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: Outcome analysis of urachal cancer (UraC) is limited due to the scarcity of cases and different staging methods compared to urothelial bladder cancer (UroBC). We attempted to assess survival outcomes of UraC and compare to UroBC after stage-matched analyses. Materials and Methods: Total 203 UraC patients from a multicenter database and 373 UroBC patients in single institution from 2000 to 2018 were enrolled (median follow-up, 32 months). Sheldon stage conversion to corresponding TNM staging for UraC was conducted for head-to-head comparison to UroBC. Perioperative clinical variables and pathological results were recorded. Stage-matched analyses for survival by stage were conducted. Results: UraC patients were younger (mean age, 54 vs. 67 years; p < 0.001), with 163 patients (80.3%) receiving partial cystectomy and 23 patients (11.3%) radical cystectomy. UraC was more likely to harbor ≥ pT3a tumors (78.8% vs. 41.8%). While 5-year recurrence-free survival, cancer-specific survival (CSS) and overall survival were comparable between two groups (63.4%, 67%, and 62.1% in UraC and 61.5%, 75.9%, and 67.8% in UroBC, respectively), generally favorable prognosis for UraC in lower stages (pT1-2) but unfavorable outcomes in higher stages (pT4) compared to UroBC was observed, although only 5-year CSS in ≥ pT4 showed statistical significance (p=0.028). Body mass index (hazard ratio [HR], 0.929), diabetes mellitus (HR, 1.921), pathologic T category (HR, 3.846), and lymphovascular invasion (HR, 1.993) were predictors of CSS for all patients. Conclusion Despite differing histology, UraC has comparable prognosis to UroBC with relatively favorable outcome in low stages but worse prognosis in higher stages. The presented system may be useful for future grading and risk stratification of UraC.

Background: The purpose of this study was to evaluate the effect of vanishing twin (VT) on maternal serum marker concentrations and nuchal translucency (NT). Methods: This is a secondary analysis of a multicenter prospective cohort study in 12 institutions. Serum concentrations of pregnancy-associated plasma protein-A in the first trimester and alpha-fetoprotein (AFP), total human chorionic gonadotrophin, unconjugated estriol, and inhibin A in the second trimester were measured, and NT was measured between 10 and 14 weeks of gestation. Results: Among 6,793 pregnant women, 5,381 women were measured for serum markers in the first or second trimester, including 65 cases in the VT group and 5,316 cases in the normal singleton group. The cases in the VT group had a higher median multiple of the median value of AFP and inhibin A than the normal singleton group. The values of other serum markers and NT were not different between the two groups. After the permutation test with adjustment,AFP and inhibin A remained significant differences. The frequency of abnormally increased AFP was also higher in the VT group than in the normal singleton group. Conclusion VT can be considered as an adjustment factor for risk assessment in the secondtrimester serum screening test.

Purpose: The fusion of early (E) and late diastolic filling (A) on mitral inflow Doppler, even in the absence of tachycardia, is often found during assessment of left ventricular (LV) diastolic function. We evaluated the echocardiographic characteristics and clinical implications of premature E-A fusion of uncertain cause in the absence of tachycardia. Materials and Methods: We identified 1014 subjects who showed E-A fusion and normal LV ejection fraction (LVEF) between January 2019 and June 2021 at two tertiary hospitals. Among these, 105 (10.4%) subjects showed premature E-A fusion at heart rates less than 100 beats per minute (bpm). The conventional echocardiographic parameters and LV global longitudinal strain (GLS) were compared with 1:1 age-, sex-, and heart rate-matched controls without E-A fusion. Results: The premature E-A fusion group had a heart rate of 96.4±3.7 bpm. Only 4 (3.8%) subjects were classified as having LV diastolic dysfunction according to current guidelines. The group showed prolonged isovolumic relaxation time (107.2±25.3 msec vs. 61.6±15.6 msec, p<0.001), increased Tei index (0.76±0.19 vs. 0.48±0.10, p<0.001), lower LVEF (63.8±7.0% vs. 67.3±5.6%, p<0.001) and lower absolute LV GLS (|LV GLS|) (17.0±4.2% vs. 19.7±3.3%, p<0.001) than controls. As the E-A fusion occurred at lower heart rate, the |LV GLS| was also lower (p for trend=0.002). Conclusion Premature E-A fusion at heart rates less than 100 bpm is associated with subclinical LV dysfunction. Time-based indices and LV GLS are helpful for evaluating this easily overlooked population.

Background: Lumbar transforaminal epidural block (TFEB) is an effective treatment modality for radicular pain due to lumbar disc herniation (LDH). The addition of steroids is more effective than local anesthetic alone in TFEBs for patients with LDH. Moreover, the efficacy of TFEBs has been reported to be positively correlated with the volume of injectate. We hypothesized that high-volume TFEBs without steroids effectively alleviate axial back and radicular pain associated with LDH. This study compared the efficacy of high-volume TFEBs with vs. without steroids for the management of the axial and radicular pain caused by LDH. Methods: A total of 54 patients were randomly assigned to either group L or group D.Patients in group L received 8-mL injections of 0.33% lidocaine only. Patients in group D received 8-mL injections of 0.33% lidocaine with 5 mg of dexamethasone. The primary outcomes were pain intensity at baseline and 4 weeks after the procedure. The secondary outcomes included the change of functional disability between baseline and 4 weeks after the procedure, pain scores during injection, and adverse effects. Results: Both groups showed a significant reduction in axial and radicular pain and improvement in the functional status at the outpatient visit 4 weeks after TFEB. However, there were no significant differences between the groups in terms of changes in back pain (10.00 [20.00] vs. 10.00 [22.50]; P = 0.896) or radicular pain (5.00 [20.00] vs.10.00 [12.50]; P = 0.871). Conclusion High-volume TFEBs with and without steroid administration yielded similar significant pain reductions and functional improvements among LDH patients 4 weeks after the procedure.

Purpose: Rapid sequence intubation (RSI) using sedatives and neuromuscular blocking agents (NMBAs) is recommended for pediatric emergency endotracheal intubation (ETI), but is not frequently performed in Korea. This study aimed to verify factors associated with the underuse of RSI medications. Materials and Methods: This multicenter retrospective study reviewed patients aged under 18 years who underwent an ETI within 24 hours of arrival at the emergency department between 2016 and 2019. Any cases of ETI during cardiopulmonary resuscitation were excluded. We investigated the characteristics of the patients, intubators, RSI medications, and outcomes. The study cases were classified into no-medication, sedative-only, and sedative-with-NMBA groups. Multivariable logistic regression analysis of RSI medication use was conducted. Results: A total of 334 cases with a median age of 3.4 years were included in this study. Sedatives and NMBAs were used in 63.8% and 32.9%, respectively. In comparing the no-medication (n=121), sedative-only (n=103), and sedative-with-NMBA (n=110) groups, patient age (median; 1.0 year vs. 2.8 years vs. 11.3 years; p<0.001), underlying medical conditions (77.7% vs. 56.3% vs. 36.4%; p<0.001), and pediatricians as intubators (76.9% vs. 54.4% vs. 17.3%; p<0.001) were different. The factors that influenced sedatives with NMBA use were patient age [for a year increment; adjusted odds ratio (aOR), 1.182; 95% confidence interval (CI), 1.120– 1.249], no underlying medical conditions (aOR, 2.109; 95% CI, 1.093–4.070), and intubators other than pediatricians (aOR, 5.123; 95% CI, 2.257–11.626). Conclusion RSI accounted for 32.9% of pediatric emergency ETI in Korea. The underuse of RSI medications is associated with younger patient age, underlying medical conditions, and pediatricians as intubators.