The tuberous sclerosis 2000 study:presentation initial assessments and implications for diagnosis and management

Aims: The Tuberous Sclerosis 2000 Study is the first comprehensive longitudinal study of tuberous sclerosis (TS) and aims to identify factors that determine prognosis. Mode of presentation and findings at initial assessments are reported here. Methods: Children aged 0-16 years newly diagnosed with TS in the UK were evaluated. Results: 125 children with TS were studied. 114 (91%) met clinical criteria for a definite diagnosis and the remaining 11 (9%) had pathogenic TSC1 or TSC2 mutations. In families with a definite clinical diagnosis, the detection rate for pathogenic mutations was 89%. 21 cases (17%) were identified prenatally, usually with abnormalities found at routine antenatal ultrasound examination. 30 cases (24%) presented before developing seizures and in 10 of these without a definite diagnosis at onset of seizures, genetic testing could have confirmed TS. 77 cases (62%) presented with seizures. Median age at recruitment assessment was 2.7 years (range:4 weeks-18 years). Dermatological features of TS were present in 81%. The detection rate of TS abnormalities was 20/107 (19%) for renal ultrasound including three cases with polycystic kidney disease, 51/88 (58%) for echocardiography, 29/35 (83%) for cranial CT and 95/104 (91%) for cranial MRI. 91% of cases had epilepsy and 65% had intellectual disability (IQ<70). Conclusions: Genetic testing can be valuable in confirming the diagnosis. Increasing numbers of cases present prenatally or in early infancy, before onset of seizures, raising important questions about whether these children should have EEG monitoring and concerning the criteria for starting anticonvulsant therapy.

Kamatlábak és árszint kis, nyitott gazdaságban = Interest rates and price levels in a small open economy

A tanulmány azt vizsgálja, hogy a különböző kamatlábaknak milyen hatásai vannak az árszintre, illetve a nominális árakra egy nyitott elsősorban kis, nyitott gazdaságban szabad tőkeáramlás mellett. Míg a zárt gazdaságban csupán a nominális és reálkamatláb megkülönböztetése a lényeges, nyitott gazdaságban a kamatlábak vizsgálatakor meg kell fontolnunk a kamatlábparitás kérdését is. Tisztáznunk kell a reálkamatláb összetevőit, amelyben fontos szerepet kap mind az árfolyam-begyűrűzés (pass-through), mind pedig a kockázati prémium mértéke. A kamatlábhatások vizsgálatakor először azt a mechanizmust elemezzük, amely által a kamatláb befolyásolja a tartós jószágok költségét (explicit vagy implicit bérleti díját). Másodszor az exportszektor termelési döntése és a hazai kamatláb viszonyára vonatkozó mechanizmust vizsgáljuk. Belátjuk, hogy az exportáló szektor döntései függetlenek lehetnek a belföldi kamatlábaktól. Harmadszor bizonyos árazási viselkedéseket tanulmányozunk. Bebizonyítjuk, hogy a kamatláb olyan növelése, ami nem változtat a jelenlegi árfolyamon, árszintnövelő az importőr ország számára. Megfogalmazható az a nézet, hogy ha van is a kamatlábaknak keresleti hatása a zárt gazdaságban, a kis, nyitott gazdaságban ez vélhetőleg sokkal gyengébb. _____ The study examines what effects various interest rates have on the price level and nomi-nal prices in an open (primarily small) economy with free flows of capital. A closed economy calls for a distinction only between nominal and real rates of interest, but in an open economy, questions of interest-rate parity have to be considered as well. It is nec-essary to clarify the factors behind the real interest rate important for price-level pass-through and for the scale of risk premium. Analysis of interest-rate effects begins with the mechanism whereby the interest rate influences the cost of fixed assets (explicit or implicit rents). Secondly, the mechanism behind the relation of export-sector production decisions and domestic interest rates is examined. It emerges that decisions of the export sector are independent of domestic interest rates. Thirdly, certain types of pricing behav-iour are studied. It is shown that a rise in the interest rate that does not alter the present exchange rate is a price-raising factor for the importing country. It can be assumed that if the interest rate has a demand effect in a closed economy, this will presumably be much weaker in a small open economy.

Federal tax deductibility for local taxes and its effect on local revenue policies

This dissertation analyzes whether and how changes in federal tax policy affect local tax policies, specifically, the elimination of the federal deductibility of state and local taxes for individual taxpayers by the Tax Reform Act of 1986 (TRA86) in 59 California cities. Two methods are used in the study: a survey of local revenue officials and a time event time-series/cross sectional sales tax reliance study.^ The reliance study uses a covariance model to pool cross-section and time-series observations. The results of the reliance study indicate a statistically significant overall decline in sales tax reliance after 1986. The results of the survey indicate that local policy makers generally do not believe that federal deductibility is an important factor when considering raising local sales taxes. Further analysis shows that local revenue officials claiming federal deductibility is not an important factor are associated mostly with cities that registered no significant decline in sales tax reliance after 1986. Similarly, local revenue officials claiming federal deductibility is an important factor when considering local tax policy are associated mostly with cities that suffered a significant decline in sales tax reliance after 1986.^ Of that group, further analysis shows that the declines in sales tax reliance are associated mostly with cities located in the southwestern part of the state. When compared to other cities in the state, an analysis of variance reveals that there are a series of statistically significant factors associated with southwestern cities which may contribute to the decline in sales tax reliance following the enactment of the Tax Reform Act of 1986. ^

Grandfamilies: Grandchildren raised by grandparents. Impact on students and school services

Historically, some grandparents have had to assume the responsibility for raising their grandchildren. More recently, with increasing frequency grandparents are serving as full-time surrogate parents to their grandchildren.^ The term "grandfamily" was coined by this researcher to identify families where children are raised by grandparents. "Supergrands" are the grandparents and "grandkin" are children in these families.^ Supergrands who raise their grandkin tend to have elevated levels of stress that negatively impact their well-being. Grandkin tend to develop problems with attachment and establishing social networks, which can lead to poor psychological adjustment. School personnel believe grandkin evidence behavioral problems and occupy disproportionate amounts of their time each day. However, there is a dearth of data to support this belief.^ This study empirically investigated the impact of grandfamilies on students and school services. The results revealed grandkin experienced significantly greater levels of emotional and behavior problems than similar schoolmates. However, they were not referred for discipline problems in substantially larger numbers.^ These results indicate the practice of education should change to allow for the development and provision of social support procedures in schools. Social support will serve to buffer the stress, manifested in emotional and behavioral problems, encountered by children living with surrogate parents. A case study was presented along with a proposed intervention project that has potential to ameliorate the problems experienced by grandfamilies. ^

Predictors of first semester attrition and their relation to retention of generic associate degree nursing students

In community college nursing programs the high rate of attrition was a major concern to faculty and administrators. Since first semester attrition could lead to permanent loss of students and low retention in nursing programs, it was important to identify at-risk students early and develop proactive approaches to assist them to be successful. The goal of nursing programs was to graduate students who were eligible to take the national council licensing examination (RN). This was especially important during a time of critical shortage in the nursing workforce. ^ This study took place at a large, multi-campus community college, and used Tinto's (1975) Student Integration Model of persistence as the framework. A correlational study was conducted to determine whether the independent variables, past academic achievement, English proficiency, achievement tendency, weekly hours of employment and financial resources, could discriminate between the two grade groups, pass and not pass. Establishing the relationship between the selected variables and successful course completion might be used to reduce attrition and improve retention. Three research instruments were used to collect data. A Demographic Information form developed by the researcher was used to obtain academic data, the research questionnaire Measure of Achieving Tendency measured achievement motivation, and the Test of Adult Basic Education (TABE), Form 8, Level A, Tests 1, 4, and 5 measured the level of English proficiency. The Department of Nursing academic policy, requiring a minimum course grade of “C” or better was used to determine the final course outcome. A stepwise discriminant analysis procedure indicated that college language level and pre-semester grade point average were significant predictors of final course outcome. ^ Based on the findings of the study recommendations focused on assessing students' English proficiency prior to admission into the nursing program, an intensive remediation plan in language comprehension for at-risk students, and the selection of alternate textbooks and readings that more closely matched the English proficiency level of the students. A pilot study should be conducted to investigate the benefit of raising the admission grade point average. ^

Essays on the selection of securities: Decision on acquisition and issuance perspectives and decision rules

This dissertation is a discourse on the capital market and its interactive framework of acquisition and issuance of financial assets that drive the economy from both sides—investors/lenders and issuers/users of capital assets. My work consists of four essays in financial economics that offer a spectrum of revisions to this significant area of study. The first essay is a delineation of the capital market over the past half a century and major developments on capital markets on issues that pertain to the investor's opportunity set and the corporation's capital-raising availability set. This chapter should have merits on two counts: (i) a comprehensive account of capital markets and return-generating assets and (ii) a backdrop against which I present my findings in Chapters 2 through 4. ^ In Chapter 2, I rework on the Markowitz-Roy-Tobin structure of the efficient frontier and of the Separation Theorem. Starting off with a 2-asset portfolio and extending the paradigm to an n-asset portfolio, I bring out the optimal choice of assets for an investor under constrained utility maximization. In this chapter, I analyze the selection and revision-theoretic construct and bring out optimum choices. The effect of a change in perceived risk or return in the mind of an investor is ascertained on the portfolio composition. ^ Chapter 3 takes a look into corporations that issue market securities. The question of how a corporation decides what kinds of securities it should issue in the marketplace to raise funds brings out the classic value invariance proposition of Modigliani and Miller and fills the gap that existed in the literature for almost half a century. I question the general validity in the classic results of Modigliani and Miller and modify the existing literature on the celebrated value invariance proposition. ^ Chapter 4 takes the Modigliani-Miller regime to its correct prescription in the presence of corporate and personal taxes. I show that Modigliani-Miller's age-old proposition needs corrections and extensions, which I derive. ^ My dissertation overall brings all of these corrections and extensions to the existing literature as my findings, showing that capital markets are in an ever-changing state of necessary revision. ^

Representation of people of color in the nineteenth century slavery novel in Cuba and Brazil

This dissertation explores the similarities and differences which characterize the depiction of people of color in certain representative nineteenth century Cuban and Brazilian slavery novels as a function of the authorial approach of each territory's literary tradition toward the issues of slavery, racial prejudice, and people of color. The selected texts, derived from the peak periods in slavery literature of each territory, include Francisco , by Anselmo Snárez y Romero; Sab, by Gertrudis Gómez de Avellaneda; Cecilia Valdés , by Cirilo Villaverde; A escrava Isaura, by Bernardo Guimarães; O mulato, by Aluísio Azevedo; and Bom-Crioulo, by Adolfo Caminha. While the present study explores the enslavement, abuse, and discrimination of people of color as a consequence of a deep-seated discourse of power, privilege and racial superiority, it focuses more extensively on the representation of people of color, particularly in their capacity to constructively appropriate the cultural values of the white dominant group and recognize their identity as ambiguous. ^ Said's theories of Orientalist discourse and geography and formation as well as Dube's perspective on subaltern-oriented studies provide a theoretical framework for exploring the response of slavery writers whose common exposure to slavery but dissimilar socio-political contexts generate some startling findings. Crafted within a period of political repression, fear of black revolt, factional in-fighting as well as strong socioeconomic ties to the slaveholding class, the Cuban texts generally fashioned an approach to slavery as one marked by moderation, reform, and cultural counter discourse and consequently depict people of color with a more passive but culturally authentic outlook. On the other hand, the Brazilian response to the issue of slavery, steeped in an ideological amalgam of liberalism, positivism, republicanism, and abolitionism, is characterized by overt opposition to slavery and a representation of people of color that is less concerned with cross-cultural input but reclaims their humanity as highly educable and socially mobile persons in search of greater freedoms. Ultimately, there is a shared message of higher significance couched in the worthwhile mission of raising slaves to the level of men. ^

A methodology to estimate time varying user responses to travel time and travel time reliability in a road pricing environment

Road pricing has emerged as an effective means of managing road traffic demand while simultaneously raising additional revenues to transportation agencies. Research on the factors that govern travel decisions has shown that user preferences may be a function of the demographic characteristics of the individuals and the perceived trip attributes. However, it is not clear what are the actual trip attributes considered in the travel decision- making process, how these attributes are perceived by travelers, and how the set of trip attributes change as a function of the time of the day or from day to day. In this study, operational Intelligent Transportation Systems (ITS) archives are mined and the aggregated preferences for a priced system are extracted at a fine time aggregation level for an extended number of days. The resulting information is related to corresponding time-varying trip attributes such as travel time, travel time reliability, charged toll, and other parameters. The time-varying user preferences and trip attributes are linked together by means of a binary choice model (Logit) with a linear utility function on trip attributes. The trip attributes weights in the utility function are then dynamically estimated for each time of day by means of an adaptive, limited-memory discrete Kalman filter (ALMF). The relationship between traveler choices and travel time is assessed using different rules to capture the logic that best represents the traveler perception and the effect of the real-time information on the observed preferences. The impact of travel time reliability on traveler choices is investigated considering its multiple definitions. It can be concluded based on the results that using the ALMF algorithm allows a robust estimation of time-varying weights in the utility function at fine time aggregation levels. The high correlations among the trip attributes severely constrain the simultaneous estimation of their weights in the utility function. Despite the data limitations, it is found that, the ALMF algorithm can provide stable estimates of the choice parameters for some periods of the day. Finally, it is found that the daily variation of the user sensitivities for different periods of the day resembles a well-defined normal distribution.

Demographic, Behavioral and Perceptual Comparisons of U.S Visitor Experience with Group Package Tours and Free Independent Travel to China

U.S. visitor demand for the China travel experience is anticipated to rise significantly through 2105, causing the Chinese government to employ 100 million service providers over the next six years and raising concern about service delivery and perceptions of the on-site China experience. In an effort to better understand these issues concerning U.S. visitors, this study investigated two specific types of U.S. travelers to China: Group Package Tour (GPT) visitors and Free Independent Travel (FIT) visitors. Results indicated that GPT visitors were more likely to be older and have higher household income than FIT visitors. Four trip-related characteristics of GPT and FIT visitors were found to be significantly different, with GPT visitors showing higher levels of satisfaction with the overall China on-site travel experience.

Mixed spatial and nonspatial problems in location based services

With hundreds of millions of users reporting locations and embracing mobile technologies, Location Based Services (LBSs) are raising new challenges. In this dissertation, we address three emerging problems in location services, where geolocation data plays a central role. First, to handle the unprecedented growth of generated geolocation data, existing location services rely on geospatial database systems. However, their inability to leverage combined geographical and textual information in analytical queries (e.g. spatial similarity joins) remains an open problem. To address this, we introduce SpsJoin, a framework for computing spatial set-similarity joins. SpsJoin handles combined similarity queries that involve textual and spatial constraints simultaneously. LBSs use this system to tackle different types of problems, such as deduplication, geolocation enhancement and record linkage. We define the spatial set-similarity join problem in a general case and propose an algorithm for its efficient computation. Our solution utilizes parallel computing with MapReduce to handle scalability issues in large geospatial databases. Second, applications that use geolocation data are seldom concerned with ensuring the privacy of participating users. To motivate participation and address privacy concerns, we propose iSafe, a privacy preserving algorithm for computing safety snapshots of co-located mobile devices as well as geosocial network users. iSafe combines geolocation data extracted from crime datasets and geosocial networks such as Yelp. In order to enhance iSafe's ability to compute safety recommendations, even when crime information is incomplete or sparse, we need to identify relationships between Yelp venues and crime indices at their locations. To achieve this, we use SpsJoin on two datasets (Yelp venues and geolocated businesses) to find venues that have not been reviewed and to further compute the crime indices of their locations. Our results show a statistically significant dependence between location crime indices and Yelp features. Third, review centered LBSs (e.g., Yelp) are increasingly becoming targets of malicious campaigns that aim to bias the public image of represented businesses. Although Yelp actively attempts to detect and filter fraudulent reviews, our experiments showed that Yelp is still vulnerable. Fraudulent LBS information also impacts the ability of iSafe to provide correct safety values. We take steps toward addressing this problem by proposing SpiDeR, an algorithm that takes advantage of the richness of information available in Yelp to detect abnormal review patterns. We propose a fake venue detection solution that applies SpsJoin on Yelp and U.S. housing datasets. We validate the proposed solutions using ground truth data extracted by our experiments and reviews filtered by Yelp.

"To hold the world in contempt": The British Empire, war, and the Irish and Indian nationalist press, 1899–1914

The era between the close of the nineteenth century and the onset of the First World War witnessed a marked increase in radical agitation among Indian and Irish nationalists. The most outspoken political leaders of the day founded a series of widely circulated newspapers in India and Ireland, placing these editors in the enviable position of both reporting and creating the news. Nationalist journalists were in the vanguard of those pressing vocally for an independent India and Ireland, and together constituted an increasingly problematic contingent for the British Empire. The advanced-nationalist press in Ireland and the nationalist press in India took the lead in facilitating the exchange of provocative ideas--raising awareness of perceived imperial injustices, offering strategic advice, and cementing international solidarity. Irish and Indian press coverage of Britain's imperial wars constituted one of the premier weapons in the nationalists' arsenal, permitting them to build support for their ideology and forward their agenda in a manner both rapid and definitive. Directing their readers' attention to conflicts overseas proved instructive in how the Empire dealt with those who resisted its policies, and also showcased how it conducted its affairs with its allies. As such, critical press coverage of the Boxer Rebellion, Boer War, Russo-Japanese War, and World War I bred disaffection for the Empire, while attempts by the Empire to suppress the critiques further alienated the public. This dissertation offers the first comparative analysis of the major nationalist press organs in India and Ireland, using the prism of war to illustrate the increasingly persuasive role of the press in promoting resistance to the Empire. It focuses on how the leading Indian and Irish editors not only fostered a nationalist agenda within their own countries, but also worked in concert to construct a global anti-imperialist platform. By highlighting the anti-imperial rhetoric of the nationalist press in India and Ireland and illuminating their strategies for attaining self-government, this study deepens understanding of the seeds of nationalism, making a contribution to comparative imperial scholarship, and demonstrating the power of the media to alter imperial dynamics and effect political change.

Resistance Performances: (Re)constructing Spaces of Resistance and Contention in the 2010-2011 University of Puerto Rico Student Movement

On the night of April 20, 2010, a group of students from the University of Puerto Rico (UPR), Río Piedras campus, met to organize an indefinite strike that quickly broadened into a defense of accessible public higher education of excellence as a fundamental right and not a privilege. Although the history of student activism in the UPR can be traced back to the early 1900s, the 2010-2011 strike will be remembered for the student activists’ use of new media technologies as resources that rapidly prompted and aided the numerous protests. This activist research entailed a critical ethnography and a critical discourse analysis (CDA) of traditional and alternative media coverage and treatment during the 2010 -2011 UPR student strike. I examined the use of the 2010-2011 UPR student activists’ resistance performances in constructing local, corporeal, and virtual spaces of resistance and contention during their movement. In particular, I analyzed the different tactics and strategies of resistance or repertoire of collective actions that student activists used (e.g. new media technologies) to frame their collective identities via alternative news media’s (re)presentation of the strike, while juxtaposing the university administration’s counter-resistance performances in counter-framing the student activists’ collective identity via traditional news media representations of the strike. I illustrated how both traditional and alternative media (re)presentations of student activism developed, maintained, and/or modified students activists’ collective identities. As such, the UPR student activism’s success should not be measured by the sum of demands granted, but by the sense of community achieved and the establishment of networks that continue to create resistance and change. These networks add to the debate surrounding Internet activism and its impact on student activism. Ultimately, the results of this study highlight the important role student movements have had in challenging different types of government policies and raising awareness of the importance of an accessible public higher education of excellence.

Standardized geo-referenced catch, fishing effort and length-frequency data for the Indian Ocean Bigeye Tuna, Thunnus obesus (1952-2014)

Geo-referenced catch and fishing effort data of the bigeye tuna fisheries in the Indian Ocean over 1952-2014 were analysed and standardized to facilitate population dynamics modelling studies. During this sixty-two years historical period of exploitation, many changes occurred both in the fishing techniques and the monitoring of activity. This study includes a series of processing steps used for standardization of spatial resolution, conversion and standardization of catch and effort units, raising of geo-referenced catch into nominal catch level, screening and correction of outliers, and detection of major catchability changes over long time series of fishing data, i.e., the Japanese longline fleet operating in the tropical Indian Ocean. A total of thirty fisheries were finally determined from longline, purse seine and other-gears data sets, from which 10 longline and four purse seine fisheries represented 96% of the whole historical catch. The geo-referenced records consists of catch, fishing effort and associated length frequency samples of all fisheries.