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.

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: Acne vulgaris is a chronic inflammatory disease of the pilosebaceous unit. Adapalene-benzoyl peroxide gel has been proven to be effective in the treatment of both inflammatory and comedonal acne. However, skin irritation characterized by erythema, scaling, and dryness may occur with the use of this formula. Objective: This study aimed to investigate the effects and safety of a new formulation of adpalene-benzoyl peroxide in combination with multi-lamellar emulsion (MLE) for acne treatment. Methods: All patients were treated with adapalene-benzoyl peroxide with MLE once daily for 12 weeks on acne lesions. The subjects visited the hospital at baseline, 2 weeks, 4 weeks, 8 weeks, and 12 weeks, and clinical effects, patient satisfaction, and adverse effects were estimated. The severity of adverse effects was measured on a 4-point scale. Results: A total of 30 subjects were enrolled. The number of lesions decreased significantly from 20.2 to 7.8 (p＜ 0.0001) after treatment. Investigator’s global assessment showed almost clear, and patient satisfaction increased from 3.57 to 4.13. The subjects had adverse effects such as tingling sensation (83.3%), scales (80.0%), erythema (63.3%), and dryness (63.3%). Severity of adverse effects had 1 point on average. Additionally, transepidermal water loss was found to be decreased. Conclusion This study suggests that adapalene-benzoyl peroxide with MLE is effective for treating acne lesions and shows high patient satisfaction. Hence, this new combination could be a safe and well-tolerated option for acne treatment.

BACKGROUND/AIMS: It is well known that gender differences are associated with clinical outcomes in patients with acute myocardial infarction (AMI). However, it is not clear whether gender differences affect the prognosis of elderly patients with AMI. METHODS: We analyzed the incidence of in-hospital complications and mortality in the Korea Acute Myocardial Infarction Registry-National Institutes of Health from November 2011 to June 2015. This study included elderly patients (≥ 75 years) diagnosed with AMI. RESULTS: A total of 2,953 patients were eligible for this study. Among them, 1,529 (51.8%) patients were female, and the mean age of the female group was older than that of the male group (80.7 ± 4.4 vs. 79.6 ± 4.0 years, respectively, p < 0.001). Elderly females utilized emergency medical services less frequently compared with elderly males (11.5 vs. 15.4%, respectively, p < 0.001). Elderly female AMI patients had a similar rate of in-hospital mortality compared with elderly males (7.1 vs. 8.4%, respectively, p = 0.196). The rate of major cardiac adverse events (MACEs) was lower in elderly females than males during a 12-month follow-up (hazard ratio [HR] 1.19, 95% confidence interval [CI] 1.00-1.41, p = 0.045). According to multivariate analysis, the male gender is an independent factor for predicting 1-year MACEs (HR 1.37, 95% CI 1.14-1.65, p < 0.001). CONCLUSIONS: No significant differences in peri-procedural complications or in-hospital mortality were observed between male and female elderly patients with AMI. However, elderly female patients had a more favorable prognosis than male patients during a 1-year clinical follow-up.
Academies and Institutes ; Aged ; Emergency Medical Services ; Female ; Follow-Up Studies ; Hospital Mortality ; Humans ; Incidence ; Korea ; Male ; Mortality ; Multivariate Analysis ; Myocardial Infarction ; Prognosis

BACKGROUND/AIMS: It is well known that gender differences are associated with clinical outcomes in patients with acute myocardial infarction (AMI). However, it is not clear whether gender differences affect the prognosis of elderly patients with AMI. METHODS: We analyzed the incidence of in-hospital complications and mortality in the Korea Acute Myocardial Infarction Registry-National Institutes of Health from November 2011 to June 2015. This study included elderly patients (≥ 75 years) diagnosed with AMI. RESULTS: A total of 2,953 patients were eligible for this study. Among them, 1,529 (51.8%) patients were female, and the mean age of the female group was older than that of the male group (80.7 ± 4.4 vs. 79.6 ± 4.0 years, respectively, p < 0.001). Elderly females utilized emergency medical services less frequently compared with elderly males (11.5 vs. 15.4%, respectively, p < 0.001). Elderly female AMI patients had a similar rate of in-hospital mortality compared with elderly males (7.1 vs. 8.4%, respectively, p = 0.196). The rate of major cardiac adverse events (MACEs) was lower in elderly females than males during a 12-month follow-up (hazard ratio [HR] 1.19, 95% confidence interval [CI] 1.00-1.41, p = 0.045). According to multivariate analysis, the male gender is an independent factor for predicting 1-year MACEs (HR 1.37, 95% CI 1.14-1.65, p < 0.001). CONCLUSIONS No significant differences in peri-procedural complications or in-hospital mortality were observed between male and female elderly patients with AMI. However, elderly female patients had a more favorable prognosis than male patients during a 1-year clinical follow-up.

BACKGROUND/AIMS: Stress has a role in the pathogenesis of functional dyspepsia (FD) and influences food intake in humans and animals. Prokinetic drugs have been used in FD, and some of these drugs reverse the feeding inhibition (FI) induced by acute restraint stress in rats. We aimed to evaluate the effect of DA-9701 on FI induced by acute restraint in rats. MATERIALS AND METHODS: Male Sprague-Dawley rats were divided into 6 groups: Control (no stress), Stress+vehicle, and Stress+DA-9701 at doses of 1, 3, 10, and 30 mg/kg (n=6~7). DA-9701 or vehicle was administered through gastric gavage 45 minutes before stress. After 60 minutes of stress, pre-weighed chow was given and the weight of remaining food was measured 30 and 60 minutes later. The effect of DA-9701 on FI was compared after pretreatment with WAY100635, a 5HT1A antagonist. RESULTS: The restraint stress group had significantly less food intake than the control group. After feeding, rats given 1 and 3 mg/kg of DA-9701 showed increased food intake at 60 minutes, but this was not statistically significant. Rats given 10 mg/kg of DA-9701 showed significantly increased food intake at 30 minutes and 60 minutes (P < 0.05). Interestingly, rats given 30 mg/kg of DA-9701 showed a significant decrease in food intake, similar to that of the vehicle group. The beneficial effect of 10 mg/kg of DA-9701 on FI was abolished by the pretreatment with WAY100635. CONCLUSIONS: Acute restraint stress reduced food intake in rats and pretreatment with DA-9701 improved stress-induced FI.
Animals ; Dyspepsia ; Eating ; Humans ; Male ; Rats ; Rats, Sprague-Dawley ; Stress, Physiological

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

The valproic acid (VPA)-induced animal model is one of the most widely utilized environmental risk factor models of autism. Autism spectrum disorder (ASD) remains an insurmountable challenge among neurodevelopmental disorders due to its heterogeneity, unresolved pathological pathways and lack of treatment. We previously reported that VPA-exposed rats and cultured rat primary neurons have increased Pax6 expression during post-midterm embryonic development which led to the sequential upregulation of glutamatergic neuronal markers. In this study, we provide experimental evidence that telomerase reverse transcriptase (TERT), a protein component of ribonucleoproteins complex of telomerase, is involved in the abnormal components caused by VPA in addition to Pax6 and its downstream signals. In embryonic rat brains and cultured rat primary neural progenitor cells (NPCs), VPA induced the increased expression of TERT as revealed by Western blot, RT-PCR, and immunostainings. The HDAC inhibitor property of VPA is responsible for the TERT upregulation. Chromatin immunoprecipitation revealed that VPA increased the histone acetylation but blocked the HDAC1 binding to both Pax6 and Tert genes. Interestingly, the VPA-induced TERT overexpression resulted to sequential upregulations of glutamatergic markers such as Ngn2 and NeuroD1, and inter-synaptic markers such as PSD-95, α-CaMKII, vGluT1 and synaptophysin. Transfection of Tert siRNA reversed the effects of VPA in cultured NPCs confirming the direct involvement of TERT in the expression of those markers. This study suggests the involvement of TERT in the VPA-induced autistic phenotypes and has important implications for the role of TERT as a modulator of balanced neuronal development and transmission in the brain.
Acetylation ; Animals ; Autism Spectrum Disorder ; Autistic Disorder ; Blotting, Western ; Brain ; Chromatin Immunoprecipitation ; Embryonic Development ; Female ; Histones ; Models, Animal ; Neurodevelopmental Disorders ; Neurons ; Phenotype ; Population Characteristics ; Pregnancy ; Rats ; Ribonucleoproteins ; Risk Factors ; RNA, Small Interfering ; Stem Cells ; Synaptophysin ; Telomerase* ; Transfection ; Up-Regulation ; Valproic Acid*

BACKGROUND: Hidradenitis suppurativa is a chronic inflammatory skin disease showing recurrent painful nodules and abscesses. Many treatment modalities, such as topical antibiotics, systemic antibiotics, retinoids, immunomodulators, biologics, and surgical treatment are used to treat the disease, but there is no single effective treatment for hidradenitis suppurativa. OBJECTIVE: We conducted this study to compare the clinical outcomes of treatment with systemic antibiotics and systemic retinoids. METHODS: We analyzed the clinical features, treatment modalities, and clinical outcomes of patients with hidradenitis suppurativa and compared the clinical outcomes of treatment modalities. RESULTS: A total of 77 patients were enrolled. Male patients were predominant, and the mean age was 28.1 years. Buttock (42.0%) was the most commonly affected site and 63 patients (81.8%) had lesions at Hurley stage I. Forty-eight patients (62.3%) were treated with systemic antibiotics and 29 (37.7%) with systemic retinoid. Seventy-one patients (92.2%) showed improvement after a mean treatment duration of 8.3 weeks, and 69 (89.6%) experienced recurrence after improvement. Systemic antibiotics showed a relatively high improvement rate, short mean treatment duration, and low recurrence rate compared with systemic retinoids, but the differences were not statistically significant. Among systemic antibiotics, both tetracycline and macrolide showed good therapeutic effects for HS, but the differences were also not statistically significant. CONCLUSION: In this study, systemic antibiotics were associated with superior treatment outcomes compared to systemic retinoids, though without statistical significance. In the case of mild lesions, treatment with systemic retinoids appears to show comparable effectiveness to systemic antibiotics.
Abscess ; Anti-Bacterial Agents* ; Biological Products ; Buttocks ; Hidradenitis Suppurativa* ; Hidradenitis* ; Humans ; Immunologic Factors ; Isotretinoin ; Male ; Recurrence ; Retinoids* ; Skin Diseases ; Tetracycline ; Therapeutic Uses