Autodeterminación en personas con discapacidad intelectual y del desarrollo: revisión del concepto, su importancia y retos emergentes

Los avances en la conceptualización de la discapacidad intelectual y el auge de movimientos de autodefensa y empoderamiento han influido, indudablemente, en la comprensión e importancia de constructos como la autodeterminación. En este trabajo el objetivo central es aportar una actualización sobre la forma de entender el constructo y la importancia del desarrollo de la autodeterminación en los diferentes contextos de vida de las personas y, en concreto, de las personas con discapacidad intelectual. Igualmente, se complementa dicha actualización del estado de la cuestión mostrando los resultados obtenidos en un estudio en el que se exploraban las percepciones de las propias personas con discapacidad, sus familiares y los profesionales que trabajan con ellas sobre la importancia que otorgan a la autodeterminación. Todo ello permite poner de relieve las necesidades y retos que los profesionales y familiares se encuentran para transformar esa importancia atribuida a la autodeterminación en la creación de oportunidades para su promoción. Se discute sobre la necesidad de seguir ahondando para conocer y dar respuestas a dichos retos. Advances in the conceptualization of intellectual disability and the emergence of self-advocacy movements have undoubtedly influenced constructs such as self-determination. In this study, an update on the way of understanding the construct is presented, as well as its importance on the development of self-determination across contexts of people with intellectual and developmental disabilities. This state of the issue is as well exemplified with the preliminary results of a study aiming to explore perceptions of self-determination importance for people with disabilities themselves, their families and the professionals who work with them. Even if professionals and families attribute high importance to self-determination, they still face needs and challenges in creating opportunities to foster self-determination in their contexts. The need to deeply explore and answer to these challenges is also discussed

How can rainfall insurance help dryland farmers

About 65% of the cropped area in India is dependent on rains. Because most of the rains in India are received duringthe monsoon months, the crop growing seasons are quite short. Any aberrations in the amount of rainfall or in its distribution can adversely impact the crop yields. Yield and price uncertainties often reduce the incomes of the farm households and, consequently, their consumption levels and investments. Many of the farmers in the semi-arid tropics (SAT) of India live close to subsistence level, and shielding them from the weather-induced shocks in agricultural income is vital for their survival. The SAT accounts for 37% of the country’s geographical area as well as population, 46% of the net cultivated area, 59% of the coarse cereals area, 53% of the pulses area and 60% of the oilseeds area. Even 60% of the commercial crops are grown in the SAT. If rainfed agriculture in the SAT is to remain as a means of livelihood, ex-ante risk management is a critical first step to ex-post risk coping. Weather-related risks are co-variant as they typically impact all farm households in the affected region at the same time. Local risk sharing arrangements are largely ineffective in these situations. When the probability of weatherrelated risks is high, farmers diversify and adopt low risk and often low return production practices, which involve trading expected profits for lower risk. Like in many other developing countries, rural financial markets in India are fragmented and formal credit markets are, at best, emerging. Although credit is an important means of consumption smoothing, it tends to be complimented by an array of other ex-post risk coping strategies, such as asset sales, remittances from family members and other risk-sharing arrangements. When many farmers are in distress and seek to liquidate assets, their prices are likely to fall, making it hard for affected families to smooth consumption and to recover from the stress in the future. Households can also reduce their exposure to weather risk ex-ante. This could occur through precautionary saving,or by income smoothing strategies such as implementing more conservative agricultural production strategies. In fact, rainfed farmers were earlier focusing on low risk food crops to keep down risks although it meant sacrificing high expected returns from cash crops such as cotton, castor, vegetables, among others. But over the years, the returns from food crops such as millet and sorghum declined rapidly due to declining demand and falling prices. Farmers increasingly shifted to cash crops as their incomes from nonfarm sources and seasonal migration enabled them to accept higher risks. However, in the absence of effective incomesmoothing measures during drought, farmers often fall short of their consumption needs, leading to chronic indebtedness and, in some cases, even to desperation and suicide. In the absence of risk insurance, traditional ex-ante risk management strategies and ineffective ex-post risk coping systems lock the SAT farmers in poverty and subsistence production. Insurance is one of the important ex-post risk reduction strategies (Figure 1)

Being Surveyed Can Change Later Behavior and Related Parameter Estimates

Does completing a household survey change the later behavior of those surveyed? In three field studies of health and two of microlending, we randomly assigned subjects to be surveyed about health and/or household finances and then measured subsequent use of a related product with data that does not rely on subjects' self-reports. In the three health experiments, we find that being surveyed increases use of water treatment products and take-up of medical insurance. Frequent surveys on reported diarrhea also led to biased estimates of the impact of improved source water quality. In two microlending studies, we do not find an effect of being surveyed on borrowing behavior. The results suggest that limited attention could play an important but context-dependent role in consumer choice, with the implication that researchers should reconsider whether, how, and how much to survey their subjectsEconomic

An open software development-based ecosystem of R packages for metabolomics data analysis

A frequent problem with scientific research software is the lack of support, maintenance and further development. In particular, development by a single researcher can easily result in orphaned software packages, especially if combined with poor documentation or lack of adherence to open software development standards. The RforMassSpectrometry initiative aims to develop an efficient and stable infrastructure for mass spectrometry (MS) data analysis. As part of this initiative, a growing ecosystem of R software packages is being developed covering different aspects of metabolomics and proteomics data analysis. To avoid the aforementioned problems, community contributions are fostered, and open development, documentation and long-term support emphasized. At the heart of the package ecosystem is the Spectra package that provides the core infrastructure to handle and analyze MS data. Its design allows easy expansion to support additional file or data formats including data representations with minimal memory footprint or remote data access. The xcms package for LC-MS data preprocessing was updated to reuse this infrastructure, enabling now also the analysis of very large, or remote, data. This integration simplifies in addition complete analysis workflows which can include the MsFeatures package for compounding, and the MetaboAnnotation package for annotation of untargeted metabolomics experiments. Public annotation resources can be easily accessed through packages such as MsBackendMassbank, MsBackendMgf, MsBackendMsp or CompoundDb, the latter also allowing to create and manage lab-specific compound databases. Finally, the MsCoreUtils and MetaboCoreUtils packages provide efficient implementations of commonly used algorithms, designed to be re-used in other R packages. Ultimately, and in contrast to a monolithic software design, the package ecosystem enables to build customized, modular, and reproducible analysis workflows. Future development will focus on improved data structures and analysis methods for chromatographic data, and better interoperability with other open source softwares including a direct integration with Python MS libraries

Bootstrapping Smooth Functions of Slope Parameters and Innovation Variances in VAR(∞) Models *

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/101814/1/1468-2354.t01-1-00016.pd

Real-world experience among patients with relapsed/refractory mantle cell lymphoma after Bruton tyrosine kinase inhibitor failure in Europe: The SCHOLAR-2 retrospective chart review study

Mantle cell lymphoma (MCL) after relapse is associated with poor prognosis. No standard of care exists and available evidence for treatments is limited, particularly in patients who fail Bruton tyrosine kinase inhibitor (BTKi) therapy. This multicentre retrospective chart review study, SCHOLAR-2, addresses this knowledge gap and reports on data collected from 240 patients with relapsed/refractory MCL in Europe who were treated with BTKi-based therapy between July 2012 and July 2018, and had experienced disease progression while on BTKi therapy or discontinued BTKi therapy due to intolerance. The median overall survival (OS) from initiation of first BTKi therapy was 14.6 months (95% confidence interval [CI] 11.6–20.0) in the overall cohort, 5.5 months (95% CI 3.9–8.2) in 91 patients without post-BTKi therapy, and 23.8 months (95% CI 18.9–30.1) in 149 patients who received post-BTKi therapy (excluding chimeric antigen receptor T-cell treatment). In the latter group, patients received a median of one (range, one to seven) line of post-BTKi therapy, with lenalidomide-containing regimens and bendamustine plus rituximab being the most frequently administered; the median OS from initiation of first post-BTKi therapy was 9.7 months (95% CI 6.3–12.7). These results provide a benchmark for survival in patients with R/R MCL receiving salvage therapy after BTKi failure

Inter- and Intraobserver Variation in the Assessment of Preoperative Colostograms in Male Anorectal Malformations: An ARM-Net Consortium Survey

Aim: Male patients with anorectal malformations (ARM) are classified according to presence and level of the recto-urinary fistula. This is traditionally established by a preoperative high-pressure distal colostogram that may be variably interpreted by different surgeons. The aim of this study was to evaluate the inter- and intraobserver variation in the assessment by pediatric surgeons of preoperative colostograms with respect to the level of the recto-urinary fistula. Materials and Methods: Sixteen pediatric surgeons from 14 European centers belonging to the ARM-Net Consortium twice scored 130 images of distal colostograms taken in sagittal projection at a median age of 66 days of life (range: 4–1,106 days). Surgeons were asked to classify the fistula in bulbar, prostatic, bladder-neck, no fistula, and “unclear anatomy” example. Their assessments were compared with the intraoperative findings (kappa) for two scoring rounds with an interval of 6 months (intraobserver variation). Agreement among the surgeons' scores (interobserver variation) was also calculated using Krippendorff's alpha. A kappa over 0.75 is considered excellent, between 0.40 and 0.75 fair to good, and below 0.40 poor. Surgeons were asked to score the images in “poor” and “good” quality and to provide their years of experience in ARM treatment. Results: Agreement between the image-based rating of surgeons and the intraoperative findings ranges from 0.06 to 0.45 (mean 0.31). Interobserver variation is higher (Krippendorff's alpha between 0.40 and 0.45). Years of experience in ARM treatment does not seem to influence the scoring. The mean intraobserver variation between the two rounds is 0.64. Overall, the quality of the images is considered poor. Images categorized as having a good quality result in a statistically significant higher kappa (mean: 0.36 and 0.37 in the first and second round, respectively) than in the group of bad-quality images (mean: 0.25 and 0.23, respectively). Conclusions: There is poor agreement among experienced pediatric colorectal surgeons on preoperative colostograms. Techniques and analyses of images need to be improved in order to generate a homogeneou

Complement component 3 (C3) expression in the hippocampus after excitotoxic injury: role of C/EBPβ

[Background] The CCAAT/enhancer-binding protein β (C/EBPβ) is a transcription factor implicated in the control of proliferation, differentiation, and inflammatory processes mainly in adipose tissue and liver; although more recent results have revealed an important role for this transcription factor in the brain. Previous studies from our laboratory indicated that CCAAT/enhancer-binding protein β is implicated in inflammatory process and brain injury, since mice lacking this gene were less susceptible to kainic acid-induced injury. More recently, we have shown that the complement component 3 gene (C3) is a downstream target of CCAAT/enhancer-binding protein β and it could be a mediator of the proinflammatory effects of this transcription factor in neural cells.[Methods] Adult male Wistar rats (8–12 weeks old) were used throughout the study. C/EBPβ+/+ and C/EBPβ–/– mice were generated from heterozygous breeding pairs. Animals were injected or not with kainic acid, brains removed, and brain slices containing the hippocampus analyzed for the expression of both CCAAT/enhancer-binding protein β and C3.[Results] In the present work, we have further extended these studies and show that CCAAT/enhancer-binding protein β and C3 co-express in the CA1 and CA3 regions of the hippocampus after an excitotoxic injury. Studies using CCAAT/enhancer-binding protein β knockout mice demonstrate a marked reduction in C3 expression after kainic acid injection in these animals, suggesting that indeed this protein is regulated by C/EBPβ in the hippocampus in vivo.[Conclusions] Altogether these results suggest that CCAAT/enhancer-binding protein β could regulate brain disorders, in which excitotoxic and inflammatory processes are involved, at least in part through the direct regulation of C3.This work was supported by MINECO, Grant SAF2014-52940-R and partially financed with FEDER funds. CIBERNED is funded by the Instituto de Salud Carlos III. JAM-G was supported by CIBERNED. We acknowledge support of the publication fee by the CSIC Open Access Publication Support Initiative through its Unit of Information Resources for Research (URICI).Peer reviewe