Question Mark Perception - example test questions

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Auriculo-condylar syndrome: mapping of a first locus and evidence for genetic heterogeneity

Auriculo-condylar syndrome (ACS), an autosomal dominant disorder of first and second pharyngeal arches, is characterized by malformed ears (`question mark ears`), prominent cheeks, microstomia, abnormal temporomandibular joint, and mandibular condyle hypoplasia. Penetrance seems to be complete, but there is high inter-and intra-familial phenotypic variation, with no evidence of genetic heterogeneity. We herein describe a new multigeneration family with 11 affected individuals (F1), in whom we confirm intra-familial clinical variability. Facial asymmetry, a clinical feature not highlighted in other ACS reports, was highly prevalent among the patients reported here. The gene responsible for ACS is still unknown and its identification will certainly contribute to the understanding of human craniofacial development. No chromosomal rearrangements have been associated with ACS, thus mapping and positional cloning is the best approach to identify this disease gene. To map the ACS gene, we conducted linkage analysis in two large ACS families, F1 and F2 (F2; reported elsewhere). Through segregation analysis, we first excluded three known loci associated with disorders of first and second pharyngeal arches (Treacher Collins syndrome, oculo-auriculo-vertebral spectrum, and Townes-Brocks syndrome). Next, we performed a wide genome search and we observed evidence of linkage to 1p21.1-q23.3 in F2 (LOD max 3.01 at theta = 0). Interestingly, this locus was not linked to the phenotype segregating in F1. Therefore, our results led to the mapping of a first locus of ACS (ACS1) and also showed evidence for genetic heterogeneity, suggesting that there are at least two loci responsible for this phenotype.

Auriculo-Condylar Syndrome. Confronting a Diagnostic Challenge

Auriculo-condylar syndrome (ACS) is characterized by typical ears malformation (so-called "question mark" ears), prominent cheeks, microstomia, and abnormality of the temporomandibular joint and condyle of the mandible. In this report we describe a new simplex case and a previously unreported family with affected individuals in three generations documenting clinical variability. Linkage study for markers located in candidate region for ACS1 (1p21.1-q23.3) was excluded in our familial case, reinforcing the hypothesis of genetic heterogeneity for this condition. A review of the literature focusing diagnostic criteria and features of ACS was performed. (C) 2011 Wiley Periodicals, Inc.

Future of the Haze Agreement : is the glass half empty or half full?

"In 1997–98, the ASEAN (Association of Southeast Asian Nations) region suffered an unprecedented health and environmental catastrophe due to choking haze created by a massive forest !re in Indonesia. It is estimated that the total losses from the fire could be US$5–6 billion after taking into account the loss of trees and other natural resources as well as the long-term impact on human health. This unprecedented anthropogenic disaster not only created a severe health and environmental hazard but also raised a question mark about the credibility and effectiveness of the ASEAN regional grouping. Against this background, ASEAN took a number of regional initiatives to try and solve the problem and finally adopted a new treaty for regional cooperation to combat forest fire and haze in 2002. This paper assesses the future success of this agreement from the perspectives of the legal, institutional and geopolitical reality of the region. Since numerous studies have examined state responsibility for transboundary environmental harm under international law and its implications on the ASEAN haze problem, this article will not touch upon that general debate nor the remedies that are possibly available to victim states. Rather, it will focus on the ASEAN regional legal and institutional initiatives to combat the haze pollution and compare them with a similar European regional agreement. Regarding the following analysis, it is important to recognise the uncertainty arising from Indonesia’s status (presently a non-party to the Agreement). A primary indication of the future effectiveness of this agreement can be drawn from an analysis of the principles involved in this agreement, bearing in mind the inherent difficulty of enforcing norms in the international environmental legal system as a whole, and the geopolitical reality of the region."

The WTO’s Trade Policy Review Mechanism – An Effective Surveillance Mechanism for Aid for Trade?

There has always been a question mark over how best to integrate developing countries into the world trading system and traditionally the WTO has used special and differential treatment (S&D) to do so. However, since 1996 the WTO has been involved with the Aid for Trade (AfT) initiative typically co-ordinated by the OECD and UN. This article firstly outlines the background to AfT since 1996 under the numerous agencies working in the area, highlighting how importance has always been placed on the monitoring and effectiveness of the process. It then turns to assessing the various methods currently used and the proposal of the WTO’s Trade Policy Review Mechanism (TPRM) as a potential monitoring tool of AfT.

Exploring the links between urban stucture and air quality

A poluição atmosférica constitui actualmente um grave problema ambiental cujos efeitos se fazem sentir a diversas escalas, desde os efeitos imediatos e de longo termo na saúde humana e nos materiais, até fenómenos regionais, como a acificação, e fenómenos globais que durante este século poderão alterar as condições de vida no globo. Apesar da redução das emissões de poluentes atmosféricos, conseguida através do uso de combustíveis mais limpos e tecnologias mais eficientes, as áreas urbanas continuam a evidenciar sinais de degradação ambiental. Para ser bem sucedida a cidade deve enfrentar as três dimensões da sustentabilidade: social, económica e ambiental. O modo de utilização do solo numa zona urbana é uma característica fundamental da cidade, com influência directa no seu desempenho ambiental e na qualidade de vida que proporciona à população. O presente trabalho explora a ligação entre a estrutura urbana e a qualidade do ar, um dos muitos aspectos do desenvolvimento urbano sustentável. A perspectiva histórica sobre o desenvolvimento urbano, a poluição atmosférica e a sua interligação é abordada, bem como o trabalho de investigação que tem vindo a ser conduzido na área. A aplicação de um sistema de modelação atmosférico a um caso de estudo idealizado demonstra a importância da estrutura espacial da cidade na sustentabilidade urbana, mostrando que cidades compactas com usos do solo misturados promovem uma melhor qualidade do ar quando comparadas com cidades dispersas, com baixa densidade populacional. De modo a explorar a relação entre a estrutura urbana e a qualidade do ar numa zona urbana real, a região urbana do Porto é identificada como um caso de estudo adequado, e o processo de crescimento urbano nas últimas décadas é analisado, assim como os níveis de qualidade do ar da região. De modo a definir a configuração do sistema de modelação mais adequada para a região de estudo, são efectuados diversos testes de sensibilidade com o modelo meteorológico. Relativamente ao modelo de qualidade do ar, é descrito e implementado um conjunto de acções de modo a melhorar o desempenho do modelo para a simulação das concentrações de poluentes na atmosfera urbana, no contexto de alterações do uso do solo. Finalmente, são desenvolvidos e testados, através da aplicação do sistema de modelação, dois cenários alternativos de desenvolvimento urbano para a área de estudo. Estes cenários alternativos implicam diferentes emissões de poluentes e diferentes distribuições espaciais dessas emissões, e como consequência, diferentes níveis de qualidade do ar. O estudo permite concluir que alterações nos padrões de uso do solo em áreas urbanas conduzem a alterações na meteorologia, emissões e qualidade do ar. As áreas urbanas dispersas, quando comparadas com estruturas urbanas compactas são responsáveis por temperaturas mais elevadas, emissões de poluentes para a atmosfera mais elevadas e maiores concentrações de poluentes.

What is poetic attention

Tese de doutoramento, Estudos de Literatura e de Cultura (Teoria da Literatura), Universidade de Lisboa, Faculdade de Letras, 2014

Heidegger's tragic Greeks : the relation between presence and deinon

Martin Heidegger's interpretation of the ancients was born out of something like a crisis in the interpretation of the Greeks, which can be characterized as nothing other than the realization of the idea that the Greek philosophers put a serious question mark over existence. This idea, which had its germination in Prussia with Jakob Burckhart and his teacher, but first came to be seriously cultivated in the Philosophy of Friedrich Nietzsche, was the first in depth investigation into whether the Greeks, on the one hand, questioned existence or, on the other hand, put a question mark over existence. To question existence is rather innocuous, since it amounts to little more, in the end, than a child looking up at the stars and asking what it all means. To put a question mark over existence, however, is another business entirely. For the Greeks, as the life work of Martin Heidegger amply demonstrates, the nature of Greek thinking and the objects towards which it is directed follows so absolutely from the tragic view of the human person that, in a certain sense, philosophy is Greek and could only have developed in Greece. Perhaps stating it a little less categorically, philosophy could have developed elsewhere at least to the extent that something like they way the Greeks understood life was at the forefront: presence, in other words. This thesis deals with the problem ofHeidegger's relation to the Greeks, specifically in terms of his understanding of the Greeks and presence. It is the position of this dissertation that the Greek notion of presence is, as Heidegger understands it, the homeliness of the hearth that radiates through all the things that humans concern themselves with. This is thought by Heidegger, as the Greeks did, specifically in contrast with the uncanninesslunhomeliness of the hqrnan apart from his or her concern with things. Therefore, the thesis is an attempt at exposing the relation between presence and the unhomely by situating it withing Greek existence and the meaning of the Greek Philosopher. In order to support this position, the thesis has been divided into five parts. The first two chapters deal with Heidegger's explanation of the relation between Greek notion of physics (Phusis), metaphysics (specifically in relation to an analysis of time and motion in Greek thought), and what Heidegger calls the fundamental attunement of Dasein (boredom). More exactly, it deals with these issues only so far as they allow us to bring out something like the notion of 'presence' in relation to things and homelessness or restlessness in relation to the human being. The rationale for these two chapters in relation to the central problem of the paper is that in Heidegger's elucidation of physics and metaphysics, he conducts his analysis in such a way that he explicitly uncovers that dimension of human existence that he calls the fundamental attunement of Dasein. This fundamental attunement is, in tum, similar to what the Greeks understood as the deinon, the uncanninesslunhomeliness of the human. The third and fourth chapters take as their explicit themes the problem of the Greek understanding of the assertion and the ways in which the person can comport himlherself toward things, two issues which are not separable. The rationale for these two chapters in relation to the central theme of the paper is that Heidegger's analysis of these two areas in Greek thought brings out precisely why the philosopher and the philosophical way of life is the highest mode of existence for the Greeks and how this is thought specifically in tenns of the uncanniness of humans. The final cijapter gives a complete elucidation of presence as the homeliness of the hearth and shows specifically how this is thought of in contradistinction to the uncanny/unhomely for the Greeks. 1I1 This last chapter also explains Martin Heidegger's reaction to the Greek's interpretation of the highest mode of existence, and what he posited as a counter-thought. The essay as a whole is an attempt to fully concertize an important dimension of Heidegger' s understanding of the Greeks, that is, the relation between presence and the deinon or Greek notion ofunhomely, which, to my la)owledge, has not been offered anywhere in commentaries on Heidegger.

Donald Ziraldo Fonds, 1968-2012, n.d.

Donald J. P. Ziraldo, C.M., BSc., LLD was born in St. Catharines, Ontario on October 13, 1948 to Fredrick and Irma (Schiratti) Ziraldo. He graduated Denis Morris High School in St. Catharines in 1967, and received his B.Sc. in Agriculture at the University of Guelph in 1971. In 1974, Ziraldo was running Ziraldo Nurseries when he met Austrian born schoolteacher, chemist and winemaker Karl J. Kaiser. They realized that there was a gap in the premium varietal wine market and decided to plant a premium traditional European variety of grape vine species, the Vitis vinifera. This was an innovation in the Niagara region because the current wine producers were not using premium European grapes at the time. Ziraldo and Kaiser founded and then formally incorporated Inniskillin Wines Inc. in Niagara-on-the-Lake, Ontario on July 31, 1975. Ziraldo successfully lobbied General George Kitching, CEO of the LCBO, for a winery license. In 1975, Kitching granted him a winery license, the first in Ontario since Prohibition ended. From the beginning, there was a division of labour where Kaiser focused on the winemaking and Ziraldo focused on the marketing and promotion of the wines. Ziraldo also became president of the company. Ziraldo and Kaiser worked on improving their winemaking techniques and promoting their products and company. Ziraldo has been called ‘one of the founding fathers of the Canadian wine industry’, and it is widely acknowledged that both men played a large role in the success and growth of the Canadian wine industry. Together they pioneered the estate winery movement in Canada. A major turning point Inniskillin came in 1984 when Karl Kaiser successfully harvested the first Icewine crop from frozen grapes on the vine and bottled Eiswein Vidal (Icewine). In 1990, Inniskillin received worldwide recognition for this Icewine when their 1989 Vidal Icewine won the most prestigious award in the wine world, the Grand Prix d’Honneur, given at Vinexpo in France. This victory has been called ‘the award heard round the world’ and it launched Inniskillin into the international wine arena. At the same time, this helped lift the profile of Canadian wines in general. Inniskillin not only became Canada’s leading producer of Icewine, but it also became known for producing ‘one of the world’s great wines’. After the 1990 award, Ziraldo began a major public relations campaign to promote Inniskillin and build Icewine into a worldwide brand. He travelled broadly every year to promote the brand and products and networked extensively with politicians, celebrities, chefs, sommeliers, etc. To ensure worldwide and long-term success, Ziraldo introduced Icewine to Asia and the United States which were new markets. He developed a new Icewine glass with George Riedel. Tony Aspler has called Ziraldo ‘Canada’s Wine Ambassador’. Ziraldo was President of Inniskillin Wines Inc. (Niagara) from 1975 to 2006. In 1992, Inniskillin merged with Cartier Wines, and in 1993 Cartier Inniskillin Vintners Inc. merged with T.G. Bright & Co. Limited, forming the new company Vincor International Inc. Inniskillin wines was now a subsidiary of Vincor. Ziraldo became a Director at Vincor International Inc. from 1993 to 2004. From 1989 to the mid 1990s, Ziraldo also became President of Inniskillin Napa, in Napa Valley, California. Inniskillin purchased Napa Valley vineyards and produced wines under the Terra label. In 1994, Ziraldo set up a subsidiary estate winery of Inniskillin in Oliver, British Columbia which was called Inniskillin Okanagan Vineyards Inc. He became President of the winery. This started as a partnership between Inniskillin and the local Inkameep Indian Band in the Okanagan. In 2006, Ziraldo left Inniskillin and since that time he has been involved in other Icewine related ventures such as running Ziraldo Estate Winery and producing Ziraldo Riesling Icewine 2007. He also is in partnership with the Niagara based Equifera Estate Winery to produce Equifera Icewine. His most recent projects include planting Picolit grapes in his parent’s hometown, in a project called Picolit Di Fagagna and becoming Managing Director of the Senhora Do Convento Port Winery in Portugal. Donald Ziraldo was instrumental in the creation of the Vintners Quality Alliance (VQA) in Ontario and was its founding Chair from 1988-1995. The VQA was established as a regulatory and appellation system which secured the quality and origin of Canadian wines made under this system. The VQA designation and bottle label gave the consumer confidence that the wines they were purchasing were 100% local products. The VQA system was set up first in Ontario and then in British Columbia.

Aplicación de las TIC a la evaluación de alumnos universitarios.

Monográfico con el título: 'Estado actual de los sistemas e-learning'. Resumen basado en el de la publicación

L'evolució dels models de turisme litoral: el Regne Unit, la Costa brava i Cuba

La tesi es centra en una anàlisi prèvia de les implicacions de la teoria del cicle de vida aplicada a les destinacions turístiques pel geògraf canadenc Richard W. Butler (1982) i en un estudi del significat de l'aparició del postmodernisme i les seves repercussions en el turisme, a la vegada que es resegueix la història que ha fet possible l'aparició del turisme massificat de sol i platja. Tot això és la base teòrica imprescindible per poder estudiar empíricament les destinacions de Saltburn (Regne Unit), l'Estartit (Costa Brava) i Cayo Coco (Cuba), utilitzant el cicle de vida per escriure la seva història i veure les polítiques de desenvolupament i regeneració turística que s'han seguit i si aquestes es poden qualificar de postfordistes. La principal conclusió és que el concepte del cicle de vida es limita a ser una eina de diagnòstic a posteriori. El cicle de vida, com a instrument prescriptiu, no serveix. Es específic per a cada destinació, amb unes etapes i punts d'inflexió que només es poden establir en restrospectiva. El cicle de vida és una eina descriptiva molt útil per entendre l'evolució de les destinacions turístiques i els seus mercats, però vigilant de no caure en falses exploracions o perillosos determinismes.

Javal: modelo de ambiente de avaliação remota multiagente baseada em tutores embarcados

Trata o presente objeto de pesquisa da proposta de desenvolvimento de um modelo de ambiente de avaliação remota para Ensino à Distância, baseado no paradigma de Orientação a Objetos e elaborado com base na tecnologia de sistemas multiagentes. Para a validação do modelo, foi desenvolvido um protótipo denominado Javal, capaz de permitir a aplicação e monitoração da realização de avaliações e testes à distância. As soluções comerciais disponíveis no mercado, como Question Mark Perception, Aula Net e WebCT possuem código fechado e custo elevado, além de serem baseadas em soluções proprietárias (QML - Question Mark, ASP - Microsoft, etc.), necessitando de plataforma específica para instalação de servidores e clientes. Quanto ao aspecto funcional, estas ferramentas possuem a base de sua lógica de funcionamento em execução de scripts no servidor. Para cada item de uma avaliação a ser respondido, torna-se necessária a efetivação de uma nova conexão. O modelo proposto traz toda a funcionalidade do sistema para o ambiente do aluno, tornando necessária apenas uma conexão para a busca da avaliação e outra para o envio dos resultados, reduzindo o tráfego na rede. Quanto ao aspecto pedagógico, estas ferramentas limitam-se apenas a apresentar uma avaliação somativa do aluno, geração de graus e estatísticas, não se preocupando em monitorar seus aspectos comportamentais, capazes de apontar indícios de possíveis falhas no aprendizado ou na elaboração da avaliação. A proposta do modelo é da apresentação de um ambiente orientado a objetos, capaz de distribuir elementos representativos das avaliações existentes no modelo tradicional de ensino, incorporando recursos capazes de possibilitar a monitoração de aspectos comportamentais do aluno, pelo emprego de agentes monitores ou tutores, que podem acompanhar o aluno e auxiliá-lo em situações de dificuldade. O modelo proposto por este trabalho envolve as avaliações formativas e somativas, aplicadas de forma assíncrona e individual. Como sugestão para trabalhos futuros, o modelo propõe o desenvolvimento de classes capazes de implementar a aplicação de avaliações síncronas e em grupo. A validação do modelo proposto foi realizado através do desenvolvimento de um protótipo que, com base no desenvolvimento de uma API Javal específica, implementa os principais tipos de questões disponíveis no sistema de ensino tradicional, além de agentes tutores de avaliação.

Investigating the role of single point mutations in the human proteome: a computational study

In the post genomic era with the massive production of biological data the understanding of factors affecting protein stability is one of the most important and challenging tasks for highlighting the role of mutations in relation to human maladies. The problem is at the basis of what is referred to as molecular medicine with the underlying idea that pathologies can be detailed at a molecular level. To this purpose scientific efforts focus on characterising mutations that hamper protein functions and by these affect biological processes at the basis of cell physiology. New techniques have been developed with the aim of detailing single nucleotide polymorphisms (SNPs) at large in all the human chromosomes and by this information in specific databases are exponentially increasing. Eventually mutations that can be found at the DNA level, when occurring in transcribed regions may then lead to mutated proteins and this can be a serious medical problem, largely affecting the phenotype. Bioinformatics tools are urgently needed to cope with the flood of genomic data stored in database and in order to analyse the role of SNPs at the protein level. In principle several experimental and theoretical observations are suggesting that protein stability in the solvent-protein space is responsible of the correct protein functioning. Then mutations that are found disease related during DNA analysis are often assumed to perturb protein stability as well. However so far no extensive analysis at the proteome level has investigated whether this is the case. Also computationally methods have been developed to infer whether a mutation is disease related and independently whether it affects protein stability. Therefore whether the perturbation of protein stability is related to what it is routinely referred to as a disease is still a big question mark. In this work we have tried for the first time to explore the relation among mutations at the protein level and their relevance to diseases with a large-scale computational study of the data from different databases. To this aim in the first part of the thesis for each mutation type we have derived two probabilistic indices (for 141 out of 150 possible SNPs): the perturbing index (Pp), which indicates the probability that a given mutation effects protein stability considering all the “in vitro” thermodynamic data available and the disease index (Pd), which indicates the probability of a mutation to be disease related, given all the mutations that have been clinically associated so far. We find with a robust statistics that the two indexes correlate with the exception of all the mutations that are somatic cancer related. By this each mutation of the 150 can be coded by two values that allow a direct comparison with data base information. Furthermore we also implement computational methods that starting from the protein structure is suited to predict the effect of a mutation on protein stability and find that overpasses a set of other predictors performing the same task. The predictor is based on support vector machines and takes as input protein tertiary structures. We show that the predicted data well correlate with the data from the databases. All our efforts therefore add to the SNP annotation process and more importantly found the relationship among protein stability perturbation and the human variome leading to the diseasome.

Imaging of total colonic Hirschsprung disease

BACKGROUND: Hirschsprung disease (HD) is a functional obstruction of the bowel caused by the absence of intrinsic enteric ganglion cells. The diagnosis of total colonic HD (TCHD) based on contrast enemas is difficult in newborns because radiological findings vary. OBJECTIVE: To evaluate the radiographic and contrast enema findings in patients with pathologically proven TCHD. MATERIALS AND METHODS: From 1966 to 2007, 17 records from a total of 31 patients with TCHD were retrospectively evaluated for diameter and shape of the colon, diameter of the small bowel, bowel wall contour, ileal reflux, abdominal calcifications, pneumoperitoneum, filling defects, transitional zones and rectosigmoid index. RESULTS: Three colonic patterns of TCHD were found: microcolon, question-mark-shape colon and normal caliber colon. Additional findings included spasmodic colon, ileal reflux, delayed evacuation and abdominal calcifications. Colonic transitional zones were found in eight patients with TCHD. CONCLUSION: The diagnosis of TCHD is difficult to establish by contrast enema studies. The length of the aganglionic small bowel and the age of the patient can influence the radiological findings in TCHD. The transitional zone and the rectosigmoid index can be false-positive in TCHD. The colon can appear normal. Consider TCHD if the contrast enema study is normal but the patient remains symptomatic and other causes of distal bowel obstruction have been excluded.

The Basement of the Deccan Traps and Its Madagascar Connection: Constraints from Xenoliths

Paleogeographic reconstructions of India and Madagascar before their late Cretaceous rifting juxtapose the Antongil Block of Madagascar against the Deccan Traps of India, indicating that the Western Dharwar Craton extends below the Deccan lavas. Some recent studies have suggested that the South Maharashtra Shear Zone along the northern Konkan coast of India limits the northern extent of the Western Dharwar Craton, implying that the craton does not extend below the Deccan Traps, raising a question mark on paleogeographic reconstructions of India and Madagascar. The continuity of the Western Dharwar Craton north of the South Maharashtra Shear Zone below the Deccan Traps—or its lack thereof—is critical for validating tectonic models correlating Madagascar with India. In this study, zircons in tonalitic basement xenoliths hosted in Deccan Trap dykes were dated in situ, using the U-Pb isotope system. The data furnish U-Pb ages that define three populations at 2527 ± 6, 2456 ± 6, and 2379 ± 9 Ma. The 2527 ± 6 Ma ages correspond to the igneous crystallization of the tonalites, whereas the 2456 ± 6 and 2379 ± 9 Ma ages date metamorphic overprints. The results help to establish for the first time that the basement is a part of the Neoarchean granitoid suite of the Western Dharwar Craton, which extends northward up to at least Talvade in central and Kihim beach in the western Deccan. By implication, the South Maharashtra Shear Zone cannot be the northern limit of the Western Dharwar Craton. The granitoids are correlated with the Neoarchean felsic intrusions (2.57–2.49) of the Masaola suite in the Antongil Block of Madagascar, supporting the existence of a Neoarchean Greater Dharwar Craton comprising the Western Dharwar Craton and the Antongil-Masora Block.