Prototipagem rápida de um modelo complexo para fabricação de moldes de gesso para barbotina cerâmica

Computer assisted designing (CAD) is well known for several decades and employed for ceramic manufacturing almost since the beginning, but usually employed in the first part of the projectual ideation processes, neither in the prototyping nor in the manufacturing stages. The rapid prototyping machines, also known as 3D printers, have the capacity to produce in a few hours real pieces using plastic materials of high resistance, with great precision and similarity with respect to the original, based on unprecedented digital models produced by means of modeling with specific design software or from the digitalization of existing parts using the so-called 3D scanners. The main objective of the work is to develop the methodology used in the entire process of building a part in ceramics from the interrelationship between traditional techniques and new technologies for the manufacture of prototypes. And to take advantage of the benefits that allow us this new reproduction technology. The experience was based on the generation of a complex piece, in digital format, which served as the model. A regular 15 cm icosahedron presented features complex enough not to advise the production of the model by means of the traditional techniques of ceramics (manual or mechanical). From this digital model, a plaster mold was made in the traditional way in order to slip cast clay based slurries, freely dried in air and fired and glazed in the traditional way. This experience has shown the working hypothesis and opens up the possibility of new lines of work to academic and technological levels that will be explored in the near future. This technology provides a wide range of options to address the formal aspect of a part to be performed for the field of design, architecture, industrial design, the traditional pottery, ceramic art, etc., which allow you to amplify the formal possibilities, save time and therefore costs when drafting the necessary and appropriate matrixes to each requirement.Projeto assistido por computador (CAD) é bem conhecido por várias décadas e empregado na fabricação cerâmica desde o início, mas usualmente empregado na primeira parte dos processos na concepção do projeto, nem na prototipagem, nem nas fases de fabricação. As máquinas de prototipagem rápida, também conhecidas como impressoras 3D têm a capacidade de produzir, em poucas horas, peças utilizando materiais plásticos de alta resistência, com grande precisão e similaridade com relação ao original, com base em modelos digitais inéditos produzidos por meio de modelagem com softwares de projeto específico ou a partir da digitalização das peças existentes, utilizando os chamados scanners 3D. O principal objetivo deste trabalho é desenvolver a metodologia utilizada em todo o processo de construção de uma parte em cerâmica da inter-relação entre as técnicas tradicionais e novas tecnologias para a fabricação de protótipos. E para aproveitar os benefícios que nos permitem esta nova tecnologia de reprodução. A experiência baseou-se na geração de uma peça complexa, em formato digital, que serviu como modelo. Um icosaedro regular de 15 cm, complexo o suficiente para não sugerir a produção do modelo por meio das técnicas tradicionais cerâmicas (manual ou mecânica). A partir deste modelo digital, um molde de gesso feito na maneira tradicional para colagem por barbotina de massas à base de argila, secagem livre ao ar, queimada e esmaltada de maneira tradicional. Esta experiência mostrou a hipótese de trabalho e abre a possibilidade de novas linhas de trabalho a níveis acadêmicos e tecnológicos que serão explorados em um futuro próximo. Esta tecnologia oferece uma grande variedade de opções para lidar com o aspecto formal de uma peça a ser executada no campo do projeto, arquitetura, projeto industrial, a cerâmica tradicional, a arte cerâmica, etc., que permitem ampliar as possibilidades formais, economizar tempo e, portanto, os custos na elaboração das matrizes necessárias e adequadas para cada exigência.Centro de Tecnología de Recursos Minerales y Cerámic

Genotypic classification of patients with Wolfram syndrome: insights into the natural history of the disease and correlation with phenotype

Purpose: Wolfram syndrome is a degenerative, recessive rare disease with an onset in childhood. It is caused by mutations in WFS1 or CISD2 genes. More than 200 different variations in WFS1 have been described in patients with Wolfram syndrome, which complicates the establishment of clear genotype-phenotype correlation. The purpose of this study was to elucidate the role of WFS1 mutations and update the natural history of the disease. Methods: This study analyzed clinical and genetic data of 412 patients with Wolfram syndrome published in the last 15 years. Results: (i) 15% of published patients do not fulfill the current ­inclusion criterion; (ii) genotypic prevalence differences may exist among countries; (iii) diabetes mellitus and optic atrophy might not be the first two clinical features in some patients; (iv) mutations are nonuniformly distributed in WFS1; (v) age at onset of diabetes mellitus, hearing defects, and diabetes insipidus may depend on the patient"s genotypic class; and (vi) disease progression rate might depend on genotypic class. Conclusion: New genotype-phenotype correlations were established, disease progression rate for the general population and for the genotypic classes has been calculated, and new diagnostic criteria have been proposed. The conclusions raised could be important for patient management and counseling as well as for the development of treatments for Wolfram syndrome

Mimicry and well known genetic friends: molecular diagnosis in an Iranian cohort of suspected Bartter syndrome and proposition of an algorithm for clinical differential diagnosis.

BACKGROUND: Bartter Syndrome is a rare, genetically heterogeneous, mainly autosomal recessively inherited condition characterized by hypochloremic hypokalemic metabolic alkalosis. Mutations in several genes encoding for ion channels localizing to the renal tubules including SLC12A1, KCNJ1, BSND, CLCNKA, CLCNKB, MAGED2 and CASR have been identified as underlying molecular cause. No genetically defined cases have been described in the Iranian population to date. Like for other rare genetic disorders, implementation of Next Generation Sequencing (NGS) technologies has greatly facilitated genetic diagnostics and counseling over the last years. In this study, we describe the clinical, biochemical and genetic characteristics of patients from 15 Iranian families with a clinical diagnosis of Bartter Syndrome. RESULTS: Age range of patients included in this study was 3 months to 6 years and all patients showed hypokalemic metabolic alkalosis. 3 patients additionally displayed hypercalciuria, with evidence of nephrocalcinosis in one case. Screening by Whole Exome Sequencing (WES) and long range PCR revealed that 12/17 patients (70%) had a deletion of the entire CLCNKB gene that was previously identified as the most common cause of Bartter Syndrome in other populations. 4/17 individuals (approximately 25% of cases) were found to suffer in fact from pseudo-Bartter syndrome resulting from congenital chloride diarrhea due to a novel homozygous mutation in the SLC26A3 gene, Pendred syndrome due to a known homozygous mutation in SLC26A4, Cystic Fibrosis (CF) due to a novel mutation in CFTR and apparent mineralocorticoid excess syndrome due to a novel homozygous loss of function mutation in HSD11B2 gene. 1 case (5%) remained unsolved. CONCLUSIONS: Our findings demonstrate deletion of CLCNKB is the most common cause of Bartter syndrome in Iranian patients and we show that age of onset of clinical symptoms as well as clinical features amongst those patients are variable. Further, using WES we were able to prove that nearly 1/4 patients in fact suffered from Pseudo-Bartter Syndrome, reversing the initial clinical diagnosis with important impact on the subsequent treatment and clinical follow up pathway. Finally, we propose an algorithm for clinical differential diagnosis of Bartter Syndrome

Severity of Giardia infection associated with post-infectious fatigue and abdominal symptoms two years after

<p>Abstract</p> <p>Background</p> <p>A high rate of post-infectious fatigue and abdominal symptoms two years after a waterborne outbreak of giardiasis in Bergen, Norway in 2004 has previously been reported. The aim of this report was to identify risk factors associated with such manifestations.</p> <p>Methods</p> <p>All laboratory confirmed cases of giardiasis (n = 1262) during the outbreak in Bergen in 2004 received a postal questionnaire two years after. Degree of post-infectious abdominal symptoms and fatigue, as well as previous abdominal problems, was recorded. In the statistical analyses number of treatment courses, treatment refractory infection, delayed education and sick leave were used as indices of protracted and severe <it>Giardia </it>infection. Age, gender, previous abdominal problems and symptoms during infection were also analysed as possible risk factors. Simple and multiple ordinal logistic regression models were used for the analyses.</p> <p>Results</p> <p>The response rate was 81% (1017/1262), 64% were women and median age was 31 years (range 3-93), compared to 61% women and 30 years (range 2-93) among all 1262 cases. Factors in multiple regression analysis significantly associated with abdominal symptoms two years after infection were: More than one treatment course, treatment refractory infection, delayed education, bloating and female gender. Abdominal problems prior to <it>Giardia </it>infection were not associated with post-infectious abdominal symptoms. More than one treatment course, delayed education, sick leave more than 2 weeks, and malaise at the time of infection, were significantly associated with fatigue in the multiple regression analysis, as were increasing age and previous abdominal problems.</p> <p>Conclusion</p> <p>Protracted and severe <it>giardiasis </it>seemed to be a risk factor for post-infectious fatigue and abdominal symptoms two years after clearing the <it>Giardia </it>infection.</p

The Meaning of Body Experience Evaluation in Oncology

Evaluation of quality of life, psychic and bodily well-being is becoming increasingly important in oncology aftercare. This type of assessment is mainly carried out by medical psychologists. In this paper I will seek to show that body experience valuation has, besides its psychological usefulness, a normative and practical dimension. Body experience evaluation aims at establishing the way a person experiences and appreciates his or her physical appearance, intactness and competence. This valuation constitutes one’s ‘body image’. While, first, interpreting the meaning of body image and, second, indicating the limitations of current psychological body image assessment, I argue that the normative aspect of body image is related to the experience of bodily wholeness or bodily integrity. Since this experience is contextualized by a person’s life story, evaluation should also focus on narrative aspects. I finally suggest that the interpretation of body experience is not only valuable to assess a person’s quality of life after treatment, but that it can also be useful in counseling prior to interventions, since it can support patients in making decisions about interventions that will change their bodies. To apply this type of evaluation to oncology practice, a rich and tailored vocabulary of body experiences has to be developed

Not gods but animals : human dignity and vulnerable subjecthood

Drawing on earlier work on the conceptual structure of dignity, this paper will suggest a particular type of connectedness between vulnerability and human dignity; namely, that the ‘‘organizing idea’’ of human dignity is the idea of a particular sort of ethical response to universal human vulnerability. It is common ground among many, if not all, approaches to ethics that vulnerability requires us to respond ethically. Here, I argue that human dignity is distinctive among ethical values in that it values us because of, rather than in spite of, or regardless of, our universal vulnerability. The term ‘‘dignity’’ is used synonymously with ‘‘human dignity’’ here, since an investigation of the dignity of non-human entities forms no part of the present examination