976 results match your criteria: "Hôpitaux Universitaires de Strasbourg et Fédération de Médecine Translationnelle de Médecine de Strasbourg[Affiliation]"

Evolution of anti-MICA antibodies after imlifidase infusion for a high immunological risk kidney transplantation.

Hum Immunol

January 2025

Service de Néphrologie, Dialyse et Transplantation, Hôpitaux Universitaires de Strasbourg, Strasbourg, France; Laboratoire d'ImmunoRhumatologie Moléculaire, Institut national de la santé et de la recherche médicale (INSERM) UMR_S1109, Faculté de Médecine, Fédération Hospitalo-Universitaire OMICARE, Fédération de Médecine Translationnelle de Strasbourg (FMTS), Université de Strasbourg, Strasbourg, France; Laboratoire d'Excellence (LabEx) TRANSPLANTEX, Faculté de Médecine, Université de Strasbourg, Strasbourg, France; Institut Thématique Interdisciplinaire (ITI) de Médecine de Précision de Strasbourg, Strasbourg, France. Electronic address:

Imlifidase is an endopeptidase known for cleaving anti-Human Leucocyte Antigen donor-specific antibodies (DSA) to allow high-risk kidney transplantation. However, it lacks comprehensive data regarding its effect on alloantibodies targeting other histocompatibility antigens, such as Major Histocompatibility Complex class I chain-related protein A (MICA). This study describes the dynamics of anti-MICA antibodies following imlifidase administration in a kidney transplant recipient with anti-MICA*002 preformed DSA.

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Background: The optimal timing for initiating dialysis and prognostic markers in chronic kidney disease (CKD) patients are under debate, with mortality and cardiovascular risks varying among patients. This study investigates whether the apoptosis inhibitor of macrophage (AIM), which is mostly bound to pentameric IgM, could serve as an effective indicator.

Methods: We prospectively followed 423 patients at dialysis initiation and 563 at various CKD stages.

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Introduction: The incidence of cervical cancer in French Guiana is among the highest worldwide. It ranks 5th among all cancers and is the 2nd most common cancer in women after breast cancer. The primary objective of this study was to establish the proportion of high-grade cytological lesions of the cervix in women under 25 years of age who underwent a cervico-uterine smear (CUS) in French Guiana over a 10-year period.

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RICTOR variants are associated with neurodevelopmental disorders.

Eur J Hum Genet

December 2024

Laboratoire d'ImmunoRhumatologie Moléculaire, plateforme GENOMAX, INSERM UMR_S 1109, Faculté de Médecine, Fédération Hospitalo-Universitaire OMICARE, Fédération de Médecine Translationnelle de Strasbourg (FMTS), Institut Thématique Interdisciplinaire TRANSPLANTEX NG, Université de Strasbourg, 4 rue Kirschleger, 67085, Strasbourg, France.

RICTOR is a key component of the mTORC2 signaling complex which is involved in the regulation of cell growth, proliferation and survival. RICTOR is highly expressed in neurons and is necessary for brain development. Here, we report eight unrelated patients presenting with intellectual disability and/or development delay and carrying variants in the RICTOR gene.

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Introduction: Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by an overactive immune response, particularly involving excessive production of type I interferons. This overproduction is driven by the phosphorylation of IRF7, a crucial factor in interferon gene activation. Current treatments for SLE are often not very effective and can have serious side effects.

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Purposes: This research highlights the need for affordable phantoms for MRI education. Current options are either expensive or limited. A phantom, easy to manufacture and distribute, is proposed to demonstrate various pedagogical concepts, aiding students in understanding MRI image quality concepts.

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Defective germinal center selection results in persistence of self-reactive B cells from the primary to the secondary repertoire in Primary Antiphospholipid Syndrome.

Nat Commun

November 2024

Department of Clinical Immunology and Internal Medicine, National Reference Center for Systemic Autoimmune Diseases (CNR RESO), Tertiary Center for Primary Immunodeficiency, Strasbourg University Hospital, Strasbourg, France.

Primary antiphospholipid syndrome (PAPS) is a life-threatening clotting disorder mediated by pathogenic autoantibodies. Here we dissect the origin of self-reactive B cells in human PAPS using peripheral blood and bone marrow of patients with triple-positive PAPS via combined single-cell RNA sequencing, B cell receptors (BCR) repertoire profiling, CITEseq analysis and single cell immortalization. We find that antiphospholipid (aPL)-specific B cells are present in the naive compartment, polyreactive, and derived from the natural repertoire.

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Early and accurate diagnosis is crucial to prevent disease development and define therapeutic strategies. Due to predominantly unspecific symptoms, diagnosis of autoimmune diseases (AID) is notoriously challenging. Clinical decision support systems (CDSS) are a promising method with the potential to enhance and expedite precise diagnostics by physicians.

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The cGAS-STING pathway in HIV-1 and Mycobacterium tuberculosis coinfection.

Infection

November 2024

Beijing Key Laboratory for HIV/AIDS Research, Clinical and Research Center for Infectious Diseases, Beijing Youan Hospital, Capital Medical University, Beijing, 100069, China.

Mycobacterium tuberculosis (M. tuberculosis) infection is the most common opportunistic infection in human immunodeficiency virus-1 (HIV-1)-infected individuals, and the mutual reinforcement of these two pathogens may accelerate disease progression and lead to rapid mortality. Therefore, HIV-1/M.

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In medicine, virological diagnosis is mainly based on the detection of the viral genome and antigens, or on the identification of specific antibodies produced in response to infection. These strategies are suitable for characterizing an active infection or past contact with an already known virus. The recent development of tests for evaluating the host's cellular immune response opens new perspectives for personalized patient care based on immunomonitoring.

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Article Synopsis
  • Primary immunodeficiency diseases with partial albinism, such as Chediak Higashi Syndrome (CHS) and Griscelli Syndrome type 2 (GS2), are autosomal recessive disorders characterized by immune deficiencies and abnormal pigmentation.
  • The study evaluated 25 patients over the past decade, using genetic analyses and examinations of leukocyte granules and hair shafts to identify these conditions, along with control groups of patients with albinism and healthy individuals.
  • The findings indicated specific genetic variants associated with CHS and HPS2, highlighting the importance of timely hematopoietic stem cell transplantation and genetic testing for accurate diagnosis and management of these syndromes.
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Cell surface scavenger receptors contribute to homoeostasis and the response to pathogens and products associated with damage by binding to common molecular features on a wide range of targets. Apoptosis inhibitor of macrophage (AIM/CD5L) is a soluble protein belonging to the scavenger receptor cysteine-rich (SRCR) superfamily that contributes to prevention of a wide range of diseases associated with infection, inflammation, and cancer. AIM forms complexes with IgM pentamers which helps maintain high-levels of circulating AIM in serum for subsequent activation on release from the complex.

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Because 20-30% of patients with sepsis deteriorate to critical illness, biomarkers that provide accurate early prognosis may identify which patients need more intensive treatment versus safe early discharge. The objective was to test the performance of sVEGFR2, suPAR and PCT, alone or combined with clinical signs and symptoms, for the prediction of clinical deterioration. This prospective observational study enrolled patients with suspected infection who met SIRS criteria without organ dysfunction (delta SOFA <2 from baseline) from 16 emergency departments.

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Detection of Borrelia burgdorferi sensu lato by proteomics: a complementary diagnosis tool on erythema migrans biopsies.

Clin Microbiol Infect

January 2025

UR3073-Pathogen-Host- Arthropod Vectors Interactions-Group Borrelia, Fédération de Médecine Translationnelle, Université de Strasbourg, Strasbourg, France; French National Reference Center for Borrelia, Hôpitaux Universitaires de Strasbourg, Strasbourg, France. Electronic address:

Objectives: We have developed targeted proteomics in the context of Lyme borreliosis (LM) as a new direct diagnostic tool for detecting Borrelia proteins in the skin of patients with erythema migrans. If satisfactory, this proteomic technique could be used in addition to culture and/or PCR for disseminated infections where Borrelia detection is essential to demonstrate active infection. In these infections, the diagnosis is indirect and relies mainly on serology.

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Narrative review of tissue-mimicking materials for MRI phantoms: Composition, fabrication, and relaxation properties.

Radiography (Lond)

October 2024

University of Strasbourg, CNRS, Inserm, ICube UMR 7357, Strasbourg, France; Pôle d'imagerie Médicale, Hôpitaux Universitaires de Strasbourg, Strasbourg, France; Faculté de Médecine, Maïeutique et Sciences de la Santé, Université de Strasbourg, Strasbourg, France; Fédération de Médecine Translationnelle de Strasbourg, Faculté de Médecine, Université de Strasbourg, Strasbourg, France. Electronic address:

Introduction: Tissue-mimicking materials (TMMs) are now essential reference objects for quality control, development and training in all medical imaging modalities. This review aims to provide a comprehensive synthesis of materials used in the fabrication of TMMs for MRI phantoms, focusing on their composition, fabrication methods, and relaxation properties (T1 and T2).

Methods: A systematic review was conducted, covering articles published between 1980 and 2023.

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Article Synopsis
  • The study looked at a special peptide called MAREMO that can help fight breast cancer by blocking a harmful molecule called tenascin-C.*
  • By doing this, it helps the body’s immune system work better against the tumor and stops the cancer from spreading.*
  • The MAREMO peptide also helps make the tumor environment less supportive for cancer growth by weakening certain cells and improving blood flow.*
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Epileptic variant in the spectrum of Alzheimer's disease - practical implications.

Seizure

September 2024

Centre Mémoire, de Ressources et de Recherche de Strasbourg, France; Unité de Neuropsychologie, Service de Neurologie des Hôpitaux Universitaires de Strasbourg, Strasbourg, France; University of Strasbourg and CNRS, ICube laboratory UMR 7357 and FMTS (Fédération de Médecine Translationnelle de Strasbourg), team IMIS/Neurocrypto Strasbourg, France; Centre de Compétences des démences rares des Hôpitaux Universitaires de Strasbourg, France. Electronic address:

Article Synopsis
  • Alzheimer's disease (AD) is linked to a higher risk of epilepsy, not only in its advanced stages (dementia) but also during earlier phases like mild cognitive impairment.
  • Epileptic seizures can even appear in the preclinical stage of AD, leading to a specific condition called the epileptic variant of Alzheimer's disease (evAD), which may be the only visible sign of the disease at that time.
  • Diagnosing evAD relies on examining amyloid and tau biomarkers and represents a crucial opportunity for early intervention with antiseizure medications to help slow down cognitive decline associated with AD.
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Unpredicted protective function of Fc-mediated inhibitory antibodies for HIV and SARS-CoV-2 vaccines.

J Infect Dis

September 2024

Laboratoire d'ImmunoRhumatologie Moléculaire, Institut national de la santé et de la recherche médicale (INSERM) UMR_S 1109, Institut thématique interdisciplinaire (ITI) de Médecine de Précision de Strasbourg, Transplantex NG, Faculté de Médecine, Fédération Hospitalo-Universitaire OMICARE, Fédération de Médecine Translationnelle de Strasbourg (FMTS), Université de Strasbourg, Strasbourg, France.

Developing effective vaccines is necessary in combating new virus pandemics. For HIV and SARS-CoV-2, the induction of neutralizing antibodies (NAb) is important for vaccine protection; however, the exact mechanisms underlying protection require further study. Recent data emphasize that even Abs that do not exhibit neutralizing activity may contribute to immune defense.

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A pleiotropic recurrent dominant variant causes a complex multisystemic disease.

Sci Adv

September 2024

Laboratoire d'ImmunoRhumatologie Moléculaire, Institut national de la santé et de la recherche médicale (INSERM) UMR_S 1109, Plateforme GENOMAX, Centre de Recherche d'Immunologie et d'Hématologie and Centre de Recherche en Biomédecine de Strasbourg (CRBS), Faculté de Médecine, Fédération Hospitalo-Universitaire OMICARE, Fédération de Médecine Translationnelle de Strasbourg (FMTS), Université de Strasbourg, Strasbourg, France.

Article Synopsis
  • The study investigates a specific genetic variant in the IP3 receptor that results in a significant disorder affecting multiple systems, characterized by immunodeficiency and disturbed calcium release in cells.
  • The variant (c.7570C>T, p.Arg2524Cys) leads to cellular defects, particularly impacting T cells, and is shown to affect calcium regulation and mitochondrial function, evidenced in laboratory models.
  • Patients exhibited a range of symptoms beyond immunodeficiency, such as ectodermal dysplasia and short stature, suggesting that this genetic mutation plays a unique and broader role in disease compared to previously documented cases.
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Article Synopsis
  • The study explores myoclonus dystonia caused by a variant in the SGCE gene, focusing on the microarchitectural brain abnormalities linked to this rare condition.
  • Researchers compared the brain structures of 18 MYC/DYT-SGCE patients with 24 healthy volunteers using advanced imaging techniques to assess neurite organization.
  • Results indicate that patients exhibited changes in cerebellar structure, with specific alterations correlating to the severity of dystonia, while no links were found between myoclonus severity and the microarchitectural measurements.
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Islet-after-kidney transplantation versus kidney alone in kidney transplant recipients with type 1 diabetes (KAIAK): a population-based target trial emulation in France.

Lancet Diabetes Endocrinol

October 2024

Translational Research Laboratory for Diabetes, Inserm, Institut Pasteur de Lille, Centre Hospitalier Universitaire de Lille, University of Lille, Lille, France; Department of General and Endocrine Surgery, Inserm, Institut Pasteur de Lille, Centre Hospitalier Universitaire de Lille, University of Lille, Lille, France. Electronic address:

Background: Islet transplantation has been associated with better metabolic control and quality of life than insulin treatment alone, but direct evidence of its effect on hard clinical endpoints is scarce. We aimed to assess the effect of islet transplantation on patient-graft survival in kidney transplant recipients with type 1 diabetes.

Methods: In this retrospective cohort study, we enrolled all patients with type 1 diabetes who received a kidney graft in France during the study period, identified from the CRISTAL nationwide registry.

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Repeat expansions in FGF14 cause autosomal dominant late-onset cerebellar ataxia (SCA27B) with estimated pathogenic thresholds of 250 (incomplete penetrance) and 300 AAG repeats (full penetrance), but the sequence of pathogenic and non-pathogenic expansions remains unexplored. Here, we demonstrate that STRling and ExpansionHunter accurately detect FGF14 expansions from short-read genome data using outlier approaches. By combining long-range PCR and nanopore sequencing in 169 patients with cerebellar ataxia and 802 controls, we compare FGF14 expansion alleles, including interruptions and flanking regions.

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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.

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Incidence, Risk Factors, and Long-Term Outcomes for Extubation Failure in ICU in Patients With Obesity: A Retrospective Analysis of a Multicenter Prospective Observational Study.

Chest

January 2025

Anesthesia and Critical Care Department B, Saint Eloi Teaching Hospital, PhyMedExp, University of Montpellier, INSERM U1046, CNRS UMR 9214, and Centre Hospitalier Universitaire Montpellier, Montpellier, France. Electronic address:

Background: To our knowledge, no large observational study has compared the incidence and risk factors for extubation failure within 48 h and during ICU stay in the same cohort of unselected critically ill patients with and without obesity.

Research Question: What are the incidence and risk factors of extubation failure in patients with and without obesity?

Study Design And Methods: In the prospective multicenter observational Practices and Risk Factors for Weaning and Extubation Airway Failure in Adult Intensive Care Unit: A Multicenter Trial (FREEREA) study in 26 ICUs, the primary objective was to compare the incidence of extubation failure within 48 h in patients with and without obesity. Secondary objectives were to describe and to identify the independent specific risk factors for extubation failure using first a logistic regression model and second a decision tree analysis.

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Background: Methotrexate, an immunosuppressant used for the treatment of inflammatory bowel disease (IBD) for over 30 years, remains underused compared to thiopurines.

Aims: To review the efficacy, safety, optimal dosing and delivery regimens of methotrexate in adults with IBD.

Methods: We conducted a systematic review of studies involving patients with IBD treated with methotrexate from inception to August 2023.

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