973 research outputs found

    Spectral properties and geology of bright and dark material on dwarf planet Ceres

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    Variations and spatial distributions of bright and dark material on dwarf planet Ceres play a key role in understanding the processes that have led to its present surface composition. We define limits for bright and dark material in order to distinguish them consistently, based on the reflectance of the average surface using Dawn Framing Camera data. A systematic classification of four types of bright material is presented based on their spectral properties, composition, spatial distribution, and association with specific geomorphological features. We found obvious correlations of reflectance with spectral shape (slopes) and age; however, this is not unique throughout the bright spots. Although impact features show generally more extreme reflectance variations, several areas can only be understood in terms of inhomogeneous distribution of composition as inferred from Dawn Visible and Infrared Spectrometer data. Additional material with anomalous composition and spectral properties are rare. The identification of the composition and origin of the dark, particularly the darkest material, remains to be explored. The spectral properties and the morphology of the dark sites suggest an endogenic origin, but it is not clear whether they are more or less primitive surficial exposures or excavated subsurface but localized material. The reflectance, spectral properties, inferred composition, and geologic context collectively suggest that the bright and dark material tends to gradually change toward the average surface over time. This could be because of multiple processes, i.e., impact gardening/space weathering, and lateral mixing, including thermal and aqueous alteration, accompanied by changes in composition and physical properties such as grain size, surface temperature, and porosity (compaction).Comment: Meteoritics and Planetary Science; Dawn at Ceres special issu

    Presenting signs and patient co-variables in Gaucher disease : outcome of the Gaucher Earlier Diagnosis Consensus (GED-C) Delphi initiative

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    © 2018 The Authors. Internal Medicine Journal by Wiley Publishing Asia Pty Ltd on behalf of Royal Australasian College of Physicians.Background: Gaucher disease (GD) presents with a range of signs and symptoms. Physicians can fail to recognise the early stages of GD owing to a lack of disease awareness, which can lead to significant diagnostic delays and sometimes irreversible but avoidable morbidities. Aim: The Gaucher Earlier Diagnosis Consensus (GED-C) initiative aimed to identify signs and co-variables considered most indicative of early type 1 and type 3 GD, to help non-specialists identify ‘at-risk’ patients who may benefit from diagnostic testing. Methods: An anonymous, three-round Delphi consensus process was deployed among a global panel of 22 specialists in GD (median experience 17.5 years, collectively managing almost 3000 patients). The rounds entailed data gathering, then importance ranking and establishment of consensus, using 5-point Likert scales and scoring thresholds defined a priori. Results: For type 1 disease, seven major signs (splenomegaly, thrombocytopenia, bone-related manifestations, anaemia, hyperferritinaemia, hepatomegaly and gammopathy) and two major co-variables (family history of GD and Ashkenazi-Jewish ancestry) were identified. For type 3 disease, nine major signs (splenomegaly, oculomotor disturbances, thrombocytopenia, epilepsy, anaemia, hepatomegaly, bone pain, motor disturbances and kyphosis) and one major co-variable (family history of GD) were identified. Lack of disease awareness, overlooking mild early signs and failure to consider GD as a diagnostic differential were considered major barriers to early diagnosis. Conclusion: The signs and co-variables identified in the GED-C initiative as potentially indicative of early GD will help to guide non-specialists and raise their index of suspicion in identifying patients potentially suitable for diagnostic testing for GD.Peer reviewedFinal Published versio

    The cognitive profile of type 1 Gaucher disease patients

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    This is the final version of the article. Available from the publisher via the DOI in this record.BACKGROUND: The absence of neurological symptoms and signs is traditionally considered mandatory for a diagnosis of type 1 Gaucher disease (GD1), but in recent years many reports have emerged on neurological manifestations in GD1 patients. Nevertheless, it has been unclear whether cognitive deficits are part of the disease as well. METHODS: Cognitive function was assessed in a large cohort of GD1 patients with the use of the CDR system, a set of computerised cognitive tests. Testing was performed at baseline and every 6 months thereafter during a two-year study period. RESULTS: Our patient cohort (84 patients, median age 40 years, median time from diagnosis 15 years) showed mild deficits relative to healthy age-matched subjects on the composite scores: power of attention (Z-score (mean ± SD) -0.9 ± 1.37) and speed of memory (Z-score (mean ± SD) -1.39 ± 1.49). No decline in cognitive function was seen during the two-year period. Age correlated with the composite scores variability of attention and quality of working memory. Moreover, severely affected patients (Zimran severity score (SSI) ≄ 15) scored more poorly compared to mildly affected patients (SSI ≀ 5) on the composite measure power of attention, reflecting the ability to concentrate. CONCLUSIONS: GD1 patients exhibit mild deficits in power of attention and speed of memory, reflecting a decreased ability to focus attention and process information, together with a slowing in the speed of retrieval of items from memory. The clinical relevance of these findings is uncertain.This work was supported by Actelion Pharmaceuticals Ltd, Allschwil, Switzerland. This study was set up under the auspices of the European Working Group on Gaucher Disease (EWGGD). MB received financial support from Actelion to conduct her activities related to this study. MB, CEMH, INvS and AM have received consultancy fees from Actelion for participation in clinical trial programs and other projects, and CEMH, INvS and AM have received speaker fees for participation in scientific congresses and sponsored events. MB and CEMH donate all fees to the Gaucher Stichting, a national foundation that supports research in the field of lysosomal storage disorders. Consulting fees for INvS are donated to the Stichting Klinische Neurologie, a local foundation that supports research in the field of neurological disorders. DH has received consultancy fees from Actelion for participation in clinical trials, grants for local laboratory projects, and speaker fees for participation in scientific congresses and sponsored events. KEM, PG and LM have received speaker fees from Actelion for participation in sponsored events. PG received consultancy fees for participation in local clinical projects. LM received a travel grant from Actelion and was financially supported by TÁMOP 4.2.1./B-09/1/KONV-2010-0007 and TÁMOP 4.2.2-08/1-2008-0015. CN got speaker fees for participation in scientific meetings. KAW was sole shareholder of Cognitive Drug Research Ltd. Cognitive Drug Research Ltd supplied the CDR System for the study and received financial support from Actelion. KAW is currently an employee of United BioSource Corporation (UBC) that owns the CDR System since August 2009. CL is an employee of Actelion Pharmaceuticals Ltd. MP and MM report no conflicts of interest

    Occator Crater in Color at Highest Spatial Resolution

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    The geology of the outstanding crater Occator on Ceres has been studied by combining highest resolution color images and other information from the DAWN mission. Thus, surface and sub-surface layers and geologic processes can be understood and interpreted in a consistent manner. In order to achieve this, morphometry, absolute surface unit ages, color, and the distribution of foci of activity were the key data. These data show that the ascent of brine from reservoir(s) at depth and deposition of its salts on the surface persisted much longer than initially thought possible as an immediate result of the primary impact. The youngest depositional processes of bright material occurred less than 2 Ma ago. Also, the bright Cerealia and Vinalia Faculae are not the only traces of this activity; updoming is present on the southwestern crater floor. Faculae coincide with fractures and vents and indicate complex mechanisms of the deposition of bright carbonate-rich material. Due to the large age difference between the Occator impact itself, modeled cooling times of heated crater material, and the recent activity at the faculae we conclude that endogenic forces were lately acting. The distribution and thickness of surface and sub-surface brine layers are far from homogeneous in the upper crust beneath Occator. Further evidence regarding the distribution of materials has been derived from the distribution of the ejecta and the transition of ejecta to background material outside the crater

    Exploring the patient journey to diagnosis of Gaucher disease from the perspective of 212 patients with Gaucher disease and 16 Gaucher expert physicians

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    Gaucher disease (GD) is a rare hereditary disorder caused by a deficiency of the lysosomal enzyme ÎČ-glucocerebrosidase. Diagnosis is challenging owing to a wide variability in clinical manifestations and severity of symptoms. Many patients may experience marked delays in obtaining a definitive diagnosis. The two surveys reported herein aimed to explore the patient journey to diagnosis of GD from the perspectives of Gaucher expert physicians and patients. Findings from the surveys revealed that many patients experienced diagnostic delays and misdiagnoses, with nearly 1 in 6 patients stating that they were not diagnosed with GD for 7years or more after first consulting a doctor. Physicians and patients both reported multiple referrals to different specialties before a diagnosis of GD was obtained, with primary care, haematology/haematology-oncology and paediatrics the main specialties to which patients first presented. Splenomegaly, thrombocytopenia, anaemia and bone pain were reported as the most common medical problems at first presentation in both surveys. These findings support a clear need for straightforward and easy-to-follow guidance designed to assist non-specialists to identify earlier patients who are at risk of GD

    Light Curves and Period Changes of Type II Cepheids in the Globular Clusters M3 and M5

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    Light curves in the B, V, and I_c passbands have been obtained for the type II Cepheids V154 in M3 and V42 and V84 in M5. Alternating cycle behavior, similar to that seen among RV Tauri variables, is confirmed for V84. Old and new observations, spanning more than a century, show that V154 has increased in period while V42 has decreased in period. V84, on the other hand, has shown large, erratic changes in period that do not appear to reflect the long term evolution of V84 through the HR diagram.Comment: 28 pages, 12 figure

    Signatures of Star-planet interactions

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    Planets interact with their host stars through gravity, radiation and magnetic fields, and for those giant planets that orbit their stars within ∌\sim10 stellar radii (∌\sim0.1 AU for a sun-like star), star-planet interactions (SPI) are observable with a wide variety of photometric, spectroscopic and spectropolarimetric studies. At such close distances, the planet orbits within the sub-alfv\'enic radius of the star in which the transfer of energy and angular momentum between the two bodies is particularly efficient. The magnetic interactions appear as enhanced stellar activity modulated by the planet as it orbits the star rather than only by stellar rotation. These SPI effects are informative for the study of the internal dynamics and atmospheric evolution of exoplanets. The nature of magnetic SPI is modeled to be strongly affected by both the stellar and planetary magnetic fields, possibly influencing the magnetic activity of both, as well as affecting the irradiation and even the migration of the planet and rotational evolution of the star. As phase-resolved observational techniques are applied to a large statistical sample of hot Jupiter systems, extensions to other tightly orbiting stellar systems, such as smaller planets close to M dwarfs become possible. In these systems, star-planet separations of tens of stellar radii begin to coincide with the radiative habitable zone where planetary magnetic fields are likely a necessary condition for surface habitability.Comment: Accepted for publication in the handbook of exoplanet

    Foliar lead uptake by lettuce exposed to atmospheric fallouts

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    Metal uptake by plants occurs by soil−root transfer but also by direct transfer of contaminants from the atmosphere to the shoots. This second pathway may be particularly important in kitchen gardens near industrial plants. The mechanisms of foliar uptake of lead by lettuce (Lactuca sativa) exposed to the atmospheric fallouts of a lead-recycling plant were studied. After 43 days of exposure, the thoroughly washed leaves contained 335 ± 50 mg Pb kg−1 (dry weight). Micro-X-ray fluorescence mappings evidenced Pb-rich spots of a few hundreds of micrometers in diameter located in necrotic zones. These spots were more abundant at the base of the central nervure. Environmental scanning electron microscopy coupled with energy dispersive X-ray microanalysis showed that smaller particles (a few micrometers in diameter) were also present in other regions of the leaves, often located beneath the leaf surface. In addition, submicrometric particles were observed inside stomatal openings. Raman microspectrometry analyses of the leaves identified smelter-originated Pb minerals but also secondary phases likely resulting from the weathering of original particles. On the basis of these observations, several pathways for foliar lead uptake are discussed. A better understanding of these mechanisms may be of interest for risk assessment of population exposure to atmospheric metal contamination
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