10,760 research outputs found

    Cutaneous findings in neurofibromatosis type 1

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    Neurofibromatosis type 1 (NF1) is characterized by major and minor cutaneous findings, whose recognition plays a key role in the early diagnosis of the disease. The disease affects multiple systems and clinical manifestation has a wide range of variability. Symptoms and clinical signs may occur over the lifetime, and the complications are very diverse. Although significant progress has been made in understanding the pathophysiology of the disease, no specific treatment has been defined. Multidisciplinary approach is required to provide optimum care for the patients. The aim of this paper is to provide the clinician with a complete guide of skin findings of NF1. Neurofibromatosis type 1 (NF1) is a complex autosomal dominant disorder associated with germline mutations in the NF1 tumor suppressor gene. NF1 belongs to a class of congenital anomaly syndromes called RASopathies, a group of rare genetic conditions caused by mutations in the Ras/mitogen-activated protein kinase pathway. Generally, NF1 patients present with dermatologic manifestations. In this review the main features of café-au-lait macules, freckling, neurofibromas, juvenile xanthogranuloma, nevus anemicus and other cutaneous findings will be discussed

    Effect of membrane character and solution chemistry on microfiltration performance

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    To help understand and predict the role of natural organic matter (NOM) in the fouling of low-pressure membranes, experiments were carried out with an apparatus that incorporates automatic backwashing and long filtration runs. Three hollow fibre membranes of varying character were included in the study, and the filtration of two different surface waters was compared. The hydrophilic membrane had greater flux recovery after backwashing than the hydrophobic membranes, but the efficiency of backwashing decreased at extended filtration times. NOM concentration of these waters (7.9 and 9.1 mg/L) had little effect on the flux of the membranes at extended filtration times, as backwashing of the membrane restored the flux to similar values regardless of the NOM concentration. The solution pH also had little effect at extended filtration times. The backwashing efficiency of the hydrophilic membrane was dramatically different for the two waters, and the presence of colloid NOM alone could not explain these differences. It is proposed that colloidal NOM forms a filter cake on the surface of the membranes and that small molecular weight organics that have an adsorption peak at 220 nm but not 254 nm were responsible for “gluing” the colloids to the membrane surface. Alum coagulation improved membrane performance in all instances, and this was suggested to be because coagulation reduced the concentration of “glue” that holds the organic colloids to the membrane surface

    A study of gas contaminants and interaction with materials in RPC closed loop systems

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    Resistive Plate Counters (RPC) detectors at the Large Hadron Collider (LHC) experiments use gas recirculation systems to cope with large gas mixture volumes and costs. In this paper a long-term systematic study about gas purifiers, gas contaminants and detector performance is discussed. The study aims at measuring the lifetime of purifiers with unused and used cartridge material along with contaminants release in the gas system. During the data-taking the response of several RPC double-gap detectors was monitored in order to characterize the correlation between dark currents, filter status and gas contaminants

    Physicochemical Characterization And Antioxidant Capacity Of Pitanga Fruits (eugenia Uniflora L.) [caracterização Fisico-química E Capacidade Antioxidante De Pitangas (eugenia Uniflora L.)]

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    This study was carried out to obtain more information about the physicochemical properties, composition, and antioxidant activity of pitanga fruits (Eugenia uniflora L.), particularly fruits from the State of Rio Grande do Sul, Brazil. Pitanga with different flesh colors (purple, red, and orange) from tree selections cultivated at Embrapa Clima Temperado (RS-Brazil) were analyzed. Only slight differences were observed in the quality parameters and in the proximate and fatty acid compositions among the fruits studied. The extracts from purple-fleshed pitanga had the highest total phenolic and anthocyanin contents along with the highest antioxidant capacity. The antioxidant capacity (DPPH and FRAP assays) of methanolic pitanga extracts was highly correlated with the total phenolic content, but in ethanolic extracts, the anthocyanin content was correlated only with the FRAP antioxidant capacity. Orange fleshed pitanga had higher β-cryptoxanthin and β-carotene levels than those of the red fruit, which had higher lycopene content. The results indicate that the purple-fleshed pitanga, cultivated in Rio Grande do Sul, is a rich source of phenolic compounds and has high antioxidant capacity. The red and orange-fleshed pitanga, on the other hand, are rich sources of carotenoids.311147154Abidille, M.D.H., Antioxidant activity of the extracts from Dillenia indica fruits (2005) Food Chemistry, 90 (4), pp. 891-896Adebajo, A.C., Oloki, K.J., Aladesanmi, A., Antimicrobial activity of the leaf extract of Eugenia uniflora (1989) Journal of Phytotherapy Resource, 3 (6), pp. 258-259Aherne, S.A., O'Brien, N.M., Dietary flavonols: Chemistry, food content, and metabolism (2002) Nutrition, 18 (1), pp. 75-81(1995) Official methods of analysis of the Association of the Official Analytical Chemists, , ASSOCIATION OF OFFICIAL ANALYTICAL CHEMISTS-AOAC, 16th ed. 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    Phosphorus species in sequentially extracted soil organic matter fractions

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    The majority of organic P (Porg) in soil is considered to be part of soil organic matter (SOM) associations, but its chemical nature is largely ‘unresolved’. In this study, we investigated the Porg composition in different SOM fractions of a Gleysol soil using the Humeomics sequential chemical fractionation (SCF) procedure combined with nuclear magnetic resonance (NMR) spectroscopy. In summary, SCF procedure with subsequent NaOH-EDTA extraction of the soil residue extracted a total of 1769 mg P/kgsoil compared to 1682 mg P/kgsoil of a single-step NaOH-EDTA extraction. Approximately 38 % of the extracted Porg was present in the form of the unresolved Porg pool, which was represented by one or two underlying broad signals in the phosphomonoester region of solution 31P NMR spectra. The SCF revealed that phosphomonoesters were recovered in each fraction: 47 % of the unresolved phosphomonoesters were associated with the SOM fraction released by breaking ester bonds (40 %) and ether bonds (7 %), whereas about 30 % of this unresolved Porg pool appeared in the SOM fraction closely associated with the soil mineral phase. Furthermore, the extractability of inositol phosphates (IP) was increased from 312 mg P/kgsoil to 534 mg P/kgsoil (factor 1.7) using the SCF procedure compared to a single-step NaOH-EDTA extraction. Previous studies have reported the presence of IP in molecular size fractions greater than 10 kDa. Our findings on the removal of IP with the fractionation of the SOM could explain the presence of IP in these large associations. We demonstrate that major pools of Porg are closely associated with SOM structures, comprising a diverse array of chemical species and bonding types. These results forward our understanding of Porg stabilisation, P transformation, and P cycling in terrestrial ecosystems towards an association point of view

    Evaluation de la sécheresse entre 2008 et 2009 dans l’ouest de la Région Pampeana (Argentine)

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    Se analiza la variabilidad de la precipitación y los efectos de la sequía en la extensión areal del Dique Paso de las Piedras y en la actividad agrícola del Oeste de la región pampeana (Argentina) durante los años 2008-2009. La metodología incluye la aplicación del Índice Estandarizado de Precipitación, el procesamiento digital de imágenes satelitales y el análisis de los rendimientos productivos de cuatro cultivos de la región. El Índice Estandarizado de Precipitación ha indicado sequía meteorológica en los años 2008 y 2009. En 2009 el rendimiento de los cultivos ha disminuido entre el 25 y 65 % con respecto a años anteriores. La extensión areal del dique ha disminuido un 52 % en 2009 en relación a períodos húmedos.The rainfall variability and drought effects in the Paso de las Piedras Dam and farming were analyzed in the western pampean region (Argentina) for the 2008-2009 years. The methodology includes the Standardized Precipitation Index application, the satellite image processing and the production yields analysis of four crops of the region. The Standardized Precipitation Index was showed meteorological drought in the 2008 and 2009 years. The production of yields has decreased between 25 and 65 % during 2009 compared to previous years. The dam area has decreased 52 % in 2009, in relation with wet periods.Les effets de la variabilité des précipitations et de la sécheresse dans le barrage de Paso de las Piedras et de l’agriculture ont été analysés dans la région occidentale Pampa (Argentine) pour les années 2008-2009. La méthodologie comprenait l’application Standardized Precipitation Index, le traitement d’images satellite et de l’analyse des rendements de production de quatre cultures de la région. L’indice de précipitations normalisé a montré la sécheresse météorologique dans les exercices 2008 et 2009. La production de rendements ont baissé entre 25 et 65 % en 2009 par rapport aux années précédentes. La zone du barrage a diminué de 52 % en 2009, en relation avec les périodes humides
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