Publications by authors named "J-P Riviere"

Background: One of the major challenges in managing allergic bronchopulmonary aspergillosis remains consistent and reproducible assessment of response to treatment.

Research Question: What are the most relevant changes in CT scan parameters over time for assessing response to treatment?

Study Design And Methods: In this ancillary study of a randomized clinical trial (NebuLamB), patients with asthma with available CT scan and without exacerbation during a 4-month allergic bronchopulmonary aspergillosis exacerbation treatment period (corticosteroids and itraconazole) were included. Changed CT scan parameters were assessed by systematic analyses of CT scan findings at initiation and end of treatment.

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Background And Objectives: Somatic and germline pathogenic variants in genes of the mammalian target of rapamycin (mTOR) signaling pathway are a common mechanism underlying a subset of focal malformations of cortical development (FMCDs) referred to as mTORopathies, which include focal cortical dysplasia (FCD) type II, subtypes of polymicrogyria, and hemimegalencephaly. Our objective is to screen resected FMCD specimens with mTORopathy features on histology for causal somatic variants in mTOR pathway genes, describe novel pathogenic variants, and examine the variant distribution in relation to neuroimaging, histopathologic classification, and clinical outcomes.

Methods: We performed ultra-deep sequencing using a custom HaloPlex Target Enrichment kit in DNA from 21 resected fresh-frozen histologically confirmed FCD type II, tuberous sclerosis complex, or hemimegalencephaly specimens.

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Article Synopsis
  • Patients with mutations in the RAG1 or RAG2 genes can develop conditions like Omenn syndrome or combined immunodeficiency, and around 60% of those treated with hematopoietic stem cell transplantation (HSCT) experienced significant health issues like infections, autoimmunity, or organ damage.
  • The study involved a diverse patient group and found that survival rates were 77.5% at one year and 67.5% at four years post-transplant, with infections being the leading cause of death.
  • Early HSCT (before age 3.5) showed better immune recovery, especially if patients had no pre-existing organ damage, highlighting the importance of timely treatment.
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Background: B-cell maturation antigen (BCMA)-directed chimeric antigen receptor (CAR) T-cell therapies have generated responses in patients with advanced myeloma, but relapses are common. G protein-coupled receptor, class C, group 5, member D (GPRC5D) has been identified as an immunotherapeutic target in multiple myeloma. Preclinical studies have shown the efficacy of GPRC5D-targeted CAR T cells, including activity in a BCMA antigen escape model.

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Background: In allergic bronchopulmonary aspergillosis (ABPA), prolonged nebulised antifungal treatment may be a strategy for maintaining remission.

Methods: We performed a randomised, single-blind, clinical trial in 30 centres. Patients with controlled ABPA after 4-month attack treatment (corticosteroids and itraconazole) were randomly assigned to nebulised liposomal amphotericin-B or placebo for 6 months.

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High-dose chemotherapy and autologous stem cell transplantation (HDT-ASCT) is the standard of care for relapsed or primary refractory (rel/ref) chemorefractory diffuse large B-cell lymphoma. Only 50% of patients are cured with this approach. We investigated safety and efficacy of CD19-specific chimeric antigen receptor (CAR) T cells administered following HDT-ASCT.

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Bone remodelling around a stem is an unavoidable long-term physiological process highly related to implant design. For some predisposed patients, it can lead to periprosthetic bone loss secondary to severe stress-shielding, which is thought to be detrimental by contributing to late loosening, late periprosthetic fracture, and thus rendering revision surgery more complicated.However, these concerns remain theoretical, since late loosening has yet to be documented among bone ingrowth cementless stems demonstrating periprosthetic bone loss associated with stress-shielding.

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The reaction of di-tert-butyl phosphate (((t)BuO)(2)P(O)(OH), dtbp-H) with copper acetate in the presence of pyridine (py) and 2,4,6-trimethylpyridine (collidine) has been investigated. Copper acetate reacts with dtbp-H in a reaction medium containing pyridine, DMSO, THF, and CH(3)OH to yield a one-dimensional polymeric complex [Cu(dtbp)(2)(py)(2)(mu-OH(2))](n) (1) as blue hollow crystalline tubes. The copper atoms in 1 are octahedral and are surrounded by two terminal phosphate ligands, two pyridine molecules, and two bridging water molecules.

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