End-User-Development for Smart Homes: Relevance and Challenges

International audienceUbiquitous computing is now mature enough to unleash the potential of Smart Homes. The obstacle is no more about hardware concerns but lies in how inhabitants can build, configure and control their Smart Home. In this paper, we defend the idea that End-User-Development (EUD), which considers inhabitants as makers rather than mere consumers, is an effective approach for tackling this obstacle. We reflect on the lifecycle of devices and services to discuss challenges that EUD system will have to address in the Smart Home context: installation and maintenance, designation, control, development (including programming, testing, and reusing), and sharing

In vitro prediction of stop-codon suppression by intravenous gentamicin in patients with cystic fibrosis: a pilot study

BACKGROUND: Cystic fibrosis (CF) is caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) protein, which acts as a chloride channel activated by cyclic AMP (cAMP). The most frequent mutation found in 70% of CF patients is F508del, while premature stop mutations are found in about 10% of patients. In vitro aminoglycoside antibiotics (e.g. gentamicin) suppress nonsense mutations located in CFTR permitting translation to continue to the natural termination codon. Pharmacologic suppression of stop mutations within the CFTR may be of benefit to a significant number of patients. Our pilot study was conducted to determine whether intravenous gentamicin suppresses stop codons in CF patients and whether it has clinical benefits. METHODS: A dual gene reporter system was used to determine the gentamicin-induced readthrough level of the most frequent stop mutations within the CFTR in the French population. We investigated readthrough efficiency in response to 10 mg/kg once-daily intravenous gentamicin perfusions in patients with and without stop mutations. Respiratory function, sweat chloride concentration, nasal potential difference (NPD) and CFTR expression in nasal epithelial cells were measured at baseline and after 15 days of treatment. RESULTS: After in vitro gentamicin incubation, the readthrough efficiency for the Y122X mutation was at least five times higher than that for G542X, R1162X, and W1282X. In six of the nine patients with the Y122X mutation, CFTR immunodetection showed protein at the membrane of the nasal epithelial cells and the CFTR-dependent Cl(- )secretion in NPD measurements increased significantly. Respiratory status also improved in these patients, irrespective of the gentamicin sensitivity of the bacteria present in the sputum. Mean sweat chloride concentration decreased significantly and normalised in two patients. Clinical status, NPD and sweat Cl(- )values did not change in the Y122X patients with no protein expression, in patients with the other stop mutations investigated in vitro and those without stop mutations. CONCLUSION: Suppression of stop mutations in the CFTR gene with parenteral gentamicin can be predicted in vitro and is associated with clinical benefit and significant modification of the CFTR-mediated Cl(- )transport in nasal and sweat gland epithelium

Technological developments as an answer to bridge management challenges

IABSE Symposium 2019, Towards a Resilient Built Environment - Risk and Asset Management, GUIMARAES, PORTUGAL, 27-/03/2019 - 29/03/2019Bridge management is a challenge as owners have to deal with limited financial resources to maintain the functionalities and safety of ageing structures. Demands on transportation networks change, due to regulatory developments, society's evolution and shifts with high expectations on the operational performance of roadway bridges with reduced congestion, delay, and accidents. To minimize intrusion in the transport flow, inspection and monitoring methods should be non?destructive, minimally invasive. They should be capable of yielding rapid and accurate inspection results allowing an adequate response from the asset manager. Research aims at including autonomously operating equipment (e.g. robotics), non?intrusive (remote or proximity) observation techniques, or other methods that ensure quality and performance control of the roadway bridges in time, more safely, more quickly and/or to a higher degree of accuracy and precision.The innovation subgroup in COST action TU1406 investigates novel condition monitoring and sensing technologies for the assessment of structural serviceability and safety. Advanced, integrated, cost-effective and reliable instrumentation solutions, techniques and concepts are looked at with the aim to provide data, that will be used to compute innovative performance indicators. In this context, this paper briefly reminds some significant challenges associated with bridge management and presents three examples of innovation in bridge monitoring and NDT investigation techniques

Online Monitoring of the Osiris Reactor with the Nucifer Neutrino Detector

Originally designed as a new nuclear reactor monitoring device, the Nucifer detector has successfully detected its first neutrinos. We provide the second shortest baseline measurement of the reactor neutrino flux. The detection of electron antineutrinos emitted in the decay chains of the fission products, combined with reactor core simulations, provides an new tool to assess both the thermal power and the fissile content of the whole nuclear core and could be used by the Inter- national Agency for Atomic Energy (IAEA) to enhance the Safeguards of civil nuclear reactors. Deployed at only 7.2m away from the compact Osiris research reactor core (70MW) operating at the Saclay research centre of the French Alternative Energies and Atomic Energy Commission (CEA), the experiment also exhibits a well-suited configuration to search for a new short baseline oscillation. We report the first results of the Nucifer experiment, describing the performances of the 0.85m3 detector remotely operating at a shallow depth equivalent to 12m of water and under intense background radiation conditions. Based on 145 (106) days of data with reactor ON (OFF), leading to the detection of an estimated 40760 electron antineutrinos, the mean number of detected antineutrinos is 281 +- 7(stat) +- 18(syst) electron antineutrinos/day, in agreement with the prediction 277(23) electron antineutrinos/day. Due the the large background no conclusive results on the existence of light sterile neutrinos could be derived, however. As a first societal application we quantify how antineutrinos could be used for the Plutonium Management and Disposition Agreement.Comment: 22 pages, 16 figures - Version

Mycobacterium abscessus and Children with Cystic Fibrosis

We prospectively studied 298 patients with cystic fibrosis (mean age 11.3 years; range 2 months to 32 years; sex ratio, 0.47) for nontuberculous mycobacteria in respiratory samples from January 1, 1996, to December 31, 1999. Mycobacterium abscessus was by far the most prevalent nontuberculous mycobacterium: 15 patients (6 male, 9 female; mean age 11.9 years; range 2.5–22 years) had at least one positive sample for this microorganism (versus 6 patients positive for M. avium complex), including 10 with >3 positive samples (versus 3 patients for M. avium complex). The M. abscessus isolates from 14 patients were typed by pulsed-field gel electrophoresis: each of the 14 patients harbored a unique strain, ruling out a common environmental reservoir or person-to-person transmission. Water samples collected in the cystic fibrosis center were negative for M. abscessus. This major mycobacterial pathogen in children and teenagers with cystic fibrosis does not appear to be acquired nosocomially

The CF-CIRC study: a French collaborative study to assess the accuracy of Cystic Fibrosis diagnosis in neonatal screening

BACKGROUND: Cystic fibrosis (CF) is caused by mutations in the gene encoding for the CF transmembrane conductance regulator (CFTR) protein, which acts as a chloride channel after activation by cyclic AMP (cAMP). Newborn screening programs for CF usually consist of an immunoreactive trypsinogen (IRT) assay, followed when IRT is elevated by testing for a panel of CF-causing mutations. Some children, however, may have persistent hypertrypsinogenemia, only one or no identified CFTR gene mutation, and sweat chloride concentrations close to normal values. In vivo demonstration of abnormal CFTR protein function would be an important diagnostic aid in this situation. Measurements of transepithelial nasal potential differences (NPD) in adults accurately characterize CFTR-related ion transport. The aim of the present study is to establish reference values for NPD measurements for healthy children and those with CF aged 3 months to 3 years, the age range of most difficult-to-diagnose patients with suspected CF. The ultimate goal of our study is to validate NPD testing as a diagnostic tool for children with borderline results in neonatal screening. METHODS/DESIGN: We adapted the standard NPD protocol for young children, designed a special catheter for them, used a slower perfusion rate, and shortened the protocol to include only measurement of basal PD, transepithelial sodium (Na(+)) transport in response to the Na(+ )channel inhibitor amiloride, and CFTR-mediated chloride (Cl(-)) secretion in response to isoproterenol, a β-agonist in a Cl(- )free solution. The study will include 20 children with CF and 20 healthy control children. CF children will be included only if they carry 2 CF-causing mutations in the CFTR gene or have sweat chloride concentrations > 60 mEq/L or both. The healthy children will be recruited among the siblings of the CF patients, after verification that they do not carry the familial mutation. DISCUSSION: A preliminary study of 3 adult control subjects and 4 children older than 12 years with CF verified that the new protocol was well tolerated and produced NPD measurements that did not differ significantly from those obtained with the standard protocol. This preliminary study will provide a basis for interpreting NPD measurements in patients with suspected CF after neonatal screening. Earlier definitive diagnosis should alleviate parental distress and allow earlier therapeutic intervention and genetic counseling

MUCOVISCIDOSE ET CANCER (UNE ETUDE FAMILIALE (DES PEDIATRIE))

PARIS5-BU Méd.Cochin (751142101) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Le concept de compétence dans la formation à l'enseignement primaire au Québec. Un cadre d'analyse

This paper presents the partial frame of analysis of a three-year research (1995-1998) on the didactic competencies of future teachers and the various contributors working in preservice teacher training at the Education Department of the Sherbrooke University, Quebec. It brings out a categorization of the concept of competence which results from a critical review of the French-speaking and English- speaking scientific literature on the subject as well as from an exploraroty study carried during the first year of the research among a limited sample of subjects. Four conceptions of the concept of competence are thus brought out : the behavioral competencies, the function competencies or generic competencies, the interactive skills and the status competence.L'article présente le cadre d'analyse partiel d'une recherche triennale (1995-1998) sur les compétences didactiques des futurs enseignants et des différents intervenants œuvrant dans la formation initiale à l'enseignement à la Faculté d'éducation de l'Université de Sherbrooke (Québec). Il dégage une catégorisation du concept de compétence qui résulte d'une recension critique de la documentation scientifique francophone et anglophone sur la question, ainsi que d'une étude exploratoire menée la première année de la recherche auprès d'un échantillon restreint de sujets. Quatre conceptions du concept de compétence sont ainsi dégagées : les compétences comportementales ou compétences béhaviorales, les compétences de fonction ou compétences génériques, les compétences escientes ou interactives et la compétence-statut.Lenoir Yves, Larose François, Biron Diane, Roy Gérard-Raymond, Spallanzani Carlo. Le concept de compétence dans la formation à l'enseignement primaire au Québec. Un cadre d'analyse. In: Recherche & Formation, N°30, 1999. La gestion des compétences dans l’Éducation nationale, sous la direction de Lise Demailly et Pierre Dasté. pp. 143-163