148 research outputs found

    Multi-minicore Disease Revisited.

    Get PDF
    Multi-minicore disease (MmD) is an infrequent congenital myopathy, defined by structural changes in optic and electron microscopy, namely, multiple small areas lacking oxidative enzyme activity and focal disorganization of contractile proteins involving at most a few sarcomeres. The classical form of the disease manifests as more or less severe hypotonia and generalized weakness with predominance in axial and proximal limb muscles. Clinical variants also exist. Usually MmD is inherited as an autosomal recessive trait. Genetic heterogeneity is recognized and up to now mutations in the genes of RYR1 and SEPN1 have been detected. We record three unrelated cases of MmD. Case 1, with the classical benign form, was followed-up for 15 years. Case 2, presenting pharyngolaryngeal involvement and severe delay of head control, improved gradually, until independent gait was acquired at age of six years. A moderate restriction of daily life activities remains. Case 3, of antenatal-onset, was expressed by arthrogryposis of hands, predominance of scapular girdle deficit and a stable course after ten years on physiotherapy. All cases were selected by the characteristic morphological abnormalities in biceps brachii samples, including electron microscopy. Emphasis is given to case 2 due to type 1 fiber uniformity and mild endomysial fibrosis, posing a difficult differential diagnosis with congenital muscular dystrophy were it not for the significant number of multi-minicores.62935-

    Noncommutative field gas driven inflation

    Full text link
    We investigate early time inflationary scenarios in an Universe filled with a dilute noncommutative bosonic gas at high temperature. A noncommutative bosonic gas is a gas composed of bosonic scalar field with noncommutative field space on a commutative spacetime. Such noncommutative field theories was recently introduced as a generalization of quantum mechanics on a noncommutative spacetime. As key features of these theories are Lorentz invariance violation and CPT violation. In the present study we use a noncommutative bosonic field theory that besides the noncommutative parameter θ\theta shows up a further parameter σ\sigma. This parameter σ\sigma controls the range of the noncommutativity and acts as a regulator for the theory. Both parameters play a key role in the modified dispersion relations of the noncommutative bosonic field, leading to possible striking consequences for phenomenology. In this work we obtain an equation of state p=ω(σ,θ;β)ρp=\omega(\sigma,\theta;\beta)\rho for the noncommutative bosonic gas relating pressure pp and energy density ρ\rho, in the limit of high temperature. We analyse possible behaviours for this gas parameters σ\sigma, θ\theta and β\beta, so that 1ω<1/3-1\leq\omega<-1/3, which is the region where the Universe enters an accelerated phase.Comment: Reference added. Version to appear in Journal of Cosmology and Astroparticle Physics - JCA

    Structuring functional groups of aquatic insects along the resistance/resilience axis when facing water flow changes

    Get PDF
    Understanding how differences in intensity and frequency of hydrological disturbances affect the resistance and resilience of aquatic organisms is key to manage aquatic systems in a fast-changing world. Some aquatic insects have strategies that improve the permanence (resistance), while others use strategies that favor recolonization (resilience). Therefore, we carried out a manipulative experiment to understand the influence of functional characteristics of aquatic insects in their permanence and recolonization against hydrological disturbances in streams in the biodiversity hotspot of the Cerrado of Brazil. We placed 200 artificial substrates in five streams and submitted them to changing water flow regimes that differed both in frequency and intensity, and we observed the response of the aquatic community for 39 days. We used a hierarchical Bayesian approach to estimate the probabilities of permanence and recolonization of each life strategy group (nine groups). We observed that the most intense changes in the water flow tended to affect the permanence of almost all groups, but the intensity of this effect reduced over time. On the other hand, less frequent disturbances, regardless of intensity, tended to reduce the permanence of most groups of aquatic insects over time. The different effects of disturbance intensity may have been related to a greater recolonization capacity of some groups. The results we present are worrisome in a scenario of reduced riparian vegetation around streams and with the expectation of precipitation becoming more concentrated in shorter periods of time due to climate change in the Cerrado hotspot, reducing the occurrence of many groups of aquatic insects in their habitat, particularly those with traits associated with resistance against hydrological disturbance

    Whipple's Disease With Neurological Manifestations: Case Report.

    Get PDF
    Whipple's disease (WD) is an uncommon multisystem condition caused by the bacillus Tropheryma whipplei. Central nervous system involvement is a classical feature of the disease observed in 20 to 40% of the patients. We report the case of a 62 year old man with WD that developed neurological manifestations during its course, and discuss the most usual signs and symptoms focusing on recent diagnostic criteria and novel treatment regimens.62342-

    Análise da gestão de resíduos sólidos em conjuntos habitacionais do Programa ‘Minha Casa, Minha Vida’ na cidade de Salvador, Bahia, Brasil

    Get PDF
    Esse artigo analisa a aderência do gerenciamento de resíduos sólidos (GRS) em condomínios construídos por meio do Programa Minha Casa Minha Vida às diretrizes da Política Nacional de Resíduos Sólidos (PNRS). A investigação foi realizada em oito conjuntos habitacionais na cidade de Salvador/BA por meio de análise documental, visitas técnicas e realização de entrevistas. Os condomínios investigados apresentam condições favoráveis à implantação do GRS conforme preconizado na PNRS, porque estão sendo implantados em grande número, são organizados na forma de condomínios, agrupam pessoas com renda per capita semelhantes e possuem áreas que podem ser usadas para implantação de práticas de manejo sustentável de resíduos como a compostagem. No entanto, verificou-se a existência de condições inadequadas de manejo e/ou desvio de função do Abrigo de Resíduo (AR) existente. Portanto, conclui-se que não há aderência do GRS às premissas da PNRS nos condomínios estudados, apesar das condições favoráveis. </p

    Análise da gestão de resíduos sólidos em conjuntos habitacionais do Programa ‘Minha Casa, Minha Vida’ na cidade de Salvador, Bahia, Brasil

    Get PDF
    Esse artigo analisa a aderência do gerenciamento de resíduos sólidos (GRS) em condomínios construídos por meio do Programa Minha Casa Minha Vida às diretrizes da Política Nacional de Resíduos Sólidos (PNRS). A investigação foi realizada em oito conjuntos habitacionais na cidade de Salvador/BA por meio de análise documental, visitas técnicas e realização de entrevistas. Os condomínios investigados apresentam condições favoráveis à implantação do GRS conforme preconizado na PNRS, porque estão sendo implantados em grande número, são organizados na forma de condomínios, agrupam pessoas com renda per capita semelhantes e possuem áreas que podem ser usadas para implantação de práticas de manejo sustentável de resíduos como a compostagem. No entanto, verificou-se a existência de condições inadequadas de manejo e/ou desvio de função do Abrigo de Resíduo (AR) existente. Portanto, conclui-se que não há aderência do GRS às premissas da PNRS nos condomínios estudados, apesar das condições favoráveis.

    The BINGO Project VI: HI Halo Occupation Distribution and Mock Building

    Get PDF
    BINGO (Baryon Acoustic Oscillations from Integrated Neutral Gas Observations.) is a radio telescope designed to survey from 980 MHz to 1260 MHz, observe the neutral Hydrogen (HI) 21-cm line and detect BAO (Baryon Acoustic Oscillation) signal with Intensity Mapping technique. Here we present our method to generate mock maps of the 21-cm Intensity Mapping signal covering the BINGO frequency range and related test results. (Abridged)Comment: 16 pages, 20 figures, 1 table. Accepted for publication in A&
    corecore