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: To assess the relevance of electrodiagnosis (EDX) in the cross-sectional area (CSA) of the nerve root of patients with cervical radiculopathy (CR) by using high-resolution ultrasonography (HRUS). Methods: The CSAs of the cervical nerve roots at C5, C6, and C7 were measured bilaterally using HRUS in 29 patients with unilateral CR whose clinical symptoms, magnetic resonance imaging (MRI) findings, and EDX results: corresponded with each other (CR-A group), and in 26 patients with unilateral CR whose clinical symptoms and MRI findings matched with each other but did not correspond with the EDX findings (CR-B group). Results The CSA of the affected side in each nerve root was significantly larger than that of the unaffected side in both the CR-A and CR-B groups. The side-to-side difference in the bilateral CSAs of the nerve root and the ratio of the CSAs between the unaffected and affected sides were statistically larger in the CR-A group than in the CR-B group. Conclusion The increased CSAs in the CR-A group reflect the physiological changes of the cervical nerve root, which is supported by the EDX findings.

Objective: To assess the relevance of electrodiagnosis (EDX) in the cross-sectional area (CSA) of the nerve root of patients with cervical radiculopathy (CR) by using high-resolution ultrasonography (HRUS). Methods: The CSAs of the cervical nerve roots at C5, C6, and C7 were measured bilaterally using HRUS in 29 patients with unilateral CR whose clinical symptoms, magnetic resonance imaging (MRI) findings, and EDX results: corresponded with each other (CR-A group), and in 26 patients with unilateral CR whose clinical symptoms and MRI findings matched with each other but did not correspond with the EDX findings (CR-B group). Results The CSA of the affected side in each nerve root was significantly larger than that of the unaffected side in both the CR-A and CR-B groups. The side-to-side difference in the bilateral CSAs of the nerve root and the ratio of the CSAs between the unaffected and affected sides were statistically larger in the CR-A group than in the CR-B group. Conclusion The increased CSAs in the CR-A group reflect the physiological changes of the cervical nerve root, which is supported by the EDX findings.

Background: Estrogen controls the pubertal growth spurt, growth plate closure, and accretion of bone mineral density (BMD) of long bones after biding estrogen receptor (ER).There are two subtypes of ER, ERα and ERβ. If each ER subtype has different effects, we may control those actions by manipulating the estrogen binding intensity to each ER subtype and increase the final adult height without markedly reducing BMD or impairing reproductive functions. The purpose of our study was to compare these effects of ERα and ERβ on long bones in ovariectomized rats. Methods: Thirty female rats were ovariectomized and randomly divided into 3 groups. The control, propylpyrazole triol (PPT), and 2,3-bis (4-hydroxyphenyl) propionitrile (DPN) groups were subcutaneously injected for 5 weeks with sesame oil, PPT as an ERα agonist, and DPN as an ERβ agonist, respectively. The crown-lump length and body weight were measured weekly.BMD, serum levels of growth hormone (GH) and estradiol were checked before and after 5 weeks of injections. Pituitary GH1 expression levels were determined with quantitative realtime polymerase chain reaction, the proximal tibias were dissected, decalcified and stained with hematoxylin-eosin, and the thicknesses of epiphyseal plates including proliferative and hypertrophic zones were measured in 20-evenly divided sites after 5 weeks of injections. Comparisons for auxological data, serum hormone and pituitary GH1 expression levels, BMD, and epiphyseal plate thicknesses among 3 groups before and after injections were conducted. Results: There was no significant difference in body lengths among 3 groups. The body weights were significantly lower, but, serum GH, pituitary GH1 expression levels, and BMDs were higher in PPT group than the other 2 groups after 5 weeks of injections. There was no significant difference in the thicknesses of the total epiphyseal plate, proliferative, and hypertrophic zone among 3 groups. Conclusion ERα is more involved in pituitary GH secretion and bone mineral deposition than ERβ. Weight gain might be prevented with the ERα agonist.

BACKGROUND: The aim of this study was to identify the painkillers preferred for self-administration by doctors working at general hospitals in the capital of the Republic of Korea.METHODS: We collected data, using a questionnaire, from 224 doctors working at secondary or tertiary hospitals in the capital of the Republic of Korea from July 1, 2017 to August 31, 2017. The questionnaire included questions on the preferred type of painkiller for each type of pain and the frequency of painkiller intake. Further, we evaluated the participants on the Likert scale to analyze the consideration and cognition of self-administration of painkillers.RESULTS: The doctors in this study tended to state the trade name of the painkillers rather than the generic name. They preferred acetaminophen for headache and nonsteroidal anti-inflammatory drugs for gastrointestinal (GI) pain, dysmenorrhea, toothache, and musculoskeletal pain. In the choice of painkiller for self-administration, they set utmost importance on the effectiveness of the medicine, followed by the potential side effects, physician's prescription, and the pharmacy's recommendation, in that order. The side effects attribute GI complications, hepatotoxicity, drug tolerance, and delayed diagnosis to painkiller use. There were some remarkable differences between surgeons and non-surgeons, men and women, and specialists and trainees in the conception of painkillers and pain control.CONCLUSION: This is the first study worldwide on the trait of the self-administration of painkillers by doctors, which can serve as a useful reference in clinical settings.
Acetaminophen ; Analgesics ; Cognition ; Delayed Diagnosis ; Drug Tolerance ; Dysmenorrhea ; Female ; Fertilization ; Headache ; Hospitals, General ; Humans ; Male ; Musculoskeletal Pain ; Prescriptions ; Republic of Korea ; Self Administration ; Self Medication ; Specialization ; Surgeons ; Tertiary Care Centers ; Toothache

In recent years, the taeniasis has been rarely reported in the Republic of Korea (Korea). But in this study, we intend to report 4 taeniasis cases caused by Taenia saginata during a 5-month period (February to June 2018) at a unversity hospital in Gwangju, Korea. Worm samples (proglottids) discharged from all cases were identified by phenotypic and molecular diagnostics. Mitochondrial cytochrome c oxidase subunit I sequences showed 99.4–99.9% identity with T. saginata but, differed by 4% from T. asiatica and by 7% from T. multiceps, respectively. We found that tapeworms in 2 cases (Cases 2 and 3) yielded exactly the same sequences between them, which differed from those in Cases 1 and 4, suggesting intra-species variation in tapeworms. These taeniasis cases by T. saginata infection in this study, which occurred within a limited time period and region, suggest the possibility of a mini-outbreak. This study highlights the need for further epidemiological investigation of potentially overlooked cases of T. saginata infection in Korea.
Cestoda ; Electron Transport Complex IV ; Gwangju ; Korea ; Pathology, Molecular ; Republic of Korea ; Taenia saginata ; Taeniasis

OBJECTIVE: To evaluate the relationship between the palmar cutaneous branch of median nerve (PCBMN) and surrounding anatomical structures by using high-resolution ultrasound (HRUS) to assess the risk of PCBMN injury. METHODS: The PCBMN course and the characteristics of bilateral distal forearms and wrists of 30 healthy volunteers were identified. The distance between PCBMN and other anatomical structures at three different levels along its course were measured using HRUS. Moreover, the depth of PCBMN from skin and its cross-sectional area (CSA) were measured. RESULTS: HRUS showed the PCBMN in all subjects. PCBMN branched off from the radial aspect of the median nerve (MN) at 4.69±0.89 cm proximal to the bistyloid line (BSL) and extended radially toward the flexor carpi radialis (FCR) tendon. PCBMN was within the ulnar edge of FCR tendon sheath, and became more superficial and perforated the antebrachial fascia between the FCR tendon laterally and the palmaris longus (PL) tendon medially. PCBMN was located at 4.08±0.72 mm on the ulnar aspect of the FCR tendon and 4.78±0.36 mm radially on the PL tendon at BSL. At the distal wrist crease level, the PCBMN was located at 5.68±0.58 mm on the ulnar side of the FCR tendon. The PCBMN depth from skin at BSL and its branching point was 1.92±0.41 and 7.95±0.79 mm, respectively. The PCBMN CSA was 0.26±0.15 mm² at BSL. CONCLUSION: HRUS can be used to identify PCBMN and its relationship with other anatomical structures. Our data can be used to predict PCBMN location, and prevent complications associated with invasive procedures involving the wrist.
Carpal Tunnel Syndrome ; Fascia ; Forearm ; Healthy Volunteers ; Median Nerve ; Risk Assessment ; Skin ; Tendons ; Ultrasonography ; Wrist

γδ T cells are abundant in the gut mucosa and play an important role in adaptive immunity as well as innate immunity. Although γδ T cells are supposed to be associated with the enhancement of Ab production, the status of γδ T cells, particularly in the synthesis of IgA isotype, remains unclear. We compared Ig expression in T cell receptor delta chain deficient (TCRδ⁻/⁻) mice with wild-type mice. The amount of IgA in fecal pellets was substantially elevated in TCRδ⁻/⁻ mice. This was paralleled by an increase in surface IgA expression and total IgA production by Peyer's patches (PPs) and mesenteric lymph node (MLN) cells. Likewise, the TCRδ⁻/⁻ mice produced much higher levels of serum IgA isotype. Here, surface IgA expression and number of IgA secreting cells were also elevated in the culture of spleen and bone marrow (BM) B cells. Germ-line α transcript, an indicator of IgA class switch recombination, higher in PP and MLN B cells from TCRδ⁻/⁻ mice, while it was not seen in inactivated B cells. Nevertheless, the frequency of IgA+ B cells was much higher in the spleen from TCRδ⁻/⁻ mice. These results suggest that γδ T cells control the early phase of B cells, in order to prevent unnecessary IgA isotype switching. Furthermore, this regulatory role of γδ T cells had lasting effects on the long-lived IgA-producing plasma cells in the BM.
Adaptive Immunity ; Animals ; B-Lymphocytes* ; Bone Marrow ; Immunity, Innate ; Immunoglobulin A* ; Immunoglobulin Class Switching* ; Lymph Nodes ; Mice ; Mucous Membrane ; Peyer's Patches ; Plasma Cells ; Receptors, Antigen, T-Cell, gamma-delta ; Recombination, Genetic ; Spleen ; T-Lymphocytes*