Neuilly-sur-Seine, France
Neuilly-sur-Seine, France

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Deroux A.,Center Hospitalier Des Alpes | Boccon-Gibod I.,Center Hospitalier Des Alpes | Fain O.,University Paris - Sud | Pralong P.,Grenoble University Hospital Center | And 7 more authors.
Clinical and Experimental Immunology | Year: 2016

Hereditary angioedema (HAE) is a rare disease associated with either a quantitative or qualitative deficiency in C1-inhibitor (C1-INH) or normal C1-INH. HAE with normal C1-INH is associated in 20% of cases with mutations in the gene for factor XII (FXII) or FXII-HAE. A recent review described 41 families, including 14 German and 15 Spanish families. We have constructed a register of French patients and their characteristics. A national survey was launched through the French National Center of Reference for Angioedema (CREAK) to study the clinical, biological and therapeutic characteristics of patients with HAE linked to a mutation of FXII gene. Fifty-seven patients were identified from 24 different families. In most cases they were young women (mean age at diagnosis: 31 years, mean age at first symptom: 21 years, female/male ratio: 76%). Twenty-one per cent of the patients experienced angioedema attacks only during pregnancy or when on oestrogen contraception. Sixty-three per cent had attacks at all times, but they were more severe during these same periods. Male carriers of the mutation were more frequently asymptomatic than females (P = 0·003). C1-INH concentrate and icatibant were both effective for treating attacks. The prophylactic use of tranexamic acid led to a 64% decrease in the number of attacks. This is one of the largest series reported of HAE patients with FXII mutation. The therapeutic management appeared to be identical to that of HAE with C1-INH deficiency. © 2016 British Society for Immunology


Dommes A.,IFSTTAR | Wu Y.-H.,Paris Center | Wu Y.-H.,University of Paris Descartes | Aquino J.-P.,Clinique Medicale de la Porte Verte | And 6 more authors.
Alzheimer Disease and Associated Disorders | Year: 2015

The overrepresentation of very old people (75 or older) in pedestrian crash statistics raises the issue of the effects of normal and pathologic ageing on gap-selection difficulties during street crossing. The present study focused on Alzheimer disease, a condition commonly associated with cognitive declines detrimental to daily life activities such as crossing the street. Twenty-five participants with mild dementia and 33 controls carried out a streetcrossing task in a simulated environment. They also took a battery of cognitive tests. The mild-dementia group was more likely than the control group to make decisions that led to collisions with approaching cars, especially when the traffic was coming from 2 directions and they were in the far lane. Regression analyses demonstrated that the increased likelihood of collisions in the dementia group was associated with impairments in processingspeed and visual-attention abilities assessed on the Useful Field of View test. This test has already proven useful for predicting driving outcomes, falls, and street-crossing difficulties in healthy old adults, and among drivers with Alzheimer disease. Clinicians are encouraged to use it to help estimate whether a patient can drive, walk, and cross a street safely. © 2015 Wolters Kluwer Health, Inc. All rights reserved.


PubMed | Paris Center, Center Hospitalier Des Alpes, Montpellier University, University Paris - Sud and 3 more.
Type: Journal Article | Journal: Clinical and experimental immunology | Year: 2016

Hereditary angioedema (HAE) is a rare disease associated with either a quantitative or qualitative deficiency in C1-inhibitor (C1-INH) or normal C1-INH. HAE with normal C1-INH is associated in 20% of cases with mutations in the gene for factor XII (FXII) or FXII-HAE. A recent review described 41 families, including 14 German and 15 Spanish families. We have constructed a register of French patients and their characteristics. A national survey was launched through the French National Center of Reference for Angioedema (CREAK) to study the clinical, biological and therapeutic characteristics of patients with HAE linked to a mutation of FXII gene. Fifty-seven patients were identified from 24 different families. In most cases they were young women (mean age at diagnosis: 31 years, mean age at first symptom: 21 years, female/male ratio: 76%). Twenty-one per cent of the patients experienced angioedema attacks only during pregnancy or when on oestrogen contraception. Sixty-three per cent had attacks at all times, but they were more severe during these same periods. Male carriers of the mutation were more frequently asymptomatic than females (P=0003). C1-INH concentrate and icatibant were both effective for treating attacks. The prophylactic use of tranexamic acid led to a 64% decrease in the number of attacks. This is one of the largest series reported of HAE patients with FXII mutation. The therapeutic management appeared to be identical to that of HAE with C1-INH deficiency.


Assouline A.,Center Clinique Of La Porte Of Saint Cloud | Assouline A.,Groupe Hospitalier Pitie Salpetriere | Levy A.,University Paris - Sud | Abdelnour-Mallet M.,Service Evaluation Pharmaceutique et Bon Usage SEPBU | And 10 more authors.
International Journal of Radiation Oncology Biology Physics | Year: 2014

Purpose This study aimed to evaluate the efficiency and the tolerance of radiation therapy (RT) on salivary glands in a large series of amyotrophic lateral sclerosis (ALS) patients with hypersalivation. Methods and Materials Fifty ALS patients that had medically failure pretreatment were included in this prospective study. RT was delivered through a conventional linear accelerator with 6-MV photons and 2 opposed beams fields including both submandibular glands and two-thirds of both parotid glands. Total RT dose was 10 Gy in 2 fractions (n=30) or 20 Gy in 4 fractions (n=20). RT efficacy was assessed with the 9-grade Sialorrhea Scoring Scale (SSS), recently prospectively validated as the most effective and sensitive tool to measure sialorrhea in ALS patients. Results At the end of RT, all patients had improved: 46 had a complete response (92% CR, SSS 1-3) and 4 had a partial response (8% PR, SSS 4-5). A significant lasting salivary reduction was observed 6 months after RT completion: there was 71% CR and 26% PR, and there was a significant SSS reduction versus baseline (P<10-6). There was no grade 3 to 4 toxicity, and most side effects (34%) occurred during RT. Nine patients (18%) underwent a second salivary gland RT course, with a 3-months mean delay from the first RT, resulting in a SSS decrease (-77%). Both RT dose regimens induced a significant SSS decrease with no significant toxicity. There were, however, more patients with CR/PR in the 20-Gy protocol (P=.02), and 8 of 9 patients (89%) receiving a second RT course had previously been treated within the 10-Gy protocol. Conclusion Radiation therapy of 20 Gy in 4 fractions is an efficient and safe treatment for ALS patients with sialorrhea. A shorter RT course (10 Gy in 2 fractions) may be proposed in patients in poor medical condition. © 2014 Elsevier Inc.


News Article | April 17, 2016
Site: motherboard.vice.com

Few things go together as poorly as science and politicians. Whether it’s Senator Ted Stevens describing the internet as a “series of tubes” during a net neutrality debate or Republican representatives reveling in their own ignorance about climate change, it’s clear that scientific illiteracy is a rampant problem in our nation’s hallowed halls of government. Yet this was precisely why it was so refreshing to see Canada’s recently elected Prime Minister Justin Trudeau explain the difference between a “normal” computer and a quantum computer completely off the cuff during a press briefing at the Perimeter Institute in Waterloo, Ontario, thereby proving that politics and science need not be mutually exclusive. Although Trudeau was at the Institute to announce $50 million in funding which will allow those working at Perimeter to continue their work on fundamental physics, he took the time to breakdown the essence of quantum computing for a clueless journalist: “Normal computers work either with power going through a wire or not, a one or a zero,” Trudeau said. “They’re binary systems. What quantum states allow for is much more complex information to be encoded into a single bit. A regular computer bit is either a one or a zero, on or off. A quantum state can be much more complex than that because as we know things can be both a particle and a wave at the same time, and the uncertainty around quantum states allows us to encode more information into a much smaller computer. That’s what’s exciting about quantum computing.” While most applauded Trudeau’s remarkably “clear and concise” explanation of quantum computing, others deemed his description as totally off the mark. I decided to ask some experts on quantum computing what they thought of the Prime Minister’s explanation to settle the debate once and for all: Romain Alléaume—Associate Professor at Telecom ParisTech and Paris Center for Quantum Computing “The beginning of Justin Trudeau’s explanation, about the difference between a classical bit and a quantum bit is absolutely correct. To be frank, the argumentation of Justin gets gradually more ‘uncertain’ when he says that the uncertainty principle implies that we can encode more information into ‘smaller computers’. Maybe he wanted to say that quantum computers can process information ‘in superposition,’ which allows to speed up some computations (i.e., solve some problems on smaller computers), but I am not certain about that. It is great to see a high level politician show enthusiasm for one of the biggest challenges in modern science.” Amr Helmy—Director, University of Toronto’s Center of Quantum Information and Quantum Control “His account of the distinction between a classical and quantum state is accurate. This is impressive that Canada’s PM has given this some thought. His comment on how superposition aides in storing information is an argument that can be equally made to explain the power which quantum computing possesses to process information in a fashion that is distinctly different from the classical paradigms. These are insights that are rarely considered by a Prime Minister. The rest of the world should be jealous!” SCORE: Too complex an issue to rank Michele Mosca—University Research Chair and Co-founder, Institute for Quantum Computing, University of Waterloo. Founding Member, Perimeter Institute for Theoretical Physics "The task is to explain quantum computing to a lay audience in a 100 words or so. It’s extremely hard, for even the best scientists and communicators, to get something like this both correct and interesting, especially in 100 words. He doesn’t say anything wrong. He conveys the essence of what quantum computing is, and why it might be more powerful. It’s understandable, and succinct. Also, keep in mind that this was something he said live, on the fly, in response to a joke from a reporter. Room for improvement? Hard to find. Can he next explain how encoding that more complex information in quantum bits leads to a more powerful computer? I’d love to hear his explanation." Aephraim Steinberg—Professor of Physics at the University of Toronto and member of Center of Quantum Information and Quantum Control “He zeroed in on the importance of how information is stored in a physical system, what a bit is, and the difference between classical bits and ‘quantum bits’ or ‘qubits’. This hinted he may have appreciated something very deep: the field of quantum computing is not just about trying to figure out how to speed up one task or another, but about understanding the fundamental role information has in the laws that govern the universe, how much information it takes to describe a physical system, and, on the flip side, what it means to store information in a physical system. “He faltered when trying to explain why a qubit is so much richer than a classical bit and threw in a few tangentially related buzzwords like ‘uncertainty’ and ‘particle and wave,’ in a way that made it clear that although he had the (accurate) sense that these concepts had something to do with quantum information he had to admit that he didn’t know what the connection was, but would throw caution to the wind and stir up some buzzword soup. “To put it bluntly, if you think about the level at which any scientist given a few minutes to try to explain quantum computing to him would have tried to pitch it, he probably got the gist and explained it back as well as you could imagine anyone doing. In any case, my joy is not because I believe our Prime Minister has become an expert at quantum physics. It is because he showed that he is ready to listen to scientists and try to understand what they are saying, what they believe is important, and why they demand support for basic research.”


Gaitch N.,Paris Center | Hubert D.,University of Paris Descartes | Gameiro C.,Paris Center | Burgel P.-R.,University of Paris Descartes | And 9 more authors.
Pancreatology | Year: 2016

Background/objectives: Currently, factors that promote the occurrence of pancreatitis episodes in patients affected with cystic fibrosis (CF) and pancreatic sufficiency (PS) are largely unknown. Methods: Six genes involved in pancreatitis or in ion transport into the pancreatic duct were investigated by next generation sequencing in 59 adult CF-PS patients with two identified CF mutations. Data on predisposing environmental factors were also recorded. Results: 19 experienced at least one episode of acute pancreatitis (AP) (AP+) and 40 patients did not (AP-). No influence of environmental factor was evidenced. No specific CFTR genotype was found predictive of pancreatitis. Patients sharing the same CFTR genotype may or may not experience AP episodes. Frequent and rare missense variants were found in 78.9% patients in group AP+ and 67.5% in group AP- but a few of them were pathogenic. Conclusions: AP or recurrent AP (RAP) is a frequent complication in our series of adult CF-PS patients. The majority of mild CFTR mutations found in group AP+ were located in the first transmembrane region. No clear other genetic factor could be found predictive of AP/RAP. Further experiments in large homogenous cohorts of CF-PS patients, including whole genome sequencing, may identify genetic predisposing factors to pancreatitis. © 2016 IAP and EPC.


PubMed | Paris Center and University of Paris Descartes
Type: Journal Article | Journal: Pancreatology : official journal of the International Association of Pancreatology (IAP) ... [et al.] | Year: 2016

Currently, factors that promote the occurrence of pancreatitis episodes in patients affected with cystic fibrosis (CF) and pancreatic sufficiency (PS) are largely unknown.Six genes involved in pancreatitis or in ion transport into the pancreatic duct were investigated by next generation sequencing in 59 adult CF-PS patients with two identified CF mutations. Data on predisposing environmental factors were also recorded.19 experienced at least one episode of acute pancreatitis (AP) (AP+) and 40 patients did not (AP-). No influence of environmental factor was evidenced. No specific CFTR genotype was found predictive of pancreatitis. Patients sharing the same CFTR genotype may or may not experience AP episodes. Frequent and rare missense variants were found in 78.9% patients in group AP+ and 67.5% in group AP- but a few of them were pathogenic.AP or recurrent AP (RAP) is a frequent complication in our series of adult CF-PS patients. The majority of mild CFTR mutations found in group AP+ were located in the first transmembrane region. No clear other genetic factor could be found predictive of AP/RAP. Further experiments in large homogenous cohorts of CF-PS patients, including whole genome sequencing, may identify genetic predisposing factors to pancreatitis.


Haferburg M.,Paris Center
ATW - Internationale Zeitschrift fur Kernenergie | Year: 2010

The World Association of Nuclear Operators (WANO) was founded in May 1989. 144 enterprises operating nuclear power plants signed the WANO Charter in Moscow as a response of industry to the Chernobyl disaster. The Association now comprises the operators of more than 430 nuclear power plants in more than 32 countries. WANO performs its activities through regional centers in Atlanta, Moscow, Paris, and Tokyo. The Coordination Center of WANO is located in London. Each regional WANO Center handles the four most important programs: - peer reviews, - exchanges of operating experience, - specialized and technical development, - technical service and exchange. The technical support and exchange program comprises proven processes, such as performance indicators, operator networks, technical support missions. WANO peer reviews are conducted on a voluntary basis and upon request by the licensees. By the end of 2008, WANO had run 388 peer reviews in 31 countries. Peer reviews serve to compare the practical operation of a nuclear power plant with the best international standards. This in-depth examination is carried out by an international, independent team of experts on an optimized objective basis. Peer reviews are conducted not only to examine compliance with all pertinent rules and regulations, but also to strive for excellent performance results.


Lefebvre A.,Paris Center | Rabbat A.,Paris Center
Reanimation | Year: 2015

Pulmonary complications are common in immunocompromised (IC) patients. These respiratory complications can lead to acute respiratory failure (ARF), which is the first reason for the patient being admitted in ICU. Noninvasive mechanical ventilation (NIV) can be applied in selected IC patients, with mild-to-moderately severe ARF. NIV with pressure support and positive expiratory pressure improves oxygenation, reduces nosocomial infection rates and prevents tracheal intubation. The survival benefit related to the use of NIV in IC patients with ARF is suggested by retrospective studies, prospective cohort studies and a few randomized controlled studies. These randomized studies were performed more than 10 years ago and they included only a limited number of patients, and therefore, their results are questionable. Furthermore, several studies suggest that intubation after failure of initial NIV is associated with excess mortality. Significant changes in the management of critically ill patients with ARF have been made during the recent years with the improvement in patient’s outcomes. Increased survival is also reported in IC patients managed with invasive mechanical ventilation (MV). In IC patients with ARF, NIV is not an alternative to intubation and invasive MV. An initial trial of NIV in IC patients with mildto- moderate ARF is reasonable with a close monitoring in an ICU. In patients showing no early (i.e., within 2 hours) clinical and arterial blood gases improvement with NIV, invasive MV should be considered. In such patients who do not respond to NIV, with hypoxemic ARF, tracheal intubation should not be delayed and invasive mechanical ventilation should be applied with lung-protective settings. During the postoperative period, NIV is useful to treat or to prevent an ARF. NIV enables to perform a bronchoscopy with bronchoalveolar lavage in patients with moderate hypoxemia and pulmonary infiltrates without diagnosis. Finally, NIV may be proposed in the context of therapeutic limitations with varying results depending on the context. © 2015, Société de réanimation de langue française (SRLF) and Springer-Verlag France.


Haferburg M.,Paris Center
ATW - Internationale Zeitschrift fur Kernenergie | Year: 2011

In the wake of the accident at the Soviet RBMK reactor unit 4 in Chernobyl the nuclear industry founded the World Association of Nuclear Operators (WANO). To this day, the purpose of the organization has been to enhance worldwide cooperation of nuclear industry and, in this way, strengthen the safety and availability of nuclear power plants. Following some first steps after 1986, the charter of the organization was signed at the WANO constituent assembly in Moscow on May 15 and 16, 1989. The member companies thus committed themselves to support WANO's mission. WANO was established for these purposes: "The mission of WANO is to maximize the safety and reliability of nuclear power plants worldwide by working together to assess, benchmark and improve performance through mutual support, exchange of information, and emulation of best practices." The WANO programs developed speedily thereafter. The focus was on peer reviews. In 2000, the first interim objective had been reached: Fifty percent of all member nuclear power plants had undergone peer reviews. In addition, plant-related peer reviews were extended throughout all operator organizations, and corporate peer reviews were developed. The other WANO programs as well, i.e. exchanges of experience, technical support, and performance indicators, exerted more and more influence on industry. Peer reviews covered entire operator organizations, and corporate peer reviews were developed. The worldwide paradigm shift in evaluating the use of nuclear power, and the associated construction programs for new nuclear power plants already in their implementation phase, assigned a new quality to the work of WANO. The organization is preparing a longterm strategy in the face of the challenges to be expected. The ultimate objective of these efforts is to support member organizations from the first preparations of a nuclear power plant project to the end of commercial operation.

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