29,205 results match your criteria: "A.K.; Pitie - Salpetriere University Hospital; ICAN[Affiliation]"

Longitudinal Insights Into Childhood Onset Facioscapulohumeral Dystrophy: A 5-Year Natural History Study.

Neurology

January 2025

From the Department of Neurology (J.N.D., H.T.M.B., N.V.A., B.G.M.V.E., N.C.V.); Department of Pediatric Neurology (J.N.D., H.T.M.B., A.K., C.E.E.), Donders Institute for Brain, Cognition and Behaviour, Amalia Children's Hospital, Radboud University Medical Centre, Nijmegen, The Netherlands; Department of Neurology (R.J.M.G.), Jönköping, and Department of Biomedical and Clinical Sciences, Linköping University, Sweden; Department of Rehabilitation (M.M.P., S.L.S.H.), Donders Institute for Brain, Cognition and Behaviour, Amalia Children's Hospital; and Department of Neurology (N.V.A.), Clinical Neuromuscular Imaging Group, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, The Netherlands.

Article Synopsis
  • FSHD is a genetic muscle disorder that can start in childhood, affecting about 20% of patients early on. Understanding its progression and outcomes is important for care and research.
  • A study followed 20 childhood-onset FSHD patients over 5 years, assessing muscle function and disease severity with various tests. Most participants did not notice changes in their condition, despite measurable progression.
  • Results showed variable disease progression, with improvements in quality of life and decreased fatigue. The study emphasizes the need for more sensitive outcome measures and larger international studies in future pediatric research.
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Carbonic anhydrases (CAs) are crucial in regulating various physiological processes in the body. The overexpression of isoforms human carbonic anhydrases (hCA) IX and hCA XII is linked to tumour progression. The selective inhibition of CA IX and CA XII isoforms can result in the development of better cancer treatment strategies.

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Stable Na/K-S Batteries with Conductive Organosulfur Polymer Microcages as Cathodes.

J Am Chem Soc

January 2025

College of Chemistry and Materials Science, Guangdong Provincial Key Laboratory of Supramolecular Coordination Chemistry, Jinan University, Guangzhou 510632, China.

Na-S and K-S batteries, with high-energy density, using naturally more abundant and affordable metals compared with rare resources like Li, Co, and Ni elements, have inspired intense research interest. However, the sulfur cathodes for Na/K storage are plagued by soluble polysulfide shuttling, larger volumetric deformation, and sluggish redox kinetics. Here, we report that a conductive organosulfur polymer microcage, fabricated facilely with the microbe and elemental sulfur as precursors, can effectively address these issues for stable high-capacity Na-S and K-S batteries.

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T cell bispecific antibodies (TCBs) are a promising new class of therapeutics for relapsed/refractory multiple myeloma. A frequently observed, yet incompletely understood effect of this treatment is the transient reduction of circulating T cell counts, also known as T cell margination (TCM). After administration of the GPRC5D-targeting TCB forimtamig (RG6234), TCM occurred in patients and correlated with cytokine release and soluble B cell maturation antigen decrease.

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K-TIG welding offers the advantages of single-sided welding and double-sided formation, making it widely used for medium/thick-plate welding. The welding quality of K-TIG is closely linked to its penetration state. However, the assembly gap in medium/thick-plate workpieces can easily result in an unstable penetration state.

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There is a shortage in the experimental research directly comparing the effectiveness of different nanoparticles in boosting asparaginase (ASNase) activity. This study assessed the impact of various nanoparticles on enhancing ASNase activity, stability, and anticancer effects through immobilization. ASNase was immobilized on different nanoparticles, and its efficiency was measured.

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Cellulosic biomass hydrolysates are rich in glucose and xylose, but most microorganisms, including , are unable to utilize xylose effectively. To address this limitation, we engineered a strain optimized for xylose metabolism through the xylose oxidoreductase pathway and promoter optimization. A promoter library with varying strengths was used to fine-tune the expression levels of the , , and genes, resulting in a strain with a strong promoter for and weaker promoters for and .

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Changes in Ibuprofen Toxicity and Degradation in Response to Immobilization of B1(2015b).

Molecules

November 2024

Institute of Biology, Biotechnology and Environmental Protection, Faculty of Natural Science, University of Silesia in Katowice, Jagiellońska 28, 40-032 Katowice, Poland.

Ibuprofen is one of the most commonly used anti-inflammatory drugs by humans, resulting in its appearance in the environment, which can negatively affect organisms living in it. The studies undertaken have shown that the immobilized B1(2015b) strain can decompose this drug at a rate of = 0.36 mg/L*h, with a constant of 0.

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Introduction: This is a structured scoping review to assess whether there is a relationship between stress-induced hyperglycemia (SIH), cytokine interactions, and mortality in trauma patients in comparison to non-diabetic normoglycemia [NDN], diabetic normoglycemia [DN], and diabetic hyperglycemia [DH].

Methods: We conducted a literature search of MEDLINE (PubMed) databases from 2000 to 2022 using a search strategy to identify observational studies. Initially, 2879 articles were retrieved.

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Enhanced Swine Behavior Detection with YOLOs and a Mixed Efficient Layer Aggregation Network in Real Time.

Animals (Basel)

November 2024

Department of Electronics Engineering, Interdisciplinary Graduate Program for BIT Medical Convergence, and Department of Data Science, Kangwon National University, Chuncheon 24341, Republic of Korea.

Effective livestock management has become essential owing to an aging workforce and the growth of large-scale farming operations in the agricultural industry. Conventional monitoring methods, primarily reliant on manual observation, are increasingly reaching their limits, necessitating the development of innovative automated solutions. This study developed a system, termed mixed-ELAN, for real-time sow and piglet behavior detection using an extended ELAN architecture with diverse kernel sizes.

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Small intestinal neuroendocrine tumors (SI-NETs) are characterized by carcinoid syndrome and carcinoid heart disease (CHD). The aim of the present study was to identify early risk markers for carcinoid heart disease and survival in a prospective median-term follow-up setting. We measured 5-HIAA and cumulative 5-HIAA exposure (Cum-5-HIAA) based on repeated measurements, proBNP, vascular function, hepatic tumor load, and transthoracic echocardiography (TTE) at baseline and during the median 5-year follow-up.

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Introduction: Latest epidemiological metrics put a global prevalence of 20.6 million people suffering from spinal cord injury (SCI), leading to a burden of functional disability, deterioration in quality of life and reduced life expectancy. A thorough statement of diagnostic methods and treatment protocols for swallowing disorders after SCI stands as a major priority to streamline patient care and cost-sharing.

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ECCENTRIC: A fast and unrestrained approach for high-resolution in vivo metabolic imaging at ultra-high field MR.

Imaging Neurosci (Camb)

October 2024

Athinoula A. Martinos Center for Biomedical Imaging, Department of Radiology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, United States.

A novel method for fast and high-resolution metabolic imaging, called ECcentric Circle ENcoding TRajectorIes for Compressed sensing (ECCENTRIC), has been developed at 7 Tesla MRI. ECCENTRIC is a non-Cartesian spatial-spectral encoding method designed to accelerate magnetic resonance spectroscopic imaging (MRSI) with high signal-to-noise at ultra-high field. The approach provides flexible and random sampling of the Fourier space without temporal interleaving to improve spatial response function and spectral quality.

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Background: No models predict future outcomes in inflammatory bowel disease (IBD) patients receiving maintenance infliximab therapy. We created a predictive model for unfavorable outcomes.

Methods: Adult patients with IBD receiving maintenance infliximab therapy at 2 centers with matched serum infliximab concentrations and blinded histologic scores (Robarts Histopathologic Index [RHI]) were included.

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Arbitrage spread prediction can provide valuable insights into the identification of arbitrage signals and assessing associated risks in algorithmic trading. However, achieving precise forecasts by increasing model complexity remains a challenging task. Moreover, uncertainty in the development and maintenance of model often results in extremely unstable returns.

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Somatic mosaicism is an important cause of disease, but mosaic and somatic variants are often challenging to detect because they exist in only a fraction of cells. To address the need for benchmarking subclonal variants in normal cell populations, we developed a benchmark containing mosaic variants in the Genome in a Bottle Consortium (GIAB) HG002 reference material DNA from a large batch of a normal lymphoblastoid cell line. First, we used a somatic variant caller with high coverage (300x) Illumina whole genome sequencing data from the Ashkenazi Jewish trio to detect variants in HG002 not detected in at least 5% of cells from the combined parental data.

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Ultrafast mapping of short reads to transcriptomic and metagenomic references via lightweight mapping techniques such as pseudoalignment has demonstrated success in substantially accelerating several types of analyses without much loss in accuracy compared to alignment-based approaches. The application of pseudoalignment to large reference sequences - like the genome - is, however, not trivial, due to the large size of the references or "targets" (i.e.

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Acute Ecotoxicity and Bioconcentration Tests for Se(IV) in Nile tilapia ().

ACS Omega

December 2024

National Institute for Alternative Technologies for Detection, Toxicological Assessment and Removal of Emerging Micropollutants and Radioactives (INCT-DATREM), Department of Chemistry and Environmental Science, São Paulo State University (UNESP), São José do Rio Preto, SP, 15054-000, Brazil.

Selenium is one of the most important trace element micronutrients for the global biota, mainly due to its role in protecting against oxidative stress. However, this element can become toxic when present at concentrations slightly higher than those needed for metabolic purposes. It can be transferred through the food chain toward higher trophic levels, with bioaccumulation and biomagnification leading to possible toxicity.

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Background: Lymphatic collecting vessels in the kidney are critical in clearing interstitial fluid, macromolecules, and infiltrating immune cells. Dysfunction of the lymphatic vessels can disrupt this process and exacerbate injury-associated inflammation in many disease conditions. We previously found that sodium accumulates within the kidney interstitium during proteinuric kidney injury and elevated sodium environments stimulate isolevuglandin production in antigen-presenting cells, stimulating T cells, and modulating inflammatory responses.

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Background: Urinalysis, an essential diagnostic tool, faces challenges in terms of standardization and accuracy. The use of artificial intelligence (AI) with mobile technology can potentially solve these challenges. Therefore, we investigated the effectiveness and accuracy of an AI-based program in automatically interpreting urine test strips using mobile phone cameras, an approach that may revolutionize point-of-care testing.

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Effectiveness and safety of VISULAS® green selective laser trabeculoplasty: 12 months retrospective data.

Lasers Med Sci

December 2024

Internationale Innovative Ophthalmochirurgie GbR, Martin-Luther-Platz 22/26, 40212, Düsseldorf, Germany.

To report the safety and effectiveness of selective laser trabeculoplasty (SLT) using the SLT mode of the VISULAS green laser in patients with primary open-angle glaucoma (POAG). Twelve months results are presented. Retrospective extension in 4 German centers of an initially prospective interventional multicenter 3-month clinical investigation using the VISULAS green SLT (Carl Zeiss Meditec AG, Jena, Germany) in patients with POAG who either needed treatment escalation or commenced treatment and had an IOP ≥ 17mmHg at baseline, with no previous glaucoma or other ocular surgery.

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Extracellular vesicles (EVs) mediate intercellular communication. EVs are composed of a lipid bilayer and contain cytosolic proteins and RNAs. Studies highlight EVs striking functions in cell-cell crosstalk.

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Background: Peanut allergy is a potentially life-threatening food allergy in children. This study explored whether dupilumab, a human monoclonal immunoglobulin (Ig)G4 antibody that blocks the activity of interleukin (IL)-4/IL-13, improved safety and desensitization to peanut exposure in children with peanut allergy.

Methods: A Phase II, 24-week, multicenter, single-arm, open-label, proof-of-concept study was conducted in the USA and Canada (NCT03793608).

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In vivo evidence for cell body loss in cortical lesions in people with multiple sclerosis.

Ann Clin Transl Neurol

January 2025

Athinoula A. Martinos Center for Biomedical Imaging, Department of Radiology, Massachusetts General Hospital, Harvard Medical School, Charlestown, Massachusetts, 02129, USA.

Objective: To quantify alterations in soma and neurite density imaging measures within and surrounding cortical lesions in people with multiple sclerosis using in vivo high-gradient diffusion MRI.

Methods: In this cross-sectional study, 41 people with multiple sclerosis and 34 age- and sex-matched healthy controls underwent 3 T high-gradient diffusion MRI. Cortical lesions were segmented on artificial intelligence-enabled double inversion recovery images.

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