Cornelia de Lange syndrome (CdLS): prenatal and autopsy findings

Cornelia de Lange Syndrome (CdLS) is a multisystem disorder characterized by somatic defects and mental retardation. Prenatal diagnosis of this severe condition is difficult in view of the non-specific ultrasound abnormalities. We report three cases with prenatally suspected CdLS based on the ultrasound findings as well as low PAPP-A detected on first trimester screening in one case, and the results of the autopsy and the NIPBL gene mutation analysis. Copyright © 2009 John Wiley & Sons, Ltd.     

Indigenous Studies Speaks to Environmental Management

This article describes the increasing connections between the fields of Indigenous studies and environmental management and examines some of the ways that an Indigenous studies perspective can guide thinking about environmental management. Indigenous groups have been involved in the management of environmental and natural resources on their lands since time immemorial. Indigenous groups have also become increasingly involved in Western practices of environmental management with the advent of co-management institutions, subsistence boards, traditional ecological knowledge forums, and environmental issues affecting Indigenous resources. Thus, it is an important time for scholarship that explores how Indigenous groups are both shaping and being affected by processes of environmental management. This article summarizes key findings and themes from eight papers situated at the intersection of these two fields of study and identify means by which environmental managers can better accommodate Indigenous rights and perspectives. It is the authors’ hope that increased dialog between Indigenous studies and environmental management can contribute to the building of sustainable and socially just environmental management practices.     

Perceptions of Species Abundance,Distribution, and Diversity: Lessons from Four Decades of Sampling on a Government-Managed Reserve

/ We examined data relative to species abundance, distribution, anddiversity patterns of reptiles and amphibians to determine how perceptionschange over time and with level of sampling effort. Location data werecompiled on more than one million individual captures or observations of 98species during a 44-year study period on the US Department of Energy's(DOE) Savannah River Site National Environmental Research Park (SRS-NERP) inSouth Carolina. We suggest that perceptions of herpetofaunal speciesdiversity are strongly dependent on level of effort and that land managementdecisions based on short-term data bases for some faunal groups could resultin serious errors in environmental management. We provide evidence thatacquiring information on biodiversity distribution patterns is compatiblewith multiyear spatially extensive research programs and also provide aperspective of what might be achieved if long-term, coordinated researchefforts were instituted nationwide.To conduct biotic surveys on government-managed lands, we recommend revisionsin the methods used by government agencies to acquire and report biodiversitydata. We suggest that government and industry employees engaged inbiodiversity survey efforts develop proficiency in field identification forone or more major taxonomic groups and be encouraged to measure the status ofpopulations quantitatively with consistent and reliable methodologies. Wealso suggest that widespread academic cooperation in the dissemination ofinformation on regional patterns of biodiversity could result byestablishment of a peer-reviewed, scientifically rigorous journal concernedwith status and trends of the biota of the United States. KEY WORDS: Abundance; Amphibian; Biodiversity; Distribution; Landmanagement; Reptile     

Effects of ultraviolet light (315 nm), temperature and relative humidity on the degradation of polylactic acid plastic films

The influence of temperature (30, 45 and 60 degrees C) and relative humidity (RH) (30%, 50% and 100%) on the degradation of poly(l-lactic acid) (PLA) films were studied. In addition, the effects of ultraviolet (UV) light (315 nm) on the degradation of PLA films were also analyzed. Various analytical techniques were applied to observe changes in the properties of PLA polymer films. FTIR spectroscopy was used as semi-quantitative method to get information about the chemistry of the degradative process. The degradation rate of PLA was enhanced by increasing temperature and RH, factors responsible for a faster reduction of the weight-average molecular weight (M(W)), of the glass transition temperature (Tg) and of the percentage of elongation at break. Moreover, UV treatment accelerated these phenomena.     

Preference for male traits in female wolf spiders varies with the choice of available males,female age and reproductive state

Individual variation in female preference for male traits may influence mate choice, especially if benefits and costs of choosiness vary with the range of available males or reproductive timing. We examined variation in female preference for male leg tuft size in Schizocosa ocreata (Hentz) wolf spiders with video playback. Dichotomous (simultaneous) choice test experiments included all possible combinations of four stimuli (i.e., modified versions of the same video male stimulus): average tuft size (control), reduced (−25%), enlarged (+25%), and no tufts (removed). Females exhibited a directional preference for larger tuft size independent of the nature of the choice (except for reduced tufts vs no tufts where no difference was seen). Female preference in the short term (over a period of 4 days) was also highly repeatable for control vs reduced tufts, but not for control vs enlarged tufts. Responses of females in ‘no-choice’ presentations of a single (control) male stimulus varied with age post-maturity; females were less receptive in weeks 1 and 2, highly receptive at week 3, and less thereafter. Mated females were least receptive and most aggressive towards a male stimulus. Females offered choices repeatedly at different ages post-maturity consistently preferred the control male vs reduced tufts over all 3 weeks but varied in their preference for enlarged tufts vs control male. In the first 2 weeks, females preferred the enlarged tuft male stimulus, but showed no preference by the third week. Females tested in week 4 showed no preference in either choice. Results suggest that the potential interaction between female preference for male traits and female reproductive timing may be a critical consideration in mate choice.     

Missing Mammals on Great Basin Mountains: Holocene Extinctions and Inadequate Knowledge

The discontinuous distribution of boreal mammals across Great Basin mountains has provided a significant body of data for the analysis of biogeographic patterns in isolated populations of small mammals. Such use assumes that species unreported from particular ranges do not, in fact, occur there. Data from the Roberts and Diamond mountains (central Nevada) demonstrate that this assumption is incorrect. The new distributional records from these ranges further decrease the slope of the log species—log area relationship for Great Basin boreal mammals, but increase the nestedness of the montane faunas involved. These data underscore the need for more-thorough assessments of the mammalian faunas of Great Basin mountains, but they also suggest that extinction of boreal mammals on these mountains is even more predictable than has appeared in the past.     

Learning to predict channel stability using biogeomorphic features

Current human land use activities are altering many components of the river landscape, resulting in unstable channels. Instability may have serious negative consequences for water quality, aquatic and riparian habitat, and for river-related human infrastructure such as bridges and roads. Resource management agencies have developed rapid bioassessment surveys to help assess stability in a fast and cost-effective way. While this assessment can be done for a single site fairly rapidly, it is still time-consuming to apply over large watersheds and assessment activities must be prioritized. We constructed a system that employs commonly available map data as inputs to cost-sensitive variants of decision tree algorithms to predict the relative channel stability of different sites. In particular, we use bagged lazy option trees (LOTs) and bagged probability estimation trees (PETs) to identify all unstable channels while making the smallest number of errors of classifying stable channels as unstable, thereby minimizing cost and maximizing safety. We measured the performance of the classifiers using ROC curves and found that the PETs performed better than the LOTs in situations where the number of instances of the stable and unstable classes were relatively balanced, but the LOTs did better where unstable examples were relatively rare compared to stable, perhaps due to the LOTs’ ability to focus on individual examples.     

Deforestation: risk of sex ratio distortion in hawksbill sea turtles.

Phenotypic sex in sea turtles is determined by nest incubation temperatures, with warmer temperatures producing females and cooler temperatures producing males. The common finding of highly skewed female-biased hatchling sex ratios in sea turtle populations could have serious repercussions for the long-term survival of these species and prompted us to examine the thermal profile of a relatively pristine hawksbill nesting beach in Guadeloupe, French West Indies. Data loggers placed at nest depth revealed that temperatures in the forested areas were significantly cooler than temperatures in the more open, deforested areas. Using these temperatures as a predictor of sex ratio, we were able to assess the relative contributions of the different beach zones to the primary sex ratio: significantly more males were likely to be produced in the forested areas. Coastal forests are therefore important male-producing areas for the hawksbill sea turtle, and this has urgent conservation implications. On Guadeloupe, as on many Caribbean islands, deforestation rates are high and show few signs of slowing, as there is continual pressure to develop beachfront areas. The destruction of coastal forest could have serious consequences both in terms of local nesting behavior and of regional demography through the effects on population sex ratios. Human alterations to nesting habitat in other reptile taxa have been shown to modify the thermal properties of nest sites in ways that can disrupt their ecology by allowing parasite transmission, increasing vulnerability to climate change, or rendering existing habitat unsuitable.