A lean six sigma framework for continuous and incremental improvement in the oil and gas sector

This article aims to explore synergies between Lean Production (LP) and Six Sigma principles in order to propose a Lean Six Sigma (LSS) framework for continuous and incremental improvement in the oil and gas sector. The Three-Dimensional LSS Framework seeks to provide various combinations about the integration between LP principles, DMAIC cycle and PDCA cycle to support operations management needs. Design/methodology/approach - The research method is composed of two main steps: (i) diagnostic of current problems and proposition of a conceptual framework that qualitatively integrates synergistic aspects of LP and Six Sigma; and (ii) analysis of the application of the construct through semi-structured interviews with leaders from oil and gas companies to assess and validate the proposed framework. Findings - As a result, a conceptual framework of LSS is developed contemplating the integration of LP and Six Sigma and providing a systemic and holistic approach to problemsolving through continuous and incremental improvement in the oil and gas sector. Originality/value - This research is different from previous studies because it integrates LP principles, DMAIC and PDCA cycles into a unique framework that fulfils a specific need of oil and gas sector. It presents a customized LSS framework that guides wastes and costs reduction, while enhances quality and reduces process variability to elevate efficiency in operations management of this sector. The paper type is an original research that present new and original scientific findings.N/

Phenotype and Genotype of Portuguese Patients with Smith-Lemli-Opitz Syndrome

A síndrome de Smith-Lemli-Opitz (SLOS) é uma síndrome polimalformativa de transmissão autossómica recessiva causada por um défice metabólico da biossíntese do colesterol, que se caracteriza por dismorfias craniofaciais, anomalias congénitas de vários órgãos (salientando-se as do esqueleto e do aparelho urogenital), restrição de crescimento intra-uterino (RCIU), alterações comportamentais e atraso mental. É causada por mutações no gene DHCR7, que codifica para a enzima 7-dehidrocolesterol reductase, responsável pelo último passo da via metabólica da síntese do colesterol. A SLOS caracteriza-se por níveis diminuídos de colesterol e concentrações altas do seu precursor, 7-dehidrocolesterol, no sangue e tecidos. Procedeu-se a uma análise comparativa dos fenótipo e genótipo de quinze casos de SLOS de origem portuguesa, e são tecidas considerações quanto às dificuldades e limitações inerentes ao diagnóstico, e ao facto de esta doença hereditária do metabolismo dever ser considerada no diagnóstico diferencial das situações de (i) hipocolesterolémia, (ii) RCIU e (iii) síndromes polimalformativas,(especialmente quando crianças com atraso de crescimento apresentam simultaneamente sindactilia do segundo e terceiro dedos do pé e microcefalia e/ou narinas antevertidas entre outras anomalias)

An objective comparison of cell-tracking algorithms

We present a combined report on the results of three editions of the Cell Tracking Challenge, an ongoing initiative aimed at promoting the development and objective evaluation of cell segmentation and tracking algorithms. With 21 participating algorithms and a data repository consisting of 13 data sets from various microscopy modalities, the challenge displays today's state-of-the-art methodology in the field. We analyzed the challenge results using performance measures for segmentation and tracking that rank all participating methods. We also analyzed the performance of all of the algorithms in terms of biological measures and practical usability. Although some methods scored high in all technical aspects, none obtained fully correct solutions. We found that methods that either take prior information into account using learning strategies or analyze cells in a global spatiotemporal video context performed better than other methods under the segmentation and tracking scenarios included in the challenge

Challenges in the Definitive Diagnosis of Niemann–Pick Type C—Leaky Variants and Alternative Transcripts

Niemann-Pick type C (NPC, ORPHA: 646) is a neuro-visceral, psychiatric disease caused predominantly by pathogenic variants in the NPC1 gene or seldom in NPC2. The rarity of the disease, and its wide range of clinical phenotypes and ages of onset, turn the diagnosis into a significant challenge. Other than the detailed clinical history, the typical diagnostic work-up for NPC includes the quantification of pathognomonic metabolites. However, the molecular basis diagnosis is still of utmost importance to fully characterize the disorder. Here, the authors provide an overview of splicing variants in the NPC1 and NPC2 genes and propose a new workflow for NPC diagnosis. Splicing variants cover a significant part of the disease-causing variants in NPC. The authors used cDNA analysis to study the impact of such variants, including the collection of data to classify them as leaky or non-leaky pathogenic variants. However, the presence of naturally occurring spliced transcripts can misdiagnose or mask a pathogenic variant and make the analysis even more difficult. Analysis of the NPC1 cDNA in NPC patients in parallel with controls is vital to assess and detect alternatively spliced forms. Moreover, nonsense-mediated mRNA decay (NMD) analysis plays an essential role in evaluating the naturally occurring transcripts during cDNA analysis and distinguishing them from other pathogenic variants' associated transcripts.info:eu-repo/semantics/publishedVersio

Defeitos congénitos da glicosilação

Congenital disorders of glycosylation are a highly variable, rapidly expanding family of genetic diseases that result from defects in the synthesis of glycans. The vast majority of these monogenic diseases are inherited in an autosomal recessive way, but some types follow an autosomal dominant or X-linked inheritance. The present work aimed to review the state of the art of congenital disorders of glycosylation, including available therapeutic options, and present a simplified diagnostic approach to this group of diseases. Congenital disorders of glycosylation can be classified into four categories: N-linked glycosylation defects, O-linked glycosylation defects, combined glycosylation defects, and glycosphingolipid and glycosylphosphatidylinositol anchor synthesis defects. The phenotype may range from mild to severe, depending on disease severity. Clinical features include dysmorphic features, neurologic, dermatologic, cardiac, endocrine, immunologic, hematologic, gastrointestinal and liver involvement, and skeletal muscle abnormalities. As there is no universal or pathognomonic sign or symptom and no sensitive diagnostic test, it is of foremost importance to keep a high index of suspicion of these diseases. When a congenital disorder of glycosylation is suspected, the first step in screening is to perform serum transferrin isoelectric focusing. Molecular genetic testing is the most specific diagnostic test. Treatment is usually symptomatic, with specific treatment only available for some of these disorders. Since congenital defects of glycosylation may affect any organ at any age and have variable clinical presentation, they should be considered in the differential diagnosis of any patient with multiorgan involvement.info:eu-repo/semantics/publishedVersio

Critical analysis of organizational change process: evidences from a steel company

© 2020, Emerald Publishing Limited. Purpose: The purpose of this paper is to evaluate the main factors that influence the organizational change in a steel company. Design/methodology/approach: The methodological procedures used were literature review and survey. The literature review allowed the listing of 24 factors and these factors were grouped into three constructs (Behavioral Aspects, Cultural Aspects and Management Aspects). The survey allowed the quantification of each factor based on information provided by employees who work in the mentioned company. The data collected were analyzed using the Partial Least Squares-Structural Equation Modeling technique. Findings: For the Behavioral Aspects, the following factors were validated: fear of the unknown; insecurity and anxiety; stress and feeling of suffering. For the Cultural Aspects the following factors were validated: multiculturalism in the company; low degree of risk acceptance and low performance acceptance; excessive concerns about consensus. Finally, for the Management Aspects, the following factors were validated: lack of clarity in communications; lack of alignment of goals; lack of leadership engagement. Originality/value: The results are valuable for the company studied and for other managers interested in subject. The findings presented here can broaden the debate about this topic and contribute with professionals that wish to evaluate the mentioned factors

Nodal prices in an integrated energy system

*Corresponding author Abstract: This paper presents a multiperiod generalised network flow model used to analyse the economic interdependencies of integrated energy systems comprising the electric network and the various fuel supply and delivery systems in a medium term operational time frame. By using a network flow programming model, one can take advantage of much faster solution procedures than standard linear programming techniques; an issue of importance considering the dimensionality of such integrated systems. The nodal prices that are obtained as a byproduct of the optimisation algorithm provide a way to analyse the economic interdependencies between the various fuel networks and the electric network. A numerical example is presented to highlight the benefits of the methodology and illustrate how nodal prices in the electric network are influenced by the dynamics of the various fuel networks