Background: and Purpose Epidemiological data on narcolepsy are rare in South Korea.We aimed to provide an overview of the burden of narcolepsy and its temporal trend in South Korea. Methods: Patients with narcolepsy were identified by their registration in the Rare and Intractable Disease (RID) register and the Health Insurance Review and Assessment database.Individuals registered in the RID program with the code V234 were considered as having ‘definite narcolepsy’, while those who claimed health insurance with G47.4 as the primary diagnostic code were considered as having ‘probable narcolepsy’. We estimated the annual prevalence, incidence, and medical costs of narcolepsy between 2010 and 2019. Results: The prevalence of definite narcolepsy was 8.4/100,000 in 2019, peaking at 32.0/100,000 in those aged 15–19 years. The prevalence was higher in males, with a relative risk of 1.72. The prevalence has increased over the past 6 years, with an average annual growth rate (AAGR) of 12.2%. The prevalence of probable narcolepsy was 10.7/100,000 in 2019. The incidence of definite narcolepsy increased up to 1.3/100,000 in 2019 with an AAGR of 7.1%. Annual medical expenditure for definite narcolepsy gradually increased up to 4.1 billion KRW in 2019, with a compound annual growth rate of 11.9%. Conclusions This study has provided the first nationwide estimates for narcolepsy in South Korea. The prevalence of diagnosed narcolepsy in South Korea was at the low end of the range of narcolepsy prevalence rates reported for other countries. However, the prevalence and incidence have been steadily increasing over the past decade.

Background: Atrial fibrillation (AF) is a well-known, long-term complication of atrial septal defect (ASD) in adults, even after device closure. Left atrial (LA) strain rate applied to the analysis of chamber function has been demon‑ strated to be an important predictor of LA reverse remodeling. This study aimed to determine the changes in cham‑ ber function after device closure of ASD. Methods: This prospective study enrolled adults with secundum ASD undergoing transcatheter device closure from December 2016 to August 2017. We analyzed the clinical characteristics and LA strain rate before and six months after ASD closure. Results: A total of 11 patients were enrolled. The mean age was 42.6 ± 9.9 years, and six (54.5%) were females. There was no significant change of conventional echocardiographic parameters after ASD closure. The mean global LA res‑ ervoir strain rate before ASD closure was 1.17 ± 0.34 and 0.85 ± 0.30 after ASD closure (p = 0.030). The median global LA conduit strain rate was 0.68 (0.42–1.16) and 0.41 (0.16–0.79) before and after ASD closure (p = 0.213), respectively. The mean global LA contractile strain rate at the six-month follow-up showed significant improvement compared with pre-procedural strain rate (0.33 ± 0.65 vs. − 0.43 ± 0.38, p = 0.006). Conclusions LA functional changes occurred during the six-month follow-up, while LA and right atrial (RA) geo‑ metric changes were not significant. LA reverse remodeling was proved by restoration of LA late diastolic contractile function.

Neuromyelitis optica spectrum disorder (NMOSD) is a rare inflammatory demyelinating disease. Anti-aquaporin-4 antibodies serve as a specific biomarker, while other factors including antecedent infection may also play a role in the development of NMOSD. Abnormal cerebrospinal fluid (CSF) findings such as leukocytosis with concentration >50/mm3 are one of the characteristics of NMOSD, but these were not specific for identifying other infective neurological diseases. Here we describe a rare case of NMOSD with CSF findings suggestive of bacterial meningomyelitis.

Purpose: Isoform 2 of tight junction protein claudin-18 (CLDN18.2) is a potential target for gastric cancer treatment. A treatment targeting CLDN18.2 has shown promising results in gastric cancer. We investigated the clinical significance of CLDN18.2 and other cell-adherens junction molecules (Rho GTPase-activating protein [RhoGAP] and E-cadherin) in metastatic diffuse-type gastric cancer (mDGC). Materials and Methods: We evaluated CLDN18.2, RhoGAP, and E-cadherin expression using two-plex immunofluorescence and quantitative data analysis of H-scores of 77 consecutive mDGC patients who received first-line platinum-based chemotherapy between March 2015 and February 2017. Results: CLDN18.2 and E-cadherin expression was significantly lower in patients with peritoneal metastasis (PM) than those without PM at the time of diagnosis (P=0.010 and 0.013, respectively), whereas it was significantly higher in patients who never developed PM from diagnosis to death than in those who did (P=0.001 and 0.003, respectively). Meanwhile, CLDN18.2 and E-cadherin expression levels were significantly higher in patients with bone metastasis than in those without bone metastasis (P=0.010 and 0.001, respectively).Moreover, we identified a positive correlation between the expression of CLDN18.2 and E-cadherin (P<0.001), RhoGAP and CLDN18.2 (P=0.004), and RhoGAP and E-cadherin (P=0.001). Conversely, CLDN18.2, RhoGAP, and E-cadherin expression was not associated with chemotherapy response and survival. Conclusions CLDN18.2 expression was reduced in patients with PM but significantly intactin those with bone metastasis. Furthermore, CLDN18.2 expression was positively correlated with other adherens junction molecules, which is clinically associated with mDGC and PM pathogenesis.

BACKGROUND: Several factors contribute to differences in Streptococcus pneumoniae serotype distribution. We investigated the serotype distribution and antimicrobial resistance of S. pneumoniae isolated between 2014 and 2016 in Korea. METHODS: We collected a total of 1,855 S. pneumoniae isolates from 44 hospitals between May 2014 and May 2016, and analyzed the serotypes by sequential multiplex PCR. We investigated the distribution of each serotype by patient age, source of the clinical specimen, and antimicrobial resistance pattern. RESULTS: The most common serotypes were 11A (10.1%), followed by 19A (8.8%), 3 (8.5%), 34 (8.1%), 23A (7.3%), and 35B (6.2%). The major invasive serotypes were 3 (12.6%), 19A (7.8%), 34 (7.8%), 10A (6.8%), and 11A (6.8%). Serotypes 10A, 15B, 19A, and 12F were more common in patients ≤5 years old, while serotype 3 was more common in patients ≥65 years old compared with the other age groups. The coverage rates of pneumococcal conjugate vaccine (PCV)7, PCV10, PCV13, and pneumococcal polysaccharide vaccine 23 were 11.8%, 12.12%, 33.3%, and 53.6%, respectively. Of the 1,855 isolates, 857 (46.2%) were multi-drug resistant (MDR), with serotypes 11A and 19A predominant among the MDR strains. The resistance rates against penicillin, cefotaxime, and levofloxacin were 22.8%, 12.5%, and 9.4%, respectively. CONCLUSIONS: There were significant changes in the major S. pneumoniae serotypes in the community. Non-PCV13 serotypes increased in patients ≤5 years old following the introduction of national immunization programs with the 10- and 13-polyvalent vaccines.
Cefotaxime ; Humans ; Immunization Programs ; Korea ; Levofloxacin ; Multiplex Polymerase Chain Reaction ; Penicillins ; Pneumococcal Vaccines ; Pneumonia ; Serogroup ; Streptococcus pneumoniae ; Streptococcus ; Vaccines

BACKGROUND: From May to July 2015, the Republic of Korea experienced the largest outbreak of Middle East respiratory syndrome (MERS) outside the Arabian Peninsula. A total of 186 patients, including 36 deaths, had been diagnosed with MERS-coronavirus (MERS-CoV) infection as of September 30th, 2015. MATERIALS AND METHODS: We obtained information of patients who were confirmed to have MERS-CoV infection. MERS-CoV infection was diagnosed using real-time reverse-transcriptase polymerase chain reaction assay. RESULTS: The median age of the patients was 55 years (range, 16 to 86). A total of 55.4% of the patients had one or more coexisting medical conditions. The most common symptom was fever (95.2%). At admission, leukopenia (42.6%), thrombocytopenia (46.6%), and elevation of aspartate aminotransferase (42.7%) were observed. Pneumonia was detected in 68.3% of patients at admission and developed in 80.8% during the disease course. Antiviral agents were used for 74.7% of patients. Mechanical ventilation, extracorporeal membrane oxygenation, and convalescent serum were employed for 24.5%, 7.1%, and 3.8% of patients, respectively. Older age, presence of coexisting medical conditions including diabetes or chronic lung disease, presence of dyspnea, hypotension, and leukocytosis at admission, and the use of mechanical ventilation were revealed to be independent predictors of death. CONCLUSION: The clinical features of MERS-CoV infection in the Republic of Korea were similar to those of previous outbreaks in the Middle East. However, the overall mortality rate (20.4%) was lower than that in previous reports. Enhanced surveillance and active management of patients during the outbreak may have resulted in improved outcomes.
Antiviral Agents ; Aspartate Aminotransferases ; Coronavirus Infections* ; Disease Outbreaks ; Dyspnea ; Extracorporeal Membrane Oxygenation ; Fever ; Humans ; Hypotension ; Leukocytosis ; Leukopenia ; Lung Diseases ; Middle East Respiratory Syndrome Coronavirus ; Middle East* ; Mortality ; Pneumonia ; Polymerase Chain Reaction ; Republic of Korea* ; Respiration, Artificial ; Thrombocytopenia

BACKGROUND: The adverse effects of the phosphodiesterase-4 inhibitor roflumilast, appear to be more frequent in clinical practice than what was observed in chronic obstructive pulmonary disease (COPD) clinical trials. Thus, we designed this study to determine whether adverse effects could be reduced by starting roflumilast at half the dose, and then increasing a few weeks later to 500 microg daily. METHODS: We retrospectively investigated 85 patients with COPD who had taken either 500 microg roflumilast, or a starting dose of 250 microg and then increased to 500 microg. We analyzed all adverse events and assessed differences between patients who continued taking the drug after dose escalation and those who had stopped. RESULTS: Adverse events were reported by 22 of the 85 patients (25.9%). The most common adverse event was diarrhea (10.6%). Of the 52 patients who had increased from a starting dose of 250 microg roflumilast to 500 microg, 43 (82.7%) successfully maintained the 500 microg roflumilast dose. No difference in factors likely to affect the risk of adverse effects, was detected between the dose-escalated and the discontinued groups. Of the 26 patients who started with the 500 microg roflumilast regimen, seven (26.9%) discontinued because of adverse effects. There was no statistically significant difference in discontinuation rate between the dose-escalated and the control groups (p=0.22). CONCLUSION: Escalating the roflumilast dose may reduce treatment-related adverse effects and improve tolerance to the full dose. This study suggests that the dose-escalated regimen reduced the rate of discontinuation. However, longer-term and larger-scale studies are needed to support the full benefit of a dose escalation strategy.
Clinical Protocols ; Cyclic Nucleotide Phosphodiesterases, Type 4 ; Diarrhea ; Humans ; Phosphodiesterase 4 Inhibitors ; Pulmonary Disease, Chronic Obstructive* ; Retrospective Studies

Gamma-glutamyltranspeptidase (GGT) was purified to electrophoretic homogeneity from the cell extract of H. pylori. The purified enzyme consisted of heavy and light subunits with molecular weights of 38 kDa and 21 kDa, respectively. N-terminal amino acid sequence of heavy and light subunits revealed that H. pylori GGT was processed into 3 parts for a signal peptide of 27 amino acid residues, a heavy subunit of 352 residues, and a light subunit of 188 residues during translation. The reaction rate for hydrolysis of gamma-GpNA was 84.4 micromol/min per milligram of protein, and that for the gamma-glutamyl transfer from gamma-GpNA to gly-gly was 23.8 micromol/min per milligram of protein. The apparent Km values of H. pylori GGT for gamma-glutamyl compounds were on the order of 10-3 to 10-4 M and those for acceptor peptides and amino acids were on the order of 10-1 to 10-2 M. The GGT protein kept approximately 80% of the initial enzymatic activity on incubation at 60degrees C for 15 min. The optimum temperature and pH for reactions of both hydrolysis and transpeptidation were 40degrees C and 9.0, respectively. The transpeptidation and hydrolysis reactions catalyzed by H. pylori GGT were strongly inhibited by L-Gln and moderately inhibited by L-Ala, L-Ser, beta-chloro-L-Ala, and L-Glu. These results demonstrated that the biochemical properties of H. pylori GGT are different from those of other bacterial GGTs. Further, H. pylori GGT might degrade glutathione in the gastric mucous layer of humans if the enzyme could be secreted in the bacterial niches.
Amino Acid Sequence ; Amino Acids ; Glutathione ; Helicobacter ; Helicobacter pylori ; Humans ; Hydrogen-Ion Concentration ; Hydrolysis ; Light ; Molecular Weight ; Peptides ; Protein Sorting Signals

Influenza vaccines are the primary method for controlling influenza and its complications. This study was conducted as a phase 3, randomized, double-blind, controlled, multi-center trial at seven university hospitals to evaluate the immunogenicity and safety of an inactivated, split, trivalent influenza vaccine (GC501, Green Cross Corporation, Yongin, Korea), which was newly manufactured in Korea in 2008. Between September 21 and 26, a total of 329 healthy subjects were recruited for the immunogenicity analysis, while 976 subjects were enrolled for the safety analysis. The GC501 vaccine met both FDA and EMEA criteria with > or = 80% of subjects achieving post-vaccination titers > or = 40 for all three subtypes, even in the elderly. The vaccine was well tolerated with only mild systemic and local adverse events. In summary, GC501 showed excellent immunogenicity and a good safety profile in both young adults and the elderly. The licensure of GC501 might be an important basis in preparation for the future influenza pandemic.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Double-Blind Method ; Humans ; Influenza Vaccines/administration & dosage/*adverse effects/*immunology ; Influenza, Human/*prevention & control ; Male ; Middle Aged ; Republic of Korea ; Vaccination ; Vaccines, Inactivated/administration & dosage/adverse effects/immunology ; Young Adult

A 51-year old man presented with generalized edema for 10 days. He was admitted to the hospital for a renal biopsy, and a pancreatic mass was incidentally detected by ultrasonography. The renal biopsy was consistent with membranous glomerulonephritis and the pancreas biopsy revealed chronic granulomatous inflammation with multinucleated giant cells, which was suggestive of tuberculosis of the pancreas. The patient was initially treated with a steroid and anti-tuberculosis drugs, but the proteinuria in the nephrotic range persisted throughout the 3-month follow-up. With the presumed diagnosis of tuberculosis-induced membranous nephropathy, immunosuppressant therapy was stopped and anti-tuberculosis drugs were administered for 31 months. With anti-tuberculous treatment, the proteinuria was reduced from 22 g/day to 0.57 g/day. A follow-up abdominal CT revealed a marked reduction in the size of the pancreatic mass. This finding suggests that the membranous nephropathy in our case was related to the pancreatic tuberculosis rather than it being related to primary nephrotic syndrome.
Biopsy ; Edema ; Follow-Up Studies ; Giant Cells ; Glomerulonephritis, Membranous ; Humans ; Inflammation ; Nephrotic Syndrome ; Pancreas ; Proteinuria ; Tuberculosis