Background: Transthyretin amyloidosis, a protein-misfolding disorder characterized by systemic amyloid deposition, can be classified as wild-type transthyretin amyloidosis (ATTRwt) or hereditary transthyretin amyloidosis (ATTRv), depending on the presence of transthyretin (TTR) gene variants. We examined the genetic distribution of TTR variants in Korean patients diagnosed with ATTRv. Methods: We retrospectively reviewed 801 participants who underwent TTR analysis at Samsung Medical Center from 2012 to 2024. The participants were categorized into two groups: in-house probands or relatives, and externally referred probands or relatives. Results: Pathogenic or likely pathogenic TTR variants were detected in 36 of 165 in-house probands (21.8%), among which D38A was the most frequent variant (50.0%; 18/36), followed by M13dup and E89K (8.3% each). Among referred probands, D38A was predominant (54.5%; 12/22), followed by M13dup (22.7%; 5/22). Cardiac amyloid involvement was the most common manifestation, observed in 97.2% (35/36) of in-house probands with ATTRv, followed by peripheral nervous system (PNS; 94.4%) and autonomic nervous system (ANS; 88.9%) involvement. In contrast, ANS involvement was most prevalent among in-house relatives who underwent organ evaluation (61.5%; 24/39), followed by cardiac (52.1%; 25/48) and PNS (48.7%; 19/39) involvement. Five of the eight in-house relatives harboring M13dup (62.5%) showed organ involvement, primarily in the ANS, supporting the pathogenicity of this variant. Conclusions This study provides the largest single-institution dataset of Korean patients with ATTRv, incorporating systematic organ assessments. The predominance of the unique TTR variants D38A and M13dup delineates a distinct genetic landscape that may facilitate accurate and timely diagnosis of ATTRv in the Korean population.

Objective: This study aimed to evaluate the feasibility, safety, and oncologic outcomes of irreversible electroporation (IRE) using a clustered electrode in patients with locally advanced pancreatic cancer (LAPC). Materials and Methods: In this single-center prospective cohort study, 13 patients with LAPC (median age, 60 years; range, 48–78 years) underwent clustered electrode IRE between September 2022 and September 2024. Patient characteristics, procedural details, and clinical outcomes were recorded. Endpoints included technical success, procedure-related complications, overall survival (OS), and progression-free survival (PFS). Results: Tumors were located in the pancreatic head in four patients (30.8%) and in the body/tail in nine (69.2%). The median tumor size was 2.4 cm (1.5–4.0 cm), and vascular invasion was present in all patients. Technical success was achieved in all patients. Intraoperative IRE was performed in 11 (84.6%) patients, and 2 (15.4%) patients underwent percutaneous IRE. Gastrointestinal bleeding events as major complications occurred in two patients (15.4%) and, both were successfully controlled by embolization. No 60-day mortality was observed. At a median follow-up of 24.5 months (range, 9.9–33.4 months) after IRE, median OS and PFS from IRE were 20.1 and 14.5 months, respectively. Conclusion IRE using clustered electrodes for LAPC appears to be a feasible therapeutic approach, offering reliable technical success and acceptable safety. Survival outcomes are encouraging; however, larger, controlled studies are required.

Background: Programmed cell death 1/programmed cell death ligand 1 (PD-1/PD-L1) inhibitors are effective treatments for non-small cell lung cancer (NSCLC), but their use can lead to severe pneumonitis. This study aims to evaluate the utility of Krebs von den Lungen-6 (KL-6) for predicting and diagnosing PD-1/PD-L1 inhibitor-related pneumonitis in NSCLC patients. Methods: We conducted a prospective observational study at a university-affiliated tertiary hospital in Korea from February 2022 to June 2023. Baseline KL-6 levels were measured immediately before initiating PD-1/PD-L1 inhibitor therapy. In patients who developed pneumonitis, KL-6 levels were monitored every 1–2 weeks from the onset of pneumonitis. For patients without pneumonitis, KL-6 levels were measured every 6 weeks. We compared clinical characteristics and serial KL-6 levels between the pneumonitis and non-pneumonitis groups. Results: Eighteen patients were enrolled, with 11 developing pneumonitis. Baseline KL-6 levels did not significantly differ between the two groups (261 U/mL in the pneumonitis group vs. 373 U/mL in the non-pneumonitis group, p=0.375). In the pneumonitis group, KL-6 levels generally showed an upward trend, with a median of 412 U/mL at pneumonitis onset. Conversely, KL-6 levels in the non-pneumonitis group showed no clear overall change. Conclusion In this pilot study, baseline KL-6 was not clearly linked to PD-1/PD-L1 inhibitor-related pneumonitis in NSCLC patients. However, increases in KL-6 levels post-baseline were more frequently observed in patients who developed pneumonitis, including cases of all-cause pneumonitis.

Background and Objectives: Clinical characteristics and outcomes in acute heart failure (AHF) vary by phenotype. We assessed phenotype-specific features, treatment patterns, and outcomes in a nationwide Korean cohort. Methods: The Korean Heart Failure III registry prospectively enrolled 7,351 AHF admissions at 47 hospitals. Among 6,777 patients with available left ventricular ejection fraction (EF), phenotypes were defined as heart failure with reduced EF (HFrEF, ≤40%), mildly reduced EF (HFmrEF,41–49%), or preserved EF (HFpEF, ≥50%). The primary endpoint was a 12-month composite of all-cause death or heart transplantation, evaluated from index admission and, among hospital survivors, from discharge. We used inverse probability weighting (multinomial generalized boosted models with stabilized, trimmed weights) and weighted Cox proportional-hazards models to estimate hazard ratios (HRs). Results: Phenotype distribution was 58.9% HFrEF, 13.6% HFmrEF, and 27.5% HFpEF. Crude 12-month composite rates from index admission were 13.4% (HFrEF), 12.7% (HFmrEF), and 16.8% (HFpEF). After weighting, from index admission, HFmrEF (HR, 0.892; 95% confidence interval [CI], 0.731–1.088) and HFpEF (HR, 1.101; 95% CI, 0.939–1.291) did not differ from HFrEF; from discharge, HFpEF had modestly higher risk (HR, 1.207; 95% CI, 1.008–1.445) whereas HFmrEF did not (HR, 1.039; 95% CI, 0.844–1.279). Hyponatremia and chronic kidney disease were consistent adverse markers, while angiotensin-converting enzyme inhibitor/ angiotensin II receptor blocker use at discharge was protective. Conclusions Across the EF spectrum, phenotypes showed distinct profiles and risk. Postdischarge risk was modestly higher in HFpEF, supporting phenotype-tailored care and systematic discharge optimization in Korean patients with AHF.

Middle meningeal arteriovenous fistulas (MMAVFs) are known as rare diseases, often associated with head trauma, with idiopathic cases being exceedingly uncommon. Here, we present a clinical case of spontaneous and simultaneous bilateral MMAVFs in a 37-year-old woman. She presented with persistent pulsatile tinnitus but no history of head trauma. Bilateral MMAVFs were identified via Time-of-Flight Magnetic Resonance Angiography. Subsequent digital subtraction angiography revealed a fistula between the middle meningeal artery (MMA) and middle meningeal vein (MMV) without definite intracranial venous reflux. We had planned coil embolization via a trans-arterial approach for both sides. Remarkably, after six days, the right-sided MMAVF observed in the previous external carotid artery (ECA) angiogram disappeared spontaneously. The left-sided MMAVF was successfully treated with coil embolization, achieving complete obliteration of fistula flow. Postoperatively, the patient’s pulsatile tinnitus disappeared, and she was discharged without medication. There are a few possible mechanisms of spontaneous MMAVFs, such as middle meningeal artery aneurysm and trivial head trauma. Trans-arterial embolization with coils appears to be an effective and safe treatment option for complete obliteration of fistula flow.

Background: Pregnant women are particularly vulnerable to oral diseases due to hormonal and behavioral changes, yet disparitiesin healthcare resources and limited access to structured oral health education persist. Plaque visualization technologies may improve self-awareness and encourage behavioral changes. Although digital education media are increasingly utilized, comparative evidence on their effectiveness and acceptability across modalities remains limited. This study aimed to compare the perceived effectiveness and preferences for plaque visualization-based digital oral health education media among pregnant women in Vietnam. Methods: In total, 497 pregnant women were recruited from obstetric and maternal health facilities in Vietnam. Participants useda mobile application with artificial intelligence-based dental plaque visualization and feedback once and viewed pregnancytailored infographics and video educational materials once. After exposure, they completed a structured post-intervention questionnaire assessing sociodemographic characteristics, self-reported periodontal symptoms, oral health behaviors, perceived effectiveness, satisfaction with each medium, and media preferences. Descriptive statistics were calculated, and t-tests were used to compare evaluation scores across media and preferences according to participant characteristics. Results: Overall, 85.1% of participants reported gingival problems during pregnancy. Media preference was highest for video-basededucation, followed by mobile applications and infographics. However, mobile applications received the highest scores for educational effectiveness. Internal consistency was acceptable to high across media (video α=0.81, infographic α=0.84, mobile application α=0.91), with the mobile application demonstrating the highest reliability. Preference for mobile applications was significantly higher among women aged 28∼34 years and those employed in the service and tourism sectors. Conclusion Perceived educational values and preferences varied by digital oral health education medium. Although video-basededucation was preferred for accessibility and initial understanding, plaque visualization-based mobile application education received higher ratings for supporting self-management and feedback. These findings suggest that a multimedia strategy integrating visual feedback technology with educational content may be effective for oral health promotion among pregnant women.

Background: The rarity and complexity of trans-ulnar basal coronoid fracture-dislocations pose significant challenges in treatment. This study aimed to categorize these fractures based on coronoid fracture patterns and propose tailored surgical approaches for each type. Additionally, we evaluated the functional and radiological outcomes among the patients managed using our treatment algorithm. Methods: A total of 19 patients who underwent open reduction and internal fixation for trans-ulnar basal coronoid fracture-dislocations between March 2018 and October 2022 were enrolled in this study. These patients were classified based on the coronoid fracture patterns associated with olecranon fractures: type 1 involved anteromedial facet (AMF) fractures, type 2 encompassed coronoid base and body fractures, and type 3 involved a combination of types 1 and 2. We made a midline longitudinal dorsal incision to facilitate the provisional fixation of the olecranon fragment to the distal metaphysis using a locking plate. Subsequently, we employed the over-the-top (type 1) and Taylor–Scham (type 3) approaches for direct coronoid process fixation with buttress plating.Type 2 fractures were approached via medial fascial exposure from the posterior ulnar cortex or through the olecranon fractures, and subsequently fixed with miniplates and screws. Bony union and joint articulation were assessed via plain radiographs, and functional outcomes were evaluated using range of motion and the Mayo Elbow Performance Score. Results: Among the 19 patients, 3 had type 1 fractures, 14 had type 2 fractures, and 2 had type 3 fractures. All fractures exhibited solid osseous union without subluxation or dislocation. The average flexion and extension arc was 119.47° ± 20.88°, with a mean flexion of 127.37° ± 13.37° and an average flexion contracture of 7.89° ± 10.04°. The average Mayo Elbow Performance Score was 82.63 ± 12.51 points. Qualitatively, patient outcomes were excellent in 5 patients, good in 9, and fair in 5. Conclusions Most of our patients presented with easily approachable coronoid base and body fractures. However, in AMF fractures of the coronoid process, a direct medial approach is required for buttress plating. We believe our study helps provide useful guidelines for making appropriate decisions in trans-ulnar basal coronoid fracture-dislocations .

Background: The treatment of Kümmell disease (KD) is controversial. Corpectomy and reconstruction or osteotomy with long-level fusion was traditionally performed for the advanced KD. However, these procedures can be disadvantageous for elderly patients.Several alternative surgical procedures including transpedicular intravertebral cage augmentation (TPICA) or vertebroplasty (VP) combined with short-segment fixation (SSF) have been suggested to minimize the surgical burden. This study aimed to compare the outcomes of percutaneous TPICA plus SSF with VP plus SSF for advanced thoracolumbar (T11–L2) KD and to introduce our novel percutaneous TPICA technique using specially designed cannulated cage trials. Methods: We devised specially designed cannulated cage trials to make the TPICA procedure safer and more reproducible, minimizing the risk of the pedicle medial wall violation. All consecutive patients who underwent percutaneous TPICA or VP combined with SSF for advanced thoracolumbar KD, from January 2021 to June 2022, with ≥ 1-year follow-up at a single institution, were included. Perioperative details, clinical outcomes (visual analog scale and Oswestry Disability Index), and radiological outcomes (anterior vertebral body compression percentage and vertebral kyphotic angle [VKA] of the fractured vertebra, and local Cobb angle [LCA]) were collected and compared between the groups. Results: A total of 42 patients were enrolled, with 21 patients in each group. There were no patients with pedicle medial wall fracture in the TPICA group. Both procedures provided significantly favorable radiological outcomes compared to those preoperatively. No significant differences were observed in the changes over time in all radiological parameters between the groups. Loss of correction during the follow-up period was significantly smaller in patients with TPICA than in those with VP in VKA (median [interquartile range], 2.15 [0.30–2.80] vs. 2.90 [0.90–6.53]; p = 0.030) and LCA (2.70 ± 2.90 vs. 5.17 ± 4.40, p = 0.037). Conclusions Both procedures are minimally invasive and useful options for advanced KD, especially for elderly patients with high comorbidity. Our novel percutaneous TPICA technique using cannulated cage trials, being safer and more reproducible, may allow spine surgeons to easily perform TPICA.

Background: Active surveillance (AS) has emerged as a viable management strategy for low-risk papillary thyroid microcarcinoma (PTMC), following pioneering trials at Kuma Hospital and the Cancer Institute Hospital in Japan. Numerous prospective cohort studies have since validated AS as a management option for low-risk PTMC, leading to its inclusion in thyroid cancer guidelines across various countries. From 2016 to 2020, the Multicenter Prospective Cohort Study of Active Surveillance on Papillary Thyroid Microcarcinoma (MAeSTro) enrolled 1,177 patients, providing comprehensive data on PTMC progression, sonographic predictors of progression, quality of life, surgical outcomes, and cost-effectiveness when comparing AS to immediate surgery. The second phase of MAeSTro (MAeSTro-EXP) expands AS to low-risk papillary thyroid carcinoma (PTC) tumors larger than 1 cm, driven by the hypothesis that overall risk assessment outweighs absolute tumor size in surgical decision-making. Methods: This protocol aims to address whether limiting AS to tumors smaller than 1 cm may result in unnecessary surgeries for low-risk PTCs detected during their rapid initial growth phase. By expanding the AS criteria to include tumors up to 1.5 cm, while simultaneously refining and standardizing the criteria for risk assessment and disease progression, we aim to minimize overtreatment and maintain rigorous monitoring to improve patient outcomes. Conclusion This study will contribute to optimizing AS guidelines and enhance our understanding of the natural course and appropriate management of low-risk PTCs. Additionally, MAeSTro-EXP involves a multinational collaboration between South Korea and Australia. This cross-country study aims to identify cultural and racial differences in the management of low-risk PTC, thereby enriching the global understanding of AS practices and their applicability across diverse populations.