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: 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.

The infection status of Centrocestus armatus metacercariae (CaMc) was broadly surveyed in freshwater fishes from major river systems in the Republic of Korea (Korea) during 2008–2017. A total of 14,977 fishes was caught and examined by the artificial digestion method. CaMc were detected in 3,818 (97.1%) (2,114 Z. platypus: 96.1% and 1,704 Z. temminckii: 98.4%) out of 3,932 Zacco spp. examined and their density was 1,867 (2,109 in Z. platypus and 1,567 in Z. temminckii) per fish infected. The prevalences with CaMc were high, 93.7–100%, in Zacco spp. from all surveyed areas. However, their densities were more or less different by the surveyed areas and fish species. They were most high in Nakdong-gang in Gyeongsangnam-do (4,201 in average), and followed by Geum-gang (2,343), Nakdong-gang in Gyeongsangbuk-do (1,623), Han-gang (1,564), Tamjin-gang and Yeongsan-gang (1,540), streams in the east coast (1,028), Seomjin-gang (488) and Mangyeong-gang (170). In another species of rasborinid fish, Opsariichthys uncirostris amurensis, CaMc were detected in 222 (74.8%) out of 297 ones examined and their density was 278 (1–4,480) per fish infected. CaMc were also detected in total 41 fish species except for the rasborinid fish, Z. platypus, Z. temminckii and O. uncirostris amurensis. Conclusively, it was confirmed that among the 3 species of rasborinid fish, Z. platypus and Z. temminckii are highly prevalent and O. uncirostris amurensis is moderately prevalent with CaMc. Additionally, we could know that variety of fish species act as the second intermediate hosts of C. armatus in Korea.
Digestion ; Fishes ; Fresh Water ; Gyeongsangbuk-do ; Gyeongsangnam-do ; Korea ; Metacercariae ; Methods ; Platypus ; Prevalence ; Republic of Korea ; Rivers ; Water

There are many different approaches to ultrasound-guided supraclavicular brachial plexus block (US-SCBPB), and each has a different success rate and complications. The most commonly performed US-SCBPB is the corner pocket approach in which the needle is advanced very close to the subclavian artery and pleura. Therefore, it may be associated with a risk of subclavian artery puncture or pneumothorax. We advanced the needle into the central part of the neural cluster after penetrating the sheath of the brachial plexus in US-SCBPB. We refer to this new method as the "central cluster approach." In this approach, the needle does not have to advance close to the subclavian artery or pleura. The aim of this study was to evaluate the clinical outcomes of the central cluster approach in US-SCBPB.
Brachial Plexus* ; Needles ; Pleura ; Pneumothorax ; Punctures ; Subclavian Artery ; Ultrasonography

BACKGROUND/AIMS: The reported prevalence of antibiotic resistance in Helicobacter pylori infection has been increasing. However, recent trends in the eradication rates of H. pylori using first-line triple regimens are rarely reported. Therefore, we determined the trend in the H. pylori eradication rates in a single center for the most recent 9 years in Daegu and Kyungpook provinces, Korea. METHODS: From January 1999 through December 2007, the eradication rates in 615 H. pylori-positive patients who received one-week triple regimens were evaluated retrospectively according to year and ulcer location. RESULTS: The overall H. pylori eradication rate was 81.6%. The eradication rate from the years 1999 to 2007 was 76.3, 78.3, 82.8, 88.5, 77.8, 91.7, 84.8, 75.4, and 83.7%, respectively, based on the per-protocol analysis. No definite evidence of a decreasing tendency of the eradication rate was seen over the 9 years (p=0.760). Furthermore, there was no significant difference in the eradication rate according to ulcer location. CONCLUSIONS: There is no decreasing trend in the H. pylori eradication rate over the past 9 years in Daegu and Kyungpook provinces. However, the eradication rates are not satisfactory, and further investigation is needed to develop more effective regimens.
Drug Resistance, Microbial ; Helicobacter ; Helicobacter pylori ; Humans ; Korea ; Prevalence ; Retrospective Studies ; Ulcer

PURPOSE: The diagnostic relevancies and characteristics and of clinical methods in the diagnosis of internal derangement (ID) were tested by comparing the results of them with those of magnetic resonance imaging (MRI). METHODS: 75 patients (150 temporomandibular joints; TMJs), who were suspected to have ID by clinical diagnoses, were included. Clinical diagnoses including mouth opening pathway and TMJ sound were conducted and MRI takings were done. Accuracies, sensitivities, specificities, positive predictive values, and negative predictive values of clinical diagnosis, mouth opening pathway, and TMJ sound were calculated by comparing with diagnoses with MRIs. RESULTS: Accuracy, sensitivity, specificity, positive predictive value, and negative predictive value of clinical diagnosis were 59.3%, 83%, 49%, 81%, and 51%. They were 59%, 82%, 25%, 73%, and 35% for mouth opening pathways. Although deviation was somewhat accurate for representing disc displacement with reduction (ADDWR), other discrepancies on opening pathways were not clinically relevant. Accuracy, sensitivity, specificity, positive predictive value, and negative predictive value of clicking sounds were 85%, 49%, 78%, 85%, and 37%. TMJs with crepitus were only three. But all TMJs with crepitus were diagnosed to have disc displacement without reduction (ADDWOR). CONCLUSION: When compared with diagnoses with MRIs, clinical diagnoses for ID were not so accurate. But they were suitable for screening tests for ID. Opening pathways and TMJ sounds were not so relevant in the diagnoses of IDs and so it was concluded that considerations for other factors must be included in the diagnoses of IDs.
Diagnosis* ; Humans ; Magnetic Resonance Imaging* ; Mass Screening ; Mouth ; Sensitivity and Specificity ; Temporomandibular Joint Disorders* ; Temporomandibular Joint*

BACKGROUND: This study compared the bronchodilator efficacy and safety of tiotropium inhalation capsules (18microgram once daily) with a ipratropium metered dose inhaler (2 puffs of 20microgram q.i.d.) in patients with chronic obstructive pulmonary disease (COPD). METHOD: After the initial screening assessment and a two-week run-in period, patients received either tiotropium 18microgram once daily or ipratropium 40microgram four times daily over a period of 4 weeks in a double blind, double dummy, parallel group study. The outcome measures were the lung function, the daily records of the peak expiratory flow rate (PEFR), the patients' questionnaire, and the use of concomitant salbutamol. The forced expiratory volume in one second (FEV1) and the forced vital capacity (FVC) were measured 5 minutes before inhalation, and 0.5, 1, 2 and 3 hours after inhaling the study drug on days 0, 14 and 28. RESULT: In 16 centers, 134 patients with a mean (SD) age of 66 (7) years and a predicted FEV1 of 42 (12)% were analyzed. The trough FEV1 response was significantly higher in the tiotropium group than in the ipratropium group after a four-week treatment period. The weekly mean morning PEFR of the tiotropium group was consistently higher than that of the ipratropium group during the 4-week treatment period with differences ranging from 12.52 to 13.88 l/min, which were statistically significant. Tiotropium was well tolerated by the COPD patients during the 4-week treatment period and had a similar safety profile to ipratropium. CONCLUSION: This study shows that tiotropium administrated once daily has a superior bronchodilator effect with a similar safety profile in treating COPD patients compared with ipratropium, inhaled four times daily.
Adult* ; Albuterol ; Bronchodilator Agents ; Capsules ; Forced Expiratory Volume ; Humans ; Inhalation ; Ipratropium* ; Lung ; Mass Screening ; Metered Dose Inhalers ; Outcome Assessment (Health Care) ; Peak Expiratory Flow Rate ; Pulmonary Disease, Chronic Obstructive* ; Surveys and Questionnaires ; Vital Capacity ; Tiotropium Bromide

BACKGROUND AND OBJECTIVES: Bcl-2 protein is related to the inhibition of apoptosis via the mitochondrial pathway and Bcl-2's anti-oxidant effect. During the development of atherosclerosis, apoptosis is known to play an important role in the pathophysiologic behavior of atherosclerotic vascular disease in the medium-sized arteries. Apoptosis may be a compensatory reaction to regulate the cellular density of various tissues during the cellular proliferation process such as happens with tissue injury and during the development of atherosclerosis. The consequences of apoptosis in atherosclerosis may be related to the formation of an acellular lipid core, plaque instability and the loss of vascular wall integrity and remodeling. We sought to determine the effect of Bcl-2 gene expression on the development of primary atherosclerosis in apolipoprotein E deficient mouse, which is one of the typical animal models that are used for the development of peripheral atherosclerosis. MATERIALS AND METHODS: Bcl-2 transgenic mice were cross hybridized with apolipoprotein E deficient mice. Systemic analysis of the distribution and severity of their atherosclerotic lesions was done by dissecting microscopy, and the histological characteristics of the lesions were evaluated in normal chow-fed, 9-month-old apolipoprotein-E deficient/Bcl-2 transgenic mice (n=6) and apolipoprotein-E deficient mice (n=6). RESULTS: The distribution and severity of atherosclerotic lesions at the peripheral arteries were less in the apolipoprotein-E deficient/Bcl-2 transgenic mice. Acellular lipid core formation, destruction of the smooth muscle cell layers in the media and infiltration of inflammatory cells in the adventitia were much less in the apolipoprotein-E deficient/Bcl-2 transgenic mice. The lipid profile was similar in both groups. CONCLUSION: The effect of Bcl-2 gene expression on the peripheral atherosclerosis was related with the inhibition or the delay of atherosclerotic lesion progression, such as the reduction of amount of the acellular lipid core, maintenance of vascular smooth muscle cell integrity and the reduction of adventitial inflammation, and this was achieved regardless of serum cholesterol level.
Adventitia ; Animals ; Antioxidants ; Apolipoproteins* ; Apoptosis ; Arteries ; Atherosclerosis ; Cell Proliferation ; Cholesterol ; Genes, bcl-2* ; Humans* ; Infant ; Inflammation ; Mice* ; Mice, Transgenic ; Microscopy ; Models, Animal ; Muscle, Smooth, Vascular ; Myocytes, Smooth Muscle ; Vascular Diseases