Nuclear-Mitochondrial Intergenomic Communication Disorders

The focus of this chapter is to review the clinical and molecular etiologies of nuclear defects involved in mtDNA stability and in mitochondrial protein synthesis. The overview done here will hopefully provide insights towards best diagnostic strategies of mitochondrial cross–talk disorders, being useful for clinicians when facing similar cases. Additionally we will present a diagnostic algorithm for these diseases based on our knowledge

Supraglottic mucopyocele with cardio-respiratory arrest

Os quistos da laringe representam um grupo de lesões benignas raras que podem causar obstrução respiratória importante e até mesmo a morte se não tratadas adequadamente. Apresentam dificuldade no seu diagnóstico, pois em regra são achados do exame objectivo, em pacientes com sintomas inespecíficos ou mesmo assintomáticos. Os autores apresentam o caso de um doente de 47 anos, previamente saudável, que num período aproximado de seis horas, evoluiu de queixas compatíveis com sensação de corpo estranho laríngeo para dificuldade respiratória extrema, que culminou com paragem cardio-respiratória por obstrução da via aérea. A mesma foi revertida através de entubação orotraqueal e administração de adrenalina EV. Após três dias de tratamento médico com corticoterapia sistémica e antibioterapia de largo espectro, o doente encontrava-se em ventilação espontânea sem necessidade de entubação orotraqueal. Nessa altura, verificou-se a presença de neoformação na face lingual da epiglote que foi removida sob microlaringoscopia em suspensão, sendo histologicamente compatível com lesão quística.info:eu-repo/semantics/publishedVersio

Performance analytics for regulation in retail water utilities: Guiding asset management by identifying peers and targets

This research evaluates the performance of water supply utilities operating at the retail level in Portugal concerning asset management practices. The study’s main innovative feature is identifying peers and targets to guide improvements in the sector. Reliable data collected by the regulatory authority for water and waste services in Portugal (ERSAR) are employed to design two composite indicators reflecting different dimensions of asset management: operational conditions and management systems. Based on the Data Envelopment Analysis technique, the Benefit-of-the-Doubt model is employed in robust and conditional formulations. The role of the context on utilities’ performance is also investigated. The results show that the direct management model is unfavourable concerning developing structured management systems, whilst urban environments favour managerial advancement. Rural and semi-urban environments favour “good” operational results in infrastructures. The pool of peers obtained for each utility and the quantification of targets based on the observed achievements by those peers facilitates the search for industry best practices and promotes continuous improvement. Given the high heterogeneity in asset management performance within the sector, the utility-specific target-setting approach illustrated in this paper can support a regulatory policy review for determining more realistic goals

The measurement of asset management performance of water companies

This study explores asset management performance of Portuguese water supply companies operating in the bulk market. The focus of the analysis are the managerial practices and the condition of infrastructures. This assessment is based on the information conveyed by the indicators collected by the Portuguese water and waste services’ regulator authority (ERSAR) between 2016 and 2020. The main contribution of this research is to propose innovative methods to enhance the knowledge on asset management practices in the water sector. Two Benefit-of-the-Doubt (BoD) Composite Indicators are developed to highlight different aspects of asset management approaches. The first reflects organisations’ performance in maintaining their infrastructures at acceptable operational levels, and the other reveals their maturity in asset management practices. Robust and conditional approaches for estimating the BoD indicators are applied, allowing to obtain results that account for the effect of contextual variables on companies’ performance. Additionally, the performance of the companies is analysed over a 5-year period. The results show that there is significant room for improvement given the indicators’ values estimated in the benchmarking analysis. The type of management systems and areas of intervention (urban, semi-urban or rural) are factors that present significant impact in asset management performance. The analysis of trends in the evolution of performance over time revealed improvements both in the companies’ managerial practices and operational results

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Panorama da cultura do feijão-caupi no Brasil.

o feijão-caupi é um importante componente da dieta alimentar das populações das regiões Nordeste e Norte do Brasil e atualmente está se expandindo para a região Centro-oeste. O feijão-caupi adapta-se bem a praticamente todos os ecossistemas dessas regiões, sendo cultivado desde o ecossistema de caatinga até ao amazônico. Além disso faz simbiose com bactérias do grupo rizóbio, constituindo em um importante fixador de nitrogênio atmosférico. Há uma grande diversidade de tipos de grãos em feijão-caupi, entretanto, os de maior valor comercial são os tipos brancão, branco, sempre verde e canapu, este tipo, principalmente, nas áreas semi-áridas da região Nordeste. O feijão-caupi é de fácil manejo. Talvez por isso, seja cultivado em uma gama muito grande de sistemas de produção, desde os consorciados com diferentes culturas até os cultivos solteiros. Por se tratar de uma cultura de grande valor alimentar e de ampla adaptação, o feijão-caupi é uma cultura de grande potencial estratégico

Water Utility Service Quality Index: A customer-centred approach for assessing the quality of service in the water sector

This work delves into the crucial role of service quality in the water supply and sanitation sector. Despite extensive research and implementation of quality management practices in this sector, a universally accepted definition of quality is still lacking, resulting in various service quality assessment procedures that are difficult to compare. To address this issue, the World Bank launched the ‘Utility of the Future’ (UoF) programme, aiming to guide water service providers in their efforts to become future-focused utilities that offer reliable, safe, inclusive, transparent, and responsive services through best-fit practices. Building upon the framework provided by the UoF programme, this study proposes the Water Utility Service Quality Index (WUSQI) - a composite indicator that reflects the quality of service provided by water supply and sanitation utilities from a customer perspective. Based on Data Envelopment Analysis, the Benefit-of-the-Doubt approach is employed to assign weights for aggregating the indicators representing the diverse performance dimensions. The study operationalises the WUSQI to assess the quality of Portuguese wholesale water and wastewater companies using data collected by the national regulator of water and waste services. A Multiple Criteria Decision Analysis technique, the Deck of Cards method, is used to specify an indicator of transparency from the information made available by the regulated utilities. The results show the effectiveness of this tool for evaluating and measuring service quality at the company level. Additionally, the findings highlight areas for improvement in the utilities’ performance. By enabling companies and regulators to identify areas for improvement, the WUSQI can support the delivery of high-quality services to customers

Electron spin resonance investigation of Mn^{2+} ions and their dynamics in manganese doped SrTiO_3

Using electron spin resonance, lattice position and dynamic properties of Mn2+ ions were studied in 0.5 and 2 % manganese doped SrTiO3 ceramics prepared by conventional mixed oxide method. The measurements showed that Mn2+ ions substitute preferably up to 97 % for Sr if the ceramics is prepared with a deficit of Sr ions. Motional narrowing of the Mn2+ ESR spectrum was observed when temperature increases from 120 K to 240-250 K that was explained as a manifestation of off-center position of this ion at the Sr site. From the analysis of the ESR spectra the activation energy Ea = 86 mV and frequency factor 1/?0 ? (2-10)x10^(-14) 1/s for jumping of the impurity between symmetrical off-center positions were determined. Both values are in agreement with those derived previously from dielectric relaxation. This proves the origin of dielectric anomalies in SrTiO3:Mn as those produced by the reorientation dynamics of Mn2+ dipoles.Comment: 16 pages, 6 figure

World state of quality: a frontier approach to benchmark the performance of countries worldwide

Purpose - The World State of Quality (WSQ) Project aims to evaluate, analyse, rank and categorise countries according to their performance in quality as a multidimensional concept. The Project involves the computation of an overall score for each country, obtained as a weighted average of ranking positions of 16 metrics, with weights determined by a panel of experts. Methodology-This work proposes an alternative strategy for that procedure, using a Benefit-of-the-Doubt (BoD) Composite Indicator approach under the framework of Data Envelopment Analysis (DEA). This strategy avoids the need of using subjective weights and normalising data by rank positions, using a more objective procedure to obtain the countries’ ranking. A new overall score of the World State of Quality is proposed, which allows the categorisation of countries’ performance. The novel insights resulting from the use of this methodology are discussed, including the identification of strengths and weaknesses of the various countries, and the peers that can be used for facilitating continuous improvements policies. Findings - The results show that the BoD approach and the original method used by the WSQ Project present comparable results. Countries’ strengths and weaknesses and their suitable peers and targets for benchmarking are presented with illustrative examples. Originality/value – A novel frontier approach for countries’ benchmarking regarding their performance in quality is proposed, incorporating new insights into the current method.FCT - Fundação para a Ciência e a Tecnologia(2021.05244)The authors acknowledge the financial support provided by FCT- Fundação para a Ciência e a Tecnologia (Portuguese National Funding Agency for Science, Research and Technology) through PhD research grants and SFRH/BD/131285/2017. This work has been supported by FCT – Fundação para a Ciência e Tecnologia within the R&D Units Project Scope: UIDB/00319/2020