Efecto de un programa de ejercicio físico en portador del Síndrome Marfan con disfunción ventricular

Marfan syndrome (MS) is an autosomal dominant disorder that affects multiple organs and systems. Several cardiac alterations are present, with the main ones being aortic root and ascending aorta dilatation, mitral valve prolapse and left ventricle (LV) dilatation. Aerobic exercise has not shown to be a non-drug therapy that promotes anti-remodeling effect in patients with heart failure. This case report describes the echocardiographic changes in a patient with Marfan syndrome during four years of cardiovascular physical therapy.El Síndrome de Marfan (SM) es un desorden autosómico dominante que afecta múltiples órganos y sistemas. Diversas alteraciones cardíacas están presentes, siendo las principales la dilatación de la raíz de la aorta y de la aorta ascendente, el Prolapso de Válvula Mitral y la dilatación del Ventrículo Izquierdo (VI). El ejercicio aeróbico ha mostrado ser un recurso terapéutico no medicamentoso, por promover efecto de antirremodelado en pacientes con insuficiencia cardíaca. Este relato de caso describe las alteraciones ecocardiográficas de un paciente con Síndrome de Marfan durante cuatro años de un programa de fisioterapia cardiovascular.A Síndrome de Marfan (SM) é uma desordem autossômica dominante que afeta múltiplos órgãos e sistemas. Diversas alterações cardíacas estão presentes, sendo as principais a dilatação da raiz da aorta e da aorta ascendente, o Prolapso de Valva Mitral e a dilatação do Ventrículo Esquerdo (VE). O exercício aeróbico tem-se mostrado um recurso terapêutico não medicamentoso, por promover efeito de antirremodelamento em pacientes com insuficiência cardíaca. Este relato de caso descreve as alterações ecocardiográficas de um paciente com Síndrome de Marfan durante quatro anos de um programa de fisioterapia cardiovascular.Universidade Federal de São Paulo (UNIFESP)Universidade de Santo AmaroUNIFESPSciEL

Anthropometric and musculoskeletal assessment of patients with Marfan syndrome

CONTEXTUALIZAÇÃO: A Síndrome de Marfan (SM) é uma doença autossômica dominante do tecido conjuntivo que envolve os sistemas ocular, cardiovascular e musculoesquelético, causada por mutações no gene da fibrilina1, gerando flacidez nos ligamentos articulares, favorecendo a hipermobilidade articular e redução na contenção do crescimento ósseo. OBJETIVOS: Avaliar as medidas antropométricas, alterações musculoesqueléticas e a frequência do tratamento fisioterapêutico nos pacientes com SM. MÉTODOS: Participaram deste estudo 26 pacientes, sendo 17 do gênero feminino, com idade de 13,23±2,77 anos, massa corpórea de 51,5±24-68 Kg, altura de 1,70±1,40-1,81 m e envergadura de 1,73±0,12 cm, e nove do gênero masculino, com idade de 14,44±2,18, massa corpórea de 61,0±42-72 Kg, altura de 1,83±1,66-1,97 m e envergadura de 1,93±0,13. Foram obtidas medidas antropométricas, alterações ME de forma padronizada, sendo o pectus e a escoliose, por avaliação radiológica, e a angulação (â) da curva escoliótica, pelo método de Cobb; a aracnodactilia, pelo sinal do polegar e teste de Walker-Murdoch, e a dolicostenomelia, pela envergadura em relação à altura. Os pacientes responderam a um questionário quanto à participação em tratamento de fisioterapia. RESULTADOS: Quando comparados com a estimativa brasileira, a massa corpórea e a altura apresentaram valores maiores no gênero feminino (p=0,001 e p<0,0005) e masculino (p=0,019 e p=0,0001). Das alterações musculoesqueléticas, encontrou-se pectus em 3 (11%), pectus e escoliose em 19 (73%), dolicostenomelia em 11 (42%) e aracnodactilia em 21(80%). Onze (42%) pacientes com SM já haviam realizado tratamento de fisioterapia. CONCLUSÕES: As alterações antropométricas e musculoesqueléticas estão presentes na SM, e o tratamento fisioterapêutico é pouco frequente. ________________________________________________________________________________________________ ABSTRACTBACKGROUND: Marfan syndrome (MS) is an autosomic dominant condition of the connective tissue that involves the ocular, cardiovascular and musculoskeletal systems. MS is caused by mutations in the fibrillin-1 gene, leading to joint ligaments flaccidity, joint hypermobility and an overgrowth of the long bones. OBJECTIVES: The aim of the present study was to assess anthropometry, musculoskeletal alterations and the prevalence of physical therapy treatments among patients with MS. METHODS: Twenty-six patients were included in this study [17 females (age: 13.23±2.77 years; body mass 51.5±24-68 Kg; height 1.70±1.40-1.81 m; arm span: 1.73±0.12 m) and 9 males (age: 14.44±2.18; body mass: 61.0±42-72 Kg; height: 1.83±1.66-1.97 m; arm span: 1.93±0.13 m)]. Anthropometric measurements and musculoskeletal abnormalities were determined in a standardized fashion: pectus and scoliosis were assessed through radiography and angulation (â) of the scoliosis curve using the Cobb method; arachnodactyly was assessed through the thumb sign and Walker-Murdoch test and dolichostenomelia was assessed by arm span in relation to height. Patients also responded to a questionnaire addressing participation in physical therapy. RESULTS: In comparison to values estimated for the Brazilian population, mass and height were greater among the patients with MS (females: p=0.001 e p<0.0005 e males p=0.019 e p=0.0001, respectively). The following musculoskeletal abnormalities were found: pectus in 3 patients (11%), pectus and scoliosis in 19 (73%), dolichostenomelia in 11 (42%) and arachnodactyly in 21 (80%). Eleven patients (42%) with MS had previously undergone physical therapy. CONCLUSIONS: Patients with MS exhibit altered musculoskeleto and anthropometry and have infrequent physical therapy treatment

Multi-temporal analysis of forestry and coastal environments using UASs

Due to strong improvements and developments achieved in the last decade, it is clear that applied research using remote sensing technology such as unmanned aerial vehicles (UAVs) can provide a flexible, efficient, non-destructive, and non-invasive means of acquiring geoscientific data, especially aerial imagery. Simultaneously, there has been an exponential increase in the development of sensors and instruments that can be installed in UAV platforms. By combining the aforementioned factors, unmanned aerial system (UAS) setups composed of UAVs, sensors, and ground control stations, have been increasingly used for remote sensing applications, with growing potential and abilities. This paper's overall goal is to identify advantages and challenges related to the use of UAVs for aerial imagery acquisition in forestry and coastal environments for preservation/prevention contexts. Moreover, the importance of monitoring these environments over time will be demonstrated. To achieve these goals, two case studies using UASs were conducted. The first focuses on phytosanitary problem detection and monitoring of chestnut tree health (Padrela region, Valpaços, Portugal). The acquired high-resolution imagery allowed for the identification of tree canopy cover decline by means of multi-temporal analysis. The second case study enabled the rigorous and non-evasive registry process of topographic changes that occurred in the sandspit of Cabedelo (Douro estuary, Porto, Portugal) in different time periods. The obtained results allow us to conclude that the UAS constitutes a low-cost, rigorous, and fairly autonomous form of remote sensing technology, capable of covering large geographical areas and acquiring high precision data to aid decision support systems in forestry preservation and coastal monitoring applications. Its swift evolution makes it a potential big player in remote sensing technologies today and in the near future.info:eu-repo/semantics/publishedVersio

Immediate effects of submaximal effort on pulse wave velocity in patients with Marfan syndrome

Marfan syndrome (MS) is a dominant autosomal disease caused by mutations in chromosome 15, the locus controlling fibrillin 1 synthesis, and may exhibit skeletal, ocular, cardiovascular, and other manifestations. Pulse wave velocity (PWV) is used to measure arterial elasticity and stiffness and is related to the elastic properties of the vascular wall. Since the practice of exercise is limited in MS patients, it was of interest to analyze the acute effect of submaximal exercise on aortic distensibility using PWV and other hemodynamic variables in patients with MS with either mild or no aortic dilatation. PWV and physiological variables were evaluated before and after submaximal exercise in 33 patients with MS and 18 controls. PWV was 8.51 ± 0.58 at rest and 9.10 ± 0.63 m/s at the end of exercise (P = 0.002) in the group with MS and 8.07 ± 0.35 and 8.98 ± 0.56 m/s in the control group, respectively (P = 0.004). Comparative group analysis regarding PWV at rest and at the end of exercise revealed no statistically significant differences. The same was true for the group that used β-blockers and the one that did not. The final heart rate was 10% higher in the control group than in the MS group (P = 0.01). Final systolic arterial pressure was higher in the control group (P = 0.02). PWV in MS patients with mild or no aortic dilatation did not differ from the control group after submaximal effort.Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)Universidade Federal de São Paulo (UNIFESP) Disciplina de CardiologiaUNIFESP, Disciplina de CardiologiaSciEL

Satellite active fire data validation using drones: Protocols and initial results from prescribed fires in Brazil

Global biomass burning impacts millions of hectares annually resulting in high social, environmental and economic costs. Satellite-based active fire detection products provide key information in support of land management and science applications and are available routinely from a variety of sources. Data validation is an important aspect guiding product development and characterization and is addressed in this study with the use of miniaturized sensors paired to unmanned airborne vehicles, or drones. Specifically, we deployed a custommade broadband spectral radiometer along with a commercial off-the-shelf infrared camera (FLIR Zenmuse XT) mounted to small consumer drones (DJI's Phantom3 and Inspire) flown over small prescribed burns implemented so as to coincide with the overpass times of different earth observing satellites (e.g., NASA Terra &amp; Aqua, NOAA/NASA S-NPP, USGS Landsat-8, and ESA Sentinel-2). Near-simultaneous fire radiative power retrievals were obtained using the airborne and spaceborne data acquired during prescribed fires conducted in grasslands and savannas plots in the Brazilian states of Rio de Janeiro, Tocantins and Mato Grosso do Sul between July 2017 and September 2018. A set of standard operating procedures were defined with attention to satellite active fire data validation requirements (e.g., reference data calibration) and subsequently adopted for each of the fires sampled. Airborne and spaceborne observations were co-located and temporally paired to within 2sec, and path transmittances calculated in order to account for atmospheric attenuation of fire retrievals. Our results showed good agreement (differences as low as 5%) between drone and satellite-based fire retrievals while also serving to demonstrate the potential for fully reproducible satellite data validation protocols using small sensor and drone technologies

The Eucalyptus Cuticular Waxes Contribute in Preformed Defense Against Austropuccinia psidii

Austropuccinia psidii, the causal agent of myrtle rust, is a biotrophic pathogen whose growth and development depends on the host tissues. The uredospores of A. psidii infect Eucalyptus by engaging in close contact with the host surface and interacting with the leaf cuticle that provides important chemical and physical signals to trigger the infection process. In this study, the cuticular waxes of Eucalyptus spp. were analyzed to determine their composition or structure and correlation with susceptibility/resistance to A. psidii. Twenty-one Eucalyptus spp. in the field were classified as resistant or susceptible. The resistance/susceptibility level of six Eucalyptus spp. were validated in controlled conditions using qPCR, revealing that the pathogen can germinate on the eucalyptus surface of some species without multiplying in the host. CG-TOF-MS analysis detected 26 compounds in the Eucalyptus spp. cuticle and led to the discovery of the role of hexadecanoic acid in the susceptibility of Eucalyptus grandis and Eucalyptus phaeotricha to A. psidii. We characterized the epicuticular wax morphology of the six previously selected Eucalyptus spp. using scanning electron microscopy and observed different behavior in A. psidii germination during host infection. It was found a correlation of epicuticular morphology on the resistance to A. psidii. However, in this study, we provide the first report of considerable interspecific variation in Eucalyptus spp. on the susceptibility to A. psidii and its correlation with cuticular waxes chemical compounds that seem to play a synergistic role as a preformed defense mechanism

Pervasive gaps in Amazonian ecological research

Biodiversity loss is one of the main challenges of our time,1,2 and attempts to address it require a clear un derstanding of how ecological communities respond to environmental change across time and space.3,4 While the increasing availability of global databases on ecological communities has advanced our knowledge of biodiversity sensitivity to environmental changes,5–7 vast areas of the tropics remain understudied.8–11 In the American tropics, Amazonia stands out as the world’s most diverse rainforest and the primary source of Neotropical biodiversity,12 but it remains among the least known forests in America and is often underrepre sented in biodiversity databases.13–15 To worsen this situation, human-induced modifications16,17 may elim inate pieces of the Amazon’s biodiversity puzzle before we can use them to understand how ecological com munities are responding. To increase generalization and applicability of biodiversity knowledge,18,19 it is thus crucial to reduce biases in ecological research, particularly in regions projected to face the most pronounced environmental changes. We integrate ecological community metadata of 7,694 sampling sites for multiple or ganism groups in a machine learning model framework to map the research probability across the Brazilian Amazonia, while identifying the region’s vulnerability to environmental change. 15%–18% of the most ne glected areas in ecological research are expected to experience severe climate or land use changes by 2050. This means that unless we take immediate action, we will not be able to establish their current status, much less monitor how it is changing and what is being lostinfo:eu-repo/semantics/publishedVersio