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
Papers
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Ludwig Maximilian University of Munich1, Uludağ University2, Cincinnati Children's Hospital Medical Center3, Turku University Hospital4, Ege University5, University of Colorado Denver6, King Saud bin Abdulaziz University for Health Sciences7, Tehran University of Medical Sciences8, St George's Hospital9, University of Freiburg10, University Hospital Regensburg11, University of Debrecen12, Boston Children's Hospital13, National Institutes of Health14, Necker-Enfants Malades Hospital15, Paris Descartes University16, Aix-Marseille University17, Newcastle University18, Utrecht University19, Loyola University Medical Center20, Kuwait University21, Harvard University22, University College London23, Seattle Children's Research Institute24
TL;DR: A comprehensive evaluation of the clinical phenotype of DOCK8 deficiency in the largest cohort reported so far demonstrates the severity of the disease with relatively poor prognosis and early HSCT should be strongly considered as a potential curative measure.
Abstract: Mutations in DOCK8 result in autosomal recessive Hyper-IgE syndrome with combined immunodeficiency (CID). However, the natural course of disease, long-term prognosis, and optimal therapeutic management have not yet been clearly defined. In an international retrospective survey of patients with DOCK8 mutations, focused on clinical presentation and therapeutic measures, a total of 136 patients with a median follow-up of 11.3 years (1.3-47.7) spanning 1693 patient years, were enrolled. Eczema, recurrent respiratory tract infections, allergies, abscesses, viral infections and mucocutaneous candidiasis were the most frequent clinical manifestations. Overall survival probability in this cohort [censored for hematopoietic stem cell transplantation (HSCT)] was 87 % at 10, 47 % at 20, and 33 % at 30 years of age, respectively. Event free survival was 44, 18 and 4 % at the same time points if events were defined as death, life-threatening infections, malignancy or cerebral complications such as CNS vasculitis or stroke. Malignancy was diagnosed in 23/136 (17 %) patients (11 hematological and 9 epithelial cancers, 5 other malignancies) at a median age of 12 years. Eight of these patients died from cancer. Severe, life-threatening infections were observed in 79/136 (58 %); severe non-infectious cerebral events occurred in 14/136 (10 %). Therapeutic measures included antiviral and antibacterial prophylaxis, immunoglobulin replacement and HSCT. This study provides a comprehensive evaluation of the clinical phenotype of DOCK8 deficiency in the largest cohort reported so far and demonstrates the severity of the disease with relatively poor prognosis. Early HSCT should be strongly considered as a potential curative measure.
222 citations
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TL;DR: A virtual environment is used to characterize episodic memory profiles in an ecological fashion, which includes memory for central and perceptual details, spatiotemporal contextual elements, and binding, and it is found that the AD patients' performances were inferior to that of the aMCI and even more to the healthy aged groups, in line with the progression of hippocampal atrophy reported in the literature.
222 citations
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TL;DR: The data suggest that whereas limbic overactivation is reported consistently in patients with mood disorders, future research should consider the relevance of a wider network of regions in formulating conceptual models of BD and MDD.
222 citations
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TL;DR: The cellular and molecular mechanisms whereby autophagy functions in multiple aspects of malignant disease, including cancer initiation, progression and responses to therapy are discussed.
Abstract: Mammalian cells harness autophagy to eliminate physiological byproducts of metabolism and cope with microenvironmental perturbations. Moreover, autophagy connects cellular adaptation with extracellular circuitries that impinge on immunity and metabolism. As it links transformed and non-transformed components of the tumour microenvironment, such an autophagic network is important for cancer initiation, progression and response to therapy. Here, we discuss the mechanisms whereby the autophagic network interfaces with multiple aspects of malignant disease.
222 citations
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TL;DR: The results showed a large diversity of outcome measures used across studies, and illustrated the need for strategies to build international consensus on appropriate outcome measures for upper limb function after stroke.
Abstract: Background
Establishing which upper limb outcome measures are most commonly used in stroke studies may help in improving consensus among scientists and clinicians.
221 citations
Authors
Showing all 21023 results
Name | H-index | Papers | Citations |
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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 |