Implementação de um sistema de datação numa máquina de empacotamento

Mestrado em Engenharia e Gestão IndustrialTodas as empresas devem dirigir os seus esforços para as constantes mudanças no mercado. A criação do presente projeto teve o intuito de adaptar uma máquina de empacotamento de chocolates já existente da empresa Bosch de acordo com as exigências do mercado. Até ao momento a máquina não incluía um sistema que permitisse fazer a inscrição da data de validade nos chocolates, sendo um processo executado separadamente e da responsabilidade do cliente. Deste modo, a implementação deste sistema permite que a empresa satisfaça os requisitos dos atuais clientes e para futuros clientes, contribuindo para o aumento do seu nicho de mercado exigindo apenas um investimento inicial. A criação deste sistema envolveu um conjunto de fases sequenciais tais como processo de modelação 3D do sistema, estudo de requisitos mecânicos e financeiros e análise de diferentes cenários até ao produto final.Every organization should maximize their efforts to follow changes in the market. The current project was created in order to satisfy a market demand which involves the modification of a product made by Bosch, namely, a chocolate wrapping machine. In the past, this machine did not contain a system to include the inscription of the date on the product. Rather, this step was performed separately by the client. Therefore, the implementation of this system in the wrapping apparatus is satisfying a customer need by reducing the number of processing steps and facilitating handling on the client-side. This may allow Bosch to increase their market niche. The creation of this system involved a series of steps, including the 3D modeling and analyses of mechanical and financial requirements until completion of the final product

Avaliar a Pobreza Energética em Portugal: Uma nova abordagem metodológica

Dissertação de Mestrado em Energia para a Sustentabilidade apresentada à Faculdade de Ciências e TecnologiaIn the "Third Pan-European Report on Energy Poverty of the EU Energy Poverty Observatory" Bouzarovski et al. (2020) recognize that "energy poverty in Portugal is a largely neglected phenomenon", reinforcing the reduce work done by the country on the subject. In 2013, EU-SILC indicated that Portugal had between 20% and 29% of its households living in energy poverty (Simões et al., 2016). Seven years later, the Portuguese Government still does not define the concept and its form of measurement.This Dissertation explores the concept of energy poverty, analyzing the work done in the four countries of the European Union with an official concept and form of measurement. It also reviews the existing indicators in the literature for measuring energy poverty and its characteristics. Given the lack of official data in Portugal, a methodology for measuring energy poverty in the country was developed. This methodology presents an innovation by using data regarding the ideal energy consumption of each household to live in thermal comfort, which allows eliminating all the missing cases of households that do not consume energy because they know they cannot afford it. The methodology is based on the use of the Portal CasA+ Energy Efficiency Simulator for Building, whose results are later combined with three selected energy poverty indicators (10%, MIS, and LIHC). These indicators include in their variables data that characterize each of the 16 different types of households in the Central Region of Portugal considered.From the 864 diagnoses made, and based on the 10% indicator it is concluded that 50.7% of these households live in energy poverty, while according to the MIS indicator the percentage of households in energy poverty rises to 65.6%. If the LIHC indicator is used, the percentage of energy poor households is 17.4%. This analysis also allowed us to conclude that the equipment used for heating the houses is the one that most influenced the results.No "Terceiro relatório pan-europeu sobre pobreza energética do Observatório da Pobreza Energética da UE", Bouzarovski et al. (2020), reconhecem que "a pobreza energética em Portugal é um fenómeno amplamente negligenciado", reforçando o pouco trabalho desenvolvido pelo país sobre o assunto. Em 2013, o UE-SILC indicava que Portugal tinha entre 20% e 29% dos seus agregados familiares a viver em pobreza energética (Simões et al., 2016). Sete anos mais tarde, o Governo português continua sem definir o conceito e a sua forma de medição.A presente dissertação explora o conceito de pobreza energética, analisando o trabalho desenvolvido nos quatro países da União Europeia com um conceito e forma de medição oficiais. Faz também uma revisão sobre os indicadores existentes na literatura para medição da pobreza energética e as suas características. Face à inexistência de dados oficiais em Portugal, foi desenvolvida uma metodologia de medição de pobreza energética para o país. Esta metodologia apresenta uma inovação pelo uso de dados relativos ao consumo ideal de energia de cada agregado familiar para viver com conforto térmico, o que permite eliminar todos os casos omissos de agregados familiares que não consomem energia por saberem que não a podem pagar. A metodologia baseia-se no uso do Simulador de Eficiência Energética para Edifícios do Portal CasA+, cujos resultados obtidos são posteriormente conjugados com três indicadores de pobreza energética selecionados para o efeito (10%, MIS e LIHC). Estes indicadores incluem nas suas variáveis dados que caracterizam cada um dos 16 tipos diferentes de agregados familiares da Região Centro de Portugal considerados.A partir dos 864 diagnósticos realizados, e, tendo por base o indicador de 10% conclui-se que 50,7% desses agregados vive em pobreza energética, ao passo que segundo o indicador MIS a percentagem de agregados em pobreza energética sobe para 65,6%. Já se for utilizado indicador LIHC a percentagem de famílias pobres em termos energéticos é de 17,4%. Esta análise permitiu também concluir que os equipamentos usados para aquecimento das habitações são os que mais influenciaram os resultados

Molecular and Functional Characterization of CBAVD-Causing Mutations Located in CFTR Nucleotide-Binding Domains

Background: About 98% of male affected with cystic fibrosis (CF [MIM 219700]) are infertile due to bilateral absence of vas deferens (CBAVD [MIM 277180]), which makes up 1-2 % of all cases with male infertility. A previous screening of the entire coding region of the cystic fibrosis transmembrane conductance regulator gene (CFTR [MIM 602421]) in CBAVD patients identified three novel mutations: P439S is located in the first nucleotide binding domain (NBD1) of CFTR, whereas P1290S and E1401K are located in NBD2. Methods: We analysed the effects of these novel mutations on CFTR processing and chloride (Cl-) channel activity. Results: Although maturation patterns were not affected, total amounts of mature P439S-CFTR and P1290S-CFTR were reduced. Confocal microscopy showed correct membrane localisation of E1401K-CFTR, whereas P439S-CFTR and P1290S-CFTR mutants were located mainly in the cytoplasm. Iodide influx assay and whole-cell patch clamp demonstrated significantly reduced cAMP-dependent anion conductances for all three mutants. Conclusion: Dysfunction of CFTR is caused by either defective CFTR trafficking (P439S and P1290S) or/and Cl- channel function (P1290S and E1401K). Thus reduced Cl- conductance caused by the three CFTR mutations affects normal development of vas deferens and leads to CBAVD, but the remaining function is sufficient to prevent other typical CF symptoms

Touraine-Solente-Gole syndrome: pathogenic variant in SLCO2A1 presented with polyarthralgia and digital clubbing

Abstract Background Primary Hypertrophic Osteoarthropathy (PHO), also known as Touraine-Solente-Gole Syndrome, is a rare, multisystemic autosomal recessive disorder caused by pathogenic variants in the 15-hydroxyprostaglandin dehydrogenase (HPGD) or Solute Carrier Organic Anion Transporter Family Member 2A1 (SLCO2A1) genes. However, autosomal dominant transmission has also been described in some families with incomplete penetrance. PHO usually starts in childhood or adolescence, presenting with digital clubbing, osteoarthropathy, and pachydermia. We described a complete form of the syndrome in a male patient with a homozygous variant in the SLCO2A1 gene (c.1259G > T). Case presentation A 20-year-old male was referred to our Pediatric Rheumatology Clinic with a five-year history of painful and swollen hands, knees, ankles and feet, prolonged morning stiffness and relief with non-steroidal antiinflammatory drugs. He also reported late onset facial acne and palmoplantar hyperhidrosis. Family history was irrelevant and parents were non-consanguineous. On clinical examination, he presented clubbing of the fingers and toes, moderate acne and marked facial skin thickening with prominent scalp folds. He had hand, knee, ankles and feet swelling. Laboratory investigations showed elevated inflammatory markers. Complete blood count, renal and hepatic function, bone biochemistry were normal, as well as immunological panel. Plain radiographs revealed soft tissue swelling, periosteal ossification and cortical thickening of the skull, phalanges, femur and toe acroosteolysis. Due to the absence of other clinical signs suggesting a secondary cause, we suspected PHO. A genetic study revealed a likely pathogenic variant, c.1259G > T(p.Cys420Phe), in homozygosity in the SLCO2A1 gene, thus confirming the diagnosis. The patient started oral naproxen with significant clinical improvement. Conclusions PHO should be kept in the differential diagnosis of inflammatory arthritis affecting children, often misdiagnosed as Juvenile Idiopathic Arthritis (JIA). To the best of our knowledge, this is the second genetically confirmed case of PHO in a Portuguese patient (first variant c.644 C > T), both made at our department

Soroprevalência e fatores de risco associados a Neospora caninum em ovinos comerciais do noroeste do Rio Grande do Sul, Brasil

Neospora caninum is a widely distributed parasite, which significantly impacts reproduction in ruminants. This study aimed to survey the seroprevalence and risk factors associated with neosporosis infection in commercial herds of sheep in the northwest of Rio Grande do Sul, Brazil. Three hundred sheep serum samples were used to investigate anti-N. caninum antibodies using indirect immunofluorescence reaction at a 1:40 dilution. The presence of anti-N. caninum antibodies was detected in 20.3% (61/300) of the samples evaluated; however, positive reactions were observed in all (13/13) flocks sampled. Sheep breeds > 1 year of age had 1.2-fold higher risk for infection with N. caninum (odds ratio 2.2 [95% confidence interval 1-5.1]; P < 0.001). These findings should raise awareness on the importance of serological screening, identification of risk factors, and maintenance of preventive measures, such as not allowing dogs to contact sheep feed and not offering the placental remains of ruminants to canids.Neospora caninum é um parasita amplamente distribuído e de grande importância para a reprodução de ruminantes. O objetivo deste estudo foi realizar um levantamento da soroprevalência e dos fatores de risco associados à neosporose em rebanhos comerciais de ovinos no noroeste do Rio Grande do Sul. Foram utilizadas 300 amostras de soro de ovelhas para investigar anti-N. caninum por Reação de Imunofluorescência Indireta (IFAT) com uma diluição de 1:40. A presença de anticorpos anti-N. caninum foi detectada em 20.3 % (61/300) das amostras avaliadas. Reações positivas foram observadas em todos (13/13) os rebanhos amostrados. Ovinos com mais de um ano tiveram 1.2 mais chances de serem infectados por N. caninum (odds ratio 2.2, intervalo de confiança 1-5.1) (P < 0.001). Com esses dados, chamamos atenção para a importância da triagem sorológica, identificação de fatores de risco e manutenção de medidas de prevalência, como não permitir que os cães entrem em contato com a alimentação das ovelhas e não oferecer os restos placentários dos ruminantes aos canídeos

Association of Cystic Fibrosis Genetic Modifiers with Congenital Bilateral Absence of the Vas Deferens

Objective: To investigate whether genetic modifiers of cystic fibrosis (CF) lung disease also predispose to congenital bilateral absence of the vas deferens (CBAVD) in association with cystic fibrosis transmembrane conductance regulator (CFTR) mutations. We tested the hypothesis that polymorphisms of transforming growth factor (TGF)-beta 1 (rs 1982073, rs 1800471) and endothelin receptor type A (EDNRA) (rs 5335, rs 1801708) are associated with the CBAVD phenotype. Design: Genotyping of subjects with clinical CBAVD. Setting: Outpatient and hospital-based clinical evaluation. Patient(s): DNA samples from 80 subjects with CBAVD and 51 healthy male controls from various regions of Europe. This is one of the largest genetic studies of this disease to date. Intervention(s): None. Main Outcome Measure(s): Genotype analysis. Result(s): For single nucleotide polymorphism (SNP) rs 5335, we found increased frequency of the CC genotype among subjects with CBAVD. The difference was significant among Turkish patients versus controls (45.2% vs. 19.4%), and between all cases versus controls (36% vs. 15.7%). No associations between CBAVD penetrance and polymorphisms rs 1982073, rs 1800471, or rs 1801708 were observed. Conclusion(s): Our findings indicate that endothelin receptor type A polymorphism rs 5335 may be associated with CBAVD penetrance. To our knowledge, this is the first study to investigate genetic modifiers relevant to CBAVD. (Fertil Steril (R) 2010; 94: 2122-7. (C) 2010 by American Society for Reproductive Medicine.)Wo

Association of cystic fibrosis genetic modifiers with congenital bilateral absence of the vas deferens

Objective: To investigate whether genetic modifiers of cystic fibrosis (CF) lung disease also predispose to congenital bilateral absence of the vas deferens (CBAVD) in association with cystic fibrosis transmembrane conductance regulator (CFTR) mutations. We tested the hypothesis that polymorphisms of transforming growth factor (TGF)-beta 1 (rs 1982073, rs 1800471) and endothelin receptor type A (EDNRA) (rs 5335, rs 1801708) are associated with the CBAVD phenotype. Design: Genotyping of subjects with clinical CBAVD. Setting: Outpatient and hospital-based clinical evaluation. Patient(s): DNA samples from 80 subjects with CBAVD and 51 healthy male controls from various regions of Europe. This is one of the largest genetic studies of this disease to date. Intervention(s): None. Main Outcome Measure(s): Genotype analysis. Result(s): For single nucleotide polymorphism (SNP) rs 5335, we found increased frequency of the CC genotype among subjects with CBAVD. The difference was significant among Turkish patients versus controls (45.2% vs. 19.4%), and between all cases versus controls (36% vs. 15.7%). No associations between CBAVD penetrance and polymorphisms rs 1982073, rs 1800471, or rs 1801708 were observed. Conclusion(s): Our findings indicate that endothelin receptor type A polymorphism rs 5335 may be associated with CBAVD penetrance. To our knowledge, this is the first study to investigate genetic modifiers relevant to CBAVD. (Fertil Steril (R) 2010; 94: 2122-7. (C) 2010 by American Society for Reproductive Medicine.