Common variable immunodeficiency (CVID) is a heterogeneous primary immunodeficiency characterized by reduced levels of immunoglobulin (Ig)G, with or without IgA and/or IgM deficiency, and hypogammaglobulinemia. Clinical manifestations are diverse, ranging from recurrent infections to autoimmune and allergic diseases. While CVID has been rarely reported in the Korean population, particularly in adults, we report 2 adult cases of CVID comorbid with asthma and Behcet’s disease. The first case of a 53-year-old with severe allergic asthma and chronic rhinosinusitis experienced recurrent respiratory infections, stomatitis, and cystitis requiring frequent antibiotic treatment. Laboratory findings indicated a T2-high asthma phenotype, with elevated serum total IgE specific IgE to dog hair and fractional exhaled nitric oxide. Immunological evaluation revealed decreased serum IgG (including IgG1 and IgG2), along with hypogammaglobulinemia. She had been treated with regular anti-IgE antibody therapy and intravenous immunoglobulin replacement therapy (IVIGRT). The second case of a 38-year-old with Behçet’s disease and uveitis had bronchial asthma and rhinitis that were exacerbated by recurrent infections despite standard asthma therapy. Laboratory findings revealed a T2-low phenotype and a marked reduction in serum IgG (including IgG1, IgG2, and IgG4), and hypogammaglobulinemia, consistent with CVID.IVIGRT effectively reduced asthma exacerbations and infection episodes in both cases. These cases highlight the clinical heterogeneity of CVID and its potential overlap with allergic and autoimmune diseases. Immunological evaluation of underlying immunodeficiency should be considered in adult patients with asthma who present with frequent exacerbations and recurrent infections. Early diagnosis and IVIGRT can prevent complications and improve outcomes.

Purpose: To compare the anatomical and clinical outcomes based on the distance from the corneal limbus to the scleral tunnel in flanged intrascleral intraocular lens (IOL) fixation. Methods: We retrospectively analyzed the medical records of patients who underwent scleral fixation of flanged IOLs. Group 1 (54 eyes) had a distance of 2.1 mm from the corneal limbus to the scleral tunnel, and Group 2 (48 eyes) had a distance of 2.8 mm. We evaluated the best corrected visual acuity (BCVA), postoperative complications, IOL tilt and decentration, refractive prediction error (RPE), effective lens position, and iris-IOL distance. Results: The BCVA, postoperative complications, IOL tilt, and IOL decentration did not differ between the two groups (p > 0.05). The RPE showed a hyperopic shift in Group 1 and a myopic shift in Group 2 (Group 1: +0.24 ± 0.68 D, Group 2: -0.03 ± 0.43 D, p = 0.03). The iris-IOL distance was statistically longer in Group 1 compared to Group 2 (Group 1: 1.02 ± 0.40 mm, Group 2: 0.57 ± 0.32 mm, p = 0.02). The incidence of pupillary optic capture was significantly higher in Group 2 compared to Group 1 (Group 1; 0%, Group 2; 8.3%, p = 0.03). Conclusions It should be considered that a shorter distance from the corneal limbus to the scleral tunnel results in a postoperative hyperopic shift and reduces the incidence of pupillary optic capture when performing flanged intrascleral IOLs fixation.

The recent approval of trastuzumab deruxtecan for human epidermal growth factor receptor 2 (HER2)–low and HER2-ultralow breast cancer mandates an adequate assessment of these categories. Methods: Seven breast pathologists from the Breast Pathology Study Group of the Korean Society of Pathologists held an on-site expert consensus meeting. Fifteen sets of virtual whole slide images (WSI) of hematoxylin and eosin stain and HER2 immunohistochemistry were provided. The pathologists were given 60 minutes to submit their diagnosis of HER2 expression into null, ultralow, 1+, 2+, or 3+. Afterwards, in-depth discussion and consensus diagnoses were made by real-time visualization of the WSI. Results: After the consensus meeting, unanimous 100% agreements were seen only in five (33.3%) of the examined cases, which consisted of three 1+ cases and two 2+ cases. Two cases (13.3%) had mild disagreement, with only one pathologist’s disagreement. Of note, eight cases (53.3%) showed significant disagreement, defined by more than two pathologists’ disagreement. All HER2-null cases were reclassified as ultralow after consensus review, suggesting potential widespread underclassification of ultralow cases in clinical practice. Conclusions: Experts had significant discrepancies in interpreting HER2-low/ultralow status. It is important to assess if the distinction between HER2-low and ultralow is strictly required and if HER2-null breast cancer exists in reality.

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: and Purpose To compare the epidemiological and clinical features of the rare patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) with age- and sex-matched controls in a nationwide cohort from South Korea. Methods: This observational cohort study analyzed newly diagnosed CADASIL patients aged at least 20 years and matched controls using data from the National Health Information Database for 2004–2022. The cumulative incidence of CADASIL was assessed by age and sex, and compared between regions. Neurologic and systemic diseases were compared between the CADASIL and control groups. Results: The study analyzed 816 CADASIL patients and 816 age- and sex-matched controls aged 56.8±15.2 years (mean±standard deviation), among whom 48.3% were male. The cumulative incidence of CADASIL was 1.86 per 100,000 people (95% confidence interval [CI]=1.85– 1.87 per 100,000), and peaked at 60–69 years of age. In terms of regional distribution, the incidence was highest for Jeju, at 39.67 per 100,000 (95% CI 37.84–41.49 per 100,000). Neurologic diseases were more frequent in CADASIL patients, including Alzheimer’s disease (33.1% vs.20.0%), vascular dementia (84.9% vs. 5.0%), epilepsy (34.6% vs. 15.9%), stroke (70.7% vs. 27.6%), parkinsonism (18.9% vs. 11.0%), and depression (60.8% vs. 44.9%). Systemic diseases such as diabetes mellitus (78.9% vs. 68.9%) were also more common in CADASIL patients, while cancer (27.9% vs. 38.7%) and myocardial infarction (10.0% vs. 13.6%) were less common than in controls. The onset ages of all diseases were lower in CADASIL patients. Conclusions This study has provided a precise nationwide estimate of the CADASIL incidence and its regional distribution in South Korea. CADASIL patients showed higher incidence rates and earlier onsets of diverse clinical manifestations.

This study compared key health indicators of poor, older urban adults attending a free clinic with those of the general older population, using data from the 2023 Korea National Health and Nutrition Examination Survey (KNHANES). Methods: This cross-sectional comparative study included 60 adults aged ≥60 years who attended the Raphael Nanum Homeless Clinic in Seoul. Participants completed a questionnaire, underwent anthropometric assessment, and provided fasting blood samples for measurement of total cholesterol, low density lipoprotein (LDL)-cholesterol, and triglycerides (TG). Obesity, current smoking, monthly alcohol use, poor self-rated health, and strength exercise (≥ 2 days/week) were defined according to 2023 KNHANES criteria and compared with age-matched 2023 KNHANES estimates for adults aged ≥ 60 years using independent t-tests and two-proportion z-tests. Results: Participants were predominantly men (80.0%) with a mean age of 79.9 years; 70.0% reported no regular income, and 46.7% rated their health as poor. Compared with their 2023 KNHANES counterparts, the clinic group had a higher prevalence of obesity (50.0% vs. 35.0%), particularly among men, and a more atherogenic lipid profile characterized by higher LDL-cholesterol despite similar total cholesterol levels and lower TG. The prevalence of current smoking (3.3% vs. 10.6%) and monthly alcohol use (31.7% vs. 53.0%) was significantly lower, whereas participation in strength exercise was low in both groups, with no significant differences observed. Conclusion: Poor, older urban adults exhibited multidimensional health disparities, including obesity, adverse lipid profiles, and markedly poorer self-rated health, despite lower levels of smoking and alcohol consumption. Community-based interventions targeting nutrition, physical activity, and chronic disease management are needed to reduce health inequalities in this vulnerable population.

Background/Aims: Biliary tract cancer (BTC) is a rare malignancy with poor prognosis. We investigated genomic determinants of clinical benefit from gemcitabine, cisplatin, and nab-paclitaxel (GAP) versus gemcitabine and cisplatin (GC) in advanced BTC. Methods: Clinical and genomic data using TruSight Oncology 500 were analyzed from patients treated with GAP (N=198) or GC (N=89) as first-line therapy. Results: With a median follow-up of 33.0 months, GAP modestly improved progression-free survival (PFS) (hazard ratio [HR] 0.764; 95% confidence interval [CI] 0.591–0.989) without significant overall survival (OS) difference compared to GC. Genomic profiling revealed frequent alterations in TP53 (35.2%), KRAS (16.4%), SMAD4 (10.5%), and TNFRSF14 (10.5%), involving RTK/RAS (44.3%), TP53 (41.8%), and PI3K (20.2%) pathways. Single-gene mutations did not predict treatment benefit. However, pathway-level analysis identified PI3K pathway activation as significantly associated with inferior PFS (HR 2.148; 95% CI 1.478–3.124) and OS (HR 2.096; 95% CI 1.413–3.109) in patients receiving GAP, an effect not observed with GC. Importantly, GAP conferred clinical benefit only in patients without PI3K pathway activation, while no survival advantage was seen in those with such alterations (Pinteraction=0.023 for PFS, Pinteraction=0.003 for OS). Similar results were obtained in the independent validation cohort treated with GAP (N=103) or GC (N=64) for BTC. Conclusions Genomic profiling using next-generation sequencing identified PI3K pathway activation as key molecular determinant that differentiates patient outcomes between GAP and GC treatments in advanced BTC.

Purpose: This study aimed to analyze changes in plantar pressure distribution at the fifth metatarsal (M5) during running with different foot strike patterns. Methods: Thirty-five healthy adults ran barefoot over a foot scan pressure plate installed in a 10-m shuttle walkway.Each participant completed 10 trials per strike pattern and mean peak pressures at M1–M5 heads and M5 base of the dominant foot was analyzed. Results: The mean peak pressures at M2– M5 heads increased progressively from heel to midfoot to forefoot strike (p＜ 0.05). Significant differences among strike patterns were found at M2–M5 heads (p＜ 0.05). Post hoc analysis showed that forefoot strike produced significantly higher pressures at M2–M5 heads than heel and midfoot strikes (p＜ 0.001). At M5, peak pressure at the head increased from heel (14.54±7.82) to midfoot (15.54±7.75) to forefoot strike (19.37±7.37), whereas peak pressure at the base decreased from heel (6.27±1.69) and midfoot strikes (5.85±1.94) to forefoot strike (4.35±1.79). At M5, both the head and base demonstrated significant differences according to strike pattern. Conclusion Plantar pressure patterns vary across foot strike types, with forefoot strike producing greater M5 head loading but lower base loading. These findings suggest that forefoot-based low-intensity running may be considered as an early loading option during rehabilitation after Jones fractures, although further clinical studies are needed to confirm its clinical benefits.

Background: Baricitinib is one of the front-runners among targeted agents for the treatment of atopic dermatitis (AD). Although many studies have been conducted on the real-world use of baricitinib, the sample size is often small and data is focused primarily on Caucasians. Objective: The objective of this study was to demonstrate the real-world itch-relieving property of baricitinib in adult AD patients in South Korea. Methods: Electronic medical records of AD patients treated with baricitinib at the National Medical Center in Korea from May 2021 to April 2023 were analyzed retrospectively. Results: Seventy patients completed 16 to 52 weeks of baricitinib treatment, with most patients showing mild-to-moderate baseline lesions and moderate-to-severe baseline itch. At Week 16 of baricitinib treatment, there was a 50% reduction in Itch numerical rating scale from baseline, and 50.7% of patients showed 50% improvement in Eczema Area and Severity Index score.The efficacy of baricitinib was also reflected in the patient reported outcomes, with 55%–58% improvements in Patient-Oriented Eczema Measure, Atopic Dermatitis Control Tool, and Dermatology Life Quality Index scores seen within 2 weeks of treatment. No new safety signals were detected in this study. Conclusion Baricitinib treatment for 52 weeks in Korean patients with itch dominant AD confirmed long term effectiveness and safety.