Enzyme replacement therapy for Mucopolysaccharidosis Type I among patients followed within the MPS Brazil Network

Mucopolysaccharidosis type I (MPS I) is a rare lysosomal disorder caused by deficiency of alpha-L-iduronidase. Few clinical trials have assessed the effect of enzyme replacement therapy (ERT) for this condition. We conducted an exploratory, open-label, non-randomized, multicenter cohort study of patients with MPS I. Data were collected from questionnaires completed by attending physicians at the time of diagnosis (T1; n = 34) and at a median time of 2.5 years later (T2; n = 24/34). The 24 patients for whom data were available at T2 were allocated into groups: A, no ERT (9 patients; median age at T1 = 36 months; 6 with severe phenotype); B, on ERT (15 patients; median age at T1 = 33 months; 4 with severe phenotype). For all variables in which there was no between-group difference at baseline, a delta of ≥ ± 20% was considered clinically relevant. The following clinically relevant differences were identified in group B in T2: lower rates of mortality and reported hospitalization for respiratory infection; lower frequency of hepatosplenomegaly; increased reported rates of obstructive sleep apnea syndrome and hearing loss; and stabilization of gibbus deformity. These changes could be due to the effect of ERT or of other therapies which have also been found more frequently in group B. Our findings suggest MPS I patients on ERT also receive a better overall care. ERT may have a positive effect on respiratory morbidity and overall mortality in patients with MPS I. Additional studies focusing on these outcomes and on other therapies should be performed.

Correlação entre a maturação óssea das vértebras cervicais com a maturação óssea de mão e punho e com a mineralização dentária pelo método radiográfico

Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)

Maintenance of Manioc Diversity by Traditional Farmers in the State of Mato Grosso, Brazil: A 20-Year Comparison

Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)

Seasonal changes in the prostatic complex of Artibeus planirostris (Chiroptera: Phyllostomidae)

Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)

Surto de crescimento médio: correlação entre a estatura, a maturação óssea e a mineralização dentária pelo método radiográfico

Pós-graduação em Biopatologia Bucal - ICT

Prevalence of health promotion programs in primary health care units in Brazil

Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)

Characteristics of physical activity programs in the Brazilian primary health care system

Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)

Differences in acoustic and perceptual parameters of the voice between elderly and young women at habitual and high intensity

The present study aimed to compare elderly and young female voices in habitual and high intensity. The effect of increased intensity on the acoustic and perceptual parameters was assessed. Sound pressure level, fundamental frequency, jitter, shimmer, and harmonic to noise ratio were obtained at habitual and high intensity voice in a group of 30 elderly women and 30 young women. Perceptual assessment was also performed. Both groups demonstrated an increase in sound pressure level and fundamental frequency from habitual voice to high intensity voice. No differences were found between groups in any acoustic variables on samples recorded with habitual intensity level. No significant differences between groups were found in habitual intensity level for pitch, hoarseness, roughness, and breathiness. Asthenia and instability obtained significant higher values in elderly than young participants, whereas, the elderly demonstrated lower values for perceived tension and loudness than young subjects. Acoustic and perceptual measures do not demonstrate evident differences between elderly and young speakers in habitual intensity level. The parameters analyzed may lack the sensitivity necessary to detect differences in subjects with normal voices. Phonation with high intensity highlights differences between groups, especially in perceptual parameters. Therefore, high intensity should be included to compare elderly and young voice.

Analytical solution for transient one-dimensional couette flow considering constant and time-dependent pressure gradients

This paperaims to determine the velocity profile, in transient state, for a parallel incompressible flow known as Couette flow. The Navier-Stokes equations were applied upon this flow. Analytical solutions, based in Fourier series and integral transforms, were obtained for the one-dimensional transient Couette flow, taking into account constant and time-dependent pressure gradients acting on the fluid since the same instant when the plate starts it´s movement. Taking advantage of the orthogonality and superposition properties solutions were foundfor both considered cases. Considering a time-dependent pressure gradient, it was found a general solution for the Couette flow for a particular time function. It was found that the solution for a time-dependent pressure gradient includes the solutions for a zero pressure gradient and for a constant pressure gradient.

Ciência de serviços no Brasil: proposta de aplicação aos agronegócios

Ao se verificar nas últimas décadas a composição dos setores econômicos nos chamados países desenvolvidos e em desenvolvimento, nota-se um grande avanço e predominância do setor de serviços na composição de seu Produto Interno Bruto, chegando a ser na maioria dos casos o mais importante setor gerador de divisas, emprego e desenvolvimento. A Ciência de Serviços (CS) traz hoje a necessidade estratégica de políticas públicas, de ensino profissional e de pesquisas para o fomento e desenvolvimento desta Ciência no Brasil como fator estratégico para garantir uma maior competitividade da economia brasileira. A partir da hipótese de utilização da CS e de uma revisão bibliográfica do tema, buscou-se evidenciar suas potenciais aplicações no agronegócio, de maneira que este se torne mais eficiente, coordenado e sincronizado com o avanço dos serviços na composição da economia contemporânea nacional.

Petroleum contamination impact on macrobenthic communities under the influence of an oil refinery: Integrating chemical and biological multivariate data

Petroleum contamination impact on macrobenthic communities in the northeast portion of Todos os Santos Bay was assessed combining in multivariate analyses, chemical parameters such as aliphatic and polycyclic aromatic hydrocarbon indices and concentration ratios with benthic ecological parameters. Sediment samples were taken in August 2000 with a 0.05 m(2) van Veen grab at 28 sampling locations. The predominance of n-alkanes with more than 24 carbons, together with CPI values close to one, and the fact that most of the stations showed UCM/resolved aliphatic hydrocarbons ratios (UCM:R) higher than two, indicated a high degree of anthropogenic contribution, the presence of terrestrial plant detritus, petroleum products and evidence of chronic oil pollution. The indices used to determine the origin of PAH indicated the occurrence of a petrogenic contribution. A pyrolytic contribution constituted mainly by fossil fuel combustion derived PAH was also observed. The results of the stepwise multiple regression analysis performed with chemical data and benthic ecological descriptors demonstrated that not only total PAH concentrations but also specific concentration ratios or indices such as >= C24:< C24, An/178 and Fl/Fl + Py, are determining the structure of benthic communities within the study area. According to the BIO-ENV results petroleum related variables seemed to have a main influence on macrofauna community structure. The PCA ordination performed with the chemical data resulted in the formation of three groups of stations. The decrease in macrofauna density, number of species and diversity from groups III to I seemed to be related to the occurrence of high aliphatic hydrocarbon and PAH concentrations associated with fine sediments. Our results showed that macrobenthic communities in the northeast portion of Todos os Santos Bay are subjected to the impact of chronic oil pollution as was reflected by the reduction in the number of species and diversity. These results emphasise the importance to combine in multivariate approaches not only total hydrocarbon concentrations but also indices, isomer pair ratios and specific compound concentrations with biological data to improve the assessment of anthropogenic impact on marine ecosystems. (c) 2008 Elsevier Ltd. All rights reserved.

Blocking of Connexin-Mediated Communication Promotes Neuroprotection during Acute Degeneration Induced by Mechanical Trauma

Accruing evidence indicates that connexin (Cx) channels in the gap junctions (GJ) are involved in neurodegeneration after injury. However, studies using KO animal models endowed apparently contradictory results in relation to the role of coupling in neuroprotection. We analyzed the role of Cx-mediated communication in a focal lesion induced by mechanical trauma of the retina, a model that allows spatial and temporal definition of the lesion with high reproducibility, permitting visualization of the focus, penumbra and adjacent areas. Cx36 and Cx43 exhibited distinct gene expression and protein levels throughout the neurodegeneration progress. Cx36 was observed close to TUNEL-positive nuclei, revealing the presence of this protein surrounding apoptotic cells. The functional role of cell coupling was assessed employing GJ blockers and openers combined with lactate dehydrogenase (LDH) assay, a direct method for evaluating cell death/viability. Carbenoxolone (CBX), a broad-spectrum GJ blocker, reduced LDH release after 4 hours, whereas quinine, a Cx36-channel specific blocker, decreased LDH release as early as 1 hour after lesion. Furthermore, analysis of dying cell distribution confirmed that the use of GJ blockers reduced apoptosis spread. Accordingly, blockade of GJ communication during neurodegeneration with quinine, but not CBX, caused downregulation of initial and effector caspases. To summarize, we observed specific changes in Cx gene expression and protein distribution during the progress of retinal degeneration, indicating the participation of these elements in acute neurodegeneration processes. More importantly, our results revealed that direct control of GJ channels permeability may take part in reliable neuroprotection strategies aimed to rapid, fast treatment of mechanical trauma in the retina.

Hypoactivity of the central dopaminergic system and autistic-like behavior induced by a single early prenatal exposure to lipopolysaccharide

The aim of the present study was to evaluate the behavioral patterns associated with autism and the prevalence of these behaviors in males and females, to verify whether our model of lipopolysaccharide (LPS) administration represents an experimental model of autism. For this, we prenatally exposed Wistar rats to LPS (100 mu g/kg, intraperitoneally, on gestational day 9.5), which mimics infection by gram-negative bacteria. Furthermore, because the exact mechanisms by which autism develops are still unknown, we investigated the neurological mechanisms that might underlie the behavioral alterations that were observed. Because we previously had demonstrated that prenatal LPS decreases striatal dopamine (DA) and metabolite levels, the striatal dopaminergic system (tyrosine hydroxylase [TH] and DA receptors D1a and D2) and glial cells (astrocytes and microglia) were analyzed by using immunohistochemistry, immunoblotting, and real-time PCR. Our results show that prenatal LPS exposure impaired communication (ultrasonic vocalizations) in male pups and learning and memory (T-maze spontaneous alternation) in male adults, as well as inducing repetitive/restricted behavior, but did not change social interactions in either infancy (play behavior) or adulthood in females. Moreover, although the expression of DA receptors was unchanged, the experimental animals exhibited reduced striatal TH levels, indicating that reduced DA synthesis impaired the striatal dopaminergic system. The expression of glial cell markers was not increased, which suggests that prenatal LPS did not induce permanent neuroinflammation in the striatum. Together with our previous finding of social impairments in males, the present findings demonstrate that prenatal LPS induced autism-like effects and also a hypoactivation of the dopaminergic system. (c) 2012 Wiley Periodicals, Inc.