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.

Purpose: Tubulinopathy represents a group of disorders caused by variants in tubulin genes, which present with a wide spectrum of brain malformations. This study was conducted to provide insight into the phenotypic and genetic spectra of tubulinopathy within the Korean pediatric population. Methods: Among individuals who underwent genetic testing at a pediatric neurology clinic between June 2011 and December 2021, 15 patients with tubulin gene variants were retrospectively recruited. Clinical features, genetic information, and brain imaging findings were retrospectively reviewed. Results: The genetic spectra of the patients included TUBA1A (n=5, 33.3%), TUBB4A (n=6, 40.0%), TUBB3 (n=2, 13.3%), TUBB (n=1, 6.7%), and TUBB2A (n=1, 6.7%) variants. Two novel mutations were identified: a c.497A>G; p.(Lys166Arg) variant in TUBA1A and a c.907G>C; p.(Ala303Pro) variant in TUBB. All 15 patients exhibited developmental delays, with a broad spectrum of severity. Other common manifestations included microcephaly (n=10; 66.7%) and seizures (n=9; 60%). A review of the neuroimaging data revealed a range of findings that were both genotype-specific and overlapping across genotypes. In cases of TUBA1A mutation (n=5), four patients (80%) presented with pachygyria and polymicrogyria, while three (60%) displayed cerebellar hypoplasia and dysplasia. All patients with TUBB4A variants (n=6) exhibited hypomyelination, and three (50%) had cerebellar dysplasia. Conclusion This study represents the first cohort analysis of tubulin gene mutations associated with tubulinopathy in a Korean pediatric population. It suggests that these mutations can produce a broad spectrum of neurodevelopmental and neuroimaging findings and should be considered within the differential diagnosis in relevant clinical scenarios.

Background: The benefits of transradial access (TRA) over transfemoral access (TFA) for bifurcation percutaneous coronary intervention (PCI) are uncertain because of the limited availability of device selection. This study aimed to compare the procedural differences and the in-hospital and long-term outcomes of TRA and TFA for bifurcation PCI using secondgeneration drug-eluting stents (DESs). Methods: Based on data from the Coronary Bifurcation Stenting Registry III, a retrospective registry of 2,648 patients undergoing bifurcation PCI with second-generation DES from 21 centers in South Korea, patients were categorized into the TRA group (n = 1,507) or the TFA group (n = 1,141). After propensity score matching (PSM), procedural differences, in-hospital outcomes, and device-oriented composite outcomes (DOCOs; a composite of cardiac death, target vessel-related myocardial infarction, and target lesion revascularization) were compared between the two groups (772 matched patients each group). Results: Despite well-balanced baseline clinical and lesion characteristics after PSM, the use of the two-stent strategy (14.2% vs. 23.7%, P = 0.001) and the incidence of in-hospital adverse outcomes, primarily driven by access site complications (2.2% vs. 4.4%, P = 0.015), were significantly lower in the TRA group than in the TFA group. At the 5-year follow-up, the incidence of DOCOs was similar between the groups (6.3% vs. 7.1%, P = 0.639). Conclusion The findings suggested that TRA may be safer than TFA for bifurcation PCI using second-generation DESs. Despite differences in treatment strategy, TRA was associated with similar long-term clinical outcomes as those of TFA. Therefore, TRA might be the preferred access for bifurcation PCI using second-generation DES.

Background: Very low birth weight infants (VLBWIs) continue to face high mortality risk influenced by the care quality of neonatal intensive care units (NICUs). Under-standing the impact of workload and regional differences on these rates is crucial for improving outcomes.Purpose: This study aimed to assess how the structural and staffing attributes of NICUs influence the mortality rates of VLBWIs, emphasizing the significance of the availability of medical personnel and the regional distribution of care facilities. Methods: Data from 69 Korean NICUs collected by the Korean Neonatal Network between January 2015 and December 2016 were retrospectively analyzed. The NICUs were classified by various parameters: capacity (small, medium, large), nurse-to-bed ratio (1–4), and regional location (A, B, C). Pediatrician staffing was also analyzed and NICUs categorized by beds per pediatrician into low (≤10), medium (11–15), and high (≥16). The NICUs were classified by mortality rates into high-performance (1st and 2nd quartiles) and low-performance (3rd and 4th quartiles). Demographic, perinatal, and neonatal outcomes were analyzed using multivariate logistic regression to explore the association between NICU characteristics and mortality rates. Results: This study included 4,745 VLBWIs (mean gestational age, 28.4 weeks; mean birth weight, 1,088 g; 55.4% male) and found significant variations in survival rates across NICUs linked to performance and staffing levels. High-performing NICUs, often with lower bed-to-staff ratios and advanced care levels, had higher survival rates. Notably, NICUs with 2 rather than 1 neonatologist were associated with reduced mortality rates. The study also underscored regional disparities, with NICUs in certain areas showing less favorable survival rates. Conclusion Adequate NICU staffing and proper facility location are key to lowering the number of VLBWI deaths. Enhancing staffing and regional healthcare equity is crucial for improving the survival of this population.

Background: Very low birth weight infants (VLBWIs) continue to face high mortality risk influenced by the care quality of neonatal intensive care units (NICUs). Under-standing the impact of workload and regional differences on these rates is crucial for improving outcomes.Purpose: This study aimed to assess how the structural and staffing attributes of NICUs influence the mortality rates of VLBWIs, emphasizing the significance of the availability of medical personnel and the regional distribution of care facilities. Methods: Data from 69 Korean NICUs collected by the Korean Neonatal Network between January 2015 and December 2016 were retrospectively analyzed. The NICUs were classified by various parameters: capacity (small, medium, large), nurse-to-bed ratio (1–4), and regional location (A, B, C). Pediatrician staffing was also analyzed and NICUs categorized by beds per pediatrician into low (≤10), medium (11–15), and high (≥16). The NICUs were classified by mortality rates into high-performance (1st and 2nd quartiles) and low-performance (3rd and 4th quartiles). Demographic, perinatal, and neonatal outcomes were analyzed using multivariate logistic regression to explore the association between NICU characteristics and mortality rates. Results: This study included 4,745 VLBWIs (mean gestational age, 28.4 weeks; mean birth weight, 1,088 g; 55.4% male) and found significant variations in survival rates across NICUs linked to performance and staffing levels. High-performing NICUs, often with lower bed-to-staff ratios and advanced care levels, had higher survival rates. Notably, NICUs with 2 rather than 1 neonatologist were associated with reduced mortality rates. The study also underscored regional disparities, with NICUs in certain areas showing less favorable survival rates. Conclusion Adequate NICU staffing and proper facility location are key to lowering the number of VLBWI deaths. Enhancing staffing and regional healthcare equity is crucial for improving the survival of this population.

Background: Very low birth weight infants (VLBWIs) continue to face high mortality risk influenced by the care quality of neonatal intensive care units (NICUs). Under-standing the impact of workload and regional differences on these rates is crucial for improving outcomes.Purpose: This study aimed to assess how the structural and staffing attributes of NICUs influence the mortality rates of VLBWIs, emphasizing the significance of the availability of medical personnel and the regional distribution of care facilities. Methods: Data from 69 Korean NICUs collected by the Korean Neonatal Network between January 2015 and December 2016 were retrospectively analyzed. The NICUs were classified by various parameters: capacity (small, medium, large), nurse-to-bed ratio (1–4), and regional location (A, B, C). Pediatrician staffing was also analyzed and NICUs categorized by beds per pediatrician into low (≤10), medium (11–15), and high (≥16). The NICUs were classified by mortality rates into high-performance (1st and 2nd quartiles) and low-performance (3rd and 4th quartiles). Demographic, perinatal, and neonatal outcomes were analyzed using multivariate logistic regression to explore the association between NICU characteristics and mortality rates. Results: This study included 4,745 VLBWIs (mean gestational age, 28.4 weeks; mean birth weight, 1,088 g; 55.4% male) and found significant variations in survival rates across NICUs linked to performance and staffing levels. High-performing NICUs, often with lower bed-to-staff ratios and advanced care levels, had higher survival rates. Notably, NICUs with 2 rather than 1 neonatologist were associated with reduced mortality rates. The study also underscored regional disparities, with NICUs in certain areas showing less favorable survival rates. Conclusion Adequate NICU staffing and proper facility location are key to lowering the number of VLBWI deaths. Enhancing staffing and regional healthcare equity is crucial for improving the survival of this population.

Background: Very low birth weight infants (VLBWIs) continue to face high mortality risk influenced by the care quality of neonatal intensive care units (NICUs). Under-standing the impact of workload and regional differences on these rates is crucial for improving outcomes.Purpose: This study aimed to assess how the structural and staffing attributes of NICUs influence the mortality rates of VLBWIs, emphasizing the significance of the availability of medical personnel and the regional distribution of care facilities. Methods: Data from 69 Korean NICUs collected by the Korean Neonatal Network between January 2015 and December 2016 were retrospectively analyzed. The NICUs were classified by various parameters: capacity (small, medium, large), nurse-to-bed ratio (1–4), and regional location (A, B, C). Pediatrician staffing was also analyzed and NICUs categorized by beds per pediatrician into low (≤10), medium (11–15), and high (≥16). The NICUs were classified by mortality rates into high-performance (1st and 2nd quartiles) and low-performance (3rd and 4th quartiles). Demographic, perinatal, and neonatal outcomes were analyzed using multivariate logistic regression to explore the association between NICU characteristics and mortality rates. Results: This study included 4,745 VLBWIs (mean gestational age, 28.4 weeks; mean birth weight, 1,088 g; 55.4% male) and found significant variations in survival rates across NICUs linked to performance and staffing levels. High-performing NICUs, often with lower bed-to-staff ratios and advanced care levels, had higher survival rates. Notably, NICUs with 2 rather than 1 neonatologist were associated with reduced mortality rates. The study also underscored regional disparities, with NICUs in certain areas showing less favorable survival rates. Conclusion Adequate NICU staffing and proper facility location are key to lowering the number of VLBWI deaths. Enhancing staffing and regional healthcare equity is crucial for improving the survival of this population.

Following the previous study, which investigated the pharmacological properties of the Technekitty injection (Tc-99m), the toxicity of a single intravenous administration of the Technekittyinjection (Tc-99m) and the side effects that may occur at the diagnostic dose were confirmed.The Technekitty injection (Tc-99m) was administered intravenously once at a dose of 0, 0.67, 2.0, and 6.0 mCi/kg to 5 male and female rats per group. Mortality, general symptom obser-vation, and weight measurement were performed for 2 weeks, followed by observation of autopsy findings. There were no deaths, and no statistically significant weight change was observed. No abnormal systemic signs related to the Technekitty injection (Tc-99m) were observed. These results confirmed that Technekitty injection (Tc-99m) can be safely admin-istered intravenously at doses up to 6.0 mCi/kg. Additionally, technetium-99m at an average dose of 2 mCi (74 MBq) has been verified as a diagnostic dose without adverse effects, al-lowing the Technekitty injection (Tc-99m) to be used safely without side effects at this dosage.This study demonstrates that the Technekitty injection (Tc-99m) has a wide safety margin, supporting its potential for clinical application. Moreover, these findings align with the nonclin-ical safety standards for radiopharmaceuticals, reinforcing its utility in veterinary medicine.The Technekitty injection (Tc-99m) is expected to be applicable for clinical diagnosis as a vet-erinary drug in Korea.