Juvenile xanthogranuloma (JXG) is a rare histiocytic disorder primarily affecting children, typically presenting as self-limiting skin lesions. However, extracutaneous JXG (extra-JXG) or systemic JXG (sJXG) can involve various organs, including the central nervous system (CNS), leading to high morbidity and mortality, especially in cases of CNS involvement. Treatment approaches for CNS–JXG, including surgical resection, chemotherapy, and radiation therapy, lack consensus, with reports of relapsed or refractory cases being rare. Here, we present the case of a 2-year-old girl with refractory extra-JXG involving the CNS, characterized by multiple intracranial lesions.Initial chemotherapy with prednisolone and vinblastine showed no response, and second-line therapy, including cytarabine and vincristine combined with surgical resection, was also ineffective. However, a 6-cycle third-line chemotherapy regimen incorporating vincristine and carboplatin led to a positive response, with stable disease status, and normal growth and neurodevelopment observed. Our findings suggest that combined chemotherapy with carboplatin and vincristine is a feasible and well-tolerated treatment strategy for patients with CNS–JXG.

The submandibular displacement of a mandibular third molar residual root presents major challenges to oral and maxillofacial surgeons due to the proximity to critical anatomical structures such as the lingual nerve and sublingual artery. Preoperative imaging can approximate the location of the residual tooth root; however, accurately determining its exact position is difficult because of the dynamic nature of the mandible and the difficulty of realtime synchronization of imaging. This study presents the successful extraction of a residual mandibular third molar root in a 67-year-old female patient achieved using a magnetic field-based navigation system. The sublingually-displaced residual root was localized using the navigation system, marked using a virtual implant placement, and positioned by a hand piece using synchronized real-time sensor data. The root was successfully removed with a minimally-invasive approach. No complications occurred postoperatively, and follow-up showed no major issues. Due to the small size of the marker, ease of calibration, and independence from visual obstacles, magnetic field-based navigation systems are a promising tool for the removal of residual roots displaced into adjacent soft tissue.

Objective: There has been a rapid increase in the utilization of emergency departments (EDs), especially for non-emergent cases. The challenges of accessing medical services at night cause disparities in ED utilization between the day and night. Additionally, the coronavirus disease-2019 (COVID-19) pandemic has had a notable impact on ED visits. This study aimed to compare the characteristics of patients visiting the EDs in the daytime and at night before and after COVID-19. Methods: A retrospective observational study was conducted on adults (≥18 years) at three metropolitan EDs from January 1, 2019 to December 31, 2020. Data, including the Korean Triage Acuity System, Tenth Revision (ICD-10) codes, and ICU admissions, were collected. Characteristics of patients visiting the ED during the day (06:00-18:00) and night (18:00-06:00) periods before and after COVID-19 were analyzed. Results: During the pre-COVID-19 and COVID-19 periods, 117,896 and 92,915 patients visited the ED, respectively. Non-emergency nighttime visits were increased than daytime visits during both the pre-COVID-19 period (37.9% vs. 30.9%) and the COVID-19 period (35.4% vs. 28.8%) (P<0.01). During COVID-19, non-emergency patient visits to the ED during the nighttime decreased compared to those during the day (odds ratio, 0.96; 95% confidence interval [CI], 0.93-0.99, P=0.01). Among the top five diagnoses, the number of patients declined during the COVID-19 pandemic in most categories, except for the category of “Fever of other and unknown origin,” which saw an increase both during the day (incident rate ratio [IRR], 1.10; 95% CI, 1.05-1.17) and night (IRR, 1.23; 95% CI, 1.17-1.30). Conclusion The COVID-19 pandemic significantly influenced ED visit patterns, particularly by reducing non-emergent visits during nighttime.

Juvenile xanthogranuloma (JXG) is a rare histiocytic disorder primarily affecting children, typically presenting as self-limiting skin lesions. However, extracutaneous JXG (extra-JXG) or systemic JXG (sJXG) can involve various organs, including the central nervous system (CNS), leading to high morbidity and mortality, especially in cases of CNS involvement. Treatment approaches for CNS–JXG, including surgical resection, chemotherapy, and radiation therapy, lack consensus, with reports of relapsed or refractory cases being rare. Here, we present the case of a 2-year-old girl with refractory extra-JXG involving the CNS, characterized by multiple intracranial lesions.Initial chemotherapy with prednisolone and vinblastine showed no response, and second-line therapy, including cytarabine and vincristine combined with surgical resection, was also ineffective. However, a 6-cycle third-line chemotherapy regimen incorporating vincristine and carboplatin led to a positive response, with stable disease status, and normal growth and neurodevelopment observed. Our findings suggest that combined chemotherapy with carboplatin and vincristine is a feasible and well-tolerated treatment strategy for patients with CNS–JXG.

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.

The submandibular displacement of a mandibular third molar residual root presents major challenges to oral and maxillofacial surgeons due to the proximity to critical anatomical structures such as the lingual nerve and sublingual artery. Preoperative imaging can approximate the location of the residual tooth root; however, accurately determining its exact position is difficult because of the dynamic nature of the mandible and the difficulty of realtime synchronization of imaging. This study presents the successful extraction of a residual mandibular third molar root in a 67-year-old female patient achieved using a magnetic field-based navigation system. The sublingually-displaced residual root was localized using the navigation system, marked using a virtual implant placement, and positioned by a hand piece using synchronized real-time sensor data. The root was successfully removed with a minimally-invasive approach. No complications occurred postoperatively, and follow-up showed no major issues. Due to the small size of the marker, ease of calibration, and independence from visual obstacles, magnetic field-based navigation systems are a promising tool for the removal of residual roots displaced into adjacent soft tissue.

Objective: There has been a rapid increase in the utilization of emergency departments (EDs), especially for non-emergent cases. The challenges of accessing medical services at night cause disparities in ED utilization between the day and night. Additionally, the coronavirus disease-2019 (COVID-19) pandemic has had a notable impact on ED visits. This study aimed to compare the characteristics of patients visiting the EDs in the daytime and at night before and after COVID-19. Methods: A retrospective observational study was conducted on adults (≥18 years) at three metropolitan EDs from January 1, 2019 to December 31, 2020. Data, including the Korean Triage Acuity System, Tenth Revision (ICD-10) codes, and ICU admissions, were collected. Characteristics of patients visiting the ED during the day (06:00-18:00) and night (18:00-06:00) periods before and after COVID-19 were analyzed. Results: During the pre-COVID-19 and COVID-19 periods, 117,896 and 92,915 patients visited the ED, respectively. Non-emergency nighttime visits were increased than daytime visits during both the pre-COVID-19 period (37.9% vs. 30.9%) and the COVID-19 period (35.4% vs. 28.8%) (P<0.01). During COVID-19, non-emergency patient visits to the ED during the nighttime decreased compared to those during the day (odds ratio, 0.96; 95% confidence interval [CI], 0.93-0.99, P=0.01). Among the top five diagnoses, the number of patients declined during the COVID-19 pandemic in most categories, except for the category of “Fever of other and unknown origin,” which saw an increase both during the day (incident rate ratio [IRR], 1.10; 95% CI, 1.05-1.17) and night (IRR, 1.23; 95% CI, 1.17-1.30). Conclusion The COVID-19 pandemic significantly influenced ED visit patterns, particularly by reducing non-emergent visits during nighttime.

Purpose: We evaluated the feasibility, safety, and learning curve of extraperitoneal single-port robot-assisted radical prostatectomy (SP-RARP) and introduced innovative surgical techniques to maintain the instrument positions during the procedures. Materials and Methods: A cohort of 100 patients underwent extraperitoneal SP-RARP at our institution from December 2021 to April 2023. The procedures were performed by an experienced urology surgeon utilizing two surgical techniques for dissecting the posterior aspect of the prostate—“changing instrument roles” and “using camera inversion”—to prevent positional shifts between the camera and instruments. Results: The mean operation time for SP-RARP was 93.58 minutes, and the mean console time was 65.16 minutes. The mean estimated blood loss during the procedures was 109.30 mL. No cases necessitated conversion to multi-port robot, laparoscopy, or open surgery, and there were no major complications during the hospital stay or in the short-term follow-up. Early outcomes of post-radical prostatectomy indicated a biochemical recurrence rate of 4.0% over a mean follow-up duration of 6.40 months, with continence and potency recovery rates of 92.3% and 55.8%, respectively. Analysis of the learning curve showed no significant differences in operation time, console time, and positive surgical margin rates between the initial and latter 50 cases. Conclusions Extraperitoneal SP-RARP is a feasible and safe option for the treatment of localized prostate cancer in skilled hands.Continued accrual of cases is essential for future comparisons of SP-RARP with multiport approaches.

Juvenile xanthogranuloma (JXG) is a rare histiocytic disorder primarily affecting children, typically presenting as self-limiting skin lesions. However, extracutaneous JXG (extra-JXG) or systemic JXG (sJXG) can involve various organs, including the central nervous system (CNS), leading to high morbidity and mortality, especially in cases of CNS involvement. Treatment approaches for CNS–JXG, including surgical resection, chemotherapy, and radiation therapy, lack consensus, with reports of relapsed or refractory cases being rare. Here, we present the case of a 2-year-old girl with refractory extra-JXG involving the CNS, characterized by multiple intracranial lesions.Initial chemotherapy with prednisolone and vinblastine showed no response, and second-line therapy, including cytarabine and vincristine combined with surgical resection, was also ineffective. However, a 6-cycle third-line chemotherapy regimen incorporating vincristine and carboplatin led to a positive response, with stable disease status, and normal growth and neurodevelopment observed. Our findings suggest that combined chemotherapy with carboplatin and vincristine is a feasible and well-tolerated treatment strategy for patients with CNS–JXG.

Background: The clinical implications of myelin oligodendrocyte glycoprotein autoantibodies (MOG-Abs) are increasing. Establishing MOG-Ab assays is essential for effectively treating patients with MOG-Abs. We established an in-house cell-based assay (CBA) to detect MOG-Abs to identify correlations with patients’ clinical characteristics. Methods: We established the CBA using HEK 293 cells transiently overexpressing fulllength human MOG, tested it against 166 samples from a multicenter registry of central nervous system (CNS) inflammatory disorders, and compared the results with those of the Oxford MOG-Ab-based CBA and a commercial MOG-Ab CBA kit. We recruited additional patients with MOG-Abs and compared the clinical characteristics of MOG-Ab-associated disease (MOGAD) with those of neuromyelitis optica spectrum disorder (NMOSD). Results: Of 166 samples tested, 10 tested positive for MOG-Abs, with optic neuritis (ON) being the most common manifestation (4/15, 26.7%). The in-house and Oxford MOG-Ab CBAs agreed for 164/166 (98.8%) samples (κ = 0.883, P < 0.001); two patients (2/166, 1.2%) were only positive in our in-house CBA, and the CBA scores of the two laboratories correlated well (r = 0.663, P < 0.001). The commercial MOG-Ab CBA kit showed one falsenegative and three false-positive results. The clinical presentation at disease onset differed between MOGAD and NMOSD; ON was the most frequent manifestation in MOGAD, and transverse myelitis was most frequent in NMOSD. Conclusions The in-house CBA for MOG-Abs demonstrated reliable results and can potentially be used to evaluate CNS inflammatory disorders. A comprehensive, long-term study with a large patient population would clarify the clinical significance of MOG-Abs.