Challenges of Engaging Primary Care Providers in Specialized Telementoring Education About Sickle Cell Disease for Sickle Cell Specialists: Results from the Sickle Cell Disease Training and Mentoring Program for Primary Care Providers (STAMP) Project ECHO.

Introduction: Sickle cell disease (SCD) is an inherited blood disorder affecting approximately 100,000 individuals in the U.S. A lack of knowledgeable providers, particularly for adult patients, has led to a significant number of adults without access to high-quality care.

Matching model with mechanism: Appropriate rodent models for studying various aspects of diabetes pathophysiology.

Many rodent models are available for preclinical diabetes research making it a challenge for researchers to choose the most appropriate one for their experimental question. To aid in this, models have classically been categorized according to which type of diabetes they represent, and further into whether the model is induced, spontaneous or the result of genetic manipulation. This fails to capture the complexity of pathogenesis seen in diabetes in humans.

Associations with Taste, Diet, and Health in an Italian Population.

Background/objectives: The SNP has previously been associated with sweet taste, diet, and health status, although never comprehensively in a single study. Also, associations between and sweet taste might be body mass index (BMI)-dependent. Therefore, this study aimed to conduct a comprehensive investigation of and sweet taste intensity and liking, food liking, and diet and health status whilst considering BMI.

Exploring Dysphagia in Congenital Diaphragmatic Hernia: A Retrospective Analysis.

Congenital diaphragmatic hernia (CDH) is a complex congenital disorder often accompanied by long-term feeding difficulties. There is a paucity of published data regarding the impact of swallowing difficulties on long-term patient outcomes. Our study attempts to evaluate this phenomenon.

An artificial transcription factor that activates potent interferon-γ expression in human Jurkat T Cells.

Interferon (IFN)-γ is a central regulator of cell-mediated immunity in human health and disease, but reduced expression of the target receptors impairs signaling activity and leads to immunotherapy resistance. Although intracellular expression of IFN-γ restores the signaling and downstream functions, we lack the tools to activate the gene instead of cell surface receptors. This paper introduces the design and characterization of an artificial transcription factor (ATF) protein that recognizes the gene with six zinc finger domains, which are dovetailed to a VP64 signaling domain that promotes gene transcription and translation.

Standardized Note Template Expedites Completion of Consults for Surgical Fetal Anomalies.

Introduction: We developed standardized electronic medical record templates (EMR-temp) for use in ambulatory prenatal surgical consultations for surgical fetal anomalies (SFAs). Our aim was to evaluate EMR-temp impact in provider documentation in prenatal care of SFA.

Methods: Prenatal consultations for SFAs at a single institution were retrospectively reviewed (2019-2022).

Examining Electronic Nicotine Delivery System (ENDS) Cue Reactivity Using a Remote Platform: Cigarette and ENDS Desire in Dual Users.

Introduction: Prior research shows that in-person exposure to electronic nicotine delivery systems (ENDS) use increases desire for cigarettes and ENDS. However, less is known about the impact of cues delivered during remote interactions. This study extends previous in-person cue work by leveraging a remote confederate-delivered cue-delivery paradigm to evaluate the impact of dual nicotine vaping (vs.

Cognitive and behavioral effects of perampanel 4 mg daily dose.

Objective: The neuropsychological adverse effects of antiseizure medications (ASMs) influence the tolerability, and in turn effectiveness of these medications, which can occur in a dose-dependent fashion. In this study, we examine the neuropsychological effects of perampanel (PER) at 4 mg daily as this dose has not been previously assessed with objective cognitive tests.

Methods: The study was originally designed to assess (1) effects of perampanel at 4 mg using different titration rates, and (2) habituation over time.

LPMO-Catalyzed Oxidation of Cellulosic Fibers with Controlled Addition of a Reductant and HO.

Cellulose-derived biomaterials offer a sustainable and versatile platform for various applications. Enzymatic engineering of these fibers, particularly using lytic polysaccharide monooxygenases (LPMOs), shows promise due to the ability to introduce functional groups onto cellulose surfaces, potentially enabling further functionalization. However, harnessing LPMOs for fiber engineering remains challenging, partly because controlling the enzymatic reaction is difficult and partly because limited information is available about how LPMOs modify the fibers.

Risk Factors for Residual Unablated Tumour Following CT-Guided Percutaneous Renal Cryoablation: Lessons from the EuRECA Registry.

Objective: To assess tumour factors that reduce primary technical efficacy (PTE) from CT-guided renal cryoablation.

Methods: Patients were taken from the EuRECA registry between December 2014 and November 2020. To focus on tumour factors rather than individual technique, this study was limited to cases using CT and excluded procedures using cone beam, US, MRI, or laparoscopy.

Brain Age Modeling and Cognitive Outcomes in Young Adults With and Without Sickle Cell Anemia.

Importance: Both sickle cell anemia (SCA) and socioeconomic status have been associated with altered brain structure and cognitive disability, yet precise mechanisms underlying these associations are unclear.

Objective: To determine whether brains of individuals with and without SCA appear older than chronological age and if brain age modeling using brain age gap (BAG) can estimate cognitive outcomes and mediate the association of socioeconomic status and disease with these outcomes.

Design, Setting, And Participants: In this cross-sectional study of 230 adults with and without SCA, individuals underwent brain magnetic resonance imaging (MRI) and cognitive assessment.

An exceptionally rare case of a diffuse midline glioma with concomitant H3.1 K27M and G34R mutations in the HIST1H3C (H3C3) gene.

Histone mutations (H3 K27M, H3 G34R/V) are molecular features defining subtypes of paediatric-type diffuse high-grade gliomas (HGG) (diffuse midline glioma (DMG), H3 K27-altered, diffuse hemispheric glioma (DHG), H3 G34-mutant). The WHO classification recognises in exceptional cases, these mutations co-occur. We report one such case of a 2-year-old female presenting with neurological symptoms; MRI imaging identified a brainstem lesion which was biopsied.

Combining Top-Down and Bottom-Up: An Open Microfluidic Microtumor Model for Investigating Tumor Cell-ECM Interaction and Anti-Metastasis.

Using a combined top-down (i.e., operator-directed) and bottom-up (i.

Eliminating invisible deaths: the woeful state of global rabies data and its impact on progress towards 2030 sustainable development goals for neglected tropical diseases.

Like other neglected diseases, surveillance data for rabies is insufficient and incompatible with the need to accurately describe the burden of disease. Multiple modeling studies central to estimating global human rabies deaths have been conducted in the last two decades, with results ranging from 14,000 to 74,000 deaths annually. Yet, uncertainty in model parameters, inconsistency in modeling approaches, and discrepancies in data quality per country included in global burden studies have led to recent skepticism about the magnitude of rabies mortality.

Artificial intelligence performance in ultrasound-based lymph node diagnosis: a systematic review and meta-analysis.

Background And Objectives: Accurate classification of lymphadenopathy is essential for determining the pathological nature of lymph nodes (LNs), which plays a crucial role in treatment selection. The biopsy method is invasive and carries the risk of sampling failure, while the utilization of non-invasive approaches such as ultrasound can minimize the probability of iatrogenic injury and infection. With the advancement of artificial intelligence (AI) and machine learning, the diagnostic efficiency of LNs is further enhanced.

Jargon and Readability in Plain Language Summaries of Health Research: Cross-Sectional Observational Study.

Background: The idea of making science more accessible to nonscientists has prompted health researchers to involve patients and the public more actively in their research. This sometimes involves writing a plain language summary (PLS), a short summary intended to make research findings accessible to nonspecialists. However, whether PLSs satisfy the basic requirements of accessible language is unclear.

Prediction of future input explains lateral connectivity in primary visual cortex.

Neurons in primary visual cortex (V1) show a remarkable functional specificity in their pre- and postsynaptic partners. Recent work has revealed a variety of wiring biases describing how the short- and long-range connections of V1 neurons relate to their tuning properties. However, it is less clear whether these connectivity rules are based on some underlying principle of cortical organization.

SIV Env RhmAbs + N-803 at ART initiation prolongs viral decay without disrupting reservoir establishment in SIV-infected infant macaques.

The latent viral reservoir remains the major barrier to HIV cure, placing the burden of strict adherence to antiretroviral therapy (ART) on people living with HIV to prevent recrudescence of viremia. For infants with perinatally acquired HIV, adherence is anticipated to be a lifelong need. In this study, we tested the hypothesis that administration of ART and viral Envelope-specific rhesus-derived IgG1 monoclonal antibodies (RhmAbs) with or without the IL-15 superagonist N-803 early in infection would limit viral reservoir establishment in SIV-infected infant rhesus macaques.

Multiomics dissection of human RAG deficiency reveals distinctive patterns of immune dysregulation but a common inflammatory signature.

Human recombination-activating gene (RAG) deficiency can manifest with distinct clinical and immunological phenotypes. By applying a multiomics approach to a large group of -mutated patients, we aimed at characterizing the immunopathology associated with each phenotype. Although defective T and B cell development is common to all phenotypes, patients with hypomorphic variants can generate T and B cells with signatures of immune dysregulation and produce autoantibodies to a broad range of self-antigens, including type I interferons.

Clinical and Electromyographic Characteristics of Pediatric Laryngeal Dyskinesia.

Objective: To report the clinical and laryngeal electromyographic (LEMG) parameters of children with laryngeal dyskinesia (LD) and its prevalence among laryngeal mobility disorder (LMD) requiring full airway examination.

Study Design: Retrospective uncontrolled study.

Setting: Tertiary pediatric center.

"Due to These Restrictions, Girls Think of Themselves as Nothing": A Qualitative and Quantitative Description of Menstrual Restrictions and Stigma Among Adolescent Girls Across Religious and Other Sociocultural Contexts.

Introduction: Girls in Tanzania often experience menstrual restrictions (i.e., expectations about behaviors prohibited during menstruation) and menstrual stigma (i.

Mapping the developmental trajectory of human astrocytes reveals divergence in glioblastoma.

Glioblastoma (GBM) is defined by heterogeneous and resilient cell populations that closely reflect neurodevelopmental cell types. Although it is clear that GBM echoes early and immature cell states, identifying the specific developmental programmes disrupted in these tumours has been hindered by a lack of high-resolution trajectories of glial and neuronal lineages. Here we delineate the course of human astrocyte maturation to uncover discrete developmental stages and attributes mirrored by GBM.

New Silurian aculiferan fossils reveal complex early history of Mollusca.

Mollusca is the second most species-rich animal phylum, but the pathways of early molluscan evolution have long been controversial. Modern faunas retain only a fraction of the past forms in this hyperdiverse and long-lived group. Recent analyses have consistently recovered a fundamental split into two sister clades, Conchifera (including gastropods, bivalves and cephalopods) and Aculifera, comprising Polyplacophora ('chitons') and Aplacophora.

De novo design of ribosomally synthesized and post-translationally modified peptides.

In nature, peptides are enzymatically modified to constrain their structure and introduce functional moieties. De novo peptide structures could be built by combining enzymes from different pathways, but determining the rules of their use is difficult. We present a biophysical model to combine enzymes sourced from bacterial ribosomally synthesized and post-translationally modified peptide (RiPP) gene clusters.