Retardo de desenvolvimento associado à Osteopetrose

Osteopetrosis is a rare genetic disease, caracterized by marked radiodensity of the bones throughout the skeleton. Heterogenous clinical, biochemical, and histological features suggest that the disease is multigenic. We will describe a case with severe growth delay and bone fractures. EDO, 19 years and 9 months old, that came to us due to a skeleton alteration and growth failure. His puberty begun at 18 years old, with slow progress. Six months ago, after falling from his own high he broke his right forearm. He was 141,5 em high and weighted 38,5 kg, with bone age at 13 years and 6 months. The skeleton examination showed hyperdensity of the bones with typical alteration observed in osteopetrosis with signs of fractures in ulna and femur both on the right. The bone density were on lumbar spine and femural neck respectively 10,65 and 15,55 SD higher than the average for his own age. The dosages of the hormones showed total testosterone levels below normal for chronological age (245 ng/dl); normal basal and stimulated gonadotropins levels, GH responsive to hypoglycemia and insulin-like growth factor-I (IGF-I) level low for puberal stage. The plasma calcium (Ca), phosphorus (P), alcaline phosphatase (FA) and intact parathormone (PTH) levels were on the normal range. The incidence of osteopetrosis in our country Is very low and its association with short stature and delayed puberty is well known. The dysplasic bone associated to chronic anemia and repeated infections present mainly in the severe forms of the disease are probably the major causes for the delayed development.A osteopetrose é uma doença genética rara, com variação na sua expressividade e no seu modo de herança e que caracteriza-se, básicamente, pela esclerose óssea generalizada resultado de um desequilibrio entre formação e reabsorçâo ósseas. Descreveremos um caso desta doença em que um retardo de desenvolvimento associado a fraturas ósseas iniciaram a investigação. E.D.C., 19 anos e 9 meses, foi encaminhado ao serviço médico por alteração esquelética e baixa estatura. Iniciou aumento de volume testicular e pilificaçâo pubiana aos 18 anos, com progressão lenta da puberdade. Há 6 meses apresentou fratura de antebraço após queda da própria altura. Ao exame físico apresentava estatura de 141,5 cm; peso 38,5 kg; estadiamento puberal em GIII PII com idade óssea de 13 anos e 6 meses. O exame radiológico do esqueleto apresentou uma hiperdensidade óssea com alterações típicas de osteopetrose , com sinais de fratura em ulna e colo de fémur, ambos à direita. A densitometria óssea encontrava-se 10,65 e 15,55 desvios-padrão acima da média para sua idade em coluna lombar e fêmur respectivamente. As dosagens hormonais revelaram níveis reduzidos de testosterona total para a idade cronológica (245 ng/dl) com gonadotrofinas basais normais; hormônio de crescimento (GH) responsivo à hipoglicemia e níveis de fator de crescimento semelhante a insulina-l (IGF-I) diminuídos para o estádio puberal. Os níveis de cálcio (Ca), fósforo (P), atividade de fosfatase alcalina (FA) e paratormônio intacto (PTH) plasmáticos estavam dentro da normalidade. A incidência de osteopetrose em nosso meio é bastante rara, e sua associação com baixa estatura e retardo puberal é conhecida, sendo provavelmente de origem multifatorial, afetando mais intensamente as formas graves da doença, onde a anemia crônica e infecções de repetição estão presentes. As alterações displásicas do tecido ósseo e das cartilagens de crescimento inerentes a osteopetrose certamente desempenham um papel importante na baixa estatura.Universidade Federal de São Paulo (UNIFESP) Faculdade de Medicina de Pouso AlegreUNIFESP, Faculdade de Medicina de Pouso AlegreSciEL

Entropy bounds, monotonicity properties and scaling in CFTs

We study the ratio of the entropy to the total energy in conformal field theories at finite temperature. For the free field realizations of {\cal N}=4 super Yang-Mills theory in D=4 and the (2,0) tensor multiplet in D=6, the ratio is bounded from above. The corresponding bounds are less stringent than the recently proposed Verlinde bound. We show that entropy bounds arise generically in CFTs in connection to monotonicity properties with respect to temperature changes of a generalized C-function. For strongly coupled CFTs with AdS duals, we show that the ratio obeys the Verlinde bound even in the presence of rotation. For such CFTs, we point out an intriguing resemblance in their thermodynamic formulas with the corresponding ones of two-dimensional CFTs. We show that simple scaling forms for the free energy and entropy of CFTs with AdS duals reproduce the thermodynamical properties of (D+1)-dimensional AdS black holes.Comment: 19p, LaTeX, v2 minor clarifications and added references, v3 version to appear in NP

Gemini Observations of Disks and Jets in Young Stellar Objects and in Active Galaxies

We present first results from the Near-infrared Integral Field Spectrograph (NIFS) located at Gemini North. For the active galaxies Cygnus A and Perseus A we observe rotationally-supported accretion disks and adduce the existence of massive central black holes and estimate their masses. In Cygnus A we also see remarkable high-excitation ionization cones dominated by photoionization from the central engine. In the T-Tauri stars HV Tau C and DG Tau we see highly-collimated bipolar outflows in the [Fe II] 1.644 micron line, surrounded by a slower molecular bipolar outflow seen in the H_2 lines, in accordance with the model advocated by Pyo et al. (2002).Comment: Invited paper presented at the 5th Stromlo Symposium. 9 pages, 7 figures. Accepted for publication in Astrophysics & Space Scienc

A utilização do ARCGIS no projecto Greenurbe: mapeamento climático

O Projecto GreenUrbe (PPCDT/AMB/59174/2004) visa avaliar o impacte dos espaços verdes na qualidade do ambiente urbano (ar, ruído e confortotérmico) e no bem-estar social, de modo a formular recomendações relativas ao desenho urbano. Este estudo, com início em Outubro de 2005, está a decorrer num contexto espacial particular – a cidade de Bragança. Na avaliação das principais vertentes deste projecto, o software ArcGIS assume um papel de relevo na análise e posterior representação espacial dos parâmetros ambientais, permitindo ainda estabelecer as suas relações com o uso do solo existente. No estudo de escala urbana, o software ArcGIS é usado intensivamente no mapeamento climático urbano, elemento essencial na compreensão das condições que influenciam o microclima em espaços urbanos.FCT-MCE

The role of green spaces on urban environmental quality

Urban green areas have the potential to attenuate some of the negative effects of urbanisation, making cities a more pleasant place to live in. In addition to aesthetic value, green spaces improve air quality, reduce noise levels and generate more comfortable micro-climatic conditions, among other benefits, contributing to urban quality of life. GreenUrbe (POCI/AMB/59174/2004) – The Impacts of Green Spaces on Urban Environmental Quality – is a three year research project, started in October 2005 and aims to assess the impact of green spaces on urban environmental quality (air quality, noise and micro-climatic conditions) and social well-being, in order to formulate recommendations regarding green spaces design, as it is an essential activity in urban planning, using Bragança (Portugal) as the case study. In order to achieve these objectives an extensive fieldwork is being carried out at an urban area, involving measurements of meteorological parameters, passive sampling of ozone, nitrogen dioxide and COVs, continuous measurements of particulate matter and ozone, environmental noise measurements, experimental designs to evaluate how much vegetation interfere with noise propagation and noise absorption, haze evaluation by means of Small Format Aerial Photography (SFAP) and a survey on population attitudes and perceptions towards the overall urban green spaces. In this article, emphasis will be given to the methodological aspects and to the first results of the project with regards to urban green structure analysis and quantitative evaluation of environmental noise, air quality and climate comfort at Bragança city

Thickness Estimation of Epitaxial Graphene on SiC using Attenuation of Substrate Raman Intensity

A simple, non-invasive method using Raman spectroscopy for the estimation of the thickness of graphene layers grown epitaxially on silicon carbide (SiC) is presented, enabling simultaneous determination of thickness, grain size and disorder using the spectra. The attenuation of the substrate Raman signal due to the graphene overlayer is found to be dependent on the graphene film thickness deduced from X-ray photoelectron spectroscopy and transmission electron microscopy of the surfaces. We explain this dependence using an absorbing overlayer model. This method can be used for mapping graphene thickness over a region and is capable of estimating thickness of multilayer graphene films beyond that possible by XPS and Auger electron spectroscopy (AES).Comment: 14 pages, 9 figure

Instalação de um ensaio de produção intensiva de biomassa lenhosa para energia

No âmbito de um projecto de investigação tendo como propósito o desenvolvimento de sistemas sustentáveis de produção de biomassa lenhosa para energia, foi instalado em Bragança, em 2008, um ensaio de produção intensiva de biomassa lenhosa com base em culturas de curta rotação. Este ensaio tem como objectivos específicos a avaliação dos efeitos de factores como material vegetal, densidade de plantação, rega e fertilização no crescimento e produção de biomassa lenhosa. Nesta comunicação descrevemos a instalação do ensaio relativamente ao desenho experimental seguido, estrutura interna das parcelas de estudo, material vegetal utilizado, operações de preparação do solo e de plantação e ainda gestão da cultura com respeito a fertilização, rega e protecção. Avaliamos também a sobrevivência das plantas no final da primeira estação de crescimento, bem como apresentamos os custos gerais associados à instalação. A apresentação deste trabalho permite partilhar com a comunidade científica e técnica nacional a experiência da instalação deste tipo de culturas, muito recentes no nosso país, e dessa forma contribuir para a definição de processos ajustados e eficientes de instalação e gestão de culturas lenhosas para produção de biomassa para energia em Portugal

Nonleptonic two-body charmless B decays involving a tensor meson in ISGW2 model

Nonleptonic charmless B decays into a pseudoscalar (P) or a vector (V) meson accompanying a tensor (T) meson are re-analyzed. We scrutinize the hadronic uncertainties and ambiguities of the form factors which appear in the literature. The Isgur-Scora-Grinstein-Wise updated model (ISGW2) is adopted to evaluate the relevant hadronic matrix elements. We calculate the branching ratios and CP asymmetries for various $B\to P(V)T$ decay processes. With the ISGW2 model, the branching ratios are enhanced by about an order of magnitude compared to the previous estimates. We show that the ratios \calB(B\to VT)/\calB(B\to PT) for some strangeness-changing processes are very sensitive to the CKM angle $\gamma$ ($\phi_3$).Comment: 23 pages, REVTEX; minor clarifications included; to appear in Phys. Rev.

Sporting embodiment: sports studies and the (continuing) promise of phenomenology

Whilst in recent years sports studies have addressed the calls ‘to bring the body back in’ to theorisations of sport and physical activity, the ‘promise of phenomenology’ remains largely under-realised with regard to sporting embodiment. Relatively few accounts are grounded in the ‘flesh’ of the lived sporting body, and phenomenology offers a powerful framework for such analysis. A wide-ranging, multi-stranded, and interpretatively contested perspective, phenomenology in general has been taken up and utilised in very different ways within different disciplinary fields. The purpose of this article is to consider some selected phenomenological threads, key qualities of the phenomenological method, and the potential for existentialist phenomenology in particular to contribute fresh perspectives to the sociological study of embodiment in sport and exercise. It offers one way to convey the ‘essences’, corporeal immediacy and textured sensuosity of the lived sporting body. The use of Interpretative Phenomenological Analysis (IPA) is also critically addressed. Key words: phenomenology; existentialist phenomenology; interpretative phenomenological analysis (IPA); sporting embodiment; the lived-body; Merleau-Pont