Recurrent Hemorrhagic Stroke and Microcephaly in a Newborn with Aicardi-Goutières Syndrome Caused by a Homozygous Intronic Variant.

Aicardi-Goutières syndrome (AGS) is a progressive multisystem disorder marked by early-onset encephalopathy. This report investigates the genetic basis of AGS in a newborn from consanguineous parents with microcephaly, recurrent hemorrhagic strokes, brain calcifications, leukodystrophy, epilepsy, anemia, thrombocytopenia, and left ventricular hypertrophy. Next-generation sequencing-based targeted gene panel testing for epilepsy variant in the gene.

Surface and Functional Properties of Biosurfactant Produced by HH2 Derived from .

Surfactants play a critical role in diverse industrial applications. However, concerns over the environmental persistence and toxicity of synthetic variants have prompted a shift toward sustainable biosurfactants. In this study, the surface properties of a biosurfactant produced by HH2 isolated from , a traditional Korean fermented seafood, were investigated to evaluate its industrial applicability.

Optimizing accuracy: a comparative analysis of preoperative liver volumetry in living donor liver transplantation from a surgeon's perspective - a retrospective cohort study.

Background: Accurate preoperative graft volume assessment is fundamental to the success of living donor liver transplantation (LDLT). Although manual and automated computed tomography (CT) volume measurement methods using various volumetric tools are widely used, their accuracy remains uncertain. This study aimed to compare various CT-based volumetric measurement methods for predicting actual graft weight (AGW) in LDLT and to identify specific dry weight correction factors for each method to improve clinical reliability.

Rapid-Turnaround Co-Administration of mRNA-Based MHC-I and MHC-II-Restricted Neoantigens Enhances Immune Responses of Antigen-Specific CD8 T Cells and Anti-Cancer Efficacy in Colorectal Cancer.

Personalized cancer vaccines (PCVs) represent a promising frontier in cancer immunotherapy; however, challenges in neoantigen prediction and treatment optimization persist. This study aims to introduce an innovative mRNA-based PCV platform that addresses these limitations. Co-administration of our major histocompatibility complex (MHC)-I and MHC-II-restricted neoantigens increases antigen-specific T cell responses and exhibits strong anti-cancer efficacy, significantly inducing antigen-specific CD8 T cell-immune responses.

Real-world safety and effectiveness of rurioctocog alfa pegol in 338 patients with hemophilia A in South Korea: A postmarketing surveillance study.

Introduction: Rurioctocog alfa pegol is a recombinant coagulation factor VIII (FVIII) with an extended half-life versus the parent product. Limited real-world data exist on the treatment of hemophilia A with rurioctocog alfa pegol in South Korea. This postmarketing surveillance study assessed its safety and effectiveness in a real-world setting, according to Korean regulatory requirements.

Characteristics and Treatment Patterns of Patients with Haemophilia B Receiving Recombinant Coagulation Factor IX.

In Haemophilia B, guideline-level factor IX (FIX) prophylaxis is recommended, but real-world dosing and adherence vary. To assess treatment patterns, adherence, FIX dosing, and their associations with bleeding events in Korean patients. We conducted a retrospective chart review and one-time survey of 130 Korean patients with haemophilia B treated with FIX for ≥12 months at 12 centers (June 2022-May 2023).

The Impact of -Associated Junction Plakoglobin on Respiratory Syncytial Virus Infection.

Respiratory syncytial virus (RSV) is a leading cause of lower respiratory tract infections in infants, young children, and immunocompromised individuals. Currently, FDA-approved monoclonal antibody therapies are limited to infants and young children with severe RSV disease. As a result, there is an urgent need for comprehensive studies of RSV pathogenesis to support the development of new therapeutic strategies.

The therapeutic potential of mRNA-encoded SFTSV human monoclonal antibody encapsulated lipid nanoparticle in vivo.

Severe fever with thrombocytopenia syndrome (SFTS), caused by the SFTS virus (SFTSV), has emerged as a significant public health concern in East Asia since 2009. The high mortality rate of SFTS underscores the urgent need for effective preventive and therapeutic interventions. Although a Gn-specific human monoclonal antibody, Ab10, herein referred to as the protein S/A-TEN, has been previously reported, its development has been hindered by the economic challenges and low yields of large-scale production.

CT-P17 Adalimumab Biosimilar in Patients with Moderate-to-Severe Chronic Plaque Psoriasis: An Open-Label Extension of a Phase 3 Interchangeability Study.

Introduction: A 27-week analysis of this phase 3 study demonstrated the interchangeability of the adalimumab biosimilar CT-P17 and reference adalimumab. The current 52-week analysis reports secondary data from the open-label extension period (OLE) of the study.

Methods: In this randomized, double-blind, active-controlled phase 3 study, adults with moderate-to-severe chronic plaque psoriasis received (via prefilled syringe) 80 mg subcutaneous reference adalimumab on day 1, then 40 mg 1 week later, and every other week (EOW) until week 11.

Repeated Switching Between CT-P17 and EU Reference Adalimumab in Patients with Moderate-to-Severe Chronic Plaque Psoriasis: A Randomized, Double-Blind, Active-Controlled, Phase 3, Interchangeability Study.

Introduction: This study aimed to demonstrate the interchangeability of biosimilar CT-P17 and European Union reference adalimumab (EU-adalimumab) in a repeated-switch scenario.

Methods: In this ongoing, randomized, double-blind, active-controlled, phase 3 study, adults with moderate-to-severe plaque psoriasis received 80 mg EU-adalimumab on day 1, then 40 mg 1 week later and every other week until week 11. At week 13, patients were randomized (1:1, via an interactive web response system) to continue EU-adalimumab ("continuous" group) or undergo repeated switches between CT-P17 and EU-adalimumab ("switching" group).

Protective Effects of Peanut Sprouts from a Smart Farming System on the Barrier Function of Human Epithelial Cells.

Inflammatory bowel disease, including Crohn's disease and ulcerative colitis, poses an emerging threat as it can lead to colorectal cancer, thrombosis, and other chronic conditions. The present study demonstrated the protective effects of peanut sprout extracts (PSEs) prepared from day 2 to day 7 of germination against lipopolysaccharide (LPS)-induced epithelial barrier breakdown. Although the peanut sprout length increased in a time-dependent manner from day 1 to day 7, the extraction yields remained relatively consistent from day 2 to day 7.

Pellino-1, a therapeutic target for control of SARS-CoV-2 infection and disease severity.

Enhanced expression of Pellino-1 (Peli1), a ubiquitin ligase is known to be associated with COVID-19 susceptibility. The underlying mechanisms are not known. Here, we report that mice deficient in Peli1 (Peli1) had reduced viral load and attenuated inflammatory immune responses and tissue damage in the lung following SARS-CoV-2 infection.

Carbon dioxide removal (CDR) potential in temperate macroalgal forests: A comparative study of chemical and biological net ecosystem production (NEP).

The carbon dioxide removal (CDR) capacity of macroalgae, a crucial component in climate regulation, has gained increasing attention. However, accurately estimating the CDR potential of macroalgae in natural conditions remains challenging, necessitating the use of multiple independent methods to reduce the uncertainties in these estimates. In this study, we compared two methods for estimating net ecosystem production (NEP), a key parameter in determining CDR potential: 1) NEP, derived from seawater carbonate chemistry and 2) NEP, based on photorespiratory measurements using benthic tent incubation.

Immunogenicity and protection of a triple repeat domain III mRNA vaccine against Zika virus.

Zika virus (ZIKV) infection is primarily transmitted by mosquitoes and often asymptomatic in most individuals. Infection during pregnancy can lead to severe birth defects such as congenital microcephaly, and currently, there is no approved vaccine for ZIKV. Several studies are investigating the development of ZIKV vaccine using DNA and RNA as well as recombinant protein technologies; however, the outcomes thus far have not been consistently noteworthy.

Role of Poly(A)-Binding Protein Cytoplasmic 1, a tRNA-Derived RNA Fragment-Bound Protein, in Respiratory Syncytial Virus Infection.

Respiratory Syncytial Virus (RSV) is a significant cause of lower respiratory tract infections (LRTI) across all demographics, with increasing mortality and morbidity among high-risk groups such as infants under two years old, the elderly, and immunocompromised individuals. Although newly approved vaccines and treatments have substantially reduced RSV hospitalizations, accessibility remains limited, and response to treatment varies. This underscores the importance of comprehensive studies on host-RSV interactions.

Clinical data on treatment regimen and use of medication among patients with hemophilia B in Korea.

Background: To investigate the clinical treatment status, such as treatment regimen, bleeding events, and drug dose, in patients with hemophilia B in South Korea.

Methods: In this retrospective chart review, data of patients with hemophilia B from eight university hospitals were collected. Demographic and clinical data, treatment data, such as regimen and number of injections, dose of factor IX concentrate, and bleeding data were reviewed.

mRNA vaccine encoding Gn provides protection against severe fever with thrombocytopenia syndrome virus in mice.

We developed a promising mRNA vaccine against severe fever with thrombocytopenia syndrome (SFTS), an infectious disease caused by the SFTS virus that is primarily transmitted through tick bites. Administration of lipid nanoparticle-encapsulated mRNA-Gn successfully induced neutralizing antibodies and T-cell responses in mice. The vaccinated mice were protected against a lethal SFTS virus challenge, suggesting that this mRNA vaccine may be an effective and successful SFTS vaccine candidate.

Parent tRNA Modification Status Determines the Induction of Functional tRNA-Derived RNA by Respiratory Syncytial Virus Infection.

tRNA-derived RNA fragments (tRFs) are a recently discovered family of small noncoding RNAs (sncRNAs). We previously reported that respiratory syncytial virus (RSV) infection induces functional tRFs, which are derived from a limited subset of parent tRNAs, in airway epithelial cells. Such induction is also observed in nasopharyngeal wash samples from RSV patients and correlates to RSV genome copies, suggesting a clinical significance of tRFs in RSV infection.