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.

This study was performed to evaluate the antibacterial activity of water-soluble mangostin derivatives (WsMD) of the ethanol extract of the peel of Garcinia mangostana L. (mangosteen) and the ethanol extracts of the fruit of Psoralea corylifolia L. and the root of Glycyrrhiza uralensis (licorice) against oral bacteria associated with endodontic infections. Cytotoxicity of the three natural products was tested on human embryonic kidney 293 cells (HEK 293) using the methylthiazolyldiphenyl-tetrazolium bromide (MTT) assay or the cell counting method. Antimicrobial activity was evaluated based on the minimum inhibitory concentration (MIC) and minimum bactericidal concentration (MBC). The highest concentrations of the WsMD of the ethanol extract of the peel of mangosteen and ethanol extracts of P. corylifolia L. fruit and licorice root without cytotoxic effects on HEK 293 cells were 20, 400, and 320 µg/ml, respectively. The MIC and MBC values of the WsMD of the ethanol extract of the peel of mangosteen and ethanol extracts of P. corylifolia L. fruit against 35 isolates (23 species) of pulpitis- and periapical disease-causative bacteria were 1.25–20 µg/ml and 25–200 µg/ml, respectively, except for Dialister invisus KCOM 1973. The ethanol extract of licorice root had MBC values of 40–320 µg/ml against 27 of 35 bacterial strains. These results showed that the WsMD of the ethanol extract of mangosteen peel had the highest antibacterial activity among the three natural extracts and suggested it as a potential root canal irrigation agent.

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.

This study was conducted to evaluate the cytotoxic and anti-inflammatory effects of the ethanol extract of Syzygium aromaticum L. (clove) buds. The cytotoxicity test was performed by cell counting method using hTERT-hNOF cells, a human immortalized gingival fibroblast cell line. To test the anti-inflammatory effects, the hTERT-hNOF cells were treated with lipopolysaccharide (LPS) extracted from Porphyromonas gingivalis KCOM 2804 (PgLPS) and ethanol extract of clove buds. The expression levels of PGE2, IL-6, and IL-8 were measured by enzyme-linked immunosorbent assay. The cytotoxicity test data showed a cell viability of ≧ 82% in hTERT-hNOF cells treated with 10 to 80 µg/mL of the ethanol extract of clove buds. The anti-inflammatory test data showed that the expression of PGE2 by PgLPS treatment was reduced to the level of the negative control group by treatment with 10 µg/mL or more of the ethanol extract of clove buds. In group treated with PgLPS and 40 µg/mL of clove bud ethanol extract, the expression levels of IL-6 and IL-8 in were inhibited by 75% and 77%, respectively (p<0.05), compared to the positive control (PgLPS treatment) group. These results suggest that the ethanol extract of clove buds can be used in developing oral hygine products for preventing periodontal disease.

Purpose: Fusobacterium species can cause infections, and associations with cancer are being increasingly reported. As their clinical significance differs, accurate identification of individual species is important. However, matrix-assisted laser desorption/ionization-time of flight mass spectrometry has not been found to be effective in identifying Fusobacterium species in previous studies. In this study, we aimed to improve the accuracy and efficacy of identifying Fusobacterium species in clinical laboratories. Materials and Methods: In total, 229 Fusobacterium isolates were included in this study. All isolates were identified at the species level based on nucleotide sequences of the 16S ribosomal RNA gene and/or DNA-dependent RNA polymerase β-subunit gene (rpoB). Where necessary, isolates were identified based on whole genome sequences. Among them, 47 isolates were used for updating the ASTA database, and 182 isolates were used for the validation of Fusobacterium spp. identification. Results: Fusobacterium isolates used for validation (182/182) were correctly identified at the genus level, and most (180/182) were correctly identified at the species level using the ASTA MicroIDSys system. Most of the F. nucleatum isolates (74/75) were correctly identified at the subspecies level. Conclusion The updated ASTA MicroIDSys system can identify nine species of Fusobacterium and four subspecies of F. nucleatum in good agreement. This tool can be routinely used in clinical microbiology laboratories to identify Fusobacterium species and serve as a springboard for future research.

Background: Many chronic obstructive pulmonary disease (COPD) patients receiving monotherapy continue to experience symptoms, exacerbations and poor quality of life. This study aimed to assess the efficacy and safety of direct switch from once-daily tiotropium (TIO) 18 μg to indacaterol/glycopyrronium (IND/GLY) 110/50 μg once-daily in COPD patients in Korea. Methods: This was a randomized, open-label, parallel group, 12-week trial in mild-to-moderate COPD patients who received TIO 18 μg once-daily for ≥12 weeks prior to study initiation. Patients aged ≥40 years, with predicted postbronchodilator forced expiratory volume in 1 second (FEV1) ≥50%, post-bronchodilator FEV1/forced vital capacity <0.7 and smoking history of ≥10 pack-years were included. Eligible patients were randomized in a 1:1 ratio to either IND/GLY or TIO. The primary objective was to demonstrate superiority of IND/GLY over TIO in pre-dose trough FEV1 at week 12. Secondary endpoints included transition dyspnea index (TDI) focal score, COPD assessment test (CAT) total score, and rescue medication use following the 12-week treatment, and safety assessment. Results: Of the 442 patients screened, 379 were randomized and 347 completed the study. IND/GLY demonstrated superiority in pre-dose trough FEV1 versus TIO at week 12 (least squares mean treatment difference [Δ], 50 mL; p=0.013). Also, numerical improvements were observed with IND/GLY in the TDI focal score (Δ, 0.31), CAT total score (Δ, –0.81), and rescue medication use (Δ, –0.09 puffs/day). Both treatments were well tolerated by patients. Conclusion A direct switch from TIO to IND/GLY provided improvements in lung function and other patient-reported outcomes with an acceptable safety profile in patients with mild-to-moderate airflow limitation.

In previous studies, we introduced that mangosteen extract complex (MEC; ethanol extracts of Garcinia mangostana L. peel and propolis) had inhibitory effects on inflammation and alveolar bone loss in silk-ligature applied and Porphyromonas gingivalis lipopolysaccharide (LPS) induced periodontitis model in rats. This study was conducted to evaluate whether MEC had inhibitory effect of alveolar bone loss when a higher inflammatory state was induced by increasing the injection amount of P. gingivalis LPS by 20 times and increasing the treatment dose of MEC by twice the amount or maintaining MEC dose that used in the previous study. The data showed that alveolar bone loss was significantly reduced in the Lig+L+MEC 1:34 group (treated with mixture of 16 µg mangosteen peel extract powder and 544 µg propolis extract powder) and in the Lig+L+MEC 2:68 group (treated with mixture of 32 µg mangosteen peel extract powder and 1,088 µg propolis extract powder) by 24.3% and 28.9%, respectively. This result reveals that the mixture of MEC 1:34 could be useful in improving periodontal tissue health and may be able to be used as a therapeutic adjuvant for periodontitis.

Background/Aims: Functional dyspepsia (FD) is a chronic upper gastrointestinal symptom complex that routine diagnostic work-up, such as endoscopy, blood laboratory analysis, or radiological examination, fails to identify a cause for. It is highly prevalent in the Korean population, and its response to the various available therapeutic strategies is only modest because of the heterogeneous nature of its pathogenesis. We constituted a guidelines development committee to review the existing guidelines on the management of FD. Methods: This committee drafted statements and conducted a systematic review and meta- analysis of various studies, guidelines, and randomized control trials. External review was also conducted by selected experts. These clinical practice guidelines for FD were developed based on evidence recently accumulated with the revised version of FD guidelines released in 2011 by the Korean Society of Neurogastroenterology and Motility. Results: These guidelines apply to adults with chronic symptoms of FD and include the diagnostic role of endoscopy, Helicobacter pylori screening, and systematic review and meta-analyses of the various treatment options for FD (proton pump inhibitors, Helicobacter pylori eradication, and tricyclic antidepressants), especially according to the FD subtype. Conclusions The purpose of these new guidelines is to aid understanding, diagnosis, and treatment of FD, and the targets of the guidelines are clinicians, healthcare workers at the forefront of patient care, patients, and medical students. The guidelines will continue to be revised and updated periodically.