Congenital Titinopathy: Comprehensive Characterization of the Most Severe End of the Disease Spectrum.

Unlabelled: Congenital titinopathy has recently emerged as one of the most common congenital muscle disorders.

Objective: To better understand the presentation and clinical needs of the under-characterized extreme end of the congenital titinopathy severity spectrum.

Methods: We comprehensively analyzed the clinical, imaging, pathology, autopsy, and genetic findings in 15 severely affected individuals from 11 families.

Organoid Ethical Typology: varieties of three-dimensional stem cell constructs and the many issues they raise in bioethics.

The advancement of and prospects for stem cell research raise a number of specific ethical issues. While navigating the ethical landscape of stem cell research is often challenging for biology researchers and biotechnology innovators, it is also difficult for the public and other persons of concern (from ethicists to policy-makers) to grasp the technicalities of a burgeoning field that develops in many directions. Organoids are one of these new biotechnological constructs that are currently eliciting a rich debate in bioethics.

International Validation of a Novel PEACE Scale to Improve the Quality of Upper Gastrointestinal Mucosal Inspection During Endoscopy.

Introduction: The performance of a high quality esophagogastroduodenoscopy (EGD) is dependent on the mucosal cleanliness. Recently, the Polprep: Effective Assessment of Cleanliness in EGD (PEACE) scale was created to assess the degree of mucosal cleanliness during EGD. The aim of this study was to validate this scoring system in a cohort of international endoscopists.

Defining Health Management: A Conceptual Foundation for Excellence Through Efficiency, Sustainability and Equity.

The practice of healthcare management is essential for the efficient operation of health services, encompassing leadership, management, and direction within healthcare organisations. 'Health management' extends beyond healthcare management by integrating principles of public health and health policy. As health management is commonly practised but not cohesively recognised, the European Health Management Association (EHMA) conducted this study to develop a cohesive definition of health management.

Identification of Cell Fate Determining Transcription Factors for Generating Brain Endothelial Cells.

Reliable models of the blood-brain barrier (BBB), wherein brain microvascular endothelial cells (BMECs) play a key role in maintenance of barrier function, are essential tools for developing therapeutics and disease modeling. Recent studies explored generating BMEC-like cells from human pluripotent stem cells (hPSCs) by mimicking brain-microenvironment signals or genetic reprogramming. However, due to the lack of comprehensive transcriptional studies, the exact cellular identity of most of these cells remains poorly defined.

Surgeon attitudes toward risk stratification in emergency surgery for the elderly: an ESTES cross-sectional survey.

Purpose: Our study explores the utilization of objective tools for preoperative assessment of elderly patients by Emergency General Surgeons (EGS).

Methods: A descriptive cross-sectional survey was conducted via the European Society for Trauma and Emergency Surgery (ESTES) Research Committee. EGS were invited through the ESTES members' mailing list and social media platforms.

Different PD-L1 Assays Reveal Distinct Immunobiology and Clinical Outcomes in Urothelial Cancer.

Testing for PD-L1 expression by immunohistochemistry (IHC) is used to predict immune checkpoint blockade (ICB) benefit but has performed inconsistently in urothelial cancer (UC) clinical trials. Different approaches are used for PD-L1 IHC. We analyzed paired PD-L1 IHC data on UC samples using the SP142 and 22C3 assays from the phase 3 IMvigor130 trial and found discordant findings summarized by four phenotypes: PD-L1 positive by both assays (PD-L1 double positive; PD-L1DP), PD-L1 positive by the SP142 assay only (SP142 single positive; SP142SP), PD-L1 positive by the 22C3 assay only (22C3 single positive; 22C3SP), and PD-L1 negative by both assays double negative (PD-L1 double negative; PD-L1DN).

Patient-derived xenograft models of gastrointestinal stromal tumors: a ready-to-use platform for translational research.

Gastrointestinal stromal tumors (GIST) are the most common mesenchymal malignancy of the gastrointestinal tract. Most GIST harbor mutations in oncogenes, such as KIT, and are treated with tyrosine kinase inhibitors (TKI), such as imatinib. Most tumors develop secondary mutations inducing drug resistance against the available TKI, which requires novel therapies.

The stress-protectant molecule trehalose mediates fluconazole tolerance in .

The incidence of non- infections has witnessed a substantial rise in recent decades. ), an opportunistic human fungal pathogen, is accountable for both superficial mucosal and life-threatening bloodstream infections, particularly in immunocompromised individuals. Distinguished by its remarkable resilience to environmental stressors, exhibits intrinsic tolerance to azoles and a high propensity to swiftly develop azole resistance during treatment.

Distinct effects of glucocorticoid on pseudorabies virus infection in neuron-like and epithelial cells.

Pseudorabies virus (PRV) is a porcine neurotropic alphaherpesvirus that infects peripheral tissues of its host, spreads into the nervous system, and establishes a life-long latency in neuronal cells. During productive infection, PRV replicates rapidly and causes pseudorabies or Aujeszky's disease. Reactivation from latent infection in the nervous system may lead to anterograde axonal transport of progeny virions, leading to recurrent infection of the epithelial layer and virus spread.

Considerations and Challenges for Acute Inhalation Toxicity Testing and Classification of Zinc Sulphide Under REACH.

Zinc sulphide is a widely used inorganic powder, and its production has reached quantities greater than 1000 t/year. Therefore, in accordance with OECD guideline 436, an acute inhalation test was implemented to provide more accurate data. This study is crucial for ensuring the safety of workers exposed to zinc sulphide dust and complying with regulatory requirements for REACH.

Diversity of Mycotoxins in Stored Paddy Rice: Contamination Patterns in the Mekong Delta, Vietnam.

Rice ( L.) is the most important food in Vietnam. However, rice is often lost in post-harvest due to fungal growth and mycotoxins contamination.

A Systematic Review of Medical Treatments for Benign Prostatic Hyperplasia in Dogs: Evaluating Strategies for Reproductive Function Preservation.

Benign prostatic hyperplasia (BPH) is a common condition in intact male dogs and a leading cause of subfertility. While surgical and chemical castration are effective treatments for BPH, managing this condition in breeding dogs is challenging due to the desire to preserve reproductive function. This study systematically reviews medical treatments for BPH that maintain breeding potential.

Persistence of the Immune Response to an Intramuscular Bivalent (GI.1/GII.4) Norovirus Vaccine in Adults.

Background: Major global economic and health burdens due to norovirus gastroenteritis could be addressed by an effective vaccine.

Methods: In this study, 428 adult recipients of various compositions of the norovirus vaccine candidate, HIL-214, were followed for 5 years, to assess immune responses to its virus-like particle antigens, GI.1 and GII.

Neethling Strain-Based Homologous Live Attenuated LSDV Vaccines Provide Protection Against Infection with a Clade 2.5 Recombinant LSDV Strain.

Background: Vaccination is the main control measure to prevent Lumpy skin disease (LSD), and Neethling-based homologous vaccines have been shown to be safe and effective against infection with classical clade 1.2 strains. In 2017, recombinant clade 2 LSDV strains originating from a badly produced and insufficiently controlled vaccine were first detected in Russia.

Recombinant Anti-PF4 Antibodies Derived from Patients with Vaccine-Induced Immune Thrombocytopenia and Thrombosis (VITT) Facilitate Research and Laboratory Diagnosis of VITT.

Background/objectives: Adenoviral vector-based vaccines against COVID-19 rarely cause vaccine-induced immune thrombocytopenia and thrombosis (VITT), a severe adverse reaction caused by IgG antibodies against platelet factor 4 (PF4). To study VITT, patient samples are crucial but have become a scarce resource. Recombinant antibodies (rAbs) derived from VITT patient characteristic amino acid sequences of anti-PF4 IgG are an alternative to study VITT pathophysiology.

Prenatal exposure to chemotherapy increases the mutation burden in human neonatal hematopoietic stem cells.

Chemotherapy is included in the standard of care for cancer treatment during pregnancy. However, whether prenatal exposure to maternal chemotherapy treatment has a mutagenic impact on the fetal genome, remains unexplored. Therefore, we investigated mutation accumulation in hematopoietic stem and progenitor cells (HSPCs) from neonates born to pregnant cancer patients treated with chemotherapy, as well as healthy pregnant women and untreated pregnant cancer patients.

Wear Resistance of Light-Cure Resin Luting Cements for Ceramic Veneers.

The purpose of this study was to compare the wear resistance of light-cure resin luting cements for veneers with that of other luting materials investigated in earlier studies. An Alabama wear-testing machine was used to measure the wear resistance of four recent light-cure resin luting cements for veneers (G-Cem Veneer; Panavia V5 LC; RelyX Veneer Cement; and Vario-link Esthetic LC). The volume loss ranged from 0.

Exploring the Structural Diversity and Biotechnological Potential of the Rhodophyte Phycolectome.

Lectins are non-covalent glycan-binding proteins found in all living organisms, binding specifically to carbohydrates through glycan-binding domains. Lectins have various biological functions, including cell signaling, molecular recognition, and innate immune responses, which play multiple roles in the physiological and developmental processes of organisms. Moreover, their diversity enables biotechnological exploration as biomarkers, biosensors, drug-delivery platforms, and lead molecules for anticancer, antidiabetic, and antimicrobial drugs.

Comparison of In Vitro Biotransformation of Olive Polyphenols Between Healthy Young and Elderly.

Background: Olive leaves are a rich source of polyphenols, predominantly secoiridoids, flavonoids, and simple phenols, which exhibit various biological properties. Extracts prepared from olive leaves are associated with hypoglycemic, hypotensive, diuretic, and antiseptic properties. Upon ingestion, a substantial fraction of these polyphenols reaches the colon where they undergo extensive metabolism by the gut microbiota.

Musical Pitch Perception and Categorization in Listeners with No Musical Training Experience: Insights from Mandarin-Speaking Non-Musicians.

Pitch is a fundamental element in music. While most previous studies on musical pitch have focused on musicians, our understanding of musical pitch perception in non-musicians is still limited. This study aimed to explore how Mandarin-speaking listeners who did not receive musical training perceive and categorize musical pitch.

Growth Propagation of Liquid Spawn on Non-Woven Hemp Mats to Inform Digital Biofabrication of Mycelium-Based Composites.

Mycelium-based composites (MBCs) are highly valued for their ability to transform low-value organic materials into sustainable building materials, offering significant potential for decarbonizing the construction sector. The properties of MBCs are influenced by factors such as the mycelium species, substrate materials, fabrication growth parameters, and post-processing. Traditional fabrication methods involve combining grain spawn with loose substrates in a mold to achieve specific single functional properties, such as strength, acoustic absorption, or thermal insulation.

Environmental Performance, Financial Constraints, and Tax Avoidance Practices: Insights from FTSE All-Share Companies.

Through its initiative known as the Climate Change Act (2008), the Government of the United Kingdom encourages corporations to enhance their environmental performance with the significant aim of reducing targeted greenhouse gas emissions by the year 2050. Previous research has predominantly assessed this encouragement favourably, suggesting that improved environmental performance bolsters governmental efforts to protect the environment and fosters commendable corporate governance practices among companies. Studies indicate that organisations exhibiting strong corporate social responsibility (CSR), environmental, social, and governance (ESG) criteria, or high levels of environmental performance often engage in lower occurrences of tax avoidance.

Is Word Order Responsive to Morphology? Disentangling Cause and Effect in Morphosyntactic Change in Five Western European Languages.

This study examines the relationship between morphological complexity and word order rigidity, addressing a gap in the literature regarding causality in linguistic changes. While prior research suggests that the loss of inflectional morphology correlates with the adoption of fixed word order, this study shifts the focus from correlation to causation. By employing Kolmogorov complexity as a measure of linguistic complexity alongside Granger Causality to examine causal relationships, we analyzed data from Germanic and Romance languages over time.

ADAMTS13 Improves Endothelial Function and Reduces Inflammation in Diabetic Retinopathy.

The protease, a disintegrin and metalloproteinase with thrombospondin type 1 motif member 13 (ADAMTS13), known to cleave only the von Willebrand factor (VWF), has powerful regulatory effects on microvascular platelet adhesion, thrombosis, inflammation, and endothelial dysfunction. We study the protection against diabetes-induced retinal injury in experimental rats by supplementation with recombinant ADAMTS13. We compare human epiretinal membranes and vitreous samples from nondiabetic subjects and patients with proliferative diabetic retinopathy (PDR) and extend in vitro analyses with the use of various immunodetection and spectrofluorimetric methods on rat retina and human retinal glial and endothelial cell cultures.