Background: and Purpose To determine the clinical phenotypes, relapse timing, treatment responses, and outcomes of children with relapsing myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). Methods: We collected the demographic, clinical, laboratory, and radiological data of patients aged <18 years who had been diagnosed with MOGAD at Seoul National University Children’s Hospital between January 2010 and January 2022; 100 were identified as positive for MOG antibodies, 43 of whom experienced relapse. Results: The median age at onset was 7 years (range 2–16 years). The median number of relapses was 2 (range 1–8), and patients were followed up for a median of 65 months (range 5–214 months). The first relapse was experienced before 3 months from onset by 15 patients (34.9%). The most-common initial phenotypes were acute disseminated encephalomyelitis (n=17, 39.5%) and optic neuritis (ON; n=11, 25.6%). The most-common relapse phenotypes were neuromyelitis optica spectrum disorder (n=9, 20.9%), relapsing ON (n=6, 14.0%), and multiphasic disseminated encephalomyelitis (n=6, 14.0%). Many of the patients (n=18, 41.9%) were not specifically categorized. A high proportion of these patients had non-acute disseminated encephalomyelitis encephalitis. Atypical phenotypes such as prolonged fever or hemiplegic migraine-like episodes were also noted. Mycophenolate mofetil and cyclic immunoglobulin treatment significantly reduced the annual relapse rates. Conclusions Our 43 pediatric patients with relapsing MOGAD showed a tendency toward early relapse and various relapse phenotypes. The overall prognoses of these patients were good regardless of phenotype or response to second-line immunosuppressant treatment.

Background: and Purpose To determine the clinical phenotypes, relapse timing, treatment responses, and outcomes of children with relapsing myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). Methods: We collected the demographic, clinical, laboratory, and radiological data of patients aged <18 years who had been diagnosed with MOGAD at Seoul National University Children’s Hospital between January 2010 and January 2022; 100 were identified as positive for MOG antibodies, 43 of whom experienced relapse. Results: The median age at onset was 7 years (range 2–16 years). The median number of relapses was 2 (range 1–8), and patients were followed up for a median of 65 months (range 5–214 months). The first relapse was experienced before 3 months from onset by 15 patients (34.9%). The most-common initial phenotypes were acute disseminated encephalomyelitis (n=17, 39.5%) and optic neuritis (ON; n=11, 25.6%). The most-common relapse phenotypes were neuromyelitis optica spectrum disorder (n=9, 20.9%), relapsing ON (n=6, 14.0%), and multiphasic disseminated encephalomyelitis (n=6, 14.0%). Many of the patients (n=18, 41.9%) were not specifically categorized. A high proportion of these patients had non-acute disseminated encephalomyelitis encephalitis. Atypical phenotypes such as prolonged fever or hemiplegic migraine-like episodes were also noted. Mycophenolate mofetil and cyclic immunoglobulin treatment significantly reduced the annual relapse rates. Conclusions Our 43 pediatric patients with relapsing MOGAD showed a tendency toward early relapse and various relapse phenotypes. The overall prognoses of these patients were good regardless of phenotype or response to second-line immunosuppressant treatment.

Background: and Purpose To determine the clinical phenotypes, relapse timing, treatment responses, and outcomes of children with relapsing myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). Methods: We collected the demographic, clinical, laboratory, and radiological data of patients aged <18 years who had been diagnosed with MOGAD at Seoul National University Children’s Hospital between January 2010 and January 2022; 100 were identified as positive for MOG antibodies, 43 of whom experienced relapse. Results: The median age at onset was 7 years (range 2–16 years). The median number of relapses was 2 (range 1–8), and patients were followed up for a median of 65 months (range 5–214 months). The first relapse was experienced before 3 months from onset by 15 patients (34.9%). The most-common initial phenotypes were acute disseminated encephalomyelitis (n=17, 39.5%) and optic neuritis (ON; n=11, 25.6%). The most-common relapse phenotypes were neuromyelitis optica spectrum disorder (n=9, 20.9%), relapsing ON (n=6, 14.0%), and multiphasic disseminated encephalomyelitis (n=6, 14.0%). Many of the patients (n=18, 41.9%) were not specifically categorized. A high proportion of these patients had non-acute disseminated encephalomyelitis encephalitis. Atypical phenotypes such as prolonged fever or hemiplegic migraine-like episodes were also noted. Mycophenolate mofetil and cyclic immunoglobulin treatment significantly reduced the annual relapse rates. Conclusions Our 43 pediatric patients with relapsing MOGAD showed a tendency toward early relapse and various relapse phenotypes. The overall prognoses of these patients were good regardless of phenotype or response to second-line immunosuppressant treatment.

Background and Objectives: Obstruction of tracheostomy cannula (T-cannula) may result in devastating results, such as hypoxic brain injury and even death. Since the recent coronavirus disease 2019 (COVID-19) outbreak, nebulizing for humidification to prevent tracheostomy cannula obstruction has been controversial due to concerns about viral spreading through aerosol. The present study evaluated the risk of cannula obstruction and thereby suggest an adequate prevention method during the COVID-19 pandemic.Materials and Method From January 2020 to October 2020, we retrospectively analyzed medical records of patients who underwent tracheostomy at the Department of Otolaryngology at Asan Medical Center, Seoul, Korea. The frequency of tracheostomy tube obstruction was compared in patients who were or were not nebulized. Additional clinical variates included patient’s sex, age, smoking history, medical history, and current medical history were evaluated. Results: Enrolled 226 patients were divided into obstruction (n=62) and non-obstruction group (n=164). T-cannula obstruction was related to period of tracheostomy, smoking history, pulmonary diseases, and nebulized use. In Cox proportional hazards analysis, ex-smoking (hazard ratio [HR]=1.962, p=0.033), current smoking (HR=2.108, p=0.027), and pulmonary diseases (HR=1.740, p=0.038) were related to T-cannula obstruction. When other factors were corrected, the risk of tracheostomy obstruction was significantly decreased in the nebulized group (HR=0.216, p<0.001). Mortality rate of this group was affected by only pulmonary diseases. Conclusion Nebulizer can be applied safely and helps to avoid the risk of T-cannula obstruction.

Purpose: TFE3-rearranged/TFEB-altered renal cell carcinoma (RCC) is a rare subtype of RCC. Due to its rarity, there is an unmet medical need for effective therapies in advanced settings. The study aims to investigate the clinical and histopathological characteristics of patients with microphthalmia transcription factor family/ transcription factor E (MiTF/TFE) translocation RCC and the clinical outcomes of systemic therapies, including tyrosine kinase inhibitors (TKIs) or immune checkpoint inhibitors (ICIs). Materials and Methods: This was a single-center, retrospective study. We identified 32 eligible patients among a total of 37 patients diagnosed with MiTF/TFE translocation RCC between January 2004 and September 2021, and the study included 9 patients who were treated with systemic therapies. We collected data on clinical characteristics, targeted sequencing, and clinical outcomes. Results: The median age of the 32 patients was 45.5 years. Histologically, 26 patients (81.3%) had TFE3-rearranged RCC, and only 1 patient (3.1%) had TFEB-altered RCC. Curative or cytoreductive nephrectomy was performed in all 27 patients (84.4%), and 4 patients (12.6%) were diagnosed with metastatic disease at the time of the initial diagnosis. Nine patients (28.1%) were treated with systemic therapy with TKIs, 2 (6.3%) of whom received simultaneous TKI and ICI treatment. The response to systemic therapy (TKI or ICI) and duration of response ranged from complete response to progressive disease. Excluding 1 patient who was treated with a TKI in the adjuvant setting, the overall response rate in 8 metastatic patients was 50% and the complete response rate was 37.5%. The median follow-up period was 29 months. The median progressionfree survival was 21 months, median overall survival was not achieved, and 2 deaths occurred. Conclusions Our findings suggest that TKI for treatment for metastatic TFE3-rearranged RCC is efficacious, with an overall response rate of 50% and a median progression-free survival of 21 months.

By utilizing 3D image data from oral and facial scans, virtual patients can be created, allowing clinicians to accurately assess the occlusal plane and the aesthetic position and form of anterior teeth in relation to facial scan data. This integration enhances the predictability of aesthetic prosthetic treatments, reduces the potential for occlusal interferences and adjustments, and facilitates effective communication with patients during the diagnostic process. In this case report, a patient with skeletal Class III malocclusion had both oral and facial scans to generate a virtual patient during the diagnostic phase. Based on this virtual model, prostheses were designed and fabricated, resulting in an efficient and clinically satisfactory outcome both aesthetically and functionally.

By utilizing 3D image data from oral and facial scans, virtual patients can be created, allowing clinicians to accurately assess the occlusal plane and the aesthetic position and form of anterior teeth in relation to facial scan data. This integration enhances the predictability of aesthetic prosthetic treatments, reduces the potential for occlusal interferences and adjustments, and facilitates effective communication with patients during the diagnostic process. In this case report, a patient with skeletal Class III malocclusion had both oral and facial scans to generate a virtual patient during the diagnostic phase. Based on this virtual model, prostheses were designed and fabricated, resulting in an efficient and clinically satisfactory outcome both aesthetically and functionally.

By utilizing 3D image data from oral and facial scans, virtual patients can be created, allowing clinicians to accurately assess the occlusal plane and the aesthetic position and form of anterior teeth in relation to facial scan data. This integration enhances the predictability of aesthetic prosthetic treatments, reduces the potential for occlusal interferences and adjustments, and facilitates effective communication with patients during the diagnostic process. In this case report, a patient with skeletal Class III malocclusion had both oral and facial scans to generate a virtual patient during the diagnostic phase. Based on this virtual model, prostheses were designed and fabricated, resulting in an efficient and clinically satisfactory outcome both aesthetically and functionally.

By utilizing 3D image data from oral and facial scans, virtual patients can be created, allowing clinicians to accurately assess the occlusal plane and the aesthetic position and form of anterior teeth in relation to facial scan data. This integration enhances the predictability of aesthetic prosthetic treatments, reduces the potential for occlusal interferences and adjustments, and facilitates effective communication with patients during the diagnostic process. In this case report, a patient with skeletal Class III malocclusion had both oral and facial scans to generate a virtual patient during the diagnostic phase. Based on this virtual model, prostheses were designed and fabricated, resulting in an efficient and clinically satisfactory outcome both aesthetically and functionally.

Postmortem computed tomography (PMCT) is used in forensic medicine worldwide due to its ability to non-invasively visualize injuries, hemorrhage, and estimate volume. In the autopsy of infants, assessing nutritional conditions such as weight is crucial for identifying neglect. This study aims to evaluate the usefulness of retrospectively estimating the weight of Korean infants using PMCT-based volume and multiplication factors, even when the body has been cremated. A total of 44 cases of infant death (under 12 months) were analyzed. PMCT images were obtained before autopsy. Autopsy records and documentation provided by the police at the time of autopsy were reviewed to determine the weight (g) of the infant. PMCT-based infant volumes (mL) were estimated using a three-dimensional semi-automatic segmentation method. Multiplication factors (g/mL) were calculated by dividing the weight recorded at autopsy by the PMCT-based volume, yielding a mean of 1.047 g/mL, ranging from 1.014 g/mL to 1.085 g/mL. The mean absolute error compared to weights recorded at autopsy was 95 g. Significant discrepancies were observed between weights recorded at the scene or medical center and those measured at autopsy. This study demonstrates that PMCT-based weight estimation for Korean infants is a reliable method and has the potential for retrospectively validating incorrect weight measurements and addressing inconsistencies in recorded weight data.