Assessment of disease progression in dysferlinopathy: A 1-year cohort study

ObjectiveTo assess the ability of functional measures to detect disease progression in dysferlinopathy over 6 months and 1 year.MethodsOne hundred ninety-three patients with dysferlinopathy were recruited to the Jain Foundation's International Clinical Outcome Study for Dysferlinopathy. Baseline, 6-month, and 1-year assessments included adapted North Star Ambulatory Assessment (a-NSAA), Motor Function Measure (MFM-20), timed function tests, 6-minute walk test (6MWT), Brooke scale, Jebsen test, manual muscle testing, and hand-held dynamometry. Patients also completed the ACTIVLIM questionnaire. Change in each measure over 6 months and 1 year was calculated and compared between disease severity (ambulant [mild, moderate, or severe based on a-NSAA score] or nonambulant [unable to complete a 10-meter walk]) and clinical diagnosis.ResultsThe functional a-NSAA test was the most sensitive to deterioration for ambulant patients overall. The a-NSAA score was the most sensitive test in the mild and moderate groups, while the 6MWT was most sensitive in the severe group. The 10-meter walk test was the only test showing significant change across all ambulant severity groups. In nonambulant patients, the MFM domain 3, wrist flexion strength, and pinch grip were most sensitive. Progression rates did not differ by clinical diagnosis. Power calculations determined that 46 moderately affected patients are required to determine clinical effectiveness for a hypothetical 1-year clinical trial based on the a-NSAA as a clinical endpoint.ConclusionCertain functional outcome measures can detect changes over 6 months and 1 year in dysferlinopathy and potentially be useful in monitoring progression in clinical trials.ClinicalTrials.gov identifier:NCT01676077

Characterization of the Brazilian continental shelf adjacent to Rio Grande do Norte state, NE Brazil

This study focuses on the analysis of high-resolution seismic profiles, integrated with sedimentological, echosounder, SRTM and satellite image datasets, of the Brazilian continental shelf adjacent to the Rio Grande do Norte State, NE Brazil. Located in the northeast of Brazil, the State of Rio Grande do Norte is bounded by two main coastal and shelf systems: the eastern coastal-shelf, from the Sagi River to the Touros High, and the northern coastal-shelf, extending from Touros High to Tibau. This shelf represents a modern, highly dynamic mixed carbonate-siliciclastic system characterized by reduced width and shallow depths as compared with other parts of the Brazilian shelf. It has an average width of 40 km, the shelf-break lying at a depth of ~ 60 m. This shelf is subject to the full strength of the westerly South Equatorial current combined with high winds and moderate to high tides and waves. A sharply defined stratigraphic boundary, probably between the Pleistocene and Holocene deposits, is clearly to be observed in the seismic record. Incised-valleys extending from the main river mouths (e.g.the Potengi, Açu, and Apodi) to the shelf break dominate the area investigated and may indicate periods of lower sea level.Este estudo está direcionado ao conhecimento da plataforma continental brasileira adjacente ao Estado do Rio Grande do Norte, NE do Brasil, através da analise de perfis sismicos de alta resolução integrados a dados sedimentológicos, batimétricos, SRTM e imagens de satélites. O Estado do Rio Grande do Norte, localizado no nordeste do Brasil, apresenta dois sistemas costeiros-plataformais: Setor Este, do Rio Sagi (divisa PB-RN) ao Alto de Touros e Setor Norte, do Alto de Touros a Tibau (divisa RN-CE). Esta plataforma representa um sistema plataformal moderno misto (carbonático-siliciclástico), altamente dinâmico. É caracterizado por sua reduzida largura e águas rasas, quando comparado com outras partes da plataforma brasileira. A plataforma apresenta largura média de 40 km e a sua quebra encontra-se a profundidades médias de 60 m. A plataforma é sujeita a ação da corrente sul equatorial, combinada a ventos fortes e variação de maré moderada a alta e/ou ondas. Um limite estratigráfico fortemente definido, provavelmente entre os depósitos Pleistocênicos e Holocênicos, pode ser bem observado e vales incisos, estendendo-se a partir dos principais rios (como por ex., Potengi, Açu e Apodi) dominam a área investigada

NEOTROPICAL XENARTHRANS: a data set of occurrence of xenarthran species in the Neotropics

Xenarthrans – anteaters, sloths, and armadillos – have essential functions for ecosystem maintenance, such as insect control and nutrient cycling, playing key roles as ecosystem engineers. Because of habitat loss and fragmentation, hunting pressure, and conflicts with 24 domestic dogs, these species have been threatened locally, regionally, or even across their full distribution ranges. The Neotropics harbor 21 species of armadillos, ten anteaters, and six sloths. Our dataset includes the families Chlamyphoridae (13), Dasypodidae (7), Myrmecophagidae (3), Bradypodidae (4), and Megalonychidae (2). We have no occurrence data on Dasypus pilosus (Dasypodidae). Regarding Cyclopedidae, until recently, only one species was recognized, but new genetic studies have revealed that the group is represented by seven species. In this data-paper, we compiled a total of 42,528 records of 31 species, represented by occurrence and quantitative data, totaling 24,847 unique georeferenced records. The geographic range is from the south of the USA, Mexico, and Caribbean countries at the northern portion of the Neotropics, to its austral distribution in Argentina, Paraguay, Chile, and Uruguay. Regarding anteaters, Myrmecophaga tridactyla has the most records (n=5,941), and Cyclopes sp. has the fewest (n=240). The armadillo species with the most data is Dasypus novemcinctus (n=11,588), and the least recorded for Calyptophractus retusus (n=33). With regards to sloth species, Bradypus variegatus has the most records (n=962), and Bradypus pygmaeus has the fewest (n=12). Our main objective with Neotropical Xenarthrans is to make occurrence and quantitative data available to facilitate more ecological research, particularly if we integrate the xenarthran data with other datasets of Neotropical Series which will become available very soon (i.e. Neotropical Carnivores, Neotropical Invasive Mammals, and Neotropical Hunters and Dogs). Therefore, studies on trophic cascades, hunting pressure, habitat loss, fragmentation effects, species invasion, and climate change effects will be possible with the Neotropical Xenarthrans dataset

Impact of different land management on soil spiders (Arachnida : Araneae) in two Amazonian areas of Brazil and Colombia

The global demand for different land-use practice commodities in the Amazonia is growing, and this region is increasingly affected by the impacts of land management. The aim of this study was to evaluate the influence of land-use intensification on soil spider assemblages from six different land-use systems in Colombia and Brazil. The systems were fallows after crops and pastures, forest, crops, pastures and plantations. Spider species richness and density decreased with increasing farming management intensity. A principal component analysis (PCA) showed forests and fallows were separated from systems with stronger anthropogenic soil disturbance. The relationships of ten spider guilds differed significantly between land-uses, suggesting that they can be a reliable parameter for studies of ecological indicators