632 research outputs found

    Morphology, dynamics and plasma parameters of plumes and inter-plume regions in solar coronal holes

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    Coronal plumes, which extend from solar coronal holes (CH) into the high corona and - possibly - into the solar wind (SW), can now continuously be studied with modern telescopes and spectrometers on spacecraft, in addition to investigations from the ground, in particular, during total eclipses. Despite the large amount of data available on these prominent features and related phenomena, many questions remained unanswered as to their generation and relative contributions to the high-speed streams emanating from CHs. An understanding of the processes of plume formation and evolution requires a better knowledge of the physical conditions at the base of CHs, in plumes and in the surrounding inter-plume regions (IPR). More specifically, information is needed on the magnetic field configuration, the electron densities and temperatures, effective ion temperatures, non-thermal motions, plume cross-sections relative to the size of a CH, the plasma bulk speeds, as well as any plume signatures in the SW. In spring 2007, the authors proposed a study on "Structure and dynamics of coronal plumes and inter-plume regions in solar coronal holes" to the International Space Science Institute (ISSI) in Bern to clarify some of these aspects by considering relevant observations and the extensive literature. This review summarizes the results and conclusions of the study. Stereoscopic observations allowed us to include three-dimensional reconstructions of plumes. Multi-instrument investigations carried out during several campaigns led to progress in some areas, such as plasma densities, temperatures, plume structure and the relation to other solar phenomena, but not all questions could be answered concerning the details of plume generation process(es) and interaction with the SW.Comment: To appear on: The Astronomy and Astrophysics Review. 72 pages, 30 figure

    Fabry in the older patient: Clinical consequences and possibilities for treatment.

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    Baseline demographic and phenotypic characteristics of patients aged ≄50years in the Fabry Outcome Survey (Shire; data extracted June 2014) were compared with younger adults to investigate potential factors influencing treatment decisions in later life. Age groups were defined using age at treatment initiation or at FOS entry for untreated patients: 18-49 (n=1344; 49.5% male; 64.6% received agalsidase alfa enzyme replacement therapy [ERT]); 50-64 (n=537; 35.4% male; 74.3% treated); 65-74 (n=137; 32.1% male; 68.6% treated); and ≄75years (n=26; 26.9% male; 50.0% treated). Successive age groups showed higher median age at first symptom and diagnosis. Median alpha-galactosidase A activity, measured as percentage activity of the midpoint of the normal range, was much greater in females than males of all groups except ≄75years (33.4% in females; 27.8% in males). Patients aged ≄75years showed greater values than patients aged 18-49years for median left ventricular mass indexed to height (62.7 vs 42.4g/m(2.7)), mean ventricular wall thickness (15.0 vs 10.0mm) and prevalence of hypertension (57.7% vs 21.8%), and lower median estimated glomerular filtration rate (Modification of Diet in Renal Disease: 65.6 vs 98.5mL/min/1.73m(2)). Larger proportions in the groups aged ≄50 exhibited cardiac and/or cerebrovascular manifestations compared with patients aged 18-49years. The smaller proportion of patients receiving ERT aged ≄75years compared with the younger groups might reflect relatively milder disease burden or physician/patient reluctance to initiate/continue ERT at this age. Further studies are needed to increase knowledge of Fabry disease and ERT in later life

    Ascending aortic remodelling in Fabry disease after long-term enzyme replacement therapy.

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    Previous cross-sectional studies reported a high prevalence of ascending aorta dilations/aneurysms in male adults with Fabry disease, independently of cardiovascular risk factors. To characterise the remodelling of the ascending aorta in classic Fabry disease under long-term enzyme replacement therapy. Diameter of the ascending aorta was measured with magnetic resonance imaging at the sino-tubular junction (STJ), and proximal (pAsAo), and distal ascending aorta (dAsAo) at baseline, and after 5 and 10 years of enzyme replacement therapy in 15 adult Fabry patients (10 males; 5 females). Over a mean follow-up of 9.5 years, the annual expansion rates measured in 10 males with Fabry disease were 0.41 ± 0.16, 0.36 ± 0.25 and 0.41 ± 0.26 mm/y at the STJ, pAsAo and dAsAo, respectively. Expansion rate at the pAsAo level in male patients was significantly higher than the expected expansion projected from theoretical normal values: 0.36 ± 0.25 vs 0.13 ± 0.05, p = 0.017. In 5 females, the annual expansion rates at the STJ, pAsAo and dAsAo were 0.14 ± 0.11, 0.21 ± 0.18 and 0.26 ± 0.24 mm/y, respectively. There was no significant difference from the projected normal expansion rate at the level of the pAsAo: 0.21 ± 0.18 vs 0.13 ± 0.04, p = 0.39. Our data suggest that the remodelling of the ascending aorta is more pronounced in male patients with Fabry disease under long-term enzyme replacement therapy compared with the progression observed in a large population study

    Unconvincing statistical and functional inferences : reply to Catmur

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    A commentary on Unconvincing support for role of mirror neurons in “action understanding”: com-mentary on Michael et al. (2014) by Catmur, C. (2014). Front. Hum

    TomograPy: A Fast, Instrument-Independent, Solar Tomography Software

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    Solar tomography has progressed rapidly in recent years thanks to the development of robust algorithms and the availability of more powerful computers. It can today provide crucial insights in solving issues related to the line-of-sight integration present in the data of solar imagers and coronagraphs. However, there remain challenges such as the increase of the available volume of data, the handling of the temporal evolution of the observed structures, and the heterogeneity of the data in multi-spacecraft studies. We present a generic software package that can perform fast tomographic inversions that scales linearly with the number of measurements, linearly with the length of the reconstruction cube (and not the number of voxels) and linearly with the number of cores and can use data from different sources and with a variety of physical models: TomograPy (http://nbarbey.github.com/TomograPy/), an open-source software freely available on the Python Package Index. For performance, TomograPy uses a parallelized-projection algorithm. It relies on the World Coordinate System standard to manage various data sources. A variety of inversion algorithms are provided to perform the tomographic-map estimation. A test suite is provided along with the code to ensure software quality. Since it makes use of the Siddon algorithm it is restricted to rectangular parallelepiped voxels but the spherical geometry of the corona can be handled through proper use of priors. We describe the main features of the code and show three practical examples of multi-spacecraft tomographic inversions using STEREO/EUVI and STEREO/COR1 data. Static and smoothly varying temporal evolution models are presented.Comment: 21 pages, 6 figures, 5 table

    On peak phenomena for non-commutative H∞H^\infty

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    A non-commutative extension of Amar and Lederer's peak set result is given. As its simple applications it is shown that any non-commutative H∞H^\infty-algebra H∞(M,τ)H^\infty(M,\tau) has unique predual,and moreover some restriction in some of the results of Blecher and Labuschagne are removed, making them hold in full generality.Comment: final version (the presentation of some part is revised and one reference added

    A novel mutation in BCS1L associated with deafness, tubulopathy, growth retardation and microcephaly

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    We report a novel homozygous missense mutation in the ubiquinol-cytochrome c reductase synthesis-like (BCS1L) gene in two consanguineous Turkish families associated with deafness, Fanconi syndrome (tubulopathy), microcephaly, mental and growth retardation. All three patients presented with transitory metabolic acidosis in the neonatal period and development of persistent renal de Toni-Debr,-Fanconi-type tubulopathy, with subsequent rachitis, short stature, microcephaly, sensorineural hearing impairment, mild mental retardation and liver dysfunction. The novel missense mutation c.142A > G (p.M48V) in BCS1L is located at a highly conserved region associated with sorting to the mitochondria. Biochemical analysis revealed an isolated complex III deficiency in skeletal muscle not detected in fibroblasts. Native polyacrylamide gel electrophoresis (PAGE) revealed normal super complex formation, but a shift in mobility of complex III most likely caused by the absence of the BCS1L-mediated insertion of Rieske Fe/S protein into complex III. These findings expand the phenotypic spectrum of BCS1L mutations, highlight the importance of biochemical analysis of different primary affected tissue and underline that neonatal lactic acidosis with multi-organ involvement may resolve after the newborn period with a relatively spared neurological outcome and survival into adulthood. Conclusion: Mutation screening for BCS1L should be considered in the differential diagnosis of severe (proximal) tubulopathy in the newborn period.Peer reviewe
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