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The article presents case of a 42-year-old woman with long standing Systemic lupus erythematosus (SLE) and stage 4 chronic kidney disease (CKD). It mentions diagnosis of Hemophagocytic lymphohistiocytosis (HLH), a rare secondary to an overwhelming inflammatory process; and also mentions investigation showed thrombocytopenia, anemia, with low reticulocyte production index, high lactate dehydrogenase.

Background: The dietary management of methylmalonic acidaemia (MMA) is a low-protein diet providing sufficient energy to avoid catabolism and to limit production of methylmalonic acid. The goal is to achieve normal growth, good nutritional status and the maintenance of metabolic stability. Aim: To describe the dietary management of patients with MMA across Europe. Methods: A cross-sectional questionnaire was sent to European colleagues managing inherited metabolic disorders (IMDs) (n=53) with 27 questions about the nutritional management of organic acidaemias. Data were analysed by different age ranges (0–6 months; 7–12 months; 1–10 years; 11–16 years; >16 years). Results: Questionnaires were returned from 53 centres. Twenty-five centres cared for 80 patients with MMA vitamin B12 responsive (MMAB12r) and 43 centres managed 215 patients with MMA vitamin B12 non-responsive (MMAB12nr). For MMAB12r patients, 44% of centres (n=11/25) prescribed natural protein below the World Health Organization/Food and Agriculture Organization/United Nations University (WHO/FAO/UNU) 2007 safe levels of protein intake in at least one age range. Precursor-free amino acids (PFAA) were prescribed by 40% of centres (10/25) caring for 36% (29/80) of all the patients. For MMAB12nr patients, 72% of centres (n=31/43) prescribed natural protein below the safe levels of protein intake (WHO/FAO/UNU 2007) in at least one age range. PFAA were prescribed by 77% of centres (n=33/43) managing 81% (n=174/215) of patients. In MMAB12nr patients, 90 (42%) required tube feeding: 25 via a nasogastric tube and 65 via a gastrostomy. Conclusions: A high percentage of centres used PFAA in MMA patients together with a protein prescription that provided less than the safe levels of natural protein intake. However, there was inconsistent practices across Europe. Long-term efficacy studies are needed to study patient outcome when using PFAA with different severities of natural protein restrictions in patients with MMA to guide future practice. [ABSTRACT FROM AUTHOR]

Background: Phenylketonuria (PKU) management practices differ between and within countries. In 2007, the Portuguese Society for Metabolic Disorders (SPDM) approved the Portuguese Consensus (PC) for the nutritional treatment of PKU. The recently published European PKU Guidelines (EPG, 2017) systematically reviewed recent evidence and aimed to harmonise treatment protocols in Europe. The objective of this study was to appraise the EPG acceptance and implementation in Portuguese treatment centres. Methods: An electronic questionnaire was prepared and the link was sent to 135 SPDM members. It outlined the 10 EPG key recommendations and compared each statement with the consensus recommendations published by SPDM. Responses were recorded and descriptive analyses were performed. Results: Twenty-five professionals completed the questionnaire, and over half (56%) were nutritionists/dieticians. At least one questionnaire from each of the 10 national treatment centres was returned. In general, responders accepted most of the recommendations. However, only the recommendation about target phenylalanine (Phe) concentrations between 120 and 360 μmol/L for patients <12 years received 100% consensus with a further seven recommendations gaining over 70% consensus. Almost half of the professionals (48%, n = 12) required further discussion about the EPG-safe upper target blood Phe concentration (600 μmol/L) suggested for patients aged ≥12 years. Almost one third (32%, n = 8) failed to agree with the recommendation in the EPG-proposed classification of Phe hydroxylase (PAH) deficiency. Conclusions: The EPG received overall good acceptance, but there was divided opinion about some recommendations which require further discussion before implementation by the Portuguese treatment centres. [ABSTRACT FROM AUTHOR]

Background: Phenylketonuria (PKU), a rare, inherited metabolic condition, is treated with a strict low-phenylalanine (Phe) diet, supplemented with Phe-free protein substitute. The optimal nutritional management of a sporting individual with PKU has not been described. Therefore, guidelines for the general athlete have to be adapted. Case presentation: Three clinical scenarios of sporting patients with PKU are given, illustrating dietary adaptations to usual management and challenges to attain optimal sporting performance. Therefore, the main objectives of sports nutrition in PKU are to (1) maintain a high carbohydrate diet; (2) carefully monitor hydration status; and (3) give attention to the timing of protein substitute intake in the immediate post-exercise recovery phase. Optimal energy intake should be given prior to, during and post exercise training sessions or competition. Fortunately, a usual low-Phe diet is rich in carbohydrate, but attention is required on the types of special low-protein foods chosen. Acute exercise does not seem to influence blood Phe concentrations, but further evidence is needed. Summary: Well-treated PKU patients should be able to participate in sports activities, but this is associated with increased nutritional requirements and dietary adjustments. Conclusions: It should be the goal of all sporting patients with PKU to maintain good metabolic Phe control and attain maximal athletic performance. [ABSTRACT FROM AUTHOR]

Abstract In European countries, buildings are the major energy consumers due to the general low energy performance of the existing building stock. To achieve the ambitioned targets for emissions reduction, it will be necessary to take actions for its large scale renovation. However, today's standards are mainly focused on new buildings, guiding the improvement of the energy performance of the existing buildings into expensive processes and complex procedures that seldom are accepted by users, owners or promoters. For these buildings, the range of technical solutions is shorter and may lead to ancillary works that result in a relevant increase in costs. In this context, the IEA EBC Programme launched the Annex56 project, with the goal of investigating solutions and produce guidance suitable for European residential buildings, taking into consideration not only the benefits related to energy and emissions reduction, but also the added value resulting from the renovation process. Considering the goal of reducing emissions, measures that promote the use of renewable energy can be as effective as energy efficiency measures, therefore, it is important to determine the optimal balance between the minimization of energy demand and the use of renewable energy. The optimization process has to explore the full range of cost-effective reduction of emissions and energy use and also to take into account the additional benefits and the overall added value achieved by the building within the renovation process as well as the increasing relevance of the embodied energy associated to the materials and systems used in the interventions. Highlights • The need for a new approach to energy related building renovation is described. • Cost-effective balance between energy efficiency, renewable energy and overall added value. • Cost-effectiveness and cost-optimality are compared considering climate change mitigation. • Recommendations are delivered for policy makers and professional owners. [ABSTRACT FROM AUTHOR]

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The objective was to describe the nutritional management of a 4-year-old child with glucose-6-phosphate dehydrogenase (G6PD) deficiency. A 4-year-old male child, African descent, born from non-consanguineous parents presented with a clinical history of frequent respiratory infections, usually treated with antibiotics. At 30 months of age, G6PD diagnosis was made after eating one portion (40 - 60 g) of fava beans, resulting in severe hemolytic anemia hospitalization for 5 days. Diagnosis was confirmed by G6PD activity measurement. Nutritional counseling was given to avoid dietary oxidative stressors particularly the exclusion of fava beans and accidental ingestion of other similar beans. Dietary intake of high vitamin C containing foods was discouraged and adequate hydration advised. Nutritional management is crucial in preventing acute stress events in patients with G6PD deficiency. [ABSTRACT FROM AUTHOR]

Natural ventilation requires no energy consumption, which can constitute 25% of a mechanically ventilated building, and both shape and position of inlets and outlets ventilation openings must be properly designed for better natural ventilation performance. Wind tunnel tests are a reliable tool for the determination of the effect of natural ventilation on buildings. This paper reports on results of wind tunnel tests conducted for the evaluation of the influence of the positioning and type of grid of ventilation modules on a façade system. Three ventilation modules were positioned below the window-sill (ventilated window-sill) and three were positioned above and below the façade. Ventilation modules with grid elements positioned vertically and horizontally were tested. Wind speed measurements that considered single-sided and cross ventilation were taken inside and outside the model for the different façade configurations for the evaluation of the best performance in relation to natural ventilation. The façade system proposed is movable and interchangeable, so that the same basic model can be used for the testing of possibilities for ventilation. The results show the use of six ventilation modules positioned below the window-sill and that form a “ventilated window-sill” with a horizontal grid is the best solution regarding natural ventilation. Such a configuration has proved the grid elements of ventilation modules exert a higher influence on the results than the positioning of modules on the façade. Therefore, better conditions of natural ventilation were obtained with a thin and horizontal positioning of the elements. [ABSTRACT FROM AUTHOR]

A large variety of transparent materials is available for uses in buildings façades and all glazing properties must be considered in their choice. Such a selection should be a careful process of evaluation and weighing of tradeoffs. The correct glazing specifications for façades can reduce energy consumption in buildings, because the heat exchange and passage of radiation into the building as light and heat occur through transparent surfaces. Therefore, glazing significantly contributes to the heat transfer between outdoor and indoor spaces, which act directly on daylighting and thermal comfort. This manuscript addresses a spectrophotometric characterization of glazings for the study of components for the design of a modular façade system based on the climate of Portugal. The study focused on results of spectrophotometric measurements of an optical behavior in different solar spectrum intervals (ultraviolet, visible and near infrared), specifically the transmittance of some types of simple glazings. The results show the percentage of transmission to spectrum intervals, which enabled the analysis (OK?) of the efficiency of the glazing regarding daylighting and correlation to the thermal performance. Indications for specifications and adequate uses based on transmission of transparent surfaces have been obtained and complemented the datasheets available from the manufactures. [ABSTRACT FROM AUTHOR]

The relevance of the building sector in the global energy use as well as in the global carbon emissions, both in the developed and developing countries, makes the improvement of the overall energy performance of existing buildings an important part of the actions to mitigate climate changes. Regardless of this potential for energy and emissions saving, large scale building renovation has been found hard to trigger, mainly because present standards are mainly focused on new buildings, not responding effectively to the numerous technical, functional and economic constraints of the existing ones. One of the common problems in the assessment of building renovation scenarios is that only energy savings and costs are normally considered, despite the fact that it has been long recognized that investment on energy efficiency and low carbon technologies yield several benefits beyond the value of saved energy which can be as important as the energy cost savings process. Based on the analysis of significant literature and several case studies, the relevance of co-benefits achieved in the renovation process is highlighted. These benefits can be felt at the building level by the owner or user (like increased user comfort, fewer problems with building physics, improved aesthetics) and should therefore be considered in the definition of the renovation measures, but also at the level of the society as a whole (like health effects, job creation, energy security, impact on climate change), and from this perspective, policy makers must be aware of the possible crossed impacts among different areas of the society for the development of public policies. [ABSTRACT FROM AUTHOR]

The International Energy Agency established an Implementing Agreement within the Energy in Buildings and Communities Program to undertake research and provide an international focus on Cost Effective Energy and Carbon Emissions Optimization in Building Renovation (EBC Annex 56). The project aims at developing a new methodology to enable cost effective renovation of existing buildings while optimizing energy consumption and carbon emissions reduction. Gathering of case studies is one of the activities undertaken to reach the overall project. Of the case studies a selection of “Shining Examples” is made to encourage decision makers to promote efficient and cost effective renovations. This paper presents the results of the analyses made on the Shining Examples. [ABSTRACT FROM AUTHOR]

Building sector has become an important target for carbon emissions reduction, energy consumption and resources depletion. Due to low rates of replacement of the existing buildings, their low energy performances are a major concern. Most of the current regulations are focused on new buildings and do not account with the several technical, functional and economic constraints that have to be faced in the renovation of existing buildings. Thus, a new methodology is proposed to be used in the decision making process for energy related building renovation, allowing finding a cost-effective balance between energy consumption, carbon emissions and overall added value. [ABSTRACT FROM AUTHOR]

Construction sector is one of the major responsible for energy consumption and carbon emissions and renovation of existing buildings plays an important role in the actions to mitigate climate changes. Present work is based on the methodology developed in IEA Annex 56, allowing identifying cost optimal and cost effective renovation scenarios improving the energy performance. The analysed case study is a residential neighbourhood of the municipality of Gaia in Portugal. The analysis compares a reference renovation scenario (without improving the energy performance of the building) with a series of alternative renovation scenarios, including the one that is being implemented. [ABSTRACT FROM AUTHOR]

Nowadays natural ventilation has gained prominence because its correct use can reduce energy consumption for cooling systems and improve thermal comfort among users. In this paper, we report on the modelling initiative, based on the wind tunnel tests that were carried out for the determination of the influence of natural ventilation in buildings. Indeed, the renewal of air in a closed environment without using an air conditioning system with mechanical elements can lead to energy savings and, in addition, provide air quality.The wind tunnel tests were carried out by varying the positioning of six ventilation modules in the façade system configuration. The modules were positioned below the window-sill (ventilated window-sill) as well as separately above and below the façade. The wind speed measurements were taken inside and outside the model for the different façades configurations to evaluate the best performance in relation to natural ventilation. The results supported the positioning of the six ventilation modules below the window-sill, forming a “ventilated window-sill” as the most effective natural ventilation solution. [ABSTRACT FROM AUTHOR]

Context and Objective Based on evidence that leptin and adiponectin are removed from circulation primarily by the kidney, we designed a study to examine the longitudinal changes of these adipokines during the first week after kidney transplantation (KTx) and to test the hypothesis that higher levels of leptin and/or adiponectin could be early biomarkers of delayed graft function (DGF = dialysis requirement during the first post-transplant week) and acute rejection. Study Design Repeated-measures prospective study. Material and Methods Forty consecutive adult patients with end-stage renal disease who were undergoing KTx. Leptin and adiponectin were measured in blood samples that were collected before (day-0) and after KTx (days-1, 2, 4 and 7). Linear mixed-models, receiver operating characteristic and area under curve (AUC-ROC) were used. Results At post-transplant day-1, leptinemia and adiponectinemia declined 43% and 47%, respectively. At all times studied after KTx, the median leptin levels were significantly higher in patients developing DGF (n = 18), but not adiponectin levels. Shortly after KTx (day-1), leptin values were significantly higher in DGF recipients in contrast to patients with promptly functioning kidneys, approximately two times higher when controlling for gender and BMI. The leptin reduction rate between pre-tranplant and one-day after KTx moderately predicted DGF (AUC = 0.73). On day-1, serum leptin predicted DGF (AUC-ROC = 0.76) with a performance slightly better than serum creatinine (AUC-ROC = 0.72), even after correcting for BMI (AUC-ROC = 0.73). Separating this analysis by gender showed that the performance of leptin in predicting DGF for male gender (AUC-ROC = 0.86) improved. Conclusions Kidney graft function is an independent determinant of leptin levels, but not of adiponectin. Leptin levels at day-1 slightly outperformed serum creatinine in predicting the occurrence of DGF, and more accurately in male gender. No significant association was detected with acute rejection. [ABSTRACT FROM AUTHOR]

Abstract: In Europe, energy and climate policies started to take shape from the 1990s onwards culminating with the ambitious 20–20–20 climate goals and the Low-Carbon Europe roadmap 2050. The European Commission empower the importance of achieving the objective of the recast Directive on energy performance of buildings (EPBD) that new buildings built from 2021 onwards will have to be nearly zero-energy buildings. The general belief is that the energy performance optimization of buildings requires an integrated design approach and cross-disciplinary teamwork to optimize the building's energy use and quality of indoor environment while satisfying the occupants' needs. In this context, there is a substantial need for professionals such as architects and engineers specifically trained and educated in integrated design approach and trained to work in cross-disciplinary teams. To be able to push forward the development, it is essential that educational institutions foster professionals with such knowledge, skills and competences. An initiative toward this direction is the EU-project of IDES-EDU: “Master and Post-Graduate education and training in multi-disciplinary teams”. The paper describes the necessity of more integrated and cross-disciplinary approaches to building design through state-of-the-art of the building sector and educational initiatives in the participating countries in the project, and through theory of design processes. The paper also communicates the results of newly developed cross-disciplinary education established by fifteen different educational institutions in Europe. Finally, the paper explains and discusses the challenges encountered during development and implementation of the education across different professions and countries. [Copyright &y& Elsevier]

In contrast with structural engineering, where the focus for design is on extreme values, for the proper assessment and modelling of air change rates in natural ventilation and infiltration, one must use the full range of input variables. Most of the modelling in infiltration research relies on hourly datasets for air infiltration balance. This consideration overlooks the intra-hour variability on meteorological variables. Additionally, it is customary to assume unchanging neutral atmospheric conditions when modelling, which affects the calculated airflows. This work intends to detail and quantify these effects in a case study representative of an average single-family dwelling in a Southern European climate setup. By comparing four setups with an increasing degree of complexity, a median of 0.04 h−1 of the hourly standard deviations in air change rates (ACH) is attributed to the time step effect. Approximately 43% of the occurrences experienced non-neutral atmospheric stability, skewing for stable conditions. This effect contributed to differences in the ACHs ranging from −0.202 to 0.131 h−1 at the 5% and 95% quantiles. Overall, by using hourly uniform distributions and smart sampling of meteorological variables, one ensures that the values in between and others potentially occurring around the boundaries are being considered for air change rates calculation, and therefore providing a more detailed picture of actual conditions. • Whole year impact of changing atmospherical stability and consideration of intra-hour variation of meterorological variables in a representative dwelling. • Non neutral atmospheric stability occurs in approximately 43% of the time. • Air change rate standard deviations by the time step effect reach 0.13 h−1 at the 95% quantile. • Non neutral conditions cause air change rates variability to range between −0.20 h−1 to +0.13 h−1 in comparison with an only neutral conditions scenario. [ABSTRACT FROM AUTHOR]

Abstract: This research has the goal of determining, comparing and substantiating the actual outcome of using sustainable practices, from a financial and environmental comfort point of view. The selected context was of interest due to the challenge of quality sustainable construction at controlled costs, meant for inhabitants of the Portuguese cooperative housing, due to its economical implications and especially its social ones. Two case studies of cooperative housing developments built in Portugal are presented, the first with characteristics of traditional construction and the second including sustainable building features. With these examples, it is intended to demonstrate that it is possible to build below cost limits imposed by law in order to substantially reduce energy consumption costs for heating and cooling as well as for domestic water heating. So, calculations of energy savings in indoor heating and cooling, as well as for domestic water heating are presented in this article for two specific situations: first, for a standard comfort use of housing, as opposed to a situation of real consumption of housing developments as obtained by monitoring carried out for one year in the same dwellings studied for standard comfort. The causes that lead to a substantial decrease in energy consumption in the building of sustainable construction are also described, as a starting point to the improvement of future cooperative housing. Finally are presented, for each of the two situations, calculations of the payback period of investment, according to the specific incremental cost in sustainable construction and the benefits in reducing consumptions. The results show an effective contribution to the improvement of the environment and environmental comfort, due to sustainable construction. [Copyright &y& Elsevier]

Abstract: Conservation of ancient buildings is a major issue for modern societies, both from economical and cultural viewpoints. Information about the ancient built heritage is vital to plan adequate remedial measures. Taking a historic centre in Portugal as a case study, this paper presents an extensive survey of building typology and materials, damage in the building envelope, indoor survey of damage, and measurements in indoor air temperature and relative humidity. Water-related problems can be confirmed as the single most important defect combined with inadequate sun exposure, ventilation and heating, and excessive moisture indoor production. Extremely low temperatures, high humidity and presence of mould therefore, compromise the indoor quality of life of the inhabitants; and make urgent repair at many levels necessary. [Copyright &y& Elsevier]

• District scale renovation can achieve cost-efficiency while reducing both energy use and CO 2 -emissions. • Drivers for district renovation are not restricted to energy savings and emission reductions. • Indoor climate, improving the overall building quality, image and value plays a key role for involved stakeholders. • Available funding is the most decisive factor in carrying out district scale renovations. Renovation at district scale is a key strategy to reduce CO 2 emissions by optimising the implementation of renewable energy sources and taking advantage of economy of scale. This paper focuses on analysing good practice examples on energy renovations at district scale. The paper adapts a qualitative research methodology in four phases, including the multi-perspective analysis of nine exemplary renovation projects in six European countries, including identification of drivers and barriers of different stakeholders. It is found that the drivers for a district renovation are not restricted to energy savings, but typically also include improving the overall quality of life as well as the image and economic value of a district. Moreover, the need for financial models that can alleviate split-incentive problems between investors and resident organizations is identified. Barriers for carrying out a district renovation include that there is a need to comply with energy standards, that the renovation scope had to be limited to avoid a noticeable rent increase and that resettling of tenants during the renovation is often not possible. Lessons learned include that good communication amongst the different stakeholders, especially with residents, plays a key role for the success of the project. Furthermore, a strong leadership is needed to coordinate activities due to the great number of stakeholders. [ABSTRACT FROM AUTHOR]

Abstract: Cryptococcal infection has been increasing among immunosuppressed population. We report three cases of Cryptococcus neoformans infection in immunosuppressed patients – two renal transplanted and one with lupus nephritis. Early infection (<3months) was diagnosed in two – an allograft Cryptococcus infection and a central nervous system involvement. The third, a 10-year transplant vintage patient, presented with cryptococcal meningitis. Amphotericin B provided good clinical outcomes. We outline the importance of suspicion for cryptococcal infection in immunosuppressed patients. [Copyright &y& Elsevier]

A full characterization of a building air leakage is labour intensive. As results of laboratory and mock-up experimentation rarely portray in situ conditions, the assessment of real case studies bring added value. Still, the results of experimentation of the latter face more challenges than the former. In this work a full quantitative and qualitative assessment of air leakage paths is performed, using a light steel framing (LSF) modular building with structural insulated panels (SIPs) as case study. Blower-door measurements undergo for a sealing campaign of eleven steps, a technique often described as reductive sealing. Additionally, smoke tracer measurements were carried out to visually identify the air leakage locations. The application of three regression methods resulted in different uncertainty estimates. Less than 7% of the total air leakage was not attributed to one of the considered types of air leakage paths. Assessing less impacting leakage paths first and placing similar types of air leakage paths in a consecutive sealing order seems to be the most correct strategy when using the reductive sealing technique. On average, at a reference pressure difference of 4 Pa, the sealing step uncertainty averaged, 9.9%, 18.8%, and 27.5%, depending on the method used for regression of the blower door test results. Despite the highest calculated uncertainty, literature shows that the application of the method leading to it, Weighted Line of Organic Correlation (WLOC), provides the results in closer agreement with the observed uncertainty of measurements. • Evaluation of the air leakage distribution of a modular building; • Air leakage contribution between types of air leakage paths range from 0.4% to 17.1%; • On average, WLOC provided values 2.8 times higher than the ones of OLSy. [ABSTRACT FROM AUTHOR]