3,267 results match your criteria: "CA J.L.; and Bnei Zion Medical Center[Affiliation]"
Frequency and Longitudinal Course of Behavioral and Neuropsychiatric Symptoms in Participants With Genetic Frontotemporal Dementia.
Neurology
October 2024
From the Department of Neurology (S. Schönecker, A.D., O.W., C.P., E.W., J.V., S.V.L., A. Brauer, G.U.H., J.L.), LMU University Hospital, LMU Munich, Germany; Department of Signal Theory Networking and Communications (F.J.M.-M., J.-M.G.S.), Andalusian Research Institute in Data Science and Computational Intelligence (DasCI), University of Granada, Spain; Institute for Stroke and Dementia Research (J.D., N.F.), LMU University Hospital, LMU Munich; Munich Cluster for Systems Neurology (SyNergy) (N.F., G.U.H., J.L.), Germany; Institute of Neuroscience and Physiology and Department of Psychiatry and Neurochemistry (N.F.), The Sahlgrenska Academy, University of Gothenburg, Mölndal and Gothenburg, Sweden; German Center for Neurodegenerative Diseases (DZNE) (J.V., G.U.H., J.L.), Munich, Germany; Dementia Research Centre (A. Bouzigues, L.L.R., P.H.F., E.F.-B., J.D.R.), Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, London, United Kingdom; Department of Neurology (J.C.v.S., L.C.J., H.S.), Erasmus Medical Centre, Rotterdam, the Netherlands; Alzheimer's disease and Other Cognitive Disorders Unit (R.S.-V.), Neurology Service, Hospital Clínic, Institut d'Investigacións Biomèdiques August Pi I Sunyer, University of Barcelona, Spain; Clinique Interdisciplinaire de Mémoire (R.L.), Département des Sciences Neurologiques, CHU de Québec, and Faculté de Médecine, Université Laval, Canada; Department of Neurobiology, Care Sciences and Society (C.G.), Center for Alzheimer Research, Division of Neurogeriatrics, Bioclinium, Karolinska Institutet; Unit for Hereditary Dementias (C.G.), Theme Inflammation and Aging, Karolinska University Hospital, Solna, Sweden; Fondazione Ca' Granda (D.G.), IRCCS Ospedale Policlinico, Milan; Centro Dino Ferrari (D.G.), University of Milan, Italy; Laboratory for Cognitive Neurology (R.V.), Department of Neurosciences, KU Leuven; Neurology Service (R.V.), University Hospitals Leuven; Leuven Brain Institute (R.V.), KU Leuven, Belgium; Faculty of Medicine (A.d.M.), University of Lisbon, Portugal; Fondazione IRCCS Istituto Neurologico Carlo Besta (P.T.), Milano, Italy; University Hospital of Coimbra (HUC) (I.S.), Neurology Service, Faculty of Medicine, and Center for Neuroscience and Cell Biology (I.S.), Faculty of Medicine, University of Coimbra, Portugal; Division of Psychology Communication and Human Neuroscience Wolfson Molecular Imaging Centre (A.G.), University of Manchester, United Kingdom; Department of Nuclear Medicine (A.G.), Center for Translational Neuro- and Behavioral Sciences, University Medicine Essen; Department of Geriatric Medicine (A.G.), Klinikum Hochsauerland, Arnsberg, Germany; Department of Neurofarba (S. Sorbi), University of Florence; IRCCS Fondazione Don Carlo Gnocchi (S. Sorbi), Florence, Italy; Department of Neurology (M.O.), University of Ulm, Germany; Univ Lille (F.P.); Inserm 1172 (F.P.), Lille; CHU (F.P.), CNR-MAJ, Labex Distalz, LiCEND Lille, France; Department of Psychiatry (S.D.), McGill University Health Centre, and McConnell Brain Imaging Centre (S.D.), Montreal Neurological Institute, McGill University, Montreal, Québec, Canada; Nuffield Department of Clinical Neurosciences (C.B.), Medical Sciences Division, University of Oxford; Department of Brain Sciences (C.B.), Imperial College London, United Kingdom; Sorbonne Université (I.L.B.), Paris Brain Institute, Institut du Cerveau, ICM, Inserm U1127, CNRS UMR 7225, Centre de Référence des Démences Rares ou Précoces (I.L.B.), IM2A, and Département de Neurologie (I.L.B.), AP-HP, Hôpital Pitié-Salpêtrière, Paris, France; Department of Clinical Neurological Sciences (E.F.), University of Western Ontario, London; Tanz Centre for Research in Neurodegenerative Diseases (M.C.T.), and Sunnybrook Health Sciences Centre (M.M.), Sunnybrook Research Institute, University of Toronto, Ontario, Canada; Department of Clinical Neurosciences (J.B.R.), MRC Cognition and Brain Sciences Unit, and Cambridge University Hospitals NHS Trust, University of Cambridge, United Kingdom; Department of Neurodegenerative Diseases (M.S.), Hertie-Institute for Clinical Brain Research and Center of Neurology, University of Tübingen; Center for Neurodegenerative Diseases (DZNE) (M.S.), Tübingen, Germany; Cognitive Disorders Unit (F.M.), Department of Neurology, Donostia Universitary Hospital; Neuroscience Area (F.M.), Biodonostia Health Research Institute, San Sebastian, Gipuzkoa, Spain; Neurology Unit (B.B.), Department of Clinical and Experimental Sciences, University of Brescia, Italy; and Department of Psychiatry and Psychotherapy (J.P.), Technical University Munich, Germany.
Background And Objectives: Behavioral and neuropsychiatric symptoms are frequent in patients with genetic frontotemporal dementia (FTD). We aimed to describe behavioral and neuropsychiatric phenotypes in genetic FTD, quantify their temporal association, and investigate their regional association with brain atrophy.
Methods: We analyzed data of pathogenic variant carriers in the chromosome 9 open reading frame 72 (), progranulin (), or microtubule-associated protein tau () gene from the Genetic Frontotemporal dementia Initiative cohort study that enrolls both symptomatic pathogenic variant carriers and first-degree relatives of known carriers.
Final, 10-Year Outcomes with Nivolumab plus Ipilimumab in Advanced Melanoma.
N Engl J Med
January 2025
From the Sandra and Edward Meyer Cancer Center (J.D.W.) and the Department of Medicine (J.D.W., M.A.P.), Weill Cornell Medicine, and Memorial Sloan Kettering Cancer Center (M.A.P.) - both in New York; Istituto Oncologico Veneto, IRCCS, Padua (V.C.-S.), European Institute of Oncology, IRCCS, Milan (P.Q.), Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori, IRCCS, Meldola (M.G.), University of Siena and the Center for Immuno-Oncology, University Hospital of Siena, Siena (M.M.), and Istituto Nazionale Tumori IRCCS Fondazione Pascale, Naples (P.A.A.) - all in Italy; Maria Sklodowska-Curie National Institute of Oncology, Warsaw, Poland (P.R.); Texas Oncology-Baylor Charles A. Sammons Cancer Center, Dallas (C.L.C.); University Hospital Essen, the German Cancer Consortium, the National Center for Tumor Diseases-West, the Research Alliance Ruhr, Research Center One Health, and University Duisburg-Essen - all in Essen, Germany (D.S.); the College of Medicine, Swansea University, Swansea (J.W.), Bristol Myers Squibb, Uxbridge (A.N.), and the Royal Marsden Hospital, London (J.L.) - all in the United Kingdom; the Department of Dermatology, University of Zurich, Zurich, Switzerland (R.D.); University Health Network Princess Margaret Cancer Centre, Toronto (M.O.B.), and Cross Cancer Institute, University of Alberta, Edmonton (J.W.) - both in Canada; Tasman Oncology Research, Southport, QLD (A.G.H.), Westmead Hospital, Westmead, NSW (M.S.C.), Blacktown Hospital, Blacktown, NSW (M.S.C.), the Melanoma Institute Australia, University of Sydney (M.S.C., G.V.L.), Royal North Shore Hospital (G.V.L.), and Mater Hospital (G.V.L.), Sydney, and Peter MacCallum Cancer Centre, University of Melbourne, Melbourne, VIC (S.S.) - all in Australia; Aix-Marseille Université, Assistance Publique-Hôpitaux de Marseille, Marseille (C.G.-M.), and Université Paris Cité, Assistance Publique-Hôpitaux de Paris (AP-HP) Dermato-oncology, Clinical Investigation Center, the Cancer Institute, AP-HP Nord Paris Cité, INSERM Unité 976, and St. Louis Hospital, Paris (C.L.) - all in France; the University of Colorado Cancer Center, Aurora (T.M.); Rogel Cancer Center, University of Michigan, Ann Arbor (C.D.L.); Hospital General Universitario Gregorio Marañon, Madrid (I.M-R.); the Netherlands Cancer Institute, Amsterdam (J.B.A.G.H.); University Hospital Leuven and Leuven Cancer Institute, KU Leuven, Leuven, Belgium (P.S.); Bristol Myers Squibb, Princeton, NJ (C.R., M.A., M.P.B., W.W.); and Dana-Farber Cancer Institute, Boston (F.S.H.).
Background: Previous results from this trial showed longer overall survival after treatment with nivolumab plus ipilimumab or with nivolumab monotherapy than with ipilimumab monotherapy in patients with advanced melanoma. Given that patients with advanced melanoma are living longer than 7.5 years, longer-term data were needed to address new clinically relevant questions.
View Article and Find Full Text PDFInhibition of novel human-HPV hybrid ecDNA enhancers reduces oncogene expression and tumor growth in oropharyngeal cancer.
Res Sq
September 2024
Moores Cancer Center, University of California, San Diego, La Jolla, CA, USA.
Article Synopsis
- Extrachromosomal circular DNA (ecDNA) is commonly found in various human cancers, particularly in oropharyngeal cancer linked to the human papillomavirus (HPV), but its role in cancer development is not fully understood.
- Researchers studied the epigenetic characteristics of hybrid ecDNA in HPV-positive oropharyngeal cancer cell lines and tumor models, discovering that HPV oncogenes E6/E7 are associated with unique enhancer regions.
- By using targeted therapies, such as CRISPR interference and specific inhibitors, they were able to decrease E6/E7 gene expression and inhibit tumor growth, highlighting the potential for new treatments that target ecDNA-driven cancer biology.
Among the goals of patient-centric care are the advancement of effective personalized treatment, while minimizing toxicity. The phase 2 I-SPY2.2 trial uses a neoadjuvant sequential therapy approach in breast cancer to further these goals, testing promising new agents while optimizing individual outcomes.
View Article and Find Full Text PDFNOTCH3 p.Arg1231Cys is markedly enriched in South Asians and associated with stroke.
Nat Commun
September 2024
Columbia University, New York, NY, USA.
Article Synopsis
- The genetic factors contributing to stroke risk in South Asians remain largely unstudied, with a recent study examining 75,000 Pakistanis using exome-wide sequencing.
- A specific genetic variant, NOTCH3 p.Arg1231Cys, was found to be more common in South Asians (0.58%) compared to Western Europeans (0.019%) and was significantly linked to hemorrhagic and overall stroke risk.
- This variant accounts for about 2.0% of hemorrhagic strokes and 1.1% of all strokes in South Asians, emphasizing the importance of including diverse populations in genetic research for better understanding and treatment of stroke.
Adaptive Deep Brain Stimulation in Parkinson's Disease: A Delphi Consensus Study.
medRxiv
August 2024
"Aldo Ravelli" Center for Neurotechnology and Experimental Brain Therapeutics, Department of Health Sciences, University of Milan, Via Antonio di Rudinì 8, 20142 Milan, Italy.
Article Synopsis
- The evolution of Deep Brain Stimulation (DBS) is shifting from conventional methods to adaptive techniques (aDBS), which are expected to become standard practice in the next decade, similar to past advancements in cardiac pacing.
- Research involving 21 experts highlighted the challenges and potential of aDBS, showing consensus on its safety and effectiveness compared to conventional DBS, particularly for Parkinson's Disease patients with motor fluctuations and dyskinesias.
- Further studies are necessary to refine the algorithms used in aDBS, ensuring its widespread and effective use in clinical settings.
People who share encounters with racism are silenced online by humans and machines, but a guideline-reframing intervention holds promise.
Proc Natl Acad Sci U S A
September 2024
Department of Psychology, Stanford University, Stanford, CA 94305.
Are members of marginalized communities silenced on social media when they share personal experiences of racism? Here, we investigate the role of algorithms, humans, and platform guidelines in suppressing disclosures of racial discrimination. In a field study of actual posts from a neighborhood-based social media platform, we find that when users talk about their experiences as targets of racism, their posts are disproportionately flagged for removal as toxic by five widely used moderation algorithms from major online platforms, including the most recent large language models. We show that human users disproportionately flag these disclosures for removal as well.
View Article and Find Full Text PDFA Novel De Novo Gain-of-Function Variant in Neurodevelopmental Disease With Congenital Tremor, Seizures, and Hypotonia.
Neurol Genet
October 2024
From the Department of Pediatric Neurology (F.D., P.B., M.S., A.M.K.); Center for Chronically Sick Children (F.D., P.B., M.S., A.M.K.), Charité-Universitätsmedizin Berlin; Department of Pediatrics (A.V.M.),DRK Kliniken Berlin Westend, Berlin; Department of Neuropediatrics (J.L., S.W.), VAMED Klinik Hohenstücken, Brandenburg an der Havel, Germany; Department of Pharmacology and Toxicology (F.T., J.S., N.J.O.), Institute of Pharmacy, Center for Molecular Biosciences Innsbruck, University of Innsbruck, Austria; Center of Functional Genomics (G.S., U.I.S.), Berlin Institute of Health at Charité - Universitätsmedizin Berlin, Hessische Straße 4A, Berlin, Germany; Department of Human Genetics (M.M.M., M.F.B.), Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands; Institute for Cell Biology and Neurobiology (A.M.K.); and Department of Nephrology and Medical Intensive Care (U.I.S.), Charité - Universitätsmedizin Berlin, Germany.
Article Synopsis
- A novel mutation (c.3506G>A, p.G1169D) in the Ca1.3 gene is linked to a syndrome that causes autism, developmental delays, and other neurological and hormonal disorders in children, presenting with varying severities.
- Two patients with this mutation exhibited different symptoms: one had severe issues including respiratory problems and hearing loss, while the other had a milder phenotype possibly due to mosaicism of the mutation.
- Functional studies showed that the mutation enhances channel activity, leading to abnormal calcium channel behavior, but treatments with calcium channel blockers (isradipine and nifedipine) did not produce positive effects for the severely affected patient.
Deep Learning Based Shear Wave Detection and Segmentation Tool for Use in Point-of-Care for Chronic Liver Disease Assessments.
Ultrasound Med Biol
December 2024
Beth Israel Deaconess Medical Center, Division of Gastroenterology, Boston, MA, USA.
Objective: As metabolic dysfunction-associated steatotic liver disease (MASLD) becomes more prevalent worldwide, it is imperative to create more accurate technologies that make it easy to assess the liver in a point-of-care setting. The aim of this study is to test the performance of a new software tool implemented in Velacur (Sonic Incytes), a liver stiffness and ultrasound attenuation measurement device, on patients with MASLD. This tool employs a deep learning-based method to detect and segment shear waves in the liver tissue for subsequent analysis to improve tissue characterization for patient diagnosis.
View Article and Find Full Text PDFResults of Lucia Keratoprosthesis Implantation in Severe Corneal Disease.
Am J Ophthalmol
December 2024
From the Instituto de Oftalmologia Fundacion Conde de Valenciana IAP (G.O.-M., G.R.V.-D., D.J.-C., J.A.R., K.A.R., J.L.D.-H., C.A.M.-M., A.N., A.R.-M., E.O.G.-H.), Mexico City, Mexico. Electronic address:
Purpose: Describe complications, functional success, and retention of the Lucia Keratoprosthesis (KPro).
Design: Retrospective interventional case series METHODS: The demographic data, baseline characteristics, complications, functional success, and retention were analyzed for Lucia KPro at the Instituto de Oftalmologia Conde de Valenciana in Mexico City from 2021 to 2023. Multivariate regression analysis and Kaplan-Meier plots were performed to identify associations with functional failure.
Small-molecule GSDMD agonism in tumors stimulates antitumor immunity without toxicity.
Cell
October 2024
Department of Biological Chemistry and Molecular Pharmacology, Harvard Medical School, Boston, MA 02115, USA; Program in Cellular and Molecular Medicine, Boston Children's Hospital, Boston, MA 02115, USA. Electronic address:
Gasdermin-mediated inflammatory cell death (pyroptosis) can activate protective immunity in immunologically cold tumors. Here, we performed a high-throughput screen for compounds that could activate gasdermin D (GSDMD), which is expressed widely in tumors. We identified 6,7-dichloro-2-methylsulfonyl-3-N-tert-butylaminoquinoxaline (DMB) as a direct and selective GSDMD agonist that activates GSDMD pore formation and pyroptosis without cleaving GSDMD.
View Article and Find Full Text PDF"Synthetic" DSC Perfusion MRI with Adjustable Acquisition Parameters in Brain Tumors Using Dynamic Spin-and-Gradient-Echo Echoplanar Imaging.
AJNR Am J Neuroradiol
January 2025
From the UCLA Brain Tumor Imaging Laboratory (BTIL), Center for Computer Vision and Imaging Biomarkers (F.S., J.Y., N.S.C., C.R., B.M.E.), University of California Los Angeles, Los Angeles, California
Background And Purpose: Normalized relative cerebral blood volume (nrCBV) and percentage of signal recovery (PSR) computed from dynamic susceptibility contrast (DSC) perfusion imaging are useful biomarkers for differential diagnosis and treatment response assessment in brain tumors. However, their measurements are dependent on DSC acquisition factors, and CBV-optimized protocols technically differ from PSR-optimized protocols. This study aimed to generate "synthetic" DSC data with adjustable synthetic acquisition parameters using dual-echo gradient-echo (GE) DSC datasets extracted from dynamic spin-and-gradient-echo echoplanar imaging (dynamic SAGE-EPI).
View Article and Find Full Text PDFPlasma cell-free RNA signatures of inflammatory syndromes in children.
Proc Natl Acad Sci U S A
September 2024
Meinig School of Biomedical Engineering, Cornell University, Ithaca, NY 14850.
Inflammatory syndromes, including those caused by infection, are a major cause of hospital admissions among children and are often misdiagnosed because of a lack of advanced molecular diagnostic tools. In this study, we explored the utility of circulating cell-free RNA (cfRNA) in plasma as an analyte for the differential diagnosis and characterization of pediatric inflammatory syndromes. We profiled cfRNA in 370 plasma samples from pediatric patients with a range of inflammatory conditions, including Kawasaki disease (KD), multisystem inflammatory syndrome in children (MIS-C), viral infections, and bacterial infections.
View Article and Find Full Text PDFComparison of protection against mpox following mRNA or modified vaccinia Ankara vaccination in nonhuman primates.
Cell
October 2024
Virology Division, United States Army Medical Research Institute of Infectious Diseases, Fort Detrick, MD, USA. Electronic address:
In 2022, mpox virus (MPXV) spread worldwide, causing 99,581 mpox cases in 121 countries. Modified vaccinia Ankara (MVA) vaccine use reduced disease in at-risk populations but failed to deliver complete protection. Lag in manufacturing and distribution of MVA resulted in additional MPXV spread, with 12,000 reported cases in 2023 and an additional outbreak in Central Africa of clade I virus.
View Article and Find Full Text PDFA Clinical Tool to Relate Youth Risk Factors to Adult Cardiovascular Events and Type 2 Diabetes: The International Childhood Cardiovascular Cohort Consortium.
J Pediatr
January 2025
Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH; The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.
Article Synopsis
- The study aimed to translate childhood cardiovascular (CV) risk factors into clinically actionable values that could predict adult CV diseases and type 2 diabetes mellitus (T2DM).
- It involved a long-term observational study with nearly 39,000 participants, tracking children from ages 3 to 19 over 30 years and analyzing several CV risk factors like BMI, blood pressure, and cholesterol.
- Findings revealed that the risk for CV events and T2DM starts at levels previously deemed normal, suggesting that earlier intervention may be necessary to prevent adult diseases.
Frontostriatal salience network expansion in individuals in depression.
Nature
September 2024
Department of Psychiatry, Weill Cornell Medicine, New York, NY, USA.
Decades of neuroimaging studies have shown modest differences in brain structure and connectivity in depression, hindering mechanistic insights or the identification of risk factors for disease onset. Furthermore, whereas depression is episodic, few longitudinal neuroimaging studies exist, limiting understanding of mechanisms that drive mood-state transitions. The emerging field of precision functional mapping has used densely sampled longitudinal neuroimaging data to show behaviourally meaningful differences in brain network topography and connectivity between and in healthy individuals, but this approach has not been applied in depression.
View Article and Find Full Text PDFDifferences in Acute Graft-Versus-Host Disease (GVHD) Severity and Its Outcomes Between Black and White Patients.
Transplant Cell Ther
November 2024
The Tisch Cancer Institute, Icahn School of Medicine at Mount Sinai, New York, New York. Electronic address:
Acute graft-versus-host disease (GVHD) is a significant complication following hematopoietic stem cell transplantation (HCT). Although recent advancements in GVHD prophylaxis have resulted in successful HCT across HLA barriers and expanded access to HCT for racial minorities, less is known about how race affects the severity and outcomes of acute GVHD. This study examines differences in the clinical course of acute GVHD and the prognostic value of GVHD biomarkers for Black and White recipients.
View Article and Find Full Text PDFRestrictive or Liberal Transfusion Strategy in Patients With Acute Myocardial Infarction and Anemia: 6-Month Mortality in the MINT Trial.
Circulation
September 2024
Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ (J.L.C.).
Bacteroides ovatus alleviates dysbiotic microbiota-induced graft-versus-host disease.
Cell Host Microbe
September 2024
Department of Genomic Medicine, The University of Texas MD Anderson Cancer Center, Houston, TX 77054, USA; Department of Stem Cell Transplantation and Cellular Therapy, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, USA; CPRIT Scholar in Cancer Research, Houston, TX, USA. Electronic address:
Acute lower gastrointestinal GVHD (aLGI-GVHD) is a serious complication of allogeneic hematopoietic stem cell transplantation. Although the intestinal microbiota is associated with the incidence of aLGI-GVHD, how the intestinal microbiota impacts treatment responses in aLGI-GVHD has not been thoroughly studied. In a cohort of patients with aLGI-GVHD (n = 37), we found that non-response to standard therapy with corticosteroids was associated with prior treatment with carbapenem antibiotics and a disrupted fecal microbiome characterized by reduced abundances of Bacteroides ovatus.
View Article and Find Full Text PDFVutrisiran in Patients with Transthyretin Amyloidosis with Cardiomyopathy.
N Engl J Med
January 2025
From the National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Hospital (M.F., J.D.G.), and Richmond Pharmacology (J.T.), London, and the Institute of Cardiovascular and Medical Sciences, British Heart Foundation Glasgow Cardiovascular Research Centre, Glasgow (M.C.P.) - all in the United Kingdom; Boston University School of Medicine (J.L.B.) and the Cardiovascular Division, Brigham and Women's Hospital (S.D.S.) - both in Boston; the Division of Cardiovascular Medicine, Stanford University School of Medicine, Stanford, CA (R.M.W.); the Department of Cardiovascular Diseases, Mayo Clinic College of Medicine, Rochester, MN (M.G.); the Division of Cardiology, Penn Presbyterian Medical Center, University of Pennsylvania Health System, Philadelphia (B.D.); the Cardiology Department and French National Reference Centre for Cardiac Amyloidosis, GRC Amyloid Research Institute and Clinical Investigation Centre 1430 at Hôpitaux Universitaires Henri-Mondor, Assistance Publique-Hôpitaux de Paris (AP-HP), and Institut Mondor de Recherche Biomédicale, INSERM, Université Paris Est Creteil, Creteil (T.D.), and the Department of Cardiology, French National Reference Center for Cardiac Amyloidosis, Bichat University Hospital, AP-HP, Paris (V.A.) - all in France; the Department of Cardiology, Hospital Universitario Puerta de Hierro Majadahonda, Health Research Institute of the Puerta de Hierro Majadahonda-Segovia, Centro de Investigación Biomédica en Red Enfermedades Cardiovasculares (CIBER-CV), and Centro Nacional de Investigaciones Cardiovasculares (P.G.-P.), and CIBER-CV (J.G.-C.), Madrid, and the Department of Cardiology, Hospital Universitari de Bellvitge, Instituto de Investigación Biomédica de Bellvitge, and Universitat de Barcelona, Barcelona (J.G.-C.) - all in Spain; MedStar Heart and Vascular Institute, MedStar Health, and Georgetown University School of Medicine - both in Washington, DC (F.H.S.); the Division of Cardiovascular Medicine, Department of Medicine, Kurume University School of Medicine, Kurume (N.T.), and the Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Kumamoto University, Kumamoto (K.T.) - both in Japan; the Department of Clinical Research and Epidemiology, Comprehensive Heart Failure Center, University Hospital Würzburg, and the Department of Medicine I, University Hospital Würzburg, Würzburg (C.M.), and the Division of Cardiovascular Imaging, University Hospital Münster, Münster (A.Y.) - both in Germany; the Department of Internal Medicine and Hematology, Semmelweis University, Budapest, Hungary (Z.P.); the Department of Cardiology, University Health Network of Toronto, Toronto (D.D.); University Medical Center Groningen, University of Groningen, Groningen, the Netherlands (P.V.M.); the Victor Chang Cardiac Research Institute, the Cardiology Department, St. Vincent's Hospital, and the School of Clinical Medicine, University of New South Wales - all in Sydney (A.J.); the Department of Cardiology, Hôpital Universitaire de Bruxelles-Hôpital Erasme, Université Libre de Bruxelles, Brussels (A.B.); the Department of Medicine, Heart Vascular Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea (D.K.); the Cardiology Department, Hospital Senhora da Oliveira-Guimarães, Guimarães, and the School of Medicine, University of Minho, Braga - both in Portugal (O.A.); the Department of Cardiology, Aarhus University Hospital, Aarhus, Denmark (S.H.P.); the Division of Translational Cardiology and Clinical Registries, Institute of Heart Diseases, Wrocław Medical University, Wrocław, Poland (E.A.J.); Alnylam Pharmaceuticals, Cambridge, MA (A.S., P.P.G., K.L.B., E.Y., N.S., L.Y., J.C., S.A.E., J.V.); and Columbia University Irving Medical Center, New York (M.S.M.).
Article Synopsis
- Transthyretin amyloidosis with cardiomyopathy (ATTR-CM) is a serious, progressive disease, and vutrisiran is a new treatment that works by reducing the production of transthyretin in the liver.
- In a double-blind trial involving 655 patients, those receiving vutrisiran had a lower risk of death and cardiovascular events compared to those on placebo, demonstrating significant efficacy.
- Vutrisiran also improved patient outcomes, showing less decline in walking distance and quality of life measurements over the study period compared to placebo.
Pulmonary hypertension associated with left heart disease.
Eur Respir J
October 2024
Division of Cardiology, Department of Medicine, Medical University of South Carolina, Charleston, SC, USA.
Article Synopsis
- Left heart disease (LHD) is a leading cause of pulmonary hypertension (PH), further classified into isolated post-capillary (ipcPH) or combined pre- and post-capillary PH (cpcPH).
- Recent clinical trials emphasize avoiding pulmonary arterial hypertension treatments for PH-LHD patients outside of trials, as these treatments may cause harm.
- A new PH-LHD staging system categorizes patients into four stages based on their clinical and hemodynamic characteristics, which aims to improve diagnosis and treatment through a more individualized approach.
Anti-RGS8 paraneoplastic cerebellar ataxia is preferentially associated with a particular subtype of Hodgkin's lymphoma.
J Neurol
October 2024
French Reference Center on Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis, Hospices Civils de Lyon, Hôpital Neurologique, Bron, France.
Ataxia with anti-regulator of G-protein signaling 8 autoantibodies (RGS8-Abs) is an autoimmune disease recently described in four patients. The present study aimed to identify other patients with RGS8-Abs, describe their clinical features, including the link between RGS8-related autoimmune cerebellar ataxia (ACA) and cancer. Patients with RGS8-Abs were identified retrospectively in the biological collections of the French Reference Center for Paraneoplastic Neurological Syndrome and the University of California San Francisco Center for Encephalitis and Meningitis.
View Article and Find Full Text PDFRestrictive Versus Liberal Transfusion in Patients With Type 1 or Type 2 Myocardial Infarction: A Prespecified Analysis of the MINT Trial.
Circulation
December 2024
St Michael's Hospital, Unity Health Toronto and Peter Munk Cardiac Centre, University Health Network, University of Toronto, Toronto (S.G.G.).
Background: The MINT trial (Myocardial Ischemia and Transfusion) raised concern for harm from a restrictive versus liberal transfusion strategy in patients with acute myocardial infarction (MI) and anemia. Type 1 and type 2 MI are distinct pathophysiologic entities that may respond differently to blood transfusion. This analysis sought to determine whether the effects of transfusion varied among patients with a type 1 or a type 2 MI and anemia.
View Article and Find Full Text PDFUnveiling Molecular Diversity in Cerebral Thrombi via Spatial Transcriptomics.
Stroke
October 2024
Departments of Genomic Medicine, Human Health and Disease, and Human Microbiome, J. Craig Venter Institute, La Jolla, CA (M.W., J.L.E., C.L.D.).