1,555 research outputs found

    Relationship between ankle brachial index and arterial remodeling in pseudoxanthoma elasticum

    Get PDF
    ObjectivesPseudoxanthoma elasticum (PXE) is an inherited metabolic disease characterized by elastic fiber fragmentation and calcification in the cutaneous, ophthalmologic, and vascular tissues. Cardiovascular manifestations such as peripheral arterial disease (PAD) are frequent in PXE. Because of the changes in the elastic properties and medial calcification of the arterial wall in PXE, the impact of the arterial remodeling on the ankle brachial index (ABI), a well-established diagnostic method for the detection and follow-up of PAD, remains to be determined in this disease. Methods This was a cross-sectional, comparative, open study, which took place at the PXE Consultation Center, University Hospital of Angers. The subjects were 53 patients (mean age, 49 ± 14 years; 35 females) with PXE clinically proven on the basis of established criteria (skin changes, angioid streaks, and skin biopsy). The ABI at rest, symptoms of intermittent claudication (IC), carotid intima-media thickness (IMT), carotid-femoral pulse wave velocity (c-f PWV), compliance (CC), and ÎČ stiffness index were measured in a single-center cohort. Results Forty-five percent of the PXE patients had an ABI ≀0.90, but only one patient had an ABI >1.40. IC was found in 23% of the patients with an ABI ≀0.90. There were no significant differences between the patients with a low and normal ABI in terms of IMT (P = .566) or ÎČ stiffness index (P = .194), but differences were significant for c-f PWV (P = .010) and CC (P = .011). Adjusted multivariate linear regression for the Framingham-Laurier score showed that patients with a low ABI had less compliant carotid arteries (B = 0.318, P = .039). Conclusions PAD detected by a low ABI is very frequent in PXE, although with limited prevalence of symptomatic claudication. Unexpectedly, ABI was low in such calcifying PAD and associated with lower CC, independently of atherosclerosis risk factors. These findings demonstrate that PXE represents a unique monogenic model of PAD in which the specific arterial wall remodeling could change the diagnostic value of the ABI to detect PAD

    Modeling open-flow steam reforming of methanol over Cu/ZnO/Al2O3 catalyst in an axisymmetric reactor for hydrogen production

    Get PDF
    This paper describes a CFD study of the steam-reforming process (SRP) of methanol in a short pseudo-contact time reactor of fixed bed type, in axi-symmetric conditions. The SRP is important sake for hydrogen production, and the design /scale-up/control of the industrial processes in the future are supported by a reliable knowledge and prediction of the catalytic reaction. The difficulty of determining the reaction scheme and the associated constants is well-known, due to the necessity of identifying the reaction kinetics in purely chemical regime, meaning with a perfect homogeneity and flow independence. Practically these ideal conditions, albeit assumed, are not fulfilled so that the intrinsic chemical kinetics is not reached. For the case of SRP, we have attempted here to validate the Peppley’s model by a numerical modelling reproducing exactly the local conditions in the experimental duct, accounting for gradients in the cross section. The numerical results show the same trends than the experimental one, but with a slight shift of 20% as a consequence of the reactor heterogeneity. This result seems acceptable to validate the use of the Peepley’s model for further studies in other types of complex flow reactors

    Local and global Fokker-Planck neoclassical calculations showing flow and bootstrap current modification in a pedestal

    Full text link
    In transport barriers, particularly H-mode edge pedestals, radial scale lengths can become comparable to the ion orbit width, causing neoclassical physics to become radially nonlocal. In this work, the resulting changes to neoclassical flow and current are examined both analytically and numerically. Steep density gradients are considered, with scale lengths comparable to the poloidal ion gyroradius, together with strong radial electric fields sufficient to electrostatically confine the ions. Attention is restricted to relatively weak ion temperature gradients (but permitting arbitrary electron temperature gradients), since in this limit a delta-f (small departures from a Maxwellian distribution) rather than full-f approach is justified. This assumption is in fact consistent with measured inter-ELM H-Mode edge pedestal density and ion temperature profiles in many present experiments, and is expected to be increasingly valid in future lower collisionality experiments. In the numerical analysis, the distribution function and Rosenbluth potentials are solved for simultaneously, allowing use of the exact field term in the linearized Fokker-Planck collision operator. In the pedestal, the parallel and poloidal flows are found to deviate strongly from the best available conventional neoclassical prediction, with large poloidal variation of a different form than in the local theory. These predicted effects may be observable experimentally. In the local limit, the Sauter bootstrap current formulae appear accurate at low collisionality, but they can overestimate the bootstrap current near the plateau regime. In the pedestal ordering, ion contributions to the bootstrap and Pfirsch-Schluter currents are also modified

    The scale of population structure in Arabidopsis thaliana

    Get PDF
    The population structure of an organism reflects its evolutionary history and influences its evolutionary trajectory. It constrains the combination of genetic diversity and reveals patterns of past gene flow. Understanding it is a prerequisite for detecting genomic regions under selection, predicting the effect of population disturbances, or modeling gene flow. This paper examines the detailed global population structure of Arabidopsis thaliana. Using a set of 5,707 plants collected from around the globe and genotyped at 149 SNPs, we show that while A. thaliana as a species self-fertilizes 97% of the time, there is considerable variation among local groups. This level of outcrossing greatly limits observed heterozygosity but is sufficient to generate considerable local haplotypic diversity. We also find that in its native Eurasian range A. thaliana exhibits continuous isolation by distance at every geographic scale without natural breaks corresponding to classical notions of populations. By contrast, in North America, where it exists as an exotic species, A. thaliana exhibits little or no population structure at a continental scale but local isolation by distance that extends hundreds of km. This suggests a pattern for the development of isolation by distance that can establish itself shortly after an organism fills a new habitat range. It also raises questions about the general applicability of many standard population genetics models. Any model based on discrete clusters of interchangeable individuals will be an uneasy fit to organisms like A. thaliana which exhibit continuous isolation by distance on many scales

    Projectile Coulomb excitation with fast radioactive beams

    Get PDF
    5 pages, 5 figures, 1 table.-- PACS nrs.: 23.20.Ck; 27.20.+n.We report a search for γ rays emanating from Coulomb excitation of fast (30-46 MeV/u) radioactive projectiles He-8, Be-11, Be-12, Be-14 interacting with a lead target. These are clearly identified by their Doppler shift. The 320 keV 1/2(-) --> 1/2(+)γ transition from Be-11 was observed with a cross-section of 191 ± 26 mb which is noticeably less than expected from the known lifetime and in the perturbation limit of pure Coulomb excitation. In the other nuclei rather stringent upper limits of 0.01 to 0.2 Weisskopf units, are placed on the hypothetical transition to 1(-) states.We would like to thank F. Geoffroy, R. Hue and L. Petizon for their technical assistance during the experiment, N. Alamanos, G. Baur aud C. Bertulani for discussions and R. Lombard for drawing our attention to the Bertlmann-Martin bound. This work was partly supported by la Région Basse Normandie. One of us, G. Schrieder, would like to thank for the support by the German Federal Minister for Research and Technology (BMFT) under contract 06DA641.Peer reviewe

    Quantification of the Calcification Phenotype of Abcc6-Deficient Mice with Microcomputed Tomography

    Get PDF
    Pseudoxanthoma elasticum in humans and dystrophic cardiac calcification in mice are heritable disorders characterized by dystrophic calcification of soft connective tissues related to the defective function of the ABCC6 (human)/Abcc6 (mouse) transporter. Of particular interest is the finding of calcified vibrissae in Abcc6−/− mice, which facilitates the study of dystrophic calcification by histological techniques. We aimed to determine whether mice prone to dystrophic cardiac calcification (C3H/HeOuJ and DBA/2J strains) presented similar vibrissae changes and to evaluate the value of microcomputed tomography to quantify the extent of mystacial vibrissae calcifications. These calcifications were absent in DBA/2J and C57BL/6J control mice. In both Abcc6−/− and C3H/HeOuJ mice, calcifications progressed in a caudal-rostral direction with aging. However, the calcification process was delayed in C3H/HeOuJ mice, indicating an incomplete expression of the calcification phenotype. We also found that the calcification process in the cephalic region was not limited to mystacial vibrissae but was also present in other periorbital sensorial vibrissae. The vibrissae calcification was circular and encompassed the medial region of the vibrissae capsule, adjacent to the ring and cavernous sinuses (the areas adjacent to blood and lymphatic vessels). Collectively, our findings confirm that Abcc6 acts as an inhibitor of spontaneous chronic mineralization and that microcomputed tomography is a valuable noninvasive tool for the assessment of the calcification phenotype in Abcc6-deficient mice

    High genetic diversity at the extreme range edge: nucleotide variation at nuclear loci in Scots pine (Pinus sylvestris L.) in Scotland

    Get PDF
    Nucleotide polymorphism at 12 nuclear loci was studied in Scots pine populations across an environmental gradient in Scotland, to evaluate the impacts of demographic history and selection on genetic diversity. At eight loci, diversity patterns were compared between Scottish and continental European populations. At these loci, a similar level of diversity (Ξsil=~0.01) was found in Scottish vs mainland European populations, contrary to expectations for recent colonization, however, less rapid decay of linkage disequilibrium was observed in the former (ρ=0.0086±0.0009, ρ=0.0245±0.0022, respectively). Scottish populations also showed a deficit of rare nucleotide variants (multi-locus Tajima's D=0.316 vs D=−0.379) and differed significantly from mainland populations in allelic frequency and/or haplotype structure at several loci. Within Scotland, western populations showed slightly reduced nucleotide diversity (πtot=0.0068) compared with those from the south and east (0.0079 and 0.0083, respectively) and about three times higher recombination to diversity ratio (ρ/Ξ=0.71 vs 0.15 and 0.18, respectively). By comparison with results from coalescent simulations, the observed allelic frequency spectrum in the western populations was compatible with a relatively recent bottleneck (0.00175 × 4Ne generations) that reduced the population to about 2% of the present size. However, heterogeneity in the allelic frequency distribution among geographical regions in Scotland suggests that subsequent admixture of populations with different demographic histories may also have played a role

    Abrupt GaP/Si hetero-interface using bistepped Si buffer

    Get PDF
    We evidence the influence of the quality of the starting Si surface on the III-V/Si interface abruptness and on the formation of defects during the growth of III-V/Si heterogeneous crystal, using high resolution transmission electron microscopy and scanning transmission electron microscopy. GaP layers were grown by molecular beam epitaxy on vicinal Si (001). The strong effect of the Si substrate chemical preparation is first demonstrated by studying structural properties of both Si homoepitaxial layer and GaP/Si heterostructure. It is then shown that choosing adequate chemical preparation conditions and subsequent III-V regrowth conditions enables the quasi-suppression of micro-twins in the epilayer. Finally, the abruptness of GaP/Si interface is found to be very sensitive to the Si chemical preparation and is improved by the use of a bistepped Si buffer prior to III-V overgrowth
    • 

    corecore