Institution
Paris Descartes University
Government•Paris, France•
About: Paris Descartes University is a government organization based out in Paris, France. It is known for research contribution in the topics: Population & Transplantation. The organization has 20987 authors who have published 37456 publications receiving 1206222 citations. The organization is also known as: Université Paris V-Descartes & Université de Paris V.
Topics: Population, Transplantation, Immune system, Cancer, Pregnancy
Papers published on a yearly basis
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Stanford University1, Paris Descartes University2, Boston Children's Hospital3, Cincinnati Children's Hospital Medical Center4, Great Ormond Street Hospital5, Vita-Salute San Raffaele University6, University of Toronto7, University of Freiburg8, University of São Paulo9, Marmara University10, University of Ulm11, University of Florence12, Baylor College of Medicine13, French Institute of Health and Medical Research14, University College London15, Ludwig Maximilian University of Munich16, Tokyo Medical and Dental University17, University of Ulsan18, University of Pittsburgh19, Hannover Medical School20, Spanish National Research Council21, Ben-Gurion University of the Negev22, Ghent University Hospital23, University of California, Los Angeles24, Leiden University Medical Center25, Wrocław Medical University26, Université de Montréal27, University of Graz28, Harvard University29, University of California, San Francisco30, University of Alabama at Birmingham31
TL;DR: Patients receiving chronic IS were hampered by disease recurrence or complications, impacting long‐term disease‐free survival, and when performed in patients with a low OI score, HSCT resulted in disease resolution with better quality of life, independent of age, donor source, or conditioning regimen.
Abstract: Background Immunodysregulation polyendocrinopathy enteropathy x-linked (IPEX) syndrome is a monogenic autoimmune disease caused by FOXP3 mutations. Because it is a rare disease, the natural history and response to treatments, including allogeneic hematopoietic stem cell transplantation (HSCT) and immunosuppression (IS), have not been thoroughly examined. Objective This analysis sought to evaluate disease onset, progression, and long-term outcome of the 2 main treatments in long-term IPEX survivors. Methods Clinical histories of 96 patients with a genetically proven IPEX syndrome were collected from 38 institutions worldwide and retrospectively analyzed. To investigate possible factors suitable to predict the outcome, an organ involvement (OI) scoring system was developed. Results We confirm neonatal onset with enteropathy, type 1 diabetes, and eczema. In addition, we found less common manifestations in delayed onset patients or during disease evolution. There is no correlation between the site of mutation and the disease course or outcome, and the same genotype can present with variable phenotypes. HSCT patients (n = 58) had a median follow-up of 2.7 years (range, 1 week-15 years). Patients receiving chronic IS (n = 34) had a median follow-up of 4 years (range, 2 months-25 years). The overall survival after HSCT was 73.2% (95% CI, 59.4-83.0) and after IS was 65.1% (95% CI, 62.8-95.8). The pretreatment OI score was the only significant predictor of overall survival after transplant ( P = .035) but not under IS. Conclusions Patients receiving chronic IS were hampered by disease recurrence or complications, impacting long-term disease-free survival. When performed in patients with a low OI score, HSCT resulted in disease resolution with better quality of life, independent of age, donor source, or conditioning regimen.
216 citations
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Hannover Medical School1, University of Graz2, Vanderbilt University3, Paris Descartes University4, Katholieke Universiteit Leuven5, Aga Khan University6, University of Hamburg7, Amrita Institute of Medical Sciences and Research Centre8, University of Münster9, University of Cambridge10, National Institute for Health Research11, Boston Children's Hospital12, Charité13, Goethe University Frankfurt14, Lund University15, Sahlgrenska University Hospital16
TL;DR: Ten section task forces voted on the updated recommendations, based on the 2016 executive summary, and provided a specific, comprehensive, detailed but practical framework for the optimal clinical care of children and young adults with PH.
Abstract: The European Pediatric Pulmonary Vascular Disease Network is a registered, non-profit organization that strives to define and develop effective, innovative diagnostic methods and treatment options in all forms of pediatric pulmonary hypertensive vascular disease, including pulmonary hypertension (PH) associated with bronchopulmonary dysplasia, PH associated with congenital heart disease (CHD), persistent PH of the newborn, and related cardiac dysfunction. The executive writing group members conducted searches of the PubMed/MEDLINE bibliographic database (1990-2018) and held face-to-face and web-based meetings. Ten section task forces voted on the updated recommendations, based on the 2016 executive summary. Clinical trials, meta-analyses, guidelines, and other articles that include pediatric data were searched using the term "pulmonary hypertension" and other keywords. Class of recommendation (COR) and level of evidence (LOE) were assigned based on European Society of Cardiology/American Heart Association definitions and on pediatric data only, or on adult studies that included >10% children or studies that enrolled adults with CHD. New definitions by the World Symposium on Pulmonary Hypertension 2018 were included. We generated 10 tables with graded recommendations (COR/LOE). The topics include diagnosis/monitoring, genetics/biomarkers, cardiac catheterization, echocardiography, cardiac magnetic resonance/chest computed tomography, associated forms of PH, intensive care unit/lung transplantation, and treatment of pediatric PH. For the first time, a set of specific recommendations on the management of PH in middle- and low-income regions was developed. Taken together, these executive, up-to-date guidelines provide a specific, comprehensive, detailed but practical framework for the optimal clinical care of children and young adults with PH.
216 citations
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TL;DR: This work shows that B56, but not B, family members of PP2A increase ERK dephosphorylation, without affecting its activation by MEK, and raises a new paradigm in ERK signaling in which ERK associated to a substrate can transph phosphorylate nearby proteins.
Abstract: The protein phosphatase 2A (PP2A) acts on several kinases in the extracellular signal-regulated kinase (ERK) signaling pathway but whether a specific holoenzyme dephosphorylates ERK and whether this activity is controlled during mitogenic stimulation is unknown. By using both RNA interference and overexpression of PP2A B regulatory subunits, we show that B56, but not B, family members of PP2A increase ERK dephosphorylation, without affecting its activation by MEK. Induction of the early gene product and ERK substrate IEX-1 (ier3) by growth factors leads to opposite effects and reverses B56-PP2A-mediated ERK dephosphorylation. IEX-1 binds to B56 subunits and pERK independently, enhances B56 phosphorylation by ERK at a conserved Ser/Pro site in this complex and triggers dissociation from the catalytic subunit. This is the first demonstration of the involvement of B56-containing PP2A in ERK dephosphorylation and of a B56-specific cellular protein inhibitor regulating its activity in an ERK-dependent fashion. In addition, our results raise a new paradigm in ERK signaling in which ERK associated to a substrate can transphosphorylate nearby proteins.
216 citations
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TL;DR: Dental CBCT could be justified for presurgical diagnosis, preoperative planning and peroperative transfer for oral implant rehabilitation, whilst striving for optimisation of CBCT based machine-dependent, patient-specific and indication-oriented variables.
Abstract: In implant dentistry, three-dimensional (3D) imaging can be realised by dental cone beam computed tomography (CBCT), offering volumetric data on jaw bones and teeth with relatively low radiation doses and costs. The latter may explain why the market has been steadily growing since the first dental CBCT system appeared two decades ago. More than 85 different CBCT devices are currently available and this exponential growth has created a gap between scientific evidence and existing CBCT machines. Indeed, research for one CBCT machine cannot be automatically applied to other systems. Supported by a narrative review, recommendations for justified and optimized CBCT imaging in oral implant dentistry are provided. The huge range in dose and diagnostic image quality requires further optimization and justification prior to clinical use. Yet, indications in implant dentistry may go beyond diagnostics. In fact, the inherent 3D datasets may further allow surgical planning and transfer to surgery via 3D printing or navigation. Nonetheless, effective radiation doses of distinct dental CBCT machines and protocols may largely vary with equivalent doses ranging between 2 to 200 panoramic radiographs, even for similar indications. Likewise, such variation is also noticed for diagnostic image quality, which reveals a massive variability amongst CBCT technologies and exposure protocols. For anatomical model making, the so-called segmentation accuracy may reach up to 200 μm, but considering wide variations in machine performance, larger inaccuracies may apply. This also holds true for linear measures, with accuracies of 200 μm being feasible, while sometimes fivefold inaccuracy levels may be reached. Diagnostic image quality may also be dramatically hampered by patient factors, such as motion and metal artefacts. Apart from radiodiagnostic possibilities, CBCT may offer a huge therapeutic potential, related to surgical guides and further prosthetic rehabilitation. Those additional opportunities may surely clarify part of the success of using CBCT for presurgical implant planning and its transfer to surgery and prosthetic solutions. Hence, dental CBCT could be justified for presurgical diagnosis, preoperative planning and peroperative transfer for oral implant rehabilitation, whilst striving for optimisation of CBCT based machine-dependent, patient-specific and indication-oriented variables.
216 citations
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TL;DR: In children with LCH, BRAF(V600E) mutation was associated with high-risk features, permanent injury, and poor short-term response to chemotherapy, and the impact of BRAF inhibitors for this subgroup of patients who may benefit from targeted therapy.
Abstract: PURPOSE:
Langerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasia with a broad spectrum of clinical manifestations and outcomes in children. The somatic BRAF(V600E) mutation occurs frequently, but clinical significance remains to be determined.
PATIENTS AND METHODS:
BRAF(V600E) mutation was investigated in a French LCH cohort. We analyzed associations between mutation status and clinical presentation, extent of disease, reactivation rate, response to therapy, and long-term permanent sequelae.
RESULTS:
Among 315 patients with successfully determined BRAF status, 173 (54.6%) carried a BRAF(V600E) mutation. Patients with BRAF(V600E) manifested more severe disease than did those with wild-type BRAF. Patients with BRAF(V600E) comprised 87.8% of patients (43 of 49) with multisystem LCH with risk organ involvement (liver, spleen, hematology), 68.6% of patients (35 of 51) with multisystem LCH without risk organ involvement, 43.9% of patients (86 of 196) with single-system LCH, and 42.1% of patients (8 of 19) with lung-involved LCH (P < .001). BRAF(V600E) mutation was also associated with organ involvement that could lead to permanent, irreversible damage, such as neurologic (75%) and pituitary (72.9%) injuries. Compared with patients with wild-type BRAF, patients with BRAF(V600E) more commonly displayed resistance to combined vinblastine and corticosteroid therapy (21.9% v 3.3%; P = .001), showed a higher reactivation rate (5-year reactivation rate, 42.8% v 28.1%; P = .006), and had more permanent, long-term consequences from disease or treatment (27.9% v 12.6%; P = .001).
CONCLUSION:
In children with LCH, BRAF(V600E) mutation was associated with high-risk features, permanent injury, and poor short-term response to chemotherapy. Further population-based studies should be undertaken to confirm our observations and to assess the impact of BRAF inhibitors for this subgroup of patients who may benefit from targeted therapy.
216 citations
Authors
Showing all 21023 results
Name | H-index | Papers | Citations |
---|---|---|---|
Guido Kroemer | 236 | 1404 | 246571 |
Cyrus Cooper | 204 | 1869 | 206782 |
Jean-Laurent Casanova | 144 | 842 | 76173 |
Alain Fischer | 143 | 770 | 81680 |
Maxime Dougados | 134 | 1054 | 69979 |
Carlos López-Otín | 126 | 494 | 83933 |
Giuseppe Viale | 123 | 740 | 72799 |
Thierry Poynard | 119 | 668 | 64548 |
Lorenzo Galluzzi | 118 | 477 | 71436 |
Shahrokh F. Shariat | 118 | 1637 | 58900 |
Richard E. Tremblay | 116 | 685 | 45844 |
Olivier Hermine | 111 | 1026 | 43779 |
Yehezkel Ben-Ari | 110 | 459 | 44293 |
Loïc Guillevin | 108 | 800 | 51085 |
Gérard Socié | 107 | 920 | 44186 |