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81.
Pycnodysostosis, a rare autosomal recessive skeletal dysplasia, is caused by a deficiency of cathepsin K. Patients have impaired bone resorption in the presence of normal or increased numbers of multinucleated, but dysfunctional, osteoclasts. Cathepsin K degrades collagen type I and generates N-telopeptide (NTX) and the C-telopeptide (CTX) that can be quantified. Levels of these telopeptides are increased in lactating women and are associated with increased bone resorption. Nothing is known about the consequences of cathepsin K deficiency in lactating women. Here we present for the first time normalized blood and CTX measurements in a patient with pycnodysostosis, exclusively related to the lactation period. In vitro studies using osteoclasts derived from blood monocytes during lactation and after weaning further show consistent bone resorption before and after lactation. Increased expression of cathepsins L and S in osteoclasts derived from the lactating patient suggests that other proteinases could compensate for the lack of cathepsin K during the lactation period of pycnodysostosis patients.  相似文献   
82.
83.
Infants born after intrauterine growth restriction (IUGR) are at risk of developing arterial hypertension at adulthood. The endothelium plays a major role in the pathogenesis of hypertension. Endothelial colony-forming cells (ECFCs), critical circulating components of the endothelium, are involved in vasculo-and angiogenesis and in endothelium repair. We previously described impaired functionality of ECFCs in cord blood of low-birth-weight newborns. However, whether early ECFC alterations persist thereafter and could be associated with hypertension in individuals born after IUGR remains unknown. A rat model of IUGR was induced by a maternal low-protein diet during gestation versus a control (CTRL) diet. In six-month-old offspring, only IUGR males have increased systolic blood pressure (tail-cuff plethysmography) and microvascular rarefaction (immunofluorescence). ECFCs isolated from bone marrow of IUGR versus CTRL males displayed a decreased proportion of CD31+ versus CD146+ staining on CD45− cells, CD34 expression (flow cytometry, immunofluorescence), reduced proliferation (BrdU incorporation), and an impaired capacity to form capillary-like structures (Matrigel test), associated with an impaired angiogenic profile (immunofluorescence). These dysfunctions were associated with oxidative stress (increased superoxide anion levels (fluorescent dye), decreased superoxide dismutase protein expression, increased DNA damage (immunofluorescence), and stress-induced premature senescence (SIPS; increased beta-galactosidase activity, increased p16INK4a, and decreased sirtuin-1 protein expression). This study demonstrated an impaired functionality of ECFCs at adulthood associated with arterial hypertension in individuals born after IUGR.  相似文献   
84.
Between 20 to 25% of Crohn’s disease (CD) patients suffer from perianal fistulas, a marker of disease severity. Seton drainage combined with anti-TNFα can result in closure of the fistula in 70 to 75% of patients. For the remaining 25% of patients there is room for in situ injection of autologous or allogenic mesenchymal stem cells such as adipose-derived stem/stromal cells (ADSCs). ADSCs exert their effects on tissues and effector cells through paracrine phenomena, including the secretome and extracellular vesicles. They display anti-inflammatory, anti-apoptotic, pro-angiogenic, proliferative, and immunomodulatory properties, and a homing within the damaged tissue. They also have immuno-evasive properties allowing a clinical allogeneic approach. Numerous clinical trials have been conducted that demonstrate a complete cure rate of anoperineal fistulas in CD ranging from 46 to 90% of cases after in situ injection of autologous or allogenic ADSCs. A pivotal phase III-controlled trial using allogenic ADSCs (Alofisel®) demonstrated that prolonged clinical and radiological remission can be obtained in nearly 60% of cases with a good safety profile. Future studies should be conducted for a better knowledge of the local effect of ADSCs as well as for a standardization in terms of the number of injections and associated procedures.  相似文献   
85.
The Sacramento-San Joaquin Delta is the major drinking water source for 23 million California residents. Consequently, many studies have examined disinfection byproduct (DBP) formation in relation to Delta dissolved organic carbon (DOC) concentration. However, DOC characteristics within the Delta are not the same as those entering downstream water treatment facilities. As water is transferred to Southern California through the California Aqueduct, a 714.5 km-open channel, it is exposed to sunlight, potentially altering DBP precursors. We collected water from three sites within the Delta and one nearthe California Aqueduct, representing different DOC sources, and irradiated them in a solar simulator at a dose equivalent to that received during four days conveyance in the aqueduct. Photolytic changes in DOC were assessed by measuring CO2 and organic acid production, fluorescence, and ultraviolet absorbance over time. Trihalomethane (THM) and haloacetic acid (HAA) formation potentials, as well as the distribution of hydrophobic, transphilic, and hydrophilic acid fractions were determined at exposures equivalent to one and four days. Solar irradiation significantly decreased ultraviolet absorbance and fluorescence intensity, produced organic acids, and increased the hydrophilic fraction of waters. These changes in DOC caused a shift in bromine incorporation among the THM and HAA species. Our results are the first to demonstrate the importance of sunlight in altering DOC with respect to DBP formation.  相似文献   
86.
This paper presents initial values of global and diffuse luminous efficacy at Saint-Pierre (Reunion Island). Firstly, data used were measured for a period of 6 months, from February to June 2008. During this period, all defined day-types have been studied (clear, cloudy and intermediate). Generally, the meteorological database of Reunion does not contain information for illuminance values. On the other hand, the local meteorological center has a 60-years-old database for solar irradiance (W/m2). So it is important to determine Luminous Efficacy in order to find illuminance from solar irradiance (or luminance from solar radiance). The measured data were analyzed, and empirical constant models were developed and presented in this paper in order to determine luminous efficacy under different sky conditions. A comparison between these empirical constants (models) and existing models has been made. The method used to define sky conditions (overcast, intermediate and clear) and day-types characterization as well as classification will be presented in this work.  相似文献   
87.
The objective of the present work was to understand how the structural, surface, water vapour and gas barrier properties of wheat gluten (WG) coated paper could be influenced by the features of paper. For this purpose, a surface treated paper (TP) and an untreated paper (UTP) were compared. Penetration of WG-coating into the bulk of paper was more pronounced in UTP than TP. This led to the formation of a significant junction zone resulting in an interpenetrated structure for WG–UTP, suggesting a composite-like structure, while a bi-layer one was obtained for WG–TP. Differences in WG penetration were related to the hydrophilicity, roughness and porosity of the paper used. Even though the extent of penetration did not greatly affect the surface properties (water and oil resistance), the transfer properties (water vapour, O2 and CO2) of WG-coated papers were significantly improved when WG-coating highly penetrated: while the WG–TP behaved as a micro-perforated material, the WG–UTP behaved as WG-film.  相似文献   
88.
The context of ornamental horticulture is considered in order to extend the techniques of sensory and preference evaluation by taking the rosebush as a plant model. In a preliminary study (Boumaza, Demotes-Mainard, Huché-Thélier, & Guérin, 2009), a sensory evaluation was conducted in order to set up a list of attributes. Subsequently, this list was adapted to assess 10 rosebushes. After the control of the panel performance using a multivariate strategy of analysis, the average scores were used in product mapping. The evaluation of the preferences with regard to these rosebushes was undertaken: 253 subjects were asked to rank the products by decreasing order of liking. Thereafter, the preference data were subjected to an internal preference mapping and a cluster analysis. Six homogeneous segments of consumers were eventually retained. By way of performing an external preference mapping, the average ranks were regressed upon the sensory attributes using principal component regression: the preferences of 67% of the consumers were satisfactorily explained by the attributes.  相似文献   
89.
ABSTRACT

Energetic materials are often disposed by open-burning or open-detonation as it is a cost-effective and efficient means of destroying explosive material, and often minimizes the need to transport hazardous explosives to treatment facilities. This practice is often scrutinized for the negative environmental impact of the odorous and unsightly toxic gaseous emissions as well as the resulting deposition residues, which often contain unburned energetic materials. With the increasing use of Insensitive High Explosive compositions in munitions, it is essential that the potential environmental impact of their disposal is assessed before their extensive use to prevent the kind of contamination incidents experienced with legacy explosives. Therefore, the aim of this work was to develop a controlled laboratory experiment to identify the gaseous emissions and the energetic material residues that are generated through the combustion of the IHE components 3-nitro-1,2,4-triazol-5-one (NTO), 2,4-dinitroanisole (DNAN), and 1,3,5-trinitroperhydro-1,3,5-triazine (RDX). A sealed vial containing small (mg) quantities of energetic material was heated until the energetic material combusted. Gas chromatography/mass spectrometry (GCMS) was used to calculate the oxygen consumption and to identify the gases that were generated. The solid residues were analyzed by high-performance liquid chromatography (HPLC) to quantify unburned energetic material. Results showed that DNAN was the most resistant to burning, thus leaving significant quantities of unreacted starting material in the vial. An interesting observation for the IHE formulation was that DNAN also inhibited the combustion of NTO and RDX. The gases emitted during the open burning of IHE components and mixtures included CO, CO2, and N2O as expected, but the proportions differed when the components and mixture were compared, reflecting the influence of DNAN on the burning behavior. From our data, we concluded that open-burning DNAN-based formulations is an environmentally unfavorable waste-management practice for the disposal of IHEs mainly due to generation of solid residues as well as unburnt DNAN.  相似文献   
90.
Obtaining quantitative data concerning the relative impact of various factors that may influence bacterial growth is of great importance for microbial risk assessment and predictive microbiology. The objective of this work was to investigate the effect of the initial Listeria monocytogenes density on all the growth parameters of this pathogen (lag phase duration, growth rate and maximum population density attained) on a sterile solid model system mimicking smoked fishery products, and in real cold-smoked salmon, a product likely to be contaminated with L. monocytogenes. Growth of the pathogen was monitored using a sensitive enumeration method, recently developed, based on membrane filtration followed by the transfer of the filter on a selective media [Gnanou Besse, N., Audinet, N., Beaufort, A., Colin, P., Cornu, M. and Lombard, B., 2004. A contribution to the improvement of Listeria monocytogenes enumeration in smoked salmon. International Journal of Food Microbiology, 91, 119-127.]. Depending on the experimental conditions, we found a significant effect of the inoculum size, both on lag phase duration, and on the maximal population attained. Moreover, the effect of the inoculum size on the growth of L. monocytogenes was dependent on a complex set of interactions. Factors which have appeared to impact on this effect include the cells physiological state, the background microflora, the texture of the media and the packaging system. It is important to understand how these interactions affect the growth of Listeria in order to predict and control its development in food.  相似文献   
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