Cerebellar ataxia, neuropathy, and vestibular areflexia syndrome (CANVAS) is a neurodegenerative disorder caused by a biallelic expansion of pentanucleotide repeats in the RFC1 gene. Previous studies have reported up to 22% of patients with late-onset ataxia harbor this pathogenic repeat expansion. Despite its relatively high prevalence, CANVAS is often underdiagnosed because the disease is not well recognized and genetic testing is not performed in clinical practice. Here, we present a patient with characteristic clinical features, confirmed by genetic testing.

Background: Despite the critical importance of sleep medicine within neurology, notable gaps in education and clinical practice persist among neurologists. This study aims to explore the extent of involvement and the challenges faced by Korean neurologists in sleep medicine, focusing on awareness of polysomnography training program, the operation of sleep study facilities, and educational exposure. Methods: An online survey collected responses from 233 neurologists, focusing on their demographics, knowledge of and involvement in sleep medicine, operation of sleep study facilities, and participation in residency training. Results: The findings indicated that 84.9% of neurologists were aware of polysomnography training program, primarily through professional societies. Nonetheless, 15.1% reported unfamiliarity with these initiatives, with 72.7% of this subgroup expressing interest in sleep medicine yet lacking access to information. In terms of clinical practice, 74 neurologists operated sleep study facilities, with 63% intending to expand. Key operational challenges included staff management, maintaining patient volumes, and inadequate institutional support. Among respondents from teaching hospitals, only 36 out of 114 reported active resident involvement in sleep study interpretations, predominantly hindered by excessive workloads and insufficient staffing. Conclusions A significant number of neurologists have an interest in sleep medicine; however, substantial challenges impede effective education and clinical practice. These results underscore the need for improved educational resources and institutional support to enhance the growth and effectiveness of sleep medicine practices among neurologists.

Background and Objectives: Among various emerging catheter-based treatments for severe tricuspid regurgitation (TR), the spacer device can reduce the regurgitation orifice without manipulating the valve leaflet. However, its clinical application has been hampered by traumatic anchoring to the myocardium and the coaxial alignment of the balloon resulting in insufficient TR reduction. This study aimed to evaluate the early-stage safety, technical feasibility, and preliminary efficacy of the novel atraumatic vertical spacer in patients with isolated severe TR. Methods: All procedures were guided by fluoroscopy and transthoracic echocardiography.The maximum device placement time with an inflated balloon was 24 hours. Changes in the amount of TR, right ventricular function, and patient hemodynamics were measured during balloon deployment. Results: A total of 7 patients (median age 74), underwent successful device implantation without procedure-related complications. During balloon inflation (median 25 minutes), there were no symptoms or signs indicative of TR intolerance. TR was reduced by 1 grade or greater in all patients, with 2 patients exhibiting a reduction of 3 grades, from torrential TR to a moderate degree. Mild TR after balloon inflation was achieved in 3 patients with baseline severe TR. The TR reduction observed during initial balloon deployment was sustained during the subsequent balloon maintenance period. Conclusions The Pivot-balloon procedure was safe, technically feasible, and effective in reducing TR in patients with severe TR. No periprocedural complications or adverse cardiovascular events were reported during device placement with TR reduction observed in all patients. However, longer-term follow-up is needed to confirm safety and treatment effect.

Objective: This study aimed to compare the efficacy and safety of romosozumab, a bone anabolic agent, versus vertebroplasty, a conventional surgical intervention, in treating osteoporotic vertebral compression fractures (OVCFs). Methods: A retrospective analysis included 86 thoracic/lumbar compression fracture patients from 2014 to 2022 at a medical center. Forty-two patients received romosozumab (monthly injections for 1 year) followed by 1 year of denosumab, while 44 underwent vertebroplasty followed by denosumab injections biannually for 2 years. Outcomes were assessed using the Numerical Rating Scale (NRS) for pain, bone mineral density (BMD), vertebral compression ratio, and Cobb angle over 12 months. Results: At 12 months, the romosozumab group showed a greater reduction in NRS scores (4.90 ± 1.01 vs. 4.27 ± 1.34, p = 0.015) and a higher increase in lumbar BMD (0.8 ± 0.5 vs. 0.5 ± 0.3, p = 0.000) compared to the vertebroplasty group. There were no significant differences in changes in hip total BMD and femur neck BMD (p = 0.190, p = 0.167, respectively). Radiographic assessments showed no significant differences in vertebral compression ratio (14.7% vs. 14.8%; p = 0.960) or Cobb angle (4.2° vs. 4.9°; p = 0.302). The incidence of major osteoporotic fractures was lower in the romosozumab group (7.1% vs. 25.0%, p = 0.051), with similar rates of cardiovascular events in both groups (4.8% vs. 9.1%, p = 0.716). Conclusion Romosozumab has demonstrated superior pain reduction and lumbar BMD improvement compared to vertebroplasty at 12 months, with no significant differences in radiographic outcomes or adverse events, suggesting it as an alternative to vertebroplasty for OVCF.

Purpose: In this study, we investigated the effect of bisphenol-A (BPA) and its major analogs, bisphenol-F (BPF), and bisphenol-S (BPS), on spermatogonial stem cells (SSCs) populations using in vitro SSC culture and in vivo transplantation models. Materials and Methods: SSCs enriched from 6- to 8-day-old C57BL/6-eGFP+ male mice testes were treated with varying concentrations of bisphenols for 7 days to examine bisphenol-derived cytotoxicity and changes in SSC characteristics. We utilized flow cytometry, immunocytochemistry, real-time quantitative reverse transcription-PCR, and western blot analysis. The functional alteration of SSCs was further investigated by examining donor SSC-derived spermatogenesis evaluation through in vivo transplantation and subsequent testis analysis. Results: BPF exhibited a similar inhibitory effect on SSCs as BPA, demonstrating a significant decrease in SSC survival, inhibition of proliferation, and induction of apoptosis. On the other hand, while BPS was comparatively weaker than BPA and BPF, it still showed significant SSC cytotoxicity. Importantly, SSCs exposed to BPA, BPF, and BPS exhibited a significant reduction in donor SSC-derived germ cell colonies per total number of cultured cells, indicating that, like BPA, BPF, and BPS can induce a comparable reduction in functional SSCs in the recipient animals. However, the progress of spermatogenesis, as evidenced by histochemistry and the expressions of PCNA and SSC specific markers, collectively indicates that BPA, BPF, and BPS may not adversely affect the spermatogenesis. Conclusions Our findings indicate that the major BPA substitutes, BPF and BPS, have significant cytotoxic effects on SSCs, similar to BPA. These effects may lead to a reduction in the functional self-renewal stem cell population and potential impacts on male fertility.

Enterococcus faecium, particularly in its multidrug-resistant forms, causes invasive nosocomial infections. Given the limited data comparing the effectiveness of the European Committee on Antimicrobial Susceptibility Testing (EUCAST) and the CLSI clinical breakpoints (CBPs) for quinupristin–dalfopristin (QD) resistance and the need to evaluate their practical application, we retrospectively investigated the susceptibility patterns of 287 E.faecium bloodstream isolates from Korean hospitals to QD using the updated EUCAST and CLSI CBPs and two antimicrobial susceptibility testing methods: disk diffusion (DD) and Sensititre broth microdilution (Sensititre). QD resistance rates were 5.9% (CLSI) and 18.8% (EUCAST) for DD and 22.6% (CLSI) and 28.2% (EUCAST) for Sensititre. The most prevalent QD resistance gene types among QD-resistant isolates were ermB+msrC+ or ermB– msrC+. Categorical agreement between DD and Sensititre ranged from 77.7% to 90.7%, depending on the testing method and CBPs applied. The EUCAST zone diameter CBPs more effectively help identify QD-resistant E. faecium isolates using the DD method than the CLSI zone diameter CBPs. In comparison, the CLSI minimum inhibitory concentration (MIC) CBPs provide more reliable results for resistance classification in the Sensititre method than EUCAST MIC CBPs. These findings would help improve clinical decision-making for treating multidrug-resistant E. faecium infections.

Postoperative adhesions following hepatic resection are a significant cause of morbidity, leading to complications such as small bowel obstruction and chronic pain. However, in the context of liver surgery, these adhesions present a unique and formidable challenge, particularly when a subsequent liver transplantation may be necessary. Adhesions from a prior resection can dramatically increase the difficulty, operative time, and blood loss associated with the transplantation procedure, thereby increasing patient morbidity. Furthermore, with the prominence of living donor liver transplantation, minimizing adhesion formation in healthy donors is an ethical and clinical imperative. This review provides a comprehensive analysis of currently available anti-adhesive agents, including films, gels, and solutions. We evaluate the evidence for their efficacy and safety in hepatic resection and, most importantly, explore their critical applications and potential benefits in the specific settings of liver transplantation recipients and living donors. Finally, we propose future research directions to establish best practices for the use of these agents to improve outcomes in the field of liver transplantation.

The 2024 nationwide medical conflict in South Korea acted as a powerful diagnostic tool, exposing deep-seated structural vulnerabilities within its world-renowned liver transplantation system. This policy perspective paper argues that the crisis did not create new problems but rather accelerated pre-existing weaknesses, primarily the system’s over-reliance on resident labor and a high volume of living donor liver transplantation (LDLT). We analyze how the mass departure of resident physicians disrupted the team-based safety net, highlighting the fragility of a model heavily dependent on the trainee workforce. Furthermore, this review explores the subsequent policy debates and emerging models for a post-conflict era. We examine the potential and financial hurdles of transitioning to ‘specialist-centered hospitals’ and emphasize the imperative of revitalizing the deceased donor system to build longterm resilience. This paper concludes by proposing a multi-layered policy strategy focused on workforce restructuring, fundamental reimbursement reform, and a paradigm shift from a high-volume, LDLT-centered model to a more sustainable and resilient liver transplantation ecosystem for the future.

Background: Deceased donor liver transplantation (DDLT) and living donor liver transplantation (LDLT) are employed to address highly urgent patients, including those with acute liver failure (ALF), acute-on-chronic liver failure (ACLF), or critical cirrhosis. This study compares outcomes between LDLT and DDLT patients with ALF, ACLF, or critical cirrhosis in highly urgent LDLT (HU-LDLT) applications. Methods: This study conducted a retrospective analysis of the Korean Network for Organ Sharing (KONOS) data, which included 391 consecutive HU-LDLT applications from 2017 to 2021. Results: The proportion of DDLT was 15.1% (n=59) within the cohort of HU-LDLT applications. The prevalence of hepatorenal syndrome, duration of pre-transplant intensive care unit (ICU) care, incidence of pre-transplant continuous renal replacement therapy, and median model for end-stage liver disease scores were significantly greater and prolonged in DDLT patients compared to LDLT patients. Statistical analysis revealed no significant differences in postoperative complications or overall survival between the two groups. In the multivariate analysis, only pre-transplant ventilator care emerged as a significant predisposing factor for mortality. Conclusion The present study indicates that LDLT is a viable option, yielding comparable perioperative and long-term outcomes to DDLT for HU patients, which can encourage living liver donation to overcome organ shortages in HU patients.