Behavioral profile of adults with Prader-Willi syndrome : correlations with individual and environmental variables

Background: Maladaptive behavior has been reported as a phenotypical feature in Prader–Willi syndrome (PWS). It severely limits social adaptation and the quality of life of children and adults with the syndrome. Different factors have been linked with the intensity and form of these behavioral disturbances but there is no consensus about the cause. Consequently, there is still controversy regarding management strategies and there is a need for new data. Methods: The behavior of 100 adults with PWS attending a dedicated center was assessed using the Developmental Behavior Checklist for Adults (DBC-A) and the PWS-specific Hyperphagia Questionnaire. The DBC-A was completed separately by trained caregivers at the center and relatives or caregivers in a natural setting. Genotype, gender, age, degree of obesity and cognitive impairment were analyzed as variables with a hypothetical influence on behavioral features. Results: Patients showed a relatively high rate of behavioral disturbances other than hyperphagia. Disruptive and social relating were the highest scoring DBC-A subscales whereas anxiety/antisocial and self-absorbed were the lowest. When hospital caregiver and natural caregiver scores were compared, scores for the latter were higher for all subscales except for disruptive and anxiety/antisocial. These effects of institutional management were underlined. In the DBC-A, 22 items have descriptive indications of PWS behavior and were used for further comparisons and correlation analysis. In contrast to previous reports, rates of disturbed behavior were lower in patients with a deletion genotype. However, the behavioral profile was similar for both genotypes. No differences were found in any measurement when comparing type I and type II deletions. The other analyzed variables showed little relevance. Conclusions: Significant rates of behavioral disorders were highlighted and their typology described in a large cohort of adults with PWS. The deletion genotype was related to a lower severity of symptoms. Some major behavioral problems, such as hyperphagia, may be well controlled if living circumstances are adapted to the specific requirements of individuals with PWS.

Technologies and Methodologies for the Detection of Harmful Algae and Their Toxins, St Petersburg, Florida, Oct 22-24 2008 : workshop proceedings

The Alliance for Coastal Technologies (ACT) Workshop "Technologies and Methodologies for the Detection of Harmful Algae and their Toxins" convened in St. Petersburg, Florida, October 22- 24, 2008 and was co-sponsored by ACT (http://act-us.info); the Cooperative Institute for Coastal and Estuarine Environmental Technology (CICEET, http://ciceet.unh.edu); and the Florida Fish and Wildlife Conservation Commission (FWC, http://www.myfwc.com). Participants from various sectors, including researchers, coastal decision makers, and technology vendors, collaborated to exchange information and build consensus. They focused on the status of currently available detection technologies and methodologies for harmful algae (HA) and their toxins, provided direction for developing operational use of existing technology, and addressed requirements for future technology developments in this area. Harmful algal blooms (HABs) in marine and freshwater systems are increasingly common worldwide and are known to cause extensive ecological, economic, and human health problems. In US waters, HABs are encountered in a growing number of locations and are also increasing in duration and severity. This expansion in HABs has led to elevated incidences of poisonous seafood, toxin-contaminated drinking water, mortality of fish and other animals dependent upon aquatic resources (including protected species), public health and economic impacts in coastal and lakeside communities, losses to aquaculture enterprises, and long-term aquatic ecosystem changes. This meeting represented the fourth ACT sponsored workshop that has addressed technology developments for improved monitoring of water-born pathogens and HA species in some form. A primary motivation was to assess the need and community support for an ACT-led Performance Demonstration of Harmful Algae Detection Technologies and Methodologies in order to facilitate their integration into regional ocean observing systems operations. The workshop focused on the identification of region-specific monitoring needs and available technologies and methodologies for detection/quantification of harmful algal species and their toxins along the US marine and freshwater coasts. To address this critical environmental issue, several technologies and methodologies have been, or are being, developed to detect and quantify various harmful algae and their associated toxins in coastal marine and freshwater environments. There are many challenges to nationwide adoption of HAB detection as part of a core monitoring infrastructure: the geographic uniqueness of primary algal species of concern around the country, the variety of HAB impacts, and the need for a clear vision of the operational requirements for monitoring the various species. Nonetheless, it was a consensus of the workshop participants that ACT should support the development of HA detection technology performance demonstrations but that these would need to be tuned regionally to algal species and toxins of concern in order to promote the adoption of state of the art technologies into HAR monitoring networks. [PDF contains 36 pages]

Towed vehicles: undulating platforms as tools for maping coastal processes and water quality assessment, Seaside, California, February 5-7,2007. Workshop proceedings

The Alliance for Coastal Technologies (ACT) Workshop on Towed Vehicles: Undulating Platforms As Tools for Mapping Coastal Processes and Water Quality Assessment was convened February 5-7,2007 at The Embassy Suites Hotel, Seaside, California and sponsored by the ACT-Pacific Coast partnership at the Moss Landing Marine Laboratories (MLML). The TUV workshop was co-chaired by Richard Burt (Chelsea Technology Group) and Stewart Lamerdin (MLML Marine Operations). Invited participants were selected to provide a uniform representation of the academic researchers, private sector product developers, and existing and potential data product users from the resource management community to enable development of broad consensus opinions on the application of TUV platforms in coastal resource assessment and management. The workshop was organized to address recognized limitations of point-based monitoring programs, which, while providing valuable data, are incapable of describing the spatial heterogeneity and the extent of features distributed in the bulk solution. This is particularly true as surveys approach the coastal zone where tidal and estuarine influences result in spatially and temporally heterogeneous water masses and entrained biological components. Aerial or satellite based remote sensing can provide an assessment of the aerial extent of plumes and blooms, yet provide no information regarding the third dimension of these features. Towed vehicles offer a cost-effective solution to this problem by providing platforms, which can sample in the horizontal, vertical, and time-based domains. Towed undulating vehicles (henceforth TUVs) represent useful platforms for event-response characterization. This workshop reviewed the current status of towed vehicle technology focusing on limitations of depth, data telemetry, instrument power demands, and ship requirements in an attempt to identify means to incorporate such technology more routinely in monitoring and event-response programs. Specifically, the participants were charged to address the following: (1) Summarize the state of the art in TUV technologies; (2) Identify how TUV platforms are used and how they can assist coastal managers in fulfilling their regulatory and management responsibilities; (3) Identify barriers and challenges to the application of TUV technologies in management and research activities, and (4) Recommend a series of community actions to overcome identified barriers and challenges. A series of plenary presentation were provided to enhance subsequent breakout discussions by the participants. Dave Nelson (University of Rhode Island) provided extensive summaries and real-world assessment of the operational features of a variety of TUV platforms available in the UNOLs scientific fleet. Dr. Burke Hales (Oregon State University) described the modification of TUV to provide a novel sampling platform for high resolution mapping of chemical distributions in near real time. Dr. Sonia Batten (Sir Alister Hardy Foundation for Ocean Sciences) provided an overview on the deployment of specialized towed vehicles equipped with rugged continuous plankton recorders on ships of opportunity to obtain long-term, basin wide surveys of zooplankton community structure, enhancing our understanding of trends in secondary production in the upper ocean. [PDF contains 32 pages]

Wave Sensor Technologies, St Petersburg, Florida, March 7-9, 2007: workshop proceedings

The Alliance for Coastal Technologies (ACT) convened a workshop on "Wave Sensor Technologies" in St. Petersburg, Florida on March 7-9, 2007, hosted by the University of South Florida (USF) College of Marine Science, an ACT partner institution. The primary objectives of this workshop were to: 1) define the present state of wave measurement technologies, 2) identify the major impediments to their advancement, and 3) make strategic recommendations for future development and on the necessary steps to integrate wave measurement sensors into operational coastal ocean observing systems. The participants were from various sectors, including research scientists, technology developers and industry providers, and technology users, such as operational coastal managers and coastal decision makers. Waves consistently are ranked as a critical variable for numerous coastal issues, from maritime transportation to beach erosion to habitat restoration. For the purposes of this workshop, the participants focused on measuring "wind waves" (i.e., waves on the water surface, generated by the wind, restored by gravity and existing between approximately 3 and 30-second periods), although it was recognized that a wide range of both forced and free waves exist on and in the oceans. Also, whereas the workshop put emphasis on the nearshore coastal component of wave measurements, the participants also stressed the importance of open ocean surface waves measurement. Wave sensor technologies that are presently available for both environments include bottom-mounted pressure gauges, surface following buoys, wave staffs, acoustic Doppler current profilers, and shore-based remote sensing radar instruments. One of the recurring themes of workshop discussions was the dichotomous nature of wave data users. The two separate groups, open ocean wave data users and the nearshore/coastal wave data users, have different requirements. Generally, the user requirements increase both in spatial/temporal resolution and precision as one moves closer to shore. Most ocean going mariners are adequately satisfied with measurements of wave period and height and a wave general direction. However, most coastal and nearshore users require at least the first five Fourier parameters ("First 5"): wave energy and the first four directional Fourier coefficients. Furthermore, wave research scientists would like sensors capable of providing measurements beyond the first four Fourier coefficients. It was debated whether or not high precision wave observations in one location can take the place of a less precise measurement at a different location. This could be accomplished by advancing wave models and using wave models to extend data to nearby areas. However, the consensus was that models are no substitution for in situ wave data.[PDF contains 26 pages]

Optical Remote Sensing of Coastal Habitats, Moss Landing, California, 9-11 January, 2006: workshop proceedings

The Alliance for Coastal Technologies (ACT) Workshop on Optical Remote Sensing of Coastal Habitats was convened January 9-11, 2006 at Moss Landing Marine Laboratories in Moss Landing, California, sponsored by the ACT West Coast regional partnership comprised of the Moss Landing Marine Laboratories (MLML) and the Monterey Bay Aquarium Research Institute (MBARI). The "Optical Remote Sensing of Coastal Habitats" (ORS) Workshop completes ACT'S Remote Sensing Technology series by building upon the success of ACT'S West Coast Regional Partner Workshop "Acoustic Remote Sensing Technologies for Coastal Imaging and Resource Assessment" (ACT 04-07). Drs. Paul Bissett of the Florida Environmental Research Institute (FERI) and Scott McClean of Satlantic, Inc. were the ORS workshop co-chairs. Invited participants were selected to provide a uniform representation of the academic researchers, private sector product developers, and existing and potential data product users from the resource management community to enable development of broad consensus opinions on the role of ORS technologies in coastal resource assessment and management. The workshop was organized to examine the current state of multi- and hyper-spectral imaging technologies with the intent to assess the current limits on their routine application for habitat classification and resource monitoring of coastal watersheds, nearshore shallow water environments, and adjacent optically deep waters. Breakout discussions focused on the capabilities, advantages ,and limitations of the different technologies (e.g., spectral & spatial resolution), as well as practical issues related to instrument and platform availability, reliability, hardware, software, and technical skill levels required to exploit the data products generated by these instruments. Specifically, the participants were charged to address the following: (1) Identify the types of ORS data products currently used for coastal resource assessment and how they can assist coastal managers in fulfilling their regulatory and management responsibilities; (2) Identify barriers and challenges to the application of ORS technologies in management and research activities; (3) Recommend a series of community actions to overcome identified barriers and challenges. Plenary presentations by Drs. Curtiss 0. Davis (Oregon State University) and Stephan Lataille (ITRES Research, Ltd.) provided background summaries on the varieties of ORS technologies available, deployment platform options, and tradeoffs for application of ORS data products with specific applications to the assessment of coastal zone water quality and habitat characterization. Dr. Jim Aiken (CASIX) described how multiscale ground-truth measurements were essential for developing robust assessment of modeled biogeochemical interpretations derived from optically based earth observation data sets. While continuing improvements in sensor spectral resolution, signal to noise and dynamic range coupled with sensor-integrated GPS, improved processing algorithms for georectification, and atmospheric correction have made ORS data products invaluable synoptic tools for oceanographic research, their adoption as management tools has lagged. Seth Blitch (Apalachicola National Estuarine Research Reserve) described the obvious needs for, yet substantial challenges hindering the adoption of advanced spectroscopic imaging data products to supplement the current dominance of digital ortho-quad imagery by the resource management community, especially when they impinge on regulatory issues. (pdf contains 32 pages)

Development of appropriateness explicit criteria for cataract extraction by phacoemulsification

Background: Consensus development techniques were used in the late 1980s to create explicit criteria for the appropriateness of cataract extraction. We developed a new appropriateness of indications tool for cataract following the RAND method. We tested the validity of our panel results. Methods: Criteria were developed using a modified Delphi panel judgment process. A panel of 12 ophthalmologists was assembled. Ratings were analyzed regarding the level of agreement among panelists. We studied the influence of all variables on the final panel score using linear and logistic regression models. The explicit criteria developed were summarized by classification and regression tree analysis. Results: Of the 765 indications evaluated by the main panel in the second round, 32.9% were found appropriate, 30.1% uncertain, and 37% inappropriate. Agreement was found in 53% of the indications and disagreement in 0.9%. Seven variables were considered to create the indications and divided into three groups: simple cataract, with diabetic retinopathy, or with other ocular pathologies. The preoperative visual acuity in the cataractous eye and visual function were the variables that best explained the panel scoring. The panel results were synthesized and presented in three decision trees. Misclassification error in the decision trees, as compared with the panel original criteria, was 5.3%. Conclusion: The parameters tested showed acceptable validity for an evaluation tool. These results support the use of this indication algorithm as a screening tool for assessing the appropriateness of cataract extraction in field studies and for the development of practice guidelines.

Failure detectors and communication efficiency in the crash and general omisión failure models

122 p.

Bases para una correcta ordenación del espacio periurbano: proyecto Sudoe

[ES] El programa de cooperación transnacional del Suroeste Europeo aporta la oportunidad de contrastar la situación que atraviesan los espacios periurbanos de cuatro áreas metropolitanas y plantear toda una serie de recomendaciones para su correcta ordenación. Tras varias décadas de crecimiento urbano intenso se observa la dificultad que tienen los documentos de ordenación para regularlo y dirigirlo en los límites de la ciudad. Tan sólo las figuras de protección de espacios naturales aportan ciertas garantías de preservar determinados entornos. Las propuestas se concretan en la necesidad de resaltar el valor natural, de ocio y agrario del medio rural próximo. También se incide en la necesidad de acometer la ordenación de este espacio utilizando una metodología diferente, la mirada invertida, con el objeto de conseguir mayor consenso y respeto a las propuestas de todas las partes.

Problems and prospects of community-based resource management approach: an experience with the implementation of the ban on beach seines on Lake Kainji

Community Based Resource Management (CBRM) understood as an approach emphasizes a community's capability, responsibility and accountability with regards to managing resources. Based on the recommendations for the Nigerian-German Kainji Lake Fisheries Promotion Project (KLFPP), the Niger and Kebbi States Fisheries Edicts were promulgated in 1997. These edicts, among other things, banned the use of beach seines. Given the conviction of KLFPP, that if communities whose livelihood is linked to the fishery, understand and identify the problems and by consensus agree to the solutions of fisheries problems, they are more likely to adhere to any control measures, specifically the ban on beach seine. In 1999 a first agreement was reached between beach seiners, non-beach seiners and government authorities leading to an almost complete elimination of beach seine on the Lake Kainji. However, despite on going efforts of the Kainji Lake Fisheries Management and Conservation Unit in 2000 and possibly because of certain oversights during and after the first agreement, in May 2001 a significant number of beach seiners was observed. This led to a re-assessment of our approach, which lately culminated into another round of negotiation. The paper presents the latest results on this on-going process

TrustedPals plataformarako middlewarearen inplementazioa eta simulazioa

Sistema banatuak zenbait konputagailu edo gailu autonomoaz osaturiko sareak dira, non algoritmo banatuen bidez partaide guztien lana koordinatzen da entitate bakarra izatearen irudia emanez. Eredu honi esker sistemaren sendotasuna handitzen da, posible baita sistemak aurrera jarraitzea zenbait partaidek huts egin arren. Sistema banatuak diseinatzeak badu zenbait zailtasun, prozesu guztien arteko koordinazioa lortu behar baita. Erronka nagusietako bat adostasuna edo consensus lortzea da; hau da, prozesu guztiak ados jartzea zerbait erabaki behar dutenean. Ingurune desberdinetan planteatu badaiteke ere, lan honetan Byzantine ingurunean egingo da. Ingurune honetan partaideen hutsegiteak ausaz gerta daitezke eta edozein momentutan. Horrez gain, hutsegite horiek edozein motakoak izan daitezke, hala nola, prozesu bat bertan behera geratzea edota prozesu baten eskaera okerra edo lekuz kanpokoa egitea. Aurkeztutako consensus arazoa garrantzi handikoa da sistema banatuen arloan, honen bitartez beste hainbat helburu lortu baitaitezke. Horien artean Secure Multy-party Computation (SMC) dugu, non sare banatu bateko partaide guztiek adostasuna lotu behar dute partaide bakoitzaren informazioa gainontzekoei ezkutatuz. Horren adibide bezala “aberatsaren arazoa” azaldu ohi da, non partaide guztiek aurkitu behar dute zein den beraien artean aberatsena, partaide bakoitzak gainontzekoen “aberastasuna” ezagutu ahal izan gabe. SMC erabili daiteke soluzioa emateko planteamendu bera jarraitzen duten aplikazio erreal askori, hala nola, enkante pribatuak edo bozketak. SMC inplementatu ahal izateko TrustedPals izeneko plataforma dugu, non diseinu modularra jarraituz smartcard bat eta algoritmo banatuak konbinatzen dira lehenengo consensus eta ondoren SMC lortzeko. Karrera amaierako proiektu honen helburua TrustedPals proposamenaren alde praktikoa jorratzea izango da. Horretarako proposamenaren algoritmo banatuak inplementatu eta simulatuko dira zenbait probetako kasuetan. Simulazioak bideratzeko gertaera diskretuko NS-3 simulagailuan erabiliko da. Simulazio eszenario desberdinak inplementatuko dira eta ondoren emaitzak aztertuko dira.

Engineering thermostable fungal cellobiohydrolases

Meeting the world's growing energy demands while protecting our fragile environment is a challenging issue. Second generation biofuels are liquid fuels like long-chain alcohols produced from lignocellulosic biomass. To reduce the cost of biofuel production, we engineered fungal family 6 cellobiohydrolases (Cel6A) for enhanced thermostability using random mutagenesis and recombination of beneficial mutations. During long-time hydrolysis, engineered thermostable cellulases hydrolyze more sugars than wild-type Cel6A as single enzymes and binary mixtures at their respective optimum temperatures. Engineered thermostable cellulases exhibit synergy in binary mixtures similar to wild-type cellulases, demonstrating the utility of engineering individual cellulases to produce novel thermostable mixtures. Crystal structures of the engineered thermostable cellulases indicate that the stabilization comes from improved hydrophobic interactions and restricted loop conformations by proline substitutions. At high temperature, free cysteines contribute to irreversible thermal inactivation in engineered thermostable Cel6A and wild-type Cel6A. The mechanism of thermal inactivation in this cellulase family is consistent with disulfide bond degradation and thiol-disulfide exchange. Enhancing the thermostability of Cel6A also increases tolerance to pretreatment chemicals, demonstrated by the strong correlation between thermostability and tolerance to 1-ethyl-3-methylimidazolium acetate. Several semi-rational protein engineering approaches – on the basis of consensus sequence analysis, proline stabilization, FoldX energy calculation, and high B-factors – were evaluated to further enhance the thermostability of Cel6A.

Improving the chances for developing coastal country success in adapting to climate change

There is an unequivocal scientific consensus that increases in greenhouse gases in the atmosphere drive warming temperatures of air and sea, and acidification of the world’s oceans from carbon dioxide absorbed by the oceans. These changes in turn can induce shifts in precipitation patterns, sea level rise, and more frequent and severe extreme weather events (e.g. storms and sea surge). All of these impacts are already being witnessed in the world’s coastal regions and are projected to intensify in years to come. Taken together, these impacts are likely to result in significant alteration of natural habitats and coastal ecosystems, and increased coastal hazards in low-lying areas. They can affect fishers, coastal communities and resource users, recreation and tourism, and coastal infrastructure. Approaches to planned adaptation to these impacts can be drawn from the lessons and good practices from global experience in Integrated Coastal Management (ICM). The recently published USAID Guidebook on Adapting to Coastal Climate Change (USAID 2009) is directed at practitioners, development planners, and coastal management professionals in developing countries. It offers approaches for assessing vulnerability to climate change and climate variability in communities and outlines how to develop and implement adaptation measures at the local and national levels. Six best practices for coastal adaptation are featured in the USAID Guidebook on Adapting to Coastal Climate Change and summarized in the following sections. (PDF contains 3 pages)

Plate tectonic constraints on flat subduction and paleomagnetic constraints on rifting

Plate tectonics shapes our dynamic planet through the creation and destruction of lithosphere. This work focuses on increasing our understanding of the processes at convergent and divergent boundaries through geologic and geophysical observations at modern plate boundaries. Recent work had shown that the subducting slab in central Mexico is most likely the flattest on Earth, yet there was no consensus about what caused it to originate. The first chapter of this thesis sets out to systematically test all previously proposed mechanisms for slab flattening on the Mexican case. What we have discovered is that there is only one model for which we can find no contradictory evidence. The lack of applicability of the standard mechanisms used to explain flat subduction in the Mexican example led us to question their applications globally. The second chapter expands the search for a cause of flat subduction, in both space and time. We focus on the historical record of flat slabs in South America and look for a correlation between the shallowing and steepening of slab segments with relation to the inferred thickness of the subducting oceanic crust. Using plate reconstructions and the assumption that a crustal anomaly formed on a spreading ridge will produce two conjugate features, we recreate the history of subduction along the South American margin and find that there is no correlation between the subduction of a bathymetric highs and shallow subduction. These studies have proven that a subducting crustal anomaly is neither a sufficient or necessary condition of flat slab subduction. The final chapter in this thesis looks at the divergent plate boundary in the Gulf of California. Through geologic reconnaissance mapping and an intensive paleomagnetic sampling campaign, we try to constrain the location and orientation of a widespread volcanic marker unit, the Tuff of San Felipe. Although the resolution of the applied magnetic susceptibility technique proved inadequate to contain the direction of the pyroclastic flow with high precision, we have been able to detect the tectonic rotation of coherent blocks as well as rotation within blocks.

Isolation and characterization of proteins that bind to yeast origins of DNA replication

Yeast chromosomes contain sequences called ARSs which function as origins of replication in vitro and in vivo. We have carried out a systematic deletion analysis of ARS1, allowing us to define three functionally distinct domains, designated A, B, and C. Domain A is a sequence of 11 to 19bp, containing the core consensus element that is required for replication. The core consensus sequence, A/TTTTATPuTTTA/T, is conserved at all ARSs sequenced to date. A fragment containing only element A and 8 flanking nucleotides enables autonomous replication of centromeric plasmids. These plasmids replicate very inefficiently, suggesting that flanking sequences must be important for ARS function. Domain B also provides important sequences needed for efficient replication. Deletion of domain B drastically increases the doubling times of transformants and reduces plasmid stability. Domain B contains a potential consensus sequence conserved at some ARSs which overlaps a region of bent DNA. Mutational analysis suggests this bent DNA may be important for ARS function. Deletion of domain C has only a slight effect on replication of plasmids carrying those deletions.

We have identified a protein called ARS binding factor I (ABF-I) that binds to the HMR-E ARS and ARS1. We have purified this protein to homogeneity using conventional and oligonucleotide affinity chromatography. The protein has an apparent molecular weight of 135kDa and is present at about 700 molecules per diploid cell, based on the yield of purified protein and in situ antibody staining. DNaseI footprinting reveals that ABF-I binds sequence-specifically to an approximately 24bp sequence that overlaps element Bat ARSl. This same protein binds to and protects a similar size region at the HMR-E ARS.

We also find evidence for another ARS binding protein, ABF-III, based on DN asei footprint analysis and gel retardation assays. The protein protects approximately 22bp adjacent to the ABF-I site. There appears to be no interaction between ABF-I and ABF-III despite the proximity of their binding sites.

To address the function of ABF-I in DNA replication, we have cloned the ABF-I gene using rabbit polyclonal anti-sera and murine monoclonal antibodies against ABF-I to screen a λgt11 expression library. Four EcoRI restriction fragments were isolated which encoded proteins that were recognized by both polyclonal and monoclonal antibodies. A gene disruption can now be constructed to determine the in vivo function of ABF-I.

Warsaw Conference: “Small steps forward while awaiting major decisions at the 2015 Paris Conference”

6 p.