GM1 Gangliosidosis, Late Infantile Onset Dystonia, and T2 Hypointensity in the Globus Pallidus and Substantia Nigra

BACKGROUND: GM1 gangliosidosis is a rare disease due to mutations in the GLB1 gene and autosomal recessive deficiency of b-galactosidase. There is considerable overlap between classical phenotypes and clinical and imaging findings, which are often difficult to interpret. PATIENT: The patient in this study had dysmorphism, dysostosis, progressive dystonia, and T2 hypointensity in the basal ganglia. Partially similar clinical and radiologic findings were described previously in two reports. CONCLUSIONS: T2 hypointensity in the globus pallidus should, in the appropriate clinical setting, lead to consideration of thediagnosis of GM1 gangliosidosis.

MOLECULAR CHARACTERIZATION OF AMERICAN CUTANEOUS LEISHMANIASIS IN THE TRI‑BORDER AREA OF ASSIS BRASIL, ACRE STATE, BRAZIL

SUMMARY In this study, Leishmaniaspecies were identified by Polymerase Chain Reaction (PCR). The epidemiology of patients suspected of having American Cutaneous Leishmaniasis in the municipality of Assis Brasil, Acre State, located in the Brazil/Peru/Bolivia triborder was also investigated. By PCR, the DNA of Leishmaniawas detected in 100% of the cases (37 samples) and a PCR-Restriction Fragment Length Polymorphism (RFLP) of the hsp 70gene identified the species in 32 samples: Leishmania (Viannia) braziliensis (65.6%) , L. (V.) shawi (28.1%) , L. (V.) guyanensis (3.1%) and mixed infection L. (V.) guyanensis and L. (Leishmania) amazonensis (3.1%)This is the first report of L. (V.) shawiand L. (L.) amazonensis in Acre. The two predominant species were found in patients living in urban and rural areas. Most cases were found in males living in rural areas for at least three years and involved in rural work. This suggests, in most cases, a possible transmission of the disease from a rural/forest source, although some patients had not engaged in activities associated with permanence in forestall areas, which indicate a possible sandflies adaptation to the periurban setting.

Treatment of a nineteenth century male portrait in oil including the characterisation of materials, technique and a study of the lead soap aggregation in the paint composite

Dissertação para obtenção do Grau de Mestre em Conservação e Restauro

Endogenous spillovers in the trade-off between centralization and decentralization

In this paper, we revisit the classical trade-off between centralized and decentralized provision of local public goods, in a setting where interregional spillovers depend on the level of a national public good. We compare the standard benevolent planner approach with a political economy in which decisions, in a centralized system, are undertaken by a non-cooperative legislature with no separation of powers. We observe that the policy-maker in a centralized system is able to play both with local public goods and spillovers, a mechanism that is not available under a decentralized system. When compared to the traditional exogenous spillovers assumption, this improves the case for centralization under the standard benevolent planner approach. However, the same is not necessarily true in the non-cooperative legislature, as in this case the interests of the legislator do not need to be aligned with those of the society. Finally, we extend the traditional political economy analysis by considering a legislature in which decisions are undertaken by different committees (separation of powers), and show that it performs better than the original non-cooperative legislature, greatly improving the case for centralization.

Macroeconomic effects of monetary policy in the Eurozone

To study the macroeconomic effects of unconventional monetary policy across the different countries of the eurozone, I develop an identification scheme to disentangle conventional from non-conventional policy shocks, using futures contracts on overnight interest rates and the size of the European Central Bank balance sheet. Setting these shocks as endogenous variables in a structural vector autoregressive (SVAR) model, along with the CPI and the employment rate, estimated impulse response functions of policy to macroeconomic variables are studied. I find that unconventional policy shocks generated mixed effects in inflation but had a positive impact on employment, with the exception of Portugal, Spain, Greece and Italy where the employment response is close to zero or negative. The heterogeneity that characterizes the responses shows that the monetary policy measures taken in recent years were not sufficient to stabilize the economies of the eurozone countries under more severe economic conditions.

Multipacket reception in the presence of in-band full-duplex communication

In-Band Full-DupleX (IB-FDX) is defined as the ability for nodes to transmit and receive signals simultaneously on the same channel. Conventional digital wireless networks do not implement it, since a node’s own transmission signal causes interference to the signal it is trying to receive. However, recent studies attempt to overcome this obstacle, since it can potentially double the spectral efficiency of current wireless networks. Different mechanisms exist today that are able to reduce a significant part of the Self- Interference (SI), although specially tuned Medium Access Control (MAC) protocols are required to optimize its use. One of IB-FDX’s biggest problems is that the nodes’ interference range is extended, meaning the unusable space for other transmissions and receptions is broader. This dissertation proposes using MultiPacket Reception (MPR) to address this issue and adapts an already existing Single-Carrier with Frequency-Domain Equalization (SC-FDE) receiver to IB-FDX. The performance analysis suggests that MPR and IB-FDX have a strong synergy and are able to achieve higher data rates, when used together. Using analytical models, the optimal transmission patterns and transmission power were identified, which maximize the channel capacity with the minimal energy consumption. This was used to define a new MAC protocol, named Full-duplex Multipacket reception Medium Access Control (FM-MAC). FM-MAC was designed for a single-hop cellular infrastructure, where the Access Point (AP) and the terminals implement both IB-FDX and MPR. It divides the coverage range of the AP into a closer Full-DupleX (FDX) zone and a farther Half-DupleX (HDX) zone and adds a tunable fairness mechanism to avoid terminal starvation. Simulation results show that this protocol provides efficient support for both HDX and FDX terminals, maximizing its capacity when more FDX terminals are used.

Association between the degree of physical impairment from leprosy and dependence in activities of daily living among the elderly in a health unit in the State of Minas Gerais

Introduction In addition to the common alterations and diseases inherent in the aging process, elderly persons with a history of leprosy are particularly vulnerable to dependence because of disease-related impairments. Objective determine whether physical impairment from leprosy is associated with dependence among the elderly. Methods An analytical cross-sectional study of elderly individuals with a history of leprosy and no signs of cognitive impairment was conducted using a database from a former leprosy colony-hospital. The patients were evaluated for dependence in the basic activities of daily living (BADL) and instrumental activities of daily living (IADL), respectively) and subjected to standard leprosy physical disability grading. Subsequently, descriptive and univariate analyses were conducted, the latter using Pearson's chi-squared test. Results A total of 186 elderly persons were included in the study. Of these individuals, 53.8% were women, 49.5% were older than 75 years of age, 93% had four or less years of formal education, 24.2% lived in an institution for the long-term care of the elderly (ILTC), and 18.3% had lower limb amputations. Among those evaluated, 79.8% had visible physical impairments from leprosy (grade 2), 83.3% were independent in BADL, and 10.2% were independent in IADL. There was a higher impairment grade among those patients who were IADL dependent (p=0.038). Conclusion s: The leprosy physical impairment grade is associated with dependence for IADL, creating the need for greater social support and systematic monitoring by a multidisciplinary team. The results highlight the importance of early diagnosis and treatment of leprosy to prevent physical impairment and dependence in later years.

Optimization of wastewater quality in the Sines refinery

In order to address and resolve the wastewater contamination problem of the Sines refinery with the main objective of optimizing the quality of this stream and reducing the costs charged to the refinery, a dynamic mass balance was developed nd implemented for ammonia and polar oil and grease (O&G) contamination in the wastewater circuit. The inadequate routing of sour gas from the sour water stripping unit and the kerosene caustic washing unit, were identified respectively as the major source of ammonia and polar substances present in the industrial wastewater effluent. For the O&G content, a predictive model was developed for the kerosene caustic washing unit, following the Projection to Latent Structures (PLS) approach. Comparison between analytical data for ammonia and polar O&G concentrations in refinery wastewater originating from the Dissolved Air Flotation (DAF) effluent and the model predictions of the dynamic mass balance calculations are in a very good agreement and highlights the dominant impact of the identified streams for the wastewater contamination levels. The ammonia contamination problem was solved by rerouting the sour gas through an existing clogged line with ammonia salts due to a non-insulated line section, while for the O&G a dynamic mass balance was implemented as an online tool, which allows for prevision of possible contamination situations and taking the required preventive actions, and can also serve as a basis for establishing relationships between the O&G contamination in the refinery wastewater with the properties of the refined crude oils and the process operating conditions. The PLS model developed could be of great asset in both optimizing the existing and designing new refinery wastewater treatment units or reuse schemes. In order to find a possible treatment solution for the spent caustic problem, an on-site pilot plant experiments for NaOH recovery from the refinery kerosene caustic washing unit effluent using an alkaline-resistant nanofiltration (NF) polymeric membrane were performed in order to evaluate its applicability for treating these highly alkaline and contaminated streams. For a constant operating pressure and temperature and adequate operating conditions, 99.9% of oil and grease rejection and 97.7% of chemical oxygen demand (COD) rejection were observed. No noticeable membrane fouling or flux decrease were registered until a volume concentration factor of 3. These results allow for NF permeate reuse instead of fresh caustic and for significant reduction of the wastewater contamination, which can result in savings of 1.5 M€ per year at the current prices for the largest Portuguese oil refinery. The capital investments needed for implementation of the required NF membrane system are less than 10% of those associated with the traditional wet air oxidation solution of the spent caustic problem. The operating costs are very similar, but can be less than half if reusing the NF concentrate in refinery pH control applications. The payback period was estimated to be 1.1 years. Overall, the pilot plant experimental results obtained and the process economic evaluation data indicate a very competitive solution through the proposed NF treatment process, which represents a highly promising alternative to conventional and existing spent caustic treatment units.

Hormone replacement therapy and the risk of breast cancer: assessment of therapy acceptance in a cohort of previously treated breast cancer patients

INTRODUCTION: In the postmenopausal period, an average of 25% of women will present symptomatic ovarian failure requiring hormonal replacement therapy. Estrogen can relieve vasomotor symptoms. Hormonal replacement therapy is generally not recommended for breast cancer patients due to the potential risk of tumor recurrence. To answer the questions about the safety of hormonal replacement therapy in this subgroup of women, it is necessary to establish the acceptance of treatment. METHODS: Between September 1998 and February 2001, a cohort of 216 breast cancer patients were asked to complete a questionnaire. All patients had completed their treatment and were informed about survival rates after breast cancer and hormonal replacement therapy. RESULTS: Among the 216 patients, 134 (62%) would refuse hormonal replacement therapy. A hundred patients were afraid of relapse (74.6%). Adjuvant tamoxifen therapy was the only statistically significant variable (70.3% versus 29.7% p=0.003). Understanding clinical stage (p= 0.045) and type of medical assistance (private versus public , p=0.033) also seemed to influence the decision. Early stage disease (p= 0.22), type of surgical procedure (radical versus conservative, p=0.67), adjuvant chemotherapy (p=0.082) or marital status (p=0.98 ) were not statistically significant in decision making. Several patients submitted to adjuvant chemotherapy (41.6%) would accept hormonal replacement therapy under medical supervision, as did most of advanced clinical stage patients (58.3%; p=0.022). CONCLUSION: There is a high level of rejection for hormonal replacement therapy among breast cancer patients when current data on tumor cure rates, and potential risks of estrogen use is available. Adverse effects of tamoxifen in the adjuvant setting may be the reason for refusal of hormonal replacement therapy .

The quantified-Self and wearable technologies in the workplace: implications and challenges for their implementations

Introduction of technologies in the workplace have led to a dramatic change. These changes have come with an increased capacity to gather data about one’s working performance (i.e. productivity), as well as the capacity to track one’s personal responses (i.e. emotional, physiological, etc.) to this changing workplace environment. This movement of self-monitoring or self-sensing using diverse types of wearable sensors combined with the use of computing has been identified as the Quantified-Self. Miniaturization of sensors, reduction in cost and a non-stop increase in the computer power capacity has led to a panacea of wearables and sensors to track and analyze all types of information. Utilized in the personal sphere to track information, a looming question remains, should employers use the information from the Quantified-Self to track their employees’ performance or well-being in the workplace and will this benefit employees? The aim of the present work is to layout the implications and challenges associated with the use of Quantified-Self information in the workplace. The Quantified-Self movement has enabled people to understand their personal life better by tracking multiple information and signals; such an approach could allow companies to gather knowledge on what drives productivity for their business and/or well-being of their employees. A discussion about the implications of this approach will cover 1) Monitoring health and well-being, 2) Oversight and safety, and 3) Mentoring and training. Challenges will address the question of 1) Privacy and Acceptability, 2) Scalability and 3) Creativity. Even though many questions remain regarding their use in the workplace, wearable technologies and Quantified-Self data in the workplace represent an exciting opportunity for the industry and health and safety practitioners who will be using them.

Portuguese publication in the aim of regional science: a study of articles published in RPER from 2003 to 2015

This paper is a study of the full content of articles published by RPER, the Portuguese Review of Regional Studies, from the time it was launched in 2003 until the first quarter of 2015. RPER is a journal edited by the Portuguese section of the European Regional Science Association, which was established in the first half of the 1980s. The Association (APDR) and the journal are the result of contributions by researchers and technicians from different scientific fields, including mainly Economics, Geography, Sociology, Engineering and Architecture. The main focus of these contributions is the socio-economic life of concrete sites, and the way this life is conditioned by resources and capabilities, the historical and cultural heritage and institutions. Content analysis was undertaken to identify the main subjects chosen during the total period under analysis, the nature of the articles published (theoretical or empirical) and the main analytical framework used. The analysis also covers sub-periods to investigate major trends found in terms of subjects chosen and analytical methods, questioning the rationale behind them. The paper concludes with a few notes regarding the social echo the research received and an identification of the main limitations of the research. In the first part of the article, we conduct a summary review of the genesis and evolution of Regional Science at international level to serve as a basis for the empirical approach developed.

Quality costs analysis: case study in the automotive industry

Dissertação de mestrado integrado em Engenharia e Gestão Industrial

Exploring the role of O-Mannosylation in the modulation of E-Cadherin function in Gastric Cancer cell line models

Dissertação de mestrado em Bioquímica Aplicada – Biomedicina

Self-reported psychosocial factors among In Vitro Fertilization patients interviewed alone or with the partner

"Published online: 07 Nov 2015"

Caracterización, procesamiento e industrialización del grano de quinoa, cultivado en la provincia de Córdoba - Argentina. Characterization, processing and industrialization of quinoa seed harvested in the province of Córdoba - Argentina

La quinoa (Chenopodium quinoa Willd), es un pseudocereal originario de la región Andina. Fue utilizada como alimento básico por los pueblos nativos. La quinoa, la papa y el maíz constituyeron el trinomio base de la alimentación indígena de este continente. La colonización española fue desplazando su cultivo a favor del trigo europeo y otros cereales, quedando reducida a las zonas altas de la región andina. La Quínoa ha adquirido una considerable atención en los últimos tiempos, principalmente por la calidad de sus proteínas y la ausencia de gluten en ella. Su empleo está ampliamente difundido en los países andinos, especialmente Bolivia y Perú, con un notable crecimiento de la superficie sembrada. En nuestro país la explotación de este cultivo se ubica principalmente en las provincias norteñas de Salta y Jujuy. En estos últimos años se ha reivindicado su cultivo y los granos privados de saponinas son considerados como un excelente alimento, reconocido por la OMS, la FAO y la NASA. Además de la calidad de sus lípidos y vitaminas, y al elevado contenido en almidón, la quinoa posee una proteína de excelente calidad nutricional y libre de gluten, lo que hace a este grano especialmente indicado para la alimentación de personas que sufren de la enfermedad celíaca o del síndrome de intestino irritado. El presente proyecto está orientado al aprovechamiento integral del grano de quinoa. Es nuestra intensión aquí, demostrar que dicho grano, cultivado en la provincia de Córdoba, permitirá elaborar productos alimenticios asi como también derivados de su industrialización. Para este objetivo se cuenta con las instalaciones de la Planta Piloto del Instituto de Ciencia y Tecnología de los Alimentos (ICTA), de la UNC, así como de intrumental moderno y acorde, como HPLC, GC, Espectrofotómetro UV-Vis, rotavapores de laboratorio e industrial, cámara fría, balanzas analíticas y de precisión, muflas, estufas, molinos y tamices, así como también, contamos con profesionales, algunos de ellos realizando su tesis doctoral en este tema. En cuanto a los objetivos que se persiguen, se espera obtener productos tales como sopas, papillas, productos para panadería y galletería y salsas. En el plano industrial, se pretende elaborar concentrados proteicos, almidón y saponinas. Como se dijo más arriba, a nivel internacional la quinoa ha comenzado a extender sus fronteras, y es así que hoy el principal productor mundial de este grano, Bolivia, destina un porcentaje importante de su producción a la exportación. La creciente demanda mundial de quinoa a hecho que se constituya en un cultivo estratégico y de alto valor, con precios internacionales que rondan los U$S 1200 la tonelada. Si a esto unimos que la planta presenta una gran resistencia a la sequía, que se adapta bien a terrenos salitrosos, arenosos y pobres, podemos comprender la importancia que adquiere para nuestra provincia, toda vez que en la misma existen zonas geográficas potencialmente aptas para su cultivo. Quinoa (Chenopodium quinoa Willd) is a pseudocereal originating in the Andean region. It was used as a staple food by native peoples. Quinoa, potatoes and corn were the tree most important indigenous staple food to this part of South America. Spanish colonization was marginalized cultivation in favor of European wheat and other grains, displacing it to the highlands of the Andean region. Quinoa has recently gained considerable attention, mainly by its protein quality and lack of gluten. Its use is widespread in the Andean countries, especially Bolivia and Peru, with a notable increase in plantings. In our country, the exploitation of this crop is located mainly in the northern provinces of Salta and Jujuy. In recent years its cultivation has been promoted, and the grains once free of saponins are considered an excellent food, recognized by WHO, FAO and NASA. In addition to its lipid and vitamins, and high starch contain, quinoa protein has an excellent nutritional value and it is free of gluten, making it particularly suitable for this grain to feed people with celiac disease or irritable bowel syndrome. This project aims at an integral development of quinoa grain. It is our intention here to demonstrate that this grain grown in the province of Córdoba, can produce food products resulting from local industrialization. This team has access to the facilities of the Pilot Plant of the Institute of Science and Food Technology (ICTA) of the UNC, and the modern equipments in it, as HPLC, GC, UV-Vis spectrophotometer, laboratory and industrial rotary evaporators, cold storage, analytical and precision balances, flasks, ovens, grinders and screens. Also, we have an important professional staff, some of them doing their thesis on this subject. With regard to the objectives pursued, we expect to obtain products such as soups, baby food, bakery products and biscuits and sauces. At the industrial level, it aims at producing protein concentrates, starch and saponins.