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Département de pathologie et immunolog... Publications | LitMetric

274 results match your criteria: "Département de pathologie et immunologie Centre médical universitaire[Affiliation]"

Background: There is considerable practice variation in labelling, diagnosis and treatment of adults with sterile bone inflammation. We developed a expert consensus recommendations on the disease definition, diagnosis and treatment of this rare condition.

Methods: Systematic literature review and Grading of Recommendations, Assessment, Development and Evaluations-based appraisal of evidence, two Delphi surveys and three digital and in-person consensus meetings with a multidisciplinary expert panel and patient representatives.

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Comparative analysis of iPSC-derived NK cells from two differentiation strategies reveals distinct signatures and cytotoxic activities.

Front Immunol

October 2024

Institut National de la Santé et de la Recherche Médicale (INSERM), Unité Mixte de Recherche en Santé (UMR-S-1310), Villejuif, France.

Purpose: The ability to generate natural killer (NK) cells from induced pluripotent stem cells (iPSCs) has given rise to new possibilities for the large-scale production of homogeneous immunotherapeutic cellular products and opened new avenues towards the creation of "off-the-shelf" cancer immunotherapies. However, the differentiation of NK cells from iPSCs remains poorly understood, particularly regarding the ontogenic landscape of iPSC-derived NK (iNK) cells produced and the influence that the differentiation strategy employed may have on the iNK profile.

Methods: To investigate this question, we conducted a comparative analysis of two sets of iNK cells generated from the same iPSC line using two different protocols: (i) a short-term, clinically compatible feeder-free protocol corresponding to primitive hematopoiesis, and (ii) a lymphoid-based protocol representing the definitive hematopoietic step.

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Article Synopsis
  • * This study analyzed cases of C bantiana infections in France and its territories, involving patients who were diagnosed through a comprehensive surveillance program, focusing on survival rates and the presence of central nervous system (CNS) involvement.
  • * Out of 23 patients identified from 2002 to 2022, 65% had CNS involvement, with a notable increase in cases reported in 2022, indicating a possible correlation with environmental factors like rising temperatures.
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Idiopathic inflammatory myopathies (IIM) are rare, acquired muscle diseases; their diagnosis of is based on clinical, serological, and histological criteria. MHC-I-positive immunostaining, although non-specific, is used as a marker for IIM diagnosis; however, the significance of major histocompatibility complex (MHC)-II immunostaining in IIM remains debated. We investigated patterns of MHC-II immunostaining in myofibers and capillaries in muscle biopsies from 103 patients with dermatomyositis ([DM], n = 31), inclusion body myositis ([IBM], n = 24), anti-synthetase syndrome ([ASyS], n = 10), immune-mediated necrotizing myopathy ([IMNM], n = 18), or overlap myositis ([OM], n = 20).

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Purpose: High throughput technologies have identified molecular patterns in colorectal cancer (CRC) cells, aiding in modeling responses to anti-cancer treatments. The different responses observed depend on the type of cancer, the tumour grade and the functional programme of the cancer cells. Recent studies suggest that the unfolded protein response (UPR), autophagy and apoptosis could be involved in treatment resistance mechanisms by interacting with the tumour microenvironment (TME).

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Progression or relapse in the central nervous system (CNS) remains a rare but mostly fatal event for patients with diffuse large B-cell lymphoma (DLBCL). In a retrospective analysis of 5189 patients treated within 19 prospective German and French phase 2/3 trials, we identified 159 patients experiencing a CNS event (relapse: 62%, progression: 38%). Intracerebral, meningeal, intraspinal, or combined involvement was reported in 44%, 31%, 3%, and 22% of patients, respectively.

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Interactions between eosinophils and IL-5Rα-positive mast cells in nonadvanced systemic mastocytosis.

J Allergy Clin Immunol

December 2024

University of Paris, Institut Imagine, INSERM, Paris, France; French Reference Center for Mastocytosis (CEREMAST), Hôpital Necker-Enfants Malades, AP-HP, Paris, France; Department of Hematology, Hôpital Necker-Enfants Malades, AP-HP, Paris, France. Electronic address:

Background: Bidirectional interactions between eosinophils and mast cells (MCs) have been reported in various allergic diseases. Bone marrow (BM) eosinophilia, and to a lesser extent blood eosinophilia, is common in systemic mastocytosis (SM), but its significance remains unknown.

Objective: We described blood and BM eosinophil characteristics in SM.

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Immunoglobulin G (IgG) purification is a critical process for evaluating its role in autoimmune diseases, which are defined by the occurrence of autoantibodies. Affinity chromatography with protein G is widely considered to be the optimal technique for laboratory-scale purification. However, this technique has some limitations, including the exposure of IgG to low pH, which can compromise the quality of the purified IgG.

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Article Synopsis
  • Acid sphingomyelinase deficiency (ASMD) is a serious genetic disease that can affect people from childhood to adulthood, caused by problems with a gene called SMPD1.
  • A study reviewed medical records from 27 hospitals in France to learn more about the health and survival of patients with ASMD from 1990 to 2020.
  • The results showed that patients with type A usually did not live past early childhood, while those with type B lived longer, but there were still risks of early death from serious illnesses like neurodegeneration and cancer.
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Current knowledge and practice of Candida auris screening in France: A nationwide survey from the French Society of Medical Mycology (SFMM).

J Mycol Med

September 2024

Sorbonne Université, Inserm, Centre de Recherche Saint-Antoine, CRSA, AP-HP, Hôpital Saint-Antoine, Service de Parasitologie-Mycologie, F-75012 Paris, France. Electronic address:

Article Synopsis
  • Candida auris is a growing threat to healthcare facilities globally, necessitating systematic screening for patients from high-risk areas to prevent widespread outbreaks.
  • A study analyzing responses from 36 French mycologists found that only 30.6% were aware of any systematic screening protocols for C. auris in their hospitals.
  • Most post-admission screening focused on specific body sites, but only a small percentage utilized specialized testing methods, highlighting the need for increased awareness and better practices among healthcare professionals regarding systematic screening for this pathogen.
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Article Synopsis
  • Follicular helper T-cell lymphomas (TFHL) often have gene alterations affecting DNA methylation, and preliminary studies indicate that 5-azacitidine may be effective for patients with relapsed TFHL.
  • This study compared the oral azacitidine treatment to typical therapies (like gemcitabine and bendamustine) in patients over 18 with relapsed or refractory TFHL across five European countries and Japan.
  • The trial enrolled 86 patients, showing that those treated with azacitidine had a median progression-free survival of 5.6 months, significantly longer compared to 2.8 months for those receiving standard therapy.
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Article Synopsis
  • The study evaluated the effectiveness of specific IgE (sIgE) testing for wheat and rye flour in diagnosing occupational asthma compared to a specific inhalation challenge (SIC), which is considered the gold standard.
  • Results showed that sIgE levels of 0.35 kU/L or greater had similar diagnostic sensitivity and specificity to the SIC, but higher sIgE thresholds increased specificity significantly.
  • The findings suggest that high sIgE levels can effectively indicate flour-induced occupational asthma, while low levels in patients with a positive SIC may correlate with lower T2 inflammatory biomarkers.
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Article Synopsis
  • The study aimed to evaluate the effectiveness and tolerance of amenamevir (AMNV) in treating recalcitrant herpes simplex keratitis (HSK) caused by acyclovir-resistant herpes simplex virus 1 strains.
  • Six patients were analyzed retrospectively after failing traditional antiviral treatments, focusing on recurrence rates and visual acuity during AMNV therapy.
  • Results showed that 66% of patients did not have any recurrences on AMNV, leading to a significant reduction in HSK episodes and some improvement in vision, although the latter was not statistically significant likely due to the small sample size.
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Sepsis induces immune alterations, which last for months after the resolution of illness. The effect of this immunological reprogramming on the risk of developing cancer is unclear. Here we use a national claims database to show that sepsis survivors had a lower cumulative incidence of cancers than matched nonsevere infection survivors.

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Successful rescue TNF-α blocking for - Related immune reconstitution inflammatory syndrome: A case report.

Heliyon

April 2024

Service de Maladies Infectieuses et Tropicales, Hôpital Necker-Enfants-malades, Assistance publique-Hôpitaux de Paris, 149 rue de Sèvres, 75743, Paris, France.

Immune reconstitution inflammatory syndrome (IRIS) has been reported in immunocompromised patients with disseminated . Management relies on high-dose corticosteroids. We describe two cases of late-onset corticosteroid-refractory IRIS related to disseminated infection in a HIV-positive patient and a renal transplant patient who had a favorable outcome with a monoclonal TNF-α blocker.

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Lessons from prospective longitudinal follow-up of a French APECED cohort.

J Clin Endocrinol Metab

April 2024

Department of Endocrinology, Diabetology and Metabolism, Huriez Hospital, Lille University Hospital, F-59000 Lille, France.

Article Synopsis
  • APECED syndrome is a rare genetic disorder caused by mutations in the AIRE gene, typically characterized by a triad of symptoms including hypoparathyroidism, adrenal failure, and chronic mucocutaneous candidiasis (CMC), along with other non-endocrine issues.
  • In a national study involving 25 patients from 23 families, researchers identified 11 different variants of the AIRE gene, including two previously unreported variants, and found that a majority of patients displayed multiple clinical manifestations.
  • The study revealed significant immunological disturbances, such as NK cell lymphopenia and altered B lymphocyte homeostasis, and highlighted a variety of non-endocrine symptoms that could potentially be life-threatening, emphasizing the need for
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Features of cryptococcosis among 652 HIV-seronegative individuals in France: a cross-sectional observational study (2005-2020).

Clin Microbiol Infect

July 2024

Université Paris Cité, Department of Infectious Diseases and Tropical Medicine, Necker - Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), IHU Imagine, 75014 Paris, France; Mycology Department, Institut Pasteur, Université Paris Cité, National Reference Center for Invasive Mycoses and Antifungals, Mycology Translational Research Group, France.

Objectives: We aimed to describe features and outcomes of cryptococcosis among HIV-seronegative individuals in a large surveillance network for cryptococcosis in France.

Methods: We included incident cases of cryptococcosis in HIV-seronegative individuals from 2005 to 2020. We compared patient characteristics, disease presentations, cryptococcal antigen results, and induction antifungal treatments according to underlying disease.

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Article Synopsis
  • Enterovirus D68 (EV-D68) infections can cause severe respiratory issues and acute flaccid myelitis, with a significant rise reported during the fall-winter season of 2021-2022 across Europe.
  • The study by the European Non-Polio Enterovirus Network (ENPEN) analyzed over 10,481 samples from 19 countries, identifying 1,004 as EV-D68, predominantly affecting young children, where 37.9% required hospitalization.
  • Additionally, genetic analyses uncovered two new B3-derived lineages without regional patterns, indicating a notable impact of the infections and the emergence of new virus strains.
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[Fusion genes in salivary gland tumors].

Ann Pathol

February 2024

REFCORpath, France; Service d'anatomie et cytologie pathologiques, CHU de Montpellier, 191, avenue du Doyen-Gaston-Giraud, 34295 Montpellier, France. Electronic address:

Salivary gland tumors represent a diagnostic challenge for pathologists due to their rarity, their very wide histopathological and immuno-phenotypic spectrum, and the recent identification of new entities. This article presents the main molecular characteristics of these tumors in order to allow any pathologist to perceive the diagnostic tracks of these ENT tumors and to better guide the molecular approach to establish the diagnosis and guide therapy.

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Infections Differ Among Human Immunodeficiency Virus (HIV)-Seropositive and HIV-Seronegative Individuals: Results From a Nationwide Surveillance Program in France.

Open Forum Infect Dis

February 2024

Department of Infectious Diseases and Tropical Medicine, Université Paris Cité, Necker-Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Institut Hospitalo-Universitaire Imagine, Paris, France.

Among 1107 cryptococcosis cases from the French surveillance network (2005-2020), the proportion of HIV-seronegative individuals has recently surpassed that of HIV-seropositive individuals. We observed marked differences in patient characteristics, disease presentations, cryptococcal antigen results, infecting species, and mortality according to HIV serostatus.

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[Tubular basement membrane immune deposits during BK virus nephropathy].

Ann Pathol

September 2024

Département de pathologie, AP-HP, hôpitaux universitaires Henri-Mondor, 51, avenue du Maréchal-de-Lattre-de-Tassigny, 94000 Créteil, France; UnivParis Est Créteil (UPEC), Créteil, France. Electronic address:

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A predictive mortality score in ANCA-associated renal vasculitis.

Nephrol Dial Transplant

August 2024

Service de Néphrologie-Dialyse-Transplantation, Université d'Angers, CHU Angers, Angers, France.

Background: Several scores have been developed to predict mortality at anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) diagnosis. Their prognostic value in Caucasian patients with kidney involvement (AAV-GN) remains uncertain as none has been developed in this specific population. We aimed to propose a novel and more accurate score specific for them.

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The spectrum of childhood leukemia predisposition syndromes has grown significantly over last decades. These predisposition syndromes mainly involve CEBPA, ETV6, GATA2, IKZF1, PAX5, RUNX1, SAMD9/SAMD9L, TP53, RAS-MAPK pathway, DNA mismatch repair system genes, genes associated with Fanconi anemia, and trisomy 21. The clinico-biological features leading to the suspicion of a leukemia predisposition are highly heterogeneous and require varied exploration strategies.

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