946 resultados para Michel de


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Background: Hepatorenal tyrosinaemia (Tyr 1) is a rare inborn error of tyrosine metabolism. Without treatment, patients are at high risk of developing acute liver failure, renal dysfunction and in the long run hepatocellular carcinoma. The aim of our study was to collect cross-sectional data. Methods. Via questionnaires we collected retrospective data of 168 patients with Tyr 1 from 21 centres (Europe, Turkey and Israel) about diagnosis, treatment, monitoring and outcome. In a subsequent consensus workshop, we discussed data and clinical implications. Results: Early treatment by NTBC accompanied by diet is essential to prevent serious complications such as liver failure, hepatocellular carcinoma and renal disease. As patients may remain initially asymptomatic or develop uncharacteristic clinical symptoms in the first months of life newborn mass screening using succinylacetone (SA) as a screening parameter in dried blood is mandatory for early diagnosis. NTBC-treatment has to be combined with natural protein restriction supplemented with essential amino acids. NTBC dosage should be reduced to the minimal dose allowing metabolic control, once daily dosing may be an option in older children and adults in order to increase compliance. Metabolic control is judged by SA (below detection limit) in dried blood or urine, plasma tyrosine (<400 μM) and NTBC-levels in the therapeutic range (20-40 μM). Side effects of NTBC are mild and often transient. Indications for liver transplantation are hepatocellular carcinoma or failure to respond to NTBC. Follow-up procedures should include liver and kidney function tests, tumor markers and imaging, ophthalmological examination, blood count, psychomotor and intelligence testing as well as therapeutic monitoring (SA, tyrosine, NTBC in blood). Conclusion: Based on the data from 21 centres treating 168 patients we were able to characterize current practice and clinical experience in Tyr 1. This information could form the basis for clinical practice recommendations, however further prospective data are required to underpin some of the recommendations.

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SCOPUS: NotDefined.j

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The NOMAD spectrometer suite on the ExoMars Trace Gas Orbiter will map the composition and distribution of Mars atmospheric trace species in unprecedented detail, fulfilling many of the scientific objectives of the joint ESA-Roscosmos ExoMars Trace Gas Orbiter mission. The instrument is a combination of three channels, covering a spectral range from the UV to the IR, and can perform solar occultation, nadir and limb observations. In this paper, we present the science objectives of the instrument and how these objectives have influenced the design of the channels. We also discuss the expected performance of the instrument in terms of coverage and detection sensitivity.

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This paper describes the status circa 2001, of the HITRAN compilation that comprises the public edition available through 2001. The HITRAN compilation consists of several components useful for radiative transfer calculation codes: high-resolution spectroscopic parameters of molecules in the gas phase, absorption cross-sections for molecules with very dense spectral features, aerosol refractive indices, ultraviolet line-by-line parameters and absorption cross-sections, and associated database management software. The line-by-line portion of the database contains spectroscopic parameters for 38 molecules and their isotopologues and isotopomers suitable for calculating atmospheric transmission and radiance properties. Many more molecular species are presented in the infrared cross-section data than in the previous edition, especially the chlorofluorocarbons and their replacement gases. There is now sufficient representation so that quasi-quantitative simulations can be obtained with the standard radiance codes. In addition to the description and justification of new or modified data that have been incorporated since the last edition of HITRAN (1996), future modifications are indicated for cases considered to have a significant impact on remote-sensing experiments. © 2003 Elsevier Ltd. All rights reserved.

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Collection :Europto series, 6

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La utilización de una herramienta nueva, de cualquier tipo que sea, necesita de una reflexión sobre lo que hacemos, muchas veces cambia nuestro modo de trabajar (actitud) y hace surgir problemas sobre las verdades que teníamos. En matemática los conocimientos utilizados pueden ser diferentes: comparar una construcción geométrica con regla y compás o con regla y escuadra (mecánica) o solamente con compás. En este curso se explora de manera activa el software Cabri II. En una primera etapa se realiza la construcción de triángulos -sus elementos secundarios- y circunferencias inscritas y circunscritas así como exploraciones de simetría. En una segunda etapa se elaboran macro construcciones o construcciones que podemos grabar, para luego reutilizar en figuras más complejas, sin necesidad de rehacerlas. A través de la exploración ya descrita se reflexiona sobre el aporte de esta herramienta al quehacer pedagógico y/o científico. El uso del software es muy cercano a la forma de pensar en la geometría clásica, lo que permite a los estudiantes acercarse a esta disciplina y hacer conjeturas. Corresponde advertir que, como Cabri II no es un software de dibujo ni de demostración sino que está basado en un ambiente numérico, hay errores de aproximación. aunque leves. Se inicia el curso explicando brevemente el funcionamiento del software Cabri II para pasar a realizar actividades de construcción y comprobación de relaciones geométricas.

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Given the importance of occupant behavior on evacuation efficiency, a new behavioral feature has been implemented into building EXODUS. This feature concerns the response of occupants to exit selection and re-direction, given that the occupant is queuing at an external exit. This behavior is not simply pre-determined by the user as part of the initialization process, but involves the occupant taking decisions based on their previous experiences with the enclosure and the information available to them. This information concerns the occupant's prior knowledge of the enclosure and line-of-sight information concerning queues at neighboring exits. This new feature is demonstrated and reviewed through several examples.

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In this paper we present some work concerned with the development and testing of a simple solid fuel combustion model incorporated within a Computational Fluid Dynamics (CFD) framework. The model is intended for use in engineering applications of fire field modeling and represents an extension of this technique to situations involving the combustion of solid fuels. The CFD model is coupled with a simple thermal pyrolysis model for combustible solid noncharring fuels, a six-flux radiation model and an eddy-dissipation model for gaseous combustion. The model is then used to simulate a series of small-scale room fire experiments in which the target solid fuel is polymethylmethacrylate. The numerical predictions produced by this coupled model are found to be in very good agreement with experimental data. Furthermore, numerical predictions of the relationship between the air entrained into the fire compartment and the ventilation factor produce a characteristic linear correlation with constant of proportionality 0.38 kg/sm5/12. The simulation results also suggest that the model is capable of predicting the onset of "flashover" type behavior within the fire compartment.

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The reaction of the five- or six-membered C,N or C,S-palladacycles [(L)PdCl](2) with PTA (1,3,5-triaza-7-phosphaadamantane) led to the monomeric complexes [(L)Pd(PTA)Cl] 6a, 6b and 7 where LH= N,N-dimethyl-1-phenylmethanamine, benzyl(methyl)sulfane or 1-methyl-5-phenyl-1H-benzo[e][1,4]diazepin-2(3H)-one respectively. Dimeric complexes have also been synthesised: [Pd(2)L(2)(mu-dppe)Cl(2)], where LH = 1-methyl-5-phenyl-1H-benzo[e][1,4]diazepin-2(3H)-one (1a), (R)- or (S)-3-isopropyl-1-methyl-5-phenyl-1H-benzo[e][1,4]diazepin-2(3H)-one (1b, 1c), [Pd(2)L(2)(mu-dppf)Cl(2)], where L= 1-methyl-5-phenyl-1H-benzo[e][1,4]diazepin-2(3H)-one (4a) or (R)-3-isopropyl-1-methyl-5-phenyl-1H-benzo[e][1,4]diazepin-2(3H)-one (4b), respectively, and dppe = 1,2-bis(diphenylphosphino)ethane, dppf = 1,1'-bis(diphenylphosphino)ferrocene. The complexes were characterised in solution, by (1)H and (31)P NMR spectroscopy, and single crystals of complexes 6b and 7 were studied in the solid state by X-ray crystallography. The palladacycles were evaluated for in vitro activity as cytotoxic agents on A2780/S cells and also as cathepsin B inhibitors, an enzyme implicated in a number of cancer related events.

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Summary: This chapter contains sections titled: * Introduction: CC Bond Formation via Cyclopalladation Reactions * Stoichiometric CH Activation Chemistry * Catalytic Chemistry * Arylations * Direct CH CH Coupling Reactions * Alkylations * Other Reactions * Conclusion * References