Background: and Purpose Unlike other immune-mediated neuropathies, anti-myelin-associated glycoprotein (MAG) neuropathy is often refractory to immunotherapy. It is necessary to compare the relative efficacies of various immunotherapies and develop objective biomarkers in order to optimize its clinical management. Methods: This study recruited 91 patients with high anti-MAG antibody titers from 7 tertiary hospitals in South Korea. We analyzed the baseline characteristics, therapeutic outcomes, and nerve conduction study (NCS) findings of 68 patients and excluded 23 false positive cases. Results: The rate of positive responses to treatment was highest using zanubrutinib (50%) and rituximab (36.4%), followed by corticosteroids (16.7%), immunosuppressants (9.5%), intravenous immunoglobulin (5%), and plasma exchange (0%). Disability and weakness were significantly associated with multiple NCS parameters at the time of diagnosis, especially distal compound muscle action potential (CMAP) amplitudes. Moreover, the longitudinal trajectory of the average CMAP amplitudes paralleled the clinical courses, with a 16.2 percentile decrease as an optimal cutoff for predicting a clinical exacerbation (area under the receiver operating characteristic curve=0.792). Conclusions Our study supports the use of NCS as an objective marker for estimating disease burden and tracking clinical changes in patients with anti-MAG neuropathy. We have described the beneficial effects of rituximab and a new drug, zanubrutinib, compared with conventional immunotherapies.

Background: During the coronavirus disease 2019 (COVID-19) pandemic, patients with myasthenia gravis (MG) were more susceptible to poor outcomes owing to respiratory muscle weakness and immunotherapy. Several studies conducted in the early stages of the COVID-19 pandemic reported higher mortality in patients with MG compared to the general population. This study aimed to investigate the clinical course and prognosis of COVID-19 in patients with MG and to compare these parameters between vaccinated and unvaccinated patients in South Korea. Methods: This multicenter, retrospective study, which was conducted at 14 tertiary hospitals in South Korea, reviewed the medical records and identified MG patients who contracted COVID-19 between February 2022 and April 2022. The demographic and clinical characteristics associated with MG and vaccination status were collected. The clinical outcomes of COVID-19 infection and MG were investigated and compared between the vaccinated and unvaccinated patients. Results: Ninety-two patients with MG contracted COVID-19 during the study. Nine (9.8%) patients required hospitalization, 4 (4.3%) of whom were admitted to the intensive care unit. Seventy-five of 92 patients were vaccinated before contracting COVID-19 infection, and 17 were not. During the COVID-19 infection, 6 of 17 (35.3%) unvaccinated patients were hospitalized, whereas 3 of 75 (4.0%) vaccinated patients were hospitalized (P < 0.001). The frequencies of ICU admission and mechanical ventilation were significantly lower in the vaccinated patients than in the unvaccinated patients (P = 0.019 and P = 0.032, respectively). The rate of MG deterioration was significantly lower in the vaccinated patients than in the unvaccinated patients (P = 0.041). Logistic regression after weighting revealed that the risk of hospitalization and MG deterioration after COVID-19 infection was significantly lower in the vaccinated patients than in the unvaccinated patients. Conclusion This study suggests that the clinical course and prognosis of patients with MG who contracted COVID-19 during the dominance of the omicron variant of COVID-19 may be milder than those at the early phase of the COVID-19 pandemic when vaccination was unavailable. Vaccination may reduce the morbidity of COVID-19 in patients with MG and effectively prevent MG deterioration induced by COVID-19 infection.

Objectives: . This study was conducted to investigate the electroacoustic characteristics of personal sound amplification products (PSAPs), to identify whether PSAPs provide adequate gain and output for three common hearing loss (HL) configurations, and to compare the benefits of a representative PSAP (RPSAP) and a conventional hearing aid (HA) for clinical hearing outcomes as a pilot study. Methods: . The study comprised three phases: electroacoustic analysis, simulated real-ear measurements (REMs), and clinical hearing experiments. Electroacoustic analysis and simulated REMs were performed for three basic PSAPs (BeethoSOL, EarJJang, and Geniesori2) and three high-end PSAPs (Hearing Able, Olive Smart Ear, and SoriIn) using the Aurical Hearing Instrument Test box with a 2-mL coupler. Four electroacoustic characteristics (maximum output sound pressure level at 90 dB SPL, frequency range, equivalent input noise, and total harmonic distortion) were investigated. By simulated REMs, appropriate levels of the six PSAPs for three common HL configurations (mild-to-moderate high-frequency HL, moderate to moderately severe sloping HL, and moderate flat HL) were determined. Clinical experiments compared the performance of RPSAP to HA, both of which were fitted by audiologists using REMs. Clinical experiments were administered using functional gain, a word recognition test, and the Korean version of the Hearing in Noise Test in six participants with bilateral moderate sensorineural HL. Results: . The two high-end devices met all tolerances. One basic and two high-end PSAPs showed appropriate levels for three common HL configurations. In the clinical experiments, the RPSAP showed better performance than unaided, but slightly worse than HA under all test conditions. Conclusion . Certain PSAPs met all specified tolerances for electroacoustic analysis and approximated prescriptive targets in well-controlled laboratory conditions. The pilot clinical experiments explored the possibility that the RPSAP could serve as a hearing assistive device for patients with moderate HL.

Background/Aims: The prospective Crohn’s Disease Clinical Network and Cohort Study is a nationwide multicenter cohort study of patients with Crohn’s disease (CD) in Korea, aiming to prospectively investigate the clinical features and long-term prognosis associated with CD. Methods: Patients diagnosed with CD between January 2009 and September 2019 were prospectively enrolled. They were divided into two cohorts according to the year of diagnosis: cohort 1 (diagnosed between 2009 and 2011) versus cohort 2 (between 2012 and 2019). Results: A total of 1,175 patients were included, and the median follow-up duration was 68 months (interquartile range, 39.0 to 91.0 months). The treatment-free durations for thiopurines (p<0.001) and anti-tumor necrosis factor agents (p=0.018) of cohort 2 were shorter than those of cohort 1. Among 887 patients with B1 behavior at diagnosis, 149 patients (16.8%) progressed to either B2 or B3 behavior during follow-up. Early use of thiopurine was associated with a reduced risk of behavioral progression (adjusted hazard ratio [aHR], 0.69; 95% confidence interval [CI], 0.50 to 0.90), and family history of inflammatory bowel disease was associated with an increased risk of behavioral progression (aHR, 2.29; 95% CI, 1.16 to 4.50). One hundred forty-one patients (12.0%) underwent intestinal resection, and the intestinal resection-free survival time was significantly longer in cohort 2 than in cohort 1 (p=0.003). The early use of thiopurines (aHR, 0.35;95% CI, 0.23 to 0.51) was independently associated with a reduced risk of intestinal resection. Conclusions The prognosis of CD in Korea appears to have improved over time, as evidenced by the decreasing intestinal resection rate. Early use of thiopurines was associated with an improved prognosis represented by a reduced risk of intestinal resection.

BACKGROUND AND PURPOSE: Visual assessment of medial temporal-lobe atrophy (MTA) has been quick, reliable, and easy to apply in routine clinical practice. However, one of the limitations in visual assessments of MTA is the lack of widely accepted age-adjusted norms and cutoff scores for MTA for a diagnosis of Alzheimer's disease (AD). This study aimed to determine the optimal cutoff score on a T1-weighted axial MTA Visual Rating Scale (VRS) for differentiating patients with AD from cognitively normal elderly people. METHODS: The 3,430 recruited subjects comprising 1,427 with no cognitive impairment (NC) and 2003 AD patients were divided into age ranges of 50–59, 60–69, 70–79, and 80–89 years. Of these, 446 participants (218 in the NC group and 228 in the AD group) were chosen by random sampling for inclusion in this study. Each decade age group included 57 individuals, with the exception of 47 subjects being included in the 80- to 89-year NC group. The scores on the T1-weighted axial MTA VRS were graded by two neurologists. The cutoff values were evaluated from the area under the receiver operating characteristic curve. RESULTS: The optimal axial MTA VRS cutoff score from discriminating AD from NC increased with age: it was ≥as ≥1, ≥2, and ≥3 in subjects aged 50–59, 60–69, 70–79, and 80–89 years, respectively (all p < 0.001). CONCLUSIONS: These results show that the optimal cutoff score on the axial MTA VRS for diagnosing of AD differed according to the decade age group. This information could be of practical usefulness in the clinical setting.
Aged ; Alzheimer Disease* ; Atrophy* ; Cognition Disorders ; Dementia* ; Diagnosis ; Humans ; Korea* ; Pemetrexed ; ROC Curve

BACKGROUND/AIMS: The eradication rate of Helicobacter pylori (H. pylori) has been decreasing recently in Korea due to antibiotics resistance. The aim of this study was to investigate the trend of eradication rate and clinical factors affecting the eradication rate of H. pylori in the last 10 years in west Gyeonggi-do, Korea. METHODS: The trends of eradication rate of H. pylori, gender, age, concomitant mediations, and clinical factors were retrospectively evaluated in patients with H. pylori infection between 2006 and 2015 (n=2,485). RESULTS: The overall H. pylori eradication rate for the standard triple therapy was 82.5%. The annual eradication rates from 2006 to 2015 were 90%, 77.9%, 75.8%, 83.2%, 85.6%, 90.1%, 81.3%, 81.1%, 78.7%, and 78.8%, respectively, showing a significant decrement during the last five years (p < 0.001). Higher eradication rate was observed in males than in females (p < 0.001). Esomeprazole showed a higher eradication rate compared with pantoprazole between 2006 and 2010 (p < 0.022). Age and the use of probiotics and mucosal protective agents played no significant role in the H. pylori eradication rate. The overall eradication rate for bismuth-based quadruple therapy was 94.4%. CONCLUSIONS: The eradication rate of H. pylori over the last 10 years for first-line therapy ranged from 75.8 to 90.1%; the eradication rate for triple therapy has declined. However, the eradication rate for quadruple therapy has remained unchanged over the last 10 years.
Anti-Bacterial Agents ; Disease Eradication ; Esomeprazole ; Female ; Gyeonggi-do* ; Helicobacter pylori* ; Helicobacter* ; Humans ; Korea* ; Male ; Probiotics ; Protective Agents ; Retrospective Studies

No abstract available.
Colon* ; Colonic Pseudo-Obstruction* ; Herpes Zoster*

PURPOSE: The purpose of this study was to investigate the feasibility and survival benefits of combined treatment with radiotherapy and adjuvant temozolomide (TMZ) in a Korean sample. MATERIALS AND METHODS: A total of 750 Korean patients with histologically confirmed glioblastoma multiforme, who received concurrent chemoradiotherapy with TMZ (CCRT) and adjuvant TMZ from January 2006 until June 2011, were analyzed retrospectively. RESULTS: After the first operation, a gross total resection (GTR), subtotal resection (STR), partial resection (PR), biopsy alone were achieved in 388 (51.7%), 159 (21.2%), 96 (12.8%), and 107 (14.3%) patients, respectively. The methylation status of O6-methylguanine-DNA methyltransferase (MGMT) was reviewed retrospectively in 217 patients. The median follow-up period was 16.3 months and the median overall survival (OS) was 17.5 months. The actuarial survival rates at the 1-, 3-, and 5-year OS were 72.1%, 21.0%, and 9.0%, respectively. The median progression-free survival (PFS) was 10.1 months, and the actuarial PFS at 1-, 3-, and 5-year PFS were 42.2%, 13.0%, and 7.8%, respectively. The patients who received GTR showed a significantly longer OS and PFS than those who received STR, PR, or biopsy alone, regardless of the methylation status of the MGMT promoter. Patients with a methylated MGMT promoter also showed a significantly longer OS and PFS than those with an unmethylated MGMT promoter. Patients who received more than six cycles of adjuvant TMZ had a longer OS and PFS than those who received six or fewer cycles. Hematologic toxicity of grade 3 or 4 was observed in 8.4% of patients during the CCRT period and in 10.2% during the adjuvant TMZ period. CONCLUSION: Patients treated with CCRT followed by adjuvant TMZ had more favorable survival rates and tolerable toxicity than those who did not undergo this treatment.
Biopsy ; Chemoradiotherapy* ; Disease-Free Survival ; Follow-Up Studies ; Glioblastoma* ; Humans ; Korea* ; Methylation ; Radiotherapy ; Retrospective Studies* ; Survival Rate

PURPOSE: Infliximab is currently used for the treatment of active Crohn's disease (CD). We aimed to assess the efficacy and safety of infliximab therapy and to determine the predictors of response in Korean patients with CD. MATERIALS AND METHODS: A total of 317 patients who received at least one infliximab infusion for active luminal CD (n=198) and fistulizing CD (n=86) or both (n=33) were reviewed retrospectively in 29 Korean referral centers. Clinical outcomes of induction and maintenance therapy with infliximab, predictors of response, and adverse events were evaluated. RESULTS: In patients with luminal CD, the rates of clinical response and remission at week 14 were 89.2% and 60.0%, respectively. Male gender and isolated colonic disease were associated with higher remission rates at week 14. In week-14 responders, the probabilities of sustained response and remission were 96.2% and 93.3% at week 30 and 88.0% and 77.0% at week 54, respectively. In patients with fistulizing CD, clinical response and remission were observed in 85.0% and 56.2% of patients, respectively, at week 14. In week-14 responders, the probabilities of sustained response and remission were 94.0% and 97.1%, respectively, at both week 30 and week 54. Thirty-nine patients (12.3%) experienced adverse events related to infliximab. Serious adverse events developed in 19 (6.0%) patients including seven cases of active pulmonary tuberculosis. CONCLUSION: Infliximab induction and maintenance therapy are effective and well tolerable in Korean patients with luminal and fistulizing CD. However, clinicians must be aware of the risk of rare yet critical adverse events.
Colonic Diseases ; Crohn Disease* ; Humans ; Infliximab* ; Male ; Phenobarbital ; Referral and Consultation ; Retrospective Studies ; Tuberculosis ; Tuberculosis, Pulmonary

Upper gastrointestinal bleeding is a common condition and has various clinical courses and prognosis. End stage renal disease (ESRD) patients receiving hemodialysis have a high risk of vascular complications and increased risk of ischemic colitis. A 59-year-old male patient with ESRD receiving hemodialysis visited due to hematemesis. After admission, he showed recurrent hematemesis and hypovolemic shock. Upper esophagogastroduodenoscopy revealed gastric ulcer bleeding and endoscopic hemostasis was successfully performed. Blood transfusion and norepinephrine was administered for hypovolemic shock during initial 3 days. Ten days later, he exhibited hematochezia. Sigmoidoscopy revealed necrotic ischemic colitis in sigmoid colon and segmental colectomy was performed. However, recurrent leakage and ischemia were developed in colon as well as small bowel, and he finally died after 55 hospital days in spite of additional operations. Here, we report a case of peptic ulcer bleeding in patient with ESRD who suffered a severe form of ischemic colitis with transmural necrosis.
Blood Transfusion ; Colectomy ; Colitis, Ischemic* ; Colon ; Colon, Sigmoid ; Endoscopy, Digestive System ; Gastrointestinal Hemorrhage ; Hematemesis ; Hemorrhage ; Hemostasis, Endoscopic ; Humans ; Ischemia ; Kidney Failure, Chronic* ; Male ; Middle Aged ; Necrosis ; Norepinephrine ; Peptic Ulcer ; Prognosis ; Renal Dialysis* ; Shock* ; Sigmoidoscopy ; Stomach Ulcer*