PDSS1 mutations-associated steroid-resistant nephrotic syndrome: case report and review of literature.

PDSS1 mutations hamper Coenzyme Q10 biosynthesis and cause a rare multisystem mitochondrial disease characterized by diverse clinical features and limited treatment options. To date, renal involvement has been reported in only one patient. We report a new female patient with compound heterozygous PDSS1 mutations and the clinical outcome following a trial of Coenzyme Q10 therapy.

Data-Driven Framework for the Prediction of PEGDA Hydrogel Mechanics.

Poly(ethylene glycol) diacrylate (PEGDA) hydrogels are biocompatible and photo-cross-linkable, with accessible values of elastic modulus ranging from kPa to MPa, leading to their wide use in biomedical and soft material applications. However, PEGDA gels possess complex microstructures, limiting the use of standard polymer theories to describe them. As a result, we lack a foundational understanding of how to relate their composition, processing, and mechanical properties.

A pain research strategy for Europe: A European survey and position paper of the European Pain Federation EFIC.

Background: Pain is the leading cause of disability and reduced quality of life worldwide. Despite the increasing burden for patients and healthcare systems, pain research remains underfunded and under focused. Having stakeholders identify and prioritize areas that need urgent attention in the field will help focus funding topics, reduce 'research waste', improve the effectiveness of pain research and therapy and promote the uptake of research evidence.

Preventing Severe COVID-19 with Tixagevimab-Cilgavimab in Hematological Patients Treated with Anti-CD20 Monoclonal Antibodies: An International Multicenter Study.

Introduction: Despite the declining public health emergency status, COVID-19 still poses significant risks, especially for immunocompromised individuals. We aimed to evaluate the effectiveness of tixagevimab-cilgavimab (T-C) prophylaxis in preventing severe COVID-19 in patients with hematologic malignancies (HM) treated with anti-CD20 therapy during the early Omicron variant phase of the pandemic.

Methods: The European Society of Clinical Microbiology and Infectious Diseases Study Group for Respiratory Viruses (ESGREV) conducted a multicenter retrospective cohort study involving 15 centers from 5 countries.

An integral membrane constitutively active heparanase enhances the tumor infiltration capability of NK cells.

Eradication of cancer cells by the immune system requires extravasation, infiltration and progression of immune cells through the tumor extracellular matrix (ECM). These are also critical determinants for successful adoptive cell immunotherapy of solid tumors. Together with structural proteins, such as collagens and fibronectin, heparan sulfate (HS) proteoglycans are major components of the ECM.

Accelerated diradical character assessment in large datasets of polybenzenoid hydrocarbons using xTB fractional occupation.

Polybenzenoid hydrocarbons (PBHs) have garnered significant attention in the field of organic electronics due to their unique electronic properties. To facilitate the design and discovery of new functional organic materials based on these compounds, it is necessary to assess their diradical character. However, this usually requires expensive multireference calculations.

Harnessing antibody-mediated recognition of the intracellular proteome with T cell receptor-like specificity.

The clinical success of cancer immunotherapy has driven ongoing efforts to identify novel targets that can effectively guide potent effector functions to eliminate malignant cells. Traditionally, immunotherapies have focused on surface antigens; however, these represent only a small fraction of the cancer proteome, limiting their therapeutic potential. In contrast, the majority of proteins within the human proteome are intracellular, yet they are represented on the cell surface as short peptides presented by MHC class I molecules.

Development and application of a comprehensive numerical simulation model for microalgal pneumatic photobioreactors.

A novel and comprehensive multiphysics numerical simulation model for microalgal pneumatic photobioreactors was developed using the open-source Computational Fluid Dynamics (CFD) software OpenFOAM. This model integrates three distinct submodels: multiphase fluid dynamics, light transport, and microalgae growth. In particular, the light transport submodel accounts for light scattering caused by air bubbles.

The Impact of Metal-Support Interaction on the Structure and Activity of Carbon-Supported Ni Nanoparticle Catalysts for Alkaline Hydrogen Oxidation Reaction.

The effect of the carbon support─Ketjenblack (C) and Vulcan (C)─on the Ni/C catalyst properties toward alkaline hydrogen oxidation reaction (HOR) was investigated. Both Ni/C and Ni/C presented improved catalytic activity with an increase in Ni particle size; however, Ni/C showed activity significantly higher than that of Ni/C at the same particle size. The reason for the difference in the HOR activity due to the types of carbon support was revealed to be the difference in the 3D structure of Ni nanoparticles determined by metal-support interaction, which is changed by the surface structures of carbon supports.

Identifying factors associated with disparities in accessing an Integrative Oncology program.

Introduction: Integrative oncology (IO) programs provide patients with evidence-based complementary medicine therapies within a supportive and palliative cancer care setting. This study retrospectively examined characteristics of patients with lung cancer predicting utilization of a freely-provided IO consultation at two medical centers in Israel.

Methods: Electronic medical files of 832 patients with lung cancer attending/not attending the IO consultation were searched for socio-demographic (age, gender, country of birth, place of residence, primary language spoken) and personal health (Body Mass Index; smoking; disability) characteristics; cancer-related parameters (primary tumor site, localized vs.

Treatment effects of elexacaftor/tezacaftor/ivacaftor on people with cystic fibrosis heterozygous for 3849+10kbC->T and a class I variant.

Background: The splice variant 3849+10kbC->T (c.3717+12191C>T) (3849 variant) is a residual function CFTR variant, characterized by insertion of an in-frame stop codon into most CFTR transcripts. Both ivacaftor (Iva) and tezacaftor/ivacaftor (Tez/Iva) have been approved for people with CF (pwCF) carrying the 3849 variant.

Nivolumab plus relatlimab and nivolumab plus ipilimumab for patients with advanced renal cell carcinoma: results from the open-label, randomised, phase II FRACTION-RCC trial.

Background: The Fast Real-time Assessment of Combination Therapies in Immuno-ONcology study in patients with aRCC (FRACTION-RCC) was designed to assess new immuno-oncology (IO) combinations in patients with advanced renal cell carcinoma (aRCC). We present results in IO-naive patients treated with nivolumab (NIVO) + relatlimab (RELA) or NIVO + ipilimumab (IPI) in track 1.

Methods: The open-label, randomised, phase II FRACTION-RCC trial enrolled patients with aRCC from 32 hospitals and cancer centres across six countries.

Bistable Soft Shells for Programmable Mechanical Logic.

Mechanical computing promises to integrate semiconductor-based digital logic in several applications, but it needs straightforward programmable devices for changing computing rules in situ. A methodology based on strain-governed, bistable soft shells that process digital information by interchanging their internal/external surfaces is proposed. This bistable behavior, explained via model-based design, safeguards robustness by working only once for each input pulse.

Does the TruBlue Laser Set Microlaryngoscopy Equipment on Fire? A Systematic Evaluation.

Introduction: The risk of fire during laser microlaryngoscopy is well known. However, limited information is available about fire risk with the novel TruBlue laser. This study systematically evaluates its interactions with common surgical supplies, offering valuable insights into safety considerations for surgeons.

Reassessing the need for scheduled replacement of short term central venous catheters: A narrative comprehensive review.

Central venous catheters (CVCs) are essential in modern healthcare but are associated with significant risks, particularly catheter-related bloodstream infections (CRBSIs). Current guidelines do not recommend routine replacement of CVCs based on time alone. However, recent evidence challenges this recommendation.

Ammonia electro-oxidation on nickel hydroxide: phases, pH and poisoning.

Nickel hydroxide is a leading alternative to platinum group metals for electrocatalysis of the ammonia oxidation reaction (AOR), an important process for energy conversion and environmental remediation. Nevertheless, the dependence of AOR electrocatalysis on the different crystalline phases at the electrode surface (α-Ni(OH)/γ-NiOOH β-Ni(OH)/β-NiOOH) has never been investigated. Herein, the crystalline β-Ni(OH) and the disordered α-Ni(OH) were synthesized and characterized by XRD, HRSEM, and Raman and FTIR spectroscopies.

pyPCG: a Python toolbox specialized for phonocardiography analysis.

Objective: Phonocardiography has recently gained popularity in low-cost and remote monitoring, including passive fetal heart monitoring. The development of methods which analyse phonocardiographic data tries to capitalize on this opportunity, and in recent years a multitude of such algorithms and models have been published. In these approaches there is little to no standardization and multiple parts of these models have to be reimplemented on a case-by-case basis.

Fibroblast diversification is an embryonic process dependent on muscle contraction.

Fibroblasts, the most common cell type found in connective tissues, play major roles in development, homeostasis, regeneration, and disease. Although specific fibroblast subpopulations have been associated with different biological processes, the mechanisms and unique activities underlying their diversity have not been thoroughly examined. Here, we set out to dissect the variation in skeletal-muscle-resident fibroblasts (mrFibroblasts) during development.

Analysis of the ubiquitin-modified proteome identifies novel host factors in Kaposi's sarcoma herpesvirus lytic reactivation.

Unlabelled: Kaposi's sarcoma herpesvirus (KSHV) is the causative agent of Kaposi's sarcoma and is associated with primary effusion lymphoma (PEL), multicentric Castleman's disease, and two inflammatory diseases. KSHV-associated cancers are primarily associated with genes expressed during latency, while other pathologies are associated with lytic gene expression. The major lytic switch of the virus, Replication and Transcription Activator (RTA), interacts with cellular machinery to co-opt the host ubiquitin proteasome system to evade the immune response as well as activate the program of lytic replication.

Coordination among cytoskeletal organization, cell contraction, and extracellular matrix development is dependent on LOX for aneurysm prevention.

Distinct and seemingly independent cellular pathways affecting intracellular machinery or extracellular matrix (ECM) deposition and organization have been implicated in aneurysm formation. One of the key genes associated with this pathology in both humans and mice is lysyl oxidase (LOX), a secreted ECM-modifying enzyme, highly expressed in medial vascular smooth muscle cells. To dissect the mechanisms leading to aneurysm development, we conditionally deleted Lox in smooth muscle cells.

Non- Tuberculous mycobacterial infection and pulmonary disease In Northern Israel: a focus on M. simiae.

Background: The prevalence of non-Tuberculous Mycobacteria pulmonary disease (NTM-pd) is rising globally. Previously, we described a high prevalence of M. simiae isolation in Israel.

Personal Values and Cognitive Biases.

Introduction: Psychology textbooks abound with demonstrations of classic biases, yet the question why some people are more or less susceptible to those biases remains little explored. Drawing on Schwartz Values Theory (1992), we aim to show how individual differences in personal values, which express trans-situational, chronic motivations of a perceiver, impact cognitive biases.

Method: In six studies (N = 843; 42.

Child-physician rapport at a neurodevelopmental clinic: In the eyes of the beholder.

Background: Building rapport with young patients is crucial for fostering a sense of comfort that enhances their cooperation during clinical encounters and improves clinical outcomes. The available data on children with neurodevelopmental disabilities in community-based settings, including data on child-physician rapport, is limited.

Aims: To examine how children with neurodevelopmental disabilities perceive rapport with treating physicians compared to parents and treating physicians and to evaluate the relationship between the child-reported rapport and its impact on their cooperation.