been diagnosed with MERS-coronavirus (MERS-CoV) infection as of September 30th, 2015. MATERIALS AND METHODS: We obtained information of patients who were confirmed to have MERS-CoV infection. MERS-CoV infection was diagnosed using real-time reverse-transcriptase polymerase chain reaction assay. RESULTS: The median age of the patients was 55 years (range, 16 to 86). A total of 55.4% of the patients had one or more coexisting medical conditions. The most common symptom was fever (95.2%). At admission, leukopenia (42.6%), thrombocytopenia (46.6%), and elevation of aspartate aminotransferase (42.7%) were observed. Pneumonia was detected in 68.3% of patients at admission and developed in 80.8% during the disease course. Antiviral agents were used for 74.7% of patients. Mechanical ventilation, extracorporeal membrane oxygenation, and convalescent serum were employed for 24.5%, 7.1%, and 3.8% of patients, respectively. Older age, presence of coexisting medical conditions including diabetes or chronic lung disease, presence of dyspnea, hypotension, and leukocytosis at admission, and the use of mechanical ventilation were revealed to be independent predictors of death. CONCLUSION: The clinical features of MERS-CoV infection in the Republic of Korea were similar to those of previous outbreaks in the Middle East. However, the overall mortality rate (20.4%) was lower than that in previous reports. Enhanced surveillance and active management of patients during the outbreak may have resulted in improved outcomes.
Antiviral Agents ; Aspartate Aminotransferases ; Coronavirus Infections* ; Disease Outbreaks ; Dyspnea ; Extracorporeal Membrane Oxygenation ; Fever ; Humans ; Hypotension ; Leukocytosis ; Leukopenia ; Lung Diseases ; Middle East Respiratory Syndrome Coronavirus ; Middle East* ; Mortality ; Pneumonia ; Polymerase Chain Reaction ; Republic of Korea* ; Respiration, Artificial ; Thrombocytopenia

Acute necrotizing esophagitis (AEN), also called "black esophagus," is a rare disorder with an unknown pathogenesis. Endoscopic findings generally show black pigmentation throughout the esophagus. This case also offered rare views of the gross anatomy of this disorder. Histological examination revealed that the mucosal and submucosal layers of the esophagus were involved in the severe necrotizing inflammation. The chief manifestation of this disease is hematemesis from hemorrhage of the upper gastrointestinal tract with a typically multifactorial etiology. AEN is also characterized by a clear boundary at the gastroesophageal junction where the necrosis stops. In this study, we report an autopsy case of a 61-year-old man with necrotizing inflammation throughout the esophagus and esophageal necrosis from the laryngopharynx to the gastroesophageal junction. The patient was a disabled person with a history of alcohol abuse who was also diagnosed with mild coronary arteriosclerosis and fatty liver on the basis of the underlying diseases. In this case, the main etiology for poor perfusion from the distal esophageal area was likely underlying illness, history of alcoholism, and malnutrition.
Alcoholism ; Autopsy* ; Coronary Artery Disease ; Disabled Persons ; Esophagitis* ; Esophagogastric Junction ; Esophagus ; Fatty Liver ; Hematemesis ; Hemorrhage ; Humans ; Hypopharynx ; Inflammation ; Malnutrition ; Middle Aged ; Necrosis ; Perfusion ; Pigmentation ; Upper Gastrointestinal Tract

BACKGROUND: Spectacle contact allergy is not infrequent. The fine scratches on the spectacle frames which may play a role in the sensitization to the potential allergenic components have not been studied. OBJECTIVE: We sought the relationship between the scratches on the spectacle frames and the allergic contact dermatitis (ACD) in the Republic of Korea. METHODS: A total of 42 Korean patients with ACD at the spectacle contact sites were enrolled. Their spectacle frames were examined with the dimethylglyoxime (DMG) test and analyzed by the scanning electron microscopy (SEM) and energy-dispersive X-ray spectroscopy (EDS). Patch tests (thin-layer rapid use epicutaneous test [TRUE tests]) were performed to identify the skin allergens. RESULTS: The DMG-positive spectacle frames were identified in 78.5% of the frames. The SEM results showed that there were more scratches on the skin-contacting parts of the spectacle frames than the non-skin-contacting parts of the same frames. In the EDS findings, the mean nickel content (weight, %) of the spectacle frames was 15.7+/-5.5, and the mean chromium content was 20.3+/-3.4 at the skin-contacting parts. In the TRUE tests, nickel sulphate was the most common allergen (31 cases, 73.8%), and potassium dichromate was the second (9 cases, 21.4%). Three patients presented simultaneous positive reactions with nickel sulphate and potassium dichromate. CONCLUSION: Minor visible and non-visible fine scratches on the spectacle frames may present the provocation factors of the ACD. Nickel sulphate was the most common allergen suspected of provoking the spectacle frame-induced ACD, followed by potassium dichromate.
Chromium ; Dermatitis, Allergic Contact ; Humans ; Hypersensitivity ; Microscopy, Electron, Scanning ; Nickel ; Oximes ; Patch Tests ; Potassium ; Potassium Dichromate ; Skin ; Spectrum Analysis

BACKGROUND: The use of light technology in dermatology has grown rapidly over the last decade, with many developments in its use for the treatment of a wide variety of skin conditions from non-melanoma skin cancers to facial resurfacing for photo-damaged skin. Light-emitting diodes (LEDs) have attracted much attention in medical fields. OBJECTIVE: (1) To assess the optimal distance of 630 nm LEDs (OmniLux(R), (Phototherapeutics Ltd, the UK)) and 830 nm LEDs (Healite(R) (Lutronic, Korea)) for maximum power as determined by a power meter and (2) to apply theory to practical use. METHODS: Two separate hinged planar light emitting diode arrays were studied: 1) the Omnilux Revive(TM) (Phototherapeutics Ltd, the UK), which delivers non-coherent red light at a wavelength of 633+/-3 nm and 2) the Lutronic Healite (Korea), which delivers non-coherent light at a wavelength of 830+/-5 nm. An X93 power meter (Gigahertz-Optik, Germany) was placed against a black background in order to reduce the amount of reflected light. We measured the LED powers over a range of 3~25 cm in 1 cm increments. RESULTS: On the irradiation side of the LED, power increases according to the mass effect of the radiation angle. However, at a certain distance, the power decline effect predominated over the amassment effect. In this respect, the LED light was estimated to be emitted in a reverse V shape. The proper irradiation distance for use in medical fields can thus be determined. CONCLUSION: The proper irradiation distance of LED will be useful and the proper use of LED under the subjects' shape will be done in many medical fields.
Dermatology ; Enzyme Multiplied Immunoassay Technique ; Light ; Skin ; Skin Neoplasms

BACKGROUND: The imaging system that's currently being used in the field of dermatology is based on such instruments as the dermoscope, phototrichograph and camera. In recent years, the use of an image magnification system based on polarization has become popular. OBJECTIVE: In this study, optical quantification was performed based on the multiwavelength imaging analysis of the structures that form dermatologic diseases, and an attempt was made to enhance the image quality by using polarization technology. METHODS: The lesions of three patients who were clinically diagnosed with cherry angioma, melanocytic nevus and inflammatory lesions in acne and freckles were measured at the outpatient clinic of the Department of Dermatology of the authors' hospital. All the patients were female, and their mean age was 29.3 years. RESULTS: The optical characteristics of the patients' various skin lesions, including cherry angioma, melanocytic nevus and inflammatory lesions in acne and freckles, were distinguishable by their wavelength. CONCLUSION: The use of different kinds of information may be helpful for measuring and diagnosing various skin lesions that have not been differentiated with using the previous modalities. Further, if the various environmental factors that may be generated during the measurement process can be controlled, then these study results can be applied to a standard diagnostic modality in the field of dermatology.
Acne Vulgaris ; Ambulatory Care Facilities ; Dermatology ; Female ; Hemangioma ; Humans ; Melanosis ; Nevus, Pigmented ; Prunus ; Skin