Ressonância magnética na avaliação do acometimento ósseo na doença de Gaucher tipo 1.

A doença de Gaucher (DG) foi a primeira doença de armazenamento lisossomal relatada e é a lipidose mais encontrada em todo mundo. Ocorre devido à deficiência hereditária do funcionamento da enzima lisossomal glucocerebrosidase. Tal carência leva ao acúmulo deste glicolipídio principalmente no fígado, baço e medula óssea. Nesta última, pode levar a osteopenia, lesões líticas, fraturas patológicas, dor óssea crônica, crises e infartos ósseos, que tendem a repercutir a longo prazo na qualidade de vida dos pacientes acometidos. Foi realizado estudo descritivo e transversal que avaliou alterações ósseas pela ressonância magnética dos pacientes com DG tipo I, utilizando o método Bone Marrow Burden (escore BMB), relacionando-o com o volume esplênico e a qualidade de vida. Foram avaliados 18 pacientes, sendo 8 do gênero masculino e 10 do gênero feminino, com média de idade 38,2 anos (17-70 anos). Foram detectadas complicações ósseas em 9 pacientes: infarto ósseo bilateral (6 pacientes), necrose avascular da cabeça femoral (2 pacientes) e necrose avascular da cabeça femoral concomitantemente a infarto ósseo unilateral (1 paciente). Houve correlação significativa de todos os escores BMBs (fêmur, coluna e total) com o volume esplênico. No entanto, nenhum dos BMBs apresentados teve relação significativa com o escore de qualidade de vida. O escore de qualidade de vida também não apresentou relação linear com o volume esplênico. O escore BMB, mostrou-se como um bom método para estimar o envolvimento ósseo e apresentou relação linear com o volume esplênico, demonstrando maior grau de comprometimento ósseo nos indivíduos com maior esplenomegalia. A qualidade de vida não se relacionou com o BMB ou com o volume esplênico, possivelmente relacionado ao caráter progressivo e indolente da DG, que determina alterações silenciosas e irreversíveis durante o curso da doença

Comparing Aspects Of The Process Quality In Six European Early Childhood Educational Settings

The European project ‘Early Change’ (http://earlychange.teithe.gr) attempts to evaluate the quality of early childhood education (ECE) environments of six European countries, Greece, Portugal, Finland, Denmark, Cyprus and Romania. The purpose of this paper is to compare the level of two dimensions of the process quality of these environments a) Space & Furnishings, and b) Personal Care Routines. Theorists, practitioners and researchers agree that in order to provide qualitative education to young children, one of the basic needs of all children must be met; that need is the protection of their health and their safety. A high quality early childhood education program must contain a safe and stimulating environment for the child (Lindsey, 1998). Such an environment includes indoor space, outdoor space, furniture, and room arrangement, and it is considered an integral part of a high quality early childhood program. 117 early educators from the six participating countries attended the training seminars about the evaluation of ECE quality using the Early Childhood Environmental Rating Scale-R (ECERS-R). The trained educators evaluated the 8 indicators of the subscale ‘space & furnishings’ and the six indicators of the subscale ‘personal care routines’ in approximately 600 early childhood classrooms from six European countries. The results of this study highlight the similarities and differences concerning the specific dimensions of the process quality of ECE environments in six European countries, and reflect the diversity of ECE environment across these countries. The findings of this study may provide a valuable insight to researchers and educational policy makers for an enhanced understanding of the cultural diversities and the strengthening of the common values and targets of the European Union

Elemental mapping of Portuguese ceramic pieces with a full-field XRF scanner based on a 2D-THCOBRA detector

UID/FIS/04559/2020In this work, we present a novel application of the full-field energy-dispersive X-ray fluorescence (EDXRF) imaging system based on a MicroPattern Gaseous Detector (2D-THCOBRA) in the cultural heritage field. The detector has an intrinsic imaging capability with spatial resolution of 400μmFWHM, and is energy sensitive, presenting an energy resolution of approximately 1keVFWHM at 5.9keV. The full-field XRF scanner based on the 2D-THCOBRA detector allows mapping the distribution of elements in large area samples with high detection efficiency (75 % at 5.9keV), being a very promising choice for elemental mapping analysis of large area cultural heritage samples. In this work, we have demonstrated the imaging capabilities of the full-field XRF scanner and used it to assess the restoration of a Portuguese faience piece.publishersversionpublishe

Modeling Architectures and Reference Models: Development and Maintenance Open Source ERP

The adoption Enterprise Resource Planning (ERPs) by small and medium-sized businesses may not possible its cost. At same time, whenever adapt ERP specific needs company, user becomes dependent developers due to the lack access and knowledge respective code. Free and open source software can promote advantages companies, however, for their adoption, it is necessary to develop techniques tools facilitate implementation and maintenance code. This article highlights the importance of defining modeling architectures and reference models for development and maintenance open source ERPs, especially the ERP5 project

Defining the uniqueness of monovarietal wines from native portuguese varieties of Vitis vinifera

Studies have been carried out on red and white monovarietal wines vinified during the 1995, 1996 and 1997 harvests. The essential aim being to evaluate sensory quality, correlate it with chemical analytical data and relate these to the various winemaking options available. Hence selective native varieties from the Douro, Dao, Bairrada and Vinhos Verdes regions have been studied and the analytical / sensorial data correlated to such factors as grape maturation and maceration regime. Results are presented illustrating the quality effects of variety / winemaking practice combinations, both in terms of analytical data (sensory and chemical) and taster preferences

高梁市国民健康保険成羽病院

Flavocytochrome c(3) from Shewanella frigidimarina WO is a tetrahaem periplasmic protein of 64 kDa with fumarate reductase activity. This work reports the first example of NMR techniques applied to the assignment of the thermodynamic order of oxidation of the four individual haems for such large protein, expanding its applicability to a wide range of proteins. NMR data from partially and fully oxidised samples of fcc(3) and a mutated protein with an axial ligand of haem IV replaced by alanine were compared with calculated chemical shifts, allowing the structural assignment of the signals and the unequivocal determination of the order of oxidation of the haems. As oxidation progresses the fcc(3) haem domain is polarised, with haems I and II much more oxidised than haems III and IV, haem IV being the most reduced. Thus, during catalysis as an electron is taken by the flavin adenosine dinucleotide from haem IV, haem III is eager to re-reduce haem IV, allowing the transfer of two electrons to the active site

Industry/University collaboration in product focussed œnological research in Portugal - the SOGRAPE / UCP ESB partnership 1995 - 1998

A innovative model of collaboration between a wine company and a university research group is presented. A protocol was established as a response to the perceived need to, on the part of the university, (i) focus research on specific scientific bottlenecks which would have a real impact in the producing industry and, on the part of the company, (ii) to have access to the scientific capacity to conduct in-depth studies to approach specific technical challenges. A closely managed programme was jointly established and the major characteristics are presented here together with the main results obtained during the first 2 and a half years of operation

Analysis of Zebrafish contamination with heavy metals using a FF-XRF imaging system based on a MPGD

Funding Information: F.D. Leite is grateful for the grant BII/UI96/9175/2020, R.G. Oliveira is grateful for the grant SFRH/BI/10638/2020, and P.M.S. Carvalho is grateful to FCT for the PhD grant PD/BD/128324/2017. Funding Information: The costs resulting from the FCT (Fundação para a Ciência e a Tecnologia, I.P–Portuguese Foundation for Science and Technology) hiring L.F.N.D. Carramate were funded by national funds (OE) in the scope of the framework contract CEECIND/01369/2017, and S. Pessanha in the scope of the framework contract CEECIND/00278/2018. Funding Information: This work was partially supported by projects PTDC/FIS-AQM/32536/2017, CERN/FIS-INS/0026/2019 and STRONG-2020–824093 through FCT and H2020 programs. It was also developed within the scope of the i3N (UIDB/50025/2020 & UIDP/50025/2020) and CESAM (UIDP/50017/2020 & UIDB/50017/2020 & LA/P/0094/2020), financed by national funds through the FCT/MEC COMPETE , FEDER and POCI programs. Publisher Copyright: © 2022 The AuthorsHeavy metals have been extensively used by humans and are still present in many aspects of modern-day life. Such elements tend to accumulate, degrading the quality of air, soil, and water, negatively affecting the living organisms. As such, it is of paramount importance to understand the environmental risks of heavy metals, including their bioaccumulation capacity in organisms, since they are associated with several harmful health effects, such as neurological damages. The Zebrafish (Danio rerio) has been shown to be an excellent animal model to understand physiological responses in mammals and consequently to perform toxicological studies due to characteristics such as the high genetic and physiological similarity with humans. In this work, a Full-Field Energy Dispersive X-ray Fluorescence (FF-XRF) imaging system, based on the 2D-THCOBRA micropattern gas detector, was used to monitor heavy metal accumulation in Zebrafish during a water borne exposure bioassay. The distributions of Mn, Se, and Pb in Zebrafish, exposed to different concentrations of the heavy metal compounds during different time intervals, were mapped. The results show a preferential accumulation of Mn, Se, and Pb in the Zebrafish's visceral region and highlight the suitability of the FF-XRF imaging system for quick screening of metal accumulation in fish bioassays.publishersversionpublishe

Children’s right to participate: The Lundy model applied to early childhood education and care

Children have the right to express their views in all matters affecting them, and to have them considered and given due weight. Children’s participation is most meaningful when rooted in children’s everyday lives, and its promotion should be encouraged from the youngest ages, especially in early childhood education and care (ecec). In this paper we apply the Lundy model of participation, widely used in policy, practice and professional development initiatives, to the ecec context. Based on examples provided by ecec professionals from Belgium, Greece, Poland, and Portugal, we illustrate the implementation of the elements of space, voice, audience and influence, proposed by the Lundy model. We also discuss the interrelations among these elements and the need for organisational and contextual support to enhance children’s participation. This paper adds to existing literature, highlighting theoretical and practical issues associated with the promotion of children’s right to participate in ecec.info:eu-repo/semantics/acceptedVersio