Megalencephalic Leukoencephalopathy With Subcortical Cysts (mlc) – A Case With Clinical And Magnetic Resonance Imaging (mri) Dissociation [leucoencefalopatia Megalencefálica Com Cistos Subcorticais (mlc) – Um Caso Com Dissociação Clínica E De Resonância Magnética]

[No abstract available]732171172Nunes, R.H., Pacheco, F.T., Rocha, A.J., Magnetic resonance imaging of anterior temporal lobe cysts in children: Discriminating special imaging features in a particular group of diseases (2014) Neuroradiology, 56, pp. 569-577. , http://dx.doi.org/10.1007/s00234-014-1356-9Olivier, M., Lenard, H.G., Aksu, F., Gärtner, J., A new leukoencephlapathy with bilateral anterior temporal lobe cysts (1998) Neuropediatrics, 29 (5), pp. 225-228. , http://dx.doi.org/10.1055/s-2007-973566Knaap, M.S., Boor, I., Estévez, R., Megalencephalic leukoencephalopathy with subcortical cysts: Chronic white matter oedema due to a defect in brain ion and water homoeostasis (2012) Lancet Neurol, 11 (11), pp. 973-985. , http://dx.doi.org/10.1016/S1474-4422(12)70192-8Sinhal, B.S., Gorospe, J.R., Naidu, S., Megalencephalic leukoencephalopathy with subcotical cysts (2003) J Child Neurol, 18 (9), pp. 645-652. , http://dx.doi.org/10.1177/0883073803018009120

A comparison of baleen whale density estimates derived from overlapping satellite imagery and a shipborne survey

As whales recover from commercial exploitation, they are increasing in abundance in habitats that they have been absent from for decades. However, studying the recovery and habitat use patterns of whales, particularly in remote and inaccessible regions, frequently poses logistical and economic challenges. Here we trial a new approach for measuring whale density in a remote area, using Very-High-Resolution WorldView-3 satellite imagery. This approach has capacity to provide sightings data to complement and assist traditional sightings surveys. We compare at-sea whale density estimates to estimates derived from satellite imagery collected at a similar time, and use suction-cup archival logger data to make an adjustment for surface availability. We demonstrate that satellite imagery can provide useful data on whale occurrence and density. Densities, when unadjusted for surface availability are shown to be considerably lower than those estimated by the ship survey. However, adjusted for surface availability and weather conditions (0.13 whales per km2, CV = 0.38), they fall within an order of magnitude of those derived by traditional line-transect estimates (0.33 whales per km2, CV = 0.09). Satellite surveys represent an exciting development for high-resolution image-based cetacean observation at sea, particularly in inaccessible regions, presenting opportunities for ongoing and future research

A study of emotions in requirements engineering.

Proceedings of: 3rd World Summit on the Knowledge Society (WKKS 2010), September 22-24, 2010, Corfu (Greece)Requirements engineering (RE) is a crucial activity in software development projects. This phase in the software development cycle is knowledge intensive, and thus, human capital intensive. From the human point of view, emotions play an important role in behavior and can even act as behavioral motivators. Thus, if we consider that RE represents a set of knowledge-intensive tasks, which include acceptance and negotiation activities, then the emotional factor represents a key element in these issues. However, the emotional factor in RE has not received the attention it deserves. This paper aims to integrate the stakeholder’s emotions into the requirement process, proposing to catalogue them like any other factor in the process such as clarity or stability. Results show that high arousal and low pleasure levels are predictors of high versioning requirements.Publicad

Effectiveness and safety of obeticholic acid in a Southern European multicenter cohort of patients with primary biliary cholangitis and suboptimal response to ursodeoxycholic acid

Background Obeticholic acid (OCA) was recently approved as the only on-label alternative for patients with primary biliary cholangitis (PBC) with intolerance or suboptimal response to ursodeoxycholic acid (UDCA). However, few data are available outside clinical trials. Aim To assess the effectiveness and safety of OCA in a real-world cohort of patients with non-effective UDCA therapy. Methods Open-label, prospective, real-world, multicentre study, enrolling consecutive patients who did not meet Paris II criteria, from 18 institutions in Spain and Portugal. Effectiveness was assessed by the changes in GLOBE and UK-PBC scores from baseline. POISE and Paris II criteria were evaluated after 12 months of OCA . Liver fibrosis was evaluated by FIB-4 and AST to platelet ratio index (APRI). Results One hundred and twenty patients were eligible, median time since PBC diagnosis 9.3 (4.0-13.8) years, 21.7% had cirrhosis, and 26.7% received had previous or concomitant treatment with fibrates. Seventy-eight patients completed at least 1 year of OCA. The Globe-PBC score decreased to 0.17 (95% CI 0.05 to 0.28; P = 0.005) and the UK-PBC score decreased to 0.81 (95% CI -0.19 to 1.80; P = 0.11). There was a significant decrease in alkaline phosphatase of 81.3 U/L (95% CI 42.5 to 120; P < 0.001), ALT 22.1 U/L (95% CI 10.4 to 33.8; P < 0.001) and bilirubin 0.12 mg/dL (95% CI 0 to 0.24; P = 0.044). FIB-4 and APRI remained stable. According to the POISE criteria, 29.5% (23 out of 78) achieved response. The adverse events rate was 35%; 11.67% discontinued (8.3% due to pruritus). Conclusions This study supports data from phase III trials with significant improvement of PBC-Globe continuous prognostic marker score among OCA-treated patients with good tolerability

DNA damage sensitivity of SWI/SNF-deficient cells depends on TFIIH subunit p62/GTF2H1

Mutations in SWI/SNF genes are amongst the most common across all human cancers, but efficient therapeutic approaches that exploit vulnerabilities caused by SWI/SNF mutations are currently lacking. Here, we show that the SWI/SNF ATPases BRM/SMARCA2 and BRG1/SMARCA4 promote the expression of p62/GTF2H1, a core subunit of the transcription factor IIH (TFIIH) complex. Inactivation of either ATPase subunit downregulates GTF2H1 and therefore compromises TFIIH stability and function in transcription and nucleotide excision repair (NER). We also demonstrate that cells with permanent BRM or BRG1 depletion have the ability to restore GTF2H1 expression. As a consequence, the sensitivity of SWI/SNF-deficient cells to DNA damag

Comparison of two strategies for the start-up of a biological reactor for the treatment of hypersaline effluents from a table olive packaging industry

Biological treatment of hypersaline effluents with high organic matter concentrations is difficult to carry out and it can require a long start-up phase. This is the case of the treatment of fermentation brines from the table olive packaging (FTOP) industries. These effluents are characterized by conductivity values around 90 mS/cm, COD around 15,000 mg/L and total phenols concentration around 1000 mg/L. In this work, FTOP has been treated in two sequencing batch reactors (SBRs) operated in parallel. In each SBR a different start-up strategy has been carried out. In the SBR-2, biomass was previously acclimated to high salinity using simulated wastewater without phenolic compounds, meanwhile in the SBR-1, FTOP was added from the beginning of the start-up. Results indicated more operational problems in the SBR-2 consisting in a higher deflocculation that drove to high turbidity values in the effluent. Besides, at the end of the start-up, the SBR-1 reached higher COD removal efficiencies than SBR-2 (88% and 73%, respectively). In both reactors, an increase in gamma-Proteobacteria in the microbial population was observed for increasing conductivities. In addition, phenols were completely removed in both reactors at the end of the start-up, what implied very low toxicity values in the effluent.The authors of this work thank the financial support of CDTI (Centre for Industrial Technological Development) depending on the Spanish Ministry of Science and Innovation.Ferrer-Polonio, E.; Mendoza Roca, JA.; Iborra Clar, A.; Alonso Molina, JL.; Pastor Alcañiz, L. (2015). Comparison of two strategies for the start-up of a biological reactor for the treatment of hypersaline effluents from a table olive packaging industry. Chemical Engineering Journal. 273:595-602. doi:10.1016/j.cej.2015.03.062S59560227

Stomatal behavior and components of the antioxidative system in coffee plants under water stress

Coffee (Coffea arabica) plants show a positive relationship between stomatal closure and formation and accumulation of H2O2. However, for coffee plants under water restriction such relationship has never been studied. The objective of the present study was evaluate the stomatal movement and the antioxidant capacity of coffee seedlings under different water regimes. Eight months old coffee seedlings of cv. Catuaí IAC 99 were submitted to field capacity, gradual and total suspension of irrigation during a period of 21 days. Evaluations of leaf water potential (&#936;w) were performed in the beginning of the morning, and stomatal resistance, transpiration rate and vapor pressure deficit were determined at 10 am and 5 pm. All biochemical and enzymatic determinations were performed in leaves collected at 5 pm. Evaluations and samplings were performed at three days intervals. There was no variation in &#936;w during the evaluated period for plants in field capacity. However, an expressive decrease of &#936;w following day 12, reaching values near -2.5 MPa at the end of the experiment was observed for plants submitted to gradual suspension of irrigation. For plants submitted to total suspension of irrigation, &#936;w decreases after the sixth day, reaching -2.5 MPa at day 15. The decay of &#936;w in plants submitted to gradual and total suspension of irrigation reflected in increased stomatal resistance and in a decreased transpiration rate leading to an increase in hydrogen peroxide formation and, on final stages, increase in lipid peroxidation. As a conclusion, an increase in the activity of antioxidant enzymes as well as in the levels of ascorbate and dehydroascorbate was observed, which act in the detoxification of free radicals formed as result of the water stress.Para o cafeeiro (Coffea arabica) existe uma comprovada relação positiva entre fechamento estomático e formação e acúmulo de H2O2. Entretanto, tal relação para a cultura sob restrição hídrica ainda não foi estudada. Avaliou-se o movimento estomático e a capacidade antioxidante em mudas de cafeeiro sob diferentes regimes hídricos. Mudas de cafeeiro cv. Catuaí IAC 99, com oito meses de idade, foram submetidas à capacidade de campo, suspensão gradativa e suspensão total da irrigação por um período de 21 dias. Foram realizadas avaliações do potencial hídrico (&#936;w) foliar na antemanhã e resistência estomática, taxa transpiratória e déficit de pressão de vapor foram avaliados as 10h00 e 17h00. As determinações bioquímicas e enzimáticas foram realizadas em folhas coletadas às 17h00. Todas as avaliações e coletas foram realizadas em intervalos de três dias. Nas plantas em capacidade de campo não houve variação no &#936;w durante o período de avaliação. Para a suspensão gradativa da irrigação, houve queda expressiva a partir dos 12 dias, chegando próximo a -2,5 Mpa, ao final do experimento. Já nas plantas em suspensão total da irrigação observou-se queda no &#936;w a partir do sexto dia, chegando a -2,5 MPa aos 15 dias. A queda no &#936;w para as plantas em suspensão gradual e total da irrigação refletiu em aumentos na resistência estomática e diminuição da taxa transpiratória, ocasionando aumento na formação de peróxido de hidrogênio e nos períodos finais, aumentos na peroxidação de lipídios. Em conseqüência obervaram-se aumentos na atividade das enzimas antioxidantes, bem como nos teores de ascorbato e dehidroascorbato, atuando na detoxificação dos radicais livres formados em função do estresse