35 research outputs found

    Etude du comportement mécanique de matériaux composites à matrice polypropylÚne et charges minérales recyclées

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    RĂ©sumé : Dans ce travail, on s’intĂ©resse Ă  l’influence de la nature de la charge sur le comportement mĂ©canique de matĂ©riaux composites Ă  matrice polypropylĂšne homopolymĂšre (PPH). On procĂšde au rajout de charges minĂ©rales qui sont, la fibre de verre, la poudre de verre rĂ©cupĂ©rĂ©e des lampes Ă  nĂ©on usagĂ©es et de la chaux. Ces charges sont rajoutĂ©es aux concentrations de 5 et 10% Ă  la matrice PPH. Le mĂ©langeage a Ă©tĂ© effectuĂ© Ă  froid puis Ă  chaud par extrusion. L’élaboration des Ă©prouvettes a Ă©tĂ© effectuĂ©e par procĂ©dĂ© d’injection aprĂšs broyage du produit d’extrusion. La caractĂ©risation mĂ©canique des Ă©chantillons a montrĂ© que la fibre de verre engendre une amĂ©lioration du module d’élasticitĂ© aux concentrations de 5 et 10%. Pour la chaux et la poudre de verre, les variations sont relativement faibles. La contrainte Ă©lastique maximale est faiblement affectĂ©e par la fibre de verre. Par contre elle diminue pour la chaux et la poudre de verre. Concernant la rĂ©silience, on enregistre un gain de 25% pour la fibre de verre, une diminution de l’ordre de 20% pour la poudre de verre et une faible variation pour 10% de chaux. L’analyse des faciĂšs de rupture par MEB a rĂ©vĂ©lĂ© le caractĂšre fragile de la rupture par traction des diffĂ©rents mĂ©langes

    Dynamique Des ElĂ©ments Nutritifs Et Du Phytoplancton Dans Le Barrage BĂ©ni Haroun Dans l’Est AlgĂ©rien

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    In order to monitor the water quality of the Beni Haroun dam, used for the drinking water production, our research work aims to provide new scientific data on the phytoplankton population of the dam. This contribution is a qualitative and quantitative investigation of this important link in the ecosystem equilibrium. To our knowledge, this study has never been the subject of a previous one, despite many works already done on this dam. Water samples were collected monthly from January to December 2015 at five stations, spread over Beni Haroun dam. The exploitation of a database, relating to fourteen physicochemical and biotic variables, made it possible to determine: the spatiotemporal evolution of each variable, the correlations between variables, the annual variability of the algal biomass, the diversity and phytoplankton dominance

    Characteristics and management of congenital esophageal stenosis: findings from a multicenter study.

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    BACKGROUND: Congenital esophageal stenosis (CES) is a rare condition frequently associated with esophageal atresia (EA). There are limited data from small series about the presentation, treatment, and outcomes of CES. METHODS: Medical records of all patients with CES included in the French Network on Esophageal Malformations and Congenital Diseases were reviewed retrospectively with regard to diagnosis, treatment, and outcome. RESULTS: Over 18 years, 61 patients (30 boys) had CES, and 29 (47%) of these patients also had EA. The mean age at diagnosis was 24 months (1 day to 14 years) and was younger in patients with CES and EA than in those with isolated CES (7 vs. 126 months, p < 0.05). Twenty-one of the 61 patients with CES had no clinical symptoms: in three patients, the findings were incidental, and in 18 of the 29 patients with associated EA, CES was diagnosed at the time of surgical repair of EA or during a postoperative systematic esophageal barium study. In the 40 other patients, at diagnosis, 50% presented with dysphasia, 40% with vomiting, 50% with food impaction, and 42% with respiratory symptoms. Diagnosis of CES was confirmed by esophageal barium study (56/61) and/or esophageal endoscopy (50/61). Sixteen patients had tracheobronchial remnants (TBR), 40 had fibromuscular stenosis (FMS), and five had membrane stenosis (MS). Thirty-four patients (56%) were treated by dilation only (13/34 remained asymptomatic at follow-up); 15 patients were treated by dilation but required later surgery because of failure (4/15 remained asymptomatic at follow-up); and nine patients had a primary surgical intervention (4/9 were asymptomatic at follow-up). Dilation was complicated by esophageal perforation in two patients (3.4%). At follow-up, dysphagia remained in 36% (21/58) of patients, but the incidence did not differ between the EA and the isolated CS groups (10/29 vs. 7/32, p = 0.27). CONCLUSIONS: CS diagnosis can be delayed when associated with EA. Dilation may be effective for treating patients with FMS and MS, but surgical repair is often required for those with TBR. Our results show clearly that, regardless of the therapeutic option, dysphagia occurs frequently, and patients with CES should be followed over the long term

    Pathogenic Variants in the Myosin Chaperone UNC-45B Cause Progressive Myopathy with Eccentric Cores

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    The myosin-directed chaperone UNC-45B is essential for sarcomeric organization and muscle function from Caenorhabditis elegans to humans. The pathological impact of UNC-45B in muscle disease remained elusive. We report ten individuals with bi-allelic variants in UNC45B who exhibit childhood-onset progressive muscle weakness. We identified a common UNC45B variant that acts as a complex hypomorph splice variant. Purified UNC-45B mutants showed changes in folding and solubility. In situ localization studies further demonstrated reduced expression of mutant UNC-45B in muscle combined with abnormal localization away from the A-band towards the Z-disk of the sarcomere. The physiological relevance of these observations was investigated in C. elegans by transgenic expression of conserved UNC-45 missense variants, which showed impaired myosin binding for one and defective muscle function for three. Together, our results demonstrate that UNC-45B impairment manifests as a chaperonopathy with progressive muscle pathology, which discovers the previously unknown conserved role of UNC-45B in myofibrillar organization

    BMJ Open

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    Introduction Guidelines for the treatment of steroid-dependent nephrotic syndrome (SDNS) and frequently relapsing nephrotic syndrome (FRNS) are lacking. Given the substantial impact of SDNS/FRNS on quality of life, strategies aiming to provide long-term remission while minimising treatment side effects are needed. Several studies confirm that rituximab is effective in preventing early relapses in SDNS/FRNS; however, the long-term relapse rate remains high (~70% at 2 years). This trial will assess the association of intravenous immunoglobulins (IVIgs) to rituximab in patients with SDNS/FRNS and inform clinicians on whether IVIg’s immunomodulatory properties can alter the course of the disease and reduce the use of immunosuppressive drugs and their side effects. Methods and analysis We conduct an open-label multicentre, randomised, parallel group in a 1:1 ratio, controlled, superiority trial to assess the safety and efficacy of a single infusion of rituximab followed by IVIg compared with rituximab alone in childhood-onset FRNS/SDNS. The primary outcome is the occurrence of first relapse within 24 months. Patients are allocated to receive either rituximab alone (375 mg/mÂČ) or rituximab followed by IVIg, which includes an initial Ig dose of 2 g/kg, followed by 1.5 g/kg injections once a month for the following 5 months (maximum dose: 100 g). Ethics and dissemination The study has been approved by the ethics committee (ComitĂ© de Protection des Personnes) of Ouest I and authorised by the French drug regulatory agency (Agence Nationale de SĂ©curitĂ© du MĂ©dicament et des Produits de SantĂ©). Results of the primary study and the secondary aims will be disseminated through peer-reviewed publications

    Pathogenic Variants in the Myosin Chaperone UNC-45B Cause Progressive Myopathy with Eccentric Cores.

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    The myosin-directed chaperone UNC-45B is essential for sarcomeric organization and muscle function from Caenorhabditis elegans to humans. The pathological impact of UNC-45B in muscle disease remained elusive. We report ten individuals with bi-allelic variants in UNC45B who exhibit childhood-onset progressive muscle weakness. We identified a common UNC45B variant that acts as a complex hypomorph splice variant. Purified UNC-45B mutants showed changes in folding and solubility. In situ localization studies further demonstrated reduced expression of mutant UNC-45B in muscle combined with abnormal localization away from the A-band towards the Z-disk of the sarcomere. The physiological relevance of these observations was investigated in C. elegans by transgenic expression of conserved UNC-45 missense variants, which showed impaired myosin binding for one and defective muscle function for three. Together, our results demonstrate that UNC-45B impairment manifests as a chaperonopathy with progressive muscle pathology, which discovers the previously unknown conserved role of UNC-45B in myofibrillar organization

    The Syenite–Carbonatite Complex of Ihouhaouene (Western Hoggar, Algeria): Interplay Between Alkaline Magma Differentiation and Hybridization of Cumulus Crystal Mushes

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    International audienceThe 2 Ga-old Ihouhaouene alkaline complex (Western Hoggar, Algeria) is among the oldest known carbonatite occurrences on Earth. The carbonatites are calciocarbonatites hosted by syenites, the predominant rock type in the complex. Both rock types are characterized by medium-grained to pegmatitic textures and contain clinopyroxene, apatite, and wollastonite, associated with K-feldspar in syenites and a groundmass of calcite in carbonatites. The rock suite shows a continuous range of compositions from 57–65 wt.% SiO 2 and 0.1–0.4 wt.% CO 2 in red syenites to 52–58 wt.% SiO 2 and 0.1–6.5 wt.% CO 2 in white syenites, 20–35 wt.% SiO 2 and 11–24 wt.% CO 2 in Si-rich carbonatites (>10% silicate minerals), and <20 wt.% SiO 2 and 24–36 wt.% CO 2 in Si-poor carbonatites (<5% silicate minerals). Calculation of mineral equilibrium melts reveals that apatite and clinopyroxene are in disequilibrium with each other and were most likely crystallized from different parental magmas before being assembled in the studied rocks. They are subtle in the red syenites, whereas the white syenites and the Si-rich carbonatites bear evidence for parental magmas of highly contrasted compositions. Apatite was equilibrated with LREE-enriched (Ce/Lu = 1,690–6,182) carbonate melts, also characterized by elevated Nb/Ta ratio (>50), whereas clinopyroxene was precipitated from silicate liquids characterized by lower LREE/HREE (Ce/Lu = 49–234) and variable Nb/Ta ratios (Nb/Ta = 2–30). The Si-poor carbonatites resemble the Si-rich carbonatites and the white syenites with elevated REE contents in apatite equilibrium melts compared to clinopyroxene. However, apatite equilibrium melt in Si-poor carbonatite shows a majority of subchondritic values (Nb/Ta<10) and clinopyroxene has chondritic-to-superchondritic values (Nb/Ta = 15–50). Although paradoxical at first sight, this Nb-Ta signature may simply reflect the segregation of the carbonatite from highly evolved silicate melts characterized by extremely low Nb/Ta values. Altogether, our results suggest an evolutionary scheme whereby slow cooling of a silico-carbonated mantle melt resulted in the segregation of both cumulus minerals and immiscible silicate and carbonate melt fractions, resulting in the overall differentiation of the complex. This process was however counterbalanced by intermingling of partially crystallized melt fractions, which resulted in the formation of hybrid alkaline cumulates composed of disequilibrium cumulus phases and variable proportions of carbonate or K-feldspar

    Single crystals of mesoporous tungstenosilicate W-MCM-48 molecular sieves for the conversion of methylcyclopentane (MCP)

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    Highly ordered W-MCM-48 mesoporous materials containing isolated W atoms in tetrahedral framework positions were successfully synthesized following the S+I- pathway, up to a Si/W of 40. When tungsten content was increased up to a Si/W of 20, the ordered cubic structure was only partially maintained, and for a Si/W of 10 an amorphous phase was obtained. Highly isolated tetrahedral framework tungsten atoms in the W-MCM-48 with a Si/W of 40, have been identified by UV-vis band at 225 nm, IR-TF band at 970cm(-1) and XRD. The W 4f XPS results suggest that the tungsten atoms exist in two oxidation states, W4+ and W5+. The morphology of the samples varies as a function of tungsten content. The W-MCM-48 samples with a Si/W ratio of 40 existed as crystals with a unique crystalline morphology consisting of cubes truncated rhombic dodecahedrons belonging to the cubic Ia3d space group, while the samples with a Si/W ratio of 20 exhibited a different morphology consisting of spheres and cubes truncated by rhombic dodecahedrons. A comparison of samples with Si/W of infinity, Si/W of 40 and Si/W of 20 was performed using the conversion of MCP carried out at 450 degrees C under H-2. (C) 2010 Elsevier B. V. All rights reserved
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