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41.
Larsen syndrome is characterized by multiple congenital joint dislocations and flattened facies. Some cases have been familial, with both autosomal dominant and recessive patterns of inheritance. Reports of a form of Larsen syndrome, lethal in the neonatal period, are reviewed. We present a family in which recurrence of the syndrome was diagnosed prenatally, but a lethal outcome again resulted despite preparation for anticipated perinatal complications. Because of the wide clinical variation and the lack of a known metabolic defect, delineation between the various forms of Larsen syndrome is difficult. While the lethal variant appears to be a combination of the Larsen phenotype and pulmonary hypoplasia, other features noted in the lethal cases, such as abnormal palmar creases and laryngotracheomalacia, are also seen in patients with Larsen syndrome who survive.  相似文献   
42.
A study of mercury concentrations in the stomach contents of fish from the north-east Irish Sea and Mersey Estuary has been shown to provide a means for surveillance of geographical and time-based changes in environmental exposure of fish biota to mercury in marine and estuarine ecosystems. This paper describes data for the flatfish dab (Limanda limanda), caught during the period 1986-1988. The low degree of variability in the data enables confirmation of clear trends in mercury concentration in stomach contents over time. As the inputs of mercury to the sewage sludge dumping ground in Liverpool Bay have decreased, there has been a corresponding decrease in mercury in fish food items. The mean mercury value in stomach contents around the dump site has declined to 100 microg kg(-1) (wet weight) which now predominates over the whole of Liverpool Bay. In 1986, mercury concentrations in stomach contents of fish ranged to over 750 microg kg(-1) although the majority of values were below 200 microg kg(-1). Most of the sites within the Mersey Estuary produced mean concentrations which were similar to those in the open sea, except for Garston which is the site closest to an inland, and principal alternative, source of mercury.  相似文献   
43.
Most population viability analyses (PVA) assume that the effects of species interactions are subsumed by population-level parameters. We examine how robust five commonly used PVA models are to violations of this assumption. We develop a stochastic, stage-structured predator-prey model and simulate prey population vital rates and abundance. We then use simulated data to parameterize and estimate risk for three demographic models (static projection matrix, stochastic projection matrix, stochastic vital rate matrix) and two time series models (diffusion approximation [DA], corrupted diffusion approximation [CDA]). Model bias is measured as the absolute deviation between estimated and observed quasi-extinction risk. Our results highlight three generalities about the application of single-species models to multi-species conservation problems. First, our collective model results suggest that most single-species PVA models overestimate extinction risk when species interactions cause periodic variation in abundance. Second, the DA model produces the most (conservatively) biased risk forecasts. Finally, the CDA model is the most robust PVA to population cycles caused by species interactions. CDA models produce virtually unbiased and relatively precise risk estimates even when populations cycle strongly. High performance of simple time series models like the CDA owes to their ability to effectively partition stochastic and deterministic sources of variation in population abundance.  相似文献   
44.
ABSTRACT

Indigenous peoples are among the most affected by environmental injustices globally, however environmental justice theory has not yet meaningfully addressed decolonisation and the resistance of Indigenous communities against extractivism in the settler-colonial context. This paper suggests that informing environmental justice through decolonial analysis and decolonising practices can help transcend the Western ontological roots of environmental justice theories and inform a more radical and emancipatory environmental justice. The Unist’ot’en Resistance and Action Camp blocking pipelines in northwestern British Columbia, Canada, their “Reimagined Free Prior and Informed Consent protocol” and the Delgamuukw case are described to discuss limitations of the state and legal framework for accommodating a decolonial and transformative environmental justice. A decolonial analysis informed by these two moments of Wet’sewet’ten history suggests limits and adaptations to the trivalent EJ framework based on recognition, participation and distribution. It is argued that a decolonising and transformative approach to environmental justice must be based on self-governing authority, relational ontologies of nature and epistemic justice and the unsettling of power through the assertion of responsibility and care through direct action. This discussion is placed in the context of the expansion of the concept of ecological rights, for example through the enshrining of the “Rights of Nature” in the constitutions of countries such as Bolivia and Ecuador, to highlight the Inherent tensions in the translation of Indigenous cosmo-visions into legal systems based on universalist values.  相似文献   
45.
The EC-funded project 'Environmental Risks from Ionising Contaminants: Assessment and Management' (ERICA) developed an 'Integrated Approach' for assessing the impact of ionising radiation on ecosystems. This paper presents the application of the ERICA Integrated Approach, supported by a software programme (the ERICA Tool) and guidance documentation, to an assessment of the Drigg coastal sand dunes (Cumbria, UK). Targeted sampling provided site-specific data for sand dune biota, including amphibians and reptiles. Radionuclides reported included (90)Sr, (99)Tc, (137)Cs, (238)Pu, (239+240)Pu and (241)Am. Site-specific data were compared to predictions derived using the ERICA Tool. Some under- and over-predictions of biota activity concentrations were identified but can be explained by the specific ecological characteristics and contamination mechanism of the dunes. Overall, the results indicated no significant impact of ionising radiation on the sand dune biota and the Integrated Approach was found to be a flexible and effective means of conducting a radiation impact assessment.  相似文献   
46.
Incentivized debt conversion is a financing mechanism that can assist countries with a heavy debt burden to bolster their long-term domestic investment in nature conservation. The Nature Conservancy, an international conservation-based nongovernmental organization, is adapting debt conversions to support marine conservation efforts by small island developing states and coastal countries. Prioritizing debt conversion opportunities according to their potential return on investment can increase the impact and effectiveness of this finance mechanism. We developed guidance on how to do so with a decision-support approach that relies on a novel threat-based adaptation of cost-effectiveness analysis. We constructed scenarios by varying parameters of the approach, including enabling conditions, expected benefits, and threat classifications. Incorporating both abatable and unabatable threats affected priorities across planning scenarios. Similarly, differences in scenario construction resulted in unique solution sets for top priorities. We show how environmental organizations, private entities, and investment banks can adopt structured prioritization frameworks for making decisions about conservation finance investments, such as debt conversions. Our guidance can accommodate a suite of social, ecological, and economic considerations, making the approach broadly applicable to other conservation finance mechanisms or investment strategies that seek to establish a transparent process for return-on-investment decision-making.  相似文献   
47.
Stable carbon isotope analysis of chlorinated aliphatic compounds was performed at an in situ biostimulation pilot test area (PTA) at a site where 1,2-dichloroethane (1,2-DCA) and trichloroethene (TCE) were present in groundwater. Chlorinated products of TCE reductive dechlorination (cis-dichloroethene (cDCE) and vinyl chloride (VC)) were present at concentrations of 17.5 to 126.4 micromol/L. Ethene, a potential degradation product of both 1,2-DCA dihaloelimination and TCE reductive dechlorination was also present in the PTA. Emulsified soybean oil and lactate were added as electron donors to stimulate anaerobic dechlorination in the PTA. Stable carbon isotope analysis provided evidence that dechlorination was occurring in the PTA during biostimulation, and a means of monitoring changes in dechlorination efficiency over the 183 day monitoring period. Stable carbon isotope analysis was also used to determine if ethene production in the PTA was due to dechlorination of TCE, 1,2-DCA, or both. Fractionation factors (alpha) were determined in the laboratory during anaerobic biotransformation of 1,2-DCA via a dihaloelimination reaction in four separate enrichment cultures. These alpha values (as well as the previously published ranges of alpha for the dechlorination of TCE, cDCE and 1,2-DCA) were used, along with isotopic values measured during the pilot test, to derive quantitative estimates of biotransformation during the pilot test. Dechlorination was found to account for 10.7 to 35.9%, 21.9 to 74.9%, and 54.4 to 67.8% of 1,2-DCA, TCE and cDCE concentration loss respectively in the PTA. Stable carbon isotope analysis indicates that dechlorination of 1,2-DCA, TCE and cDCE were all significant processes during the pilot test, while ethene production during the pilot test was dominated by 1,2-DCA dihaloelimination. This study demonstrates how stable carbon isotope analysis can provide more conservative estimates of the extent of biotransformation than do conventional protocols. In addition, in a complex mixed plume such as this, compound specific isotope analysis is shown to be one of the few methods available for clarifying dominant biotransformation pathways where breakdown products are non-exclusive (i.e. ethene).  相似文献   
48.
The knowledge produced by conservation scientists must be actionable in order to address urgent conservation challenges. To understand the process of creating actionable science, we interviewed 71 conservation scientists who had participated in 1 of 3 fellowship programs focused on training scientists to become agents of change. Using a grounded theory approach, we identified 16 activities that these researchers employed to make their scientific products more actionable. Some activities were more common than others and, arguably, more foundational. We organized these activities into 3 nested categories (motivations, strategies, and tactics). Using a co-occurrence matrix, we found that most activities were positively correlated. These correlations allowed us to identify 5 approaches, framed as profiles, to actionable science: the discloser, focused on open access; the educator, focused on science communication; the networker, focused on user needs and building relationships; the collaborator, focused on boundary spanning; and the pluralist, focused on knowledge coproduction resulting in valuable outcomes for all parties. These profiles build on one another in a hierarchy determined by their complexity and level of engagement, their potential to support actionable science, and their proximity to ideal coproduction with knowledge users. Our results provide clear guidance for conservation scientists to generate actionable science to address the global biodiversity conservation challenge.  相似文献   
49.
Despite an abundance of research reaffirming biodiversity's importance to the health of the planet and society, species continue to go extinct at an alarming rate. Why has continued research on the value of biodiversity not had the intended effect and what can be done about it? We considered biodiversity loss as a public value failure and the result of a misalignment between the logic of inquiry (which guides scientists) and the logic of action (which guides practitioners). We drew lessons from our own research to propose the creation of a national biodiversity strategy designed to link the logic of inquiry with the logic of action and coordinate the production of actionable conservation science and informed conservation action.  相似文献   
50.
Birds are commonly sexually promiscuous, which can lead to conflict between the sexes and the evolution of paternity assurance strategies, such as mate guarding. Adaptive explanations for mate guarding have tended to focus on fitness consequences for males, but mate guarding and participation in being guarded is also likely adaptive for females in certain contexts. To better understand the adaptive explanations for mate guarding as well as the observed variation in paternity patterns, it is necessary to explore the relative costs and benefits of guarding (and being guarded) from both the male and female perspective. To investigate these costs and benefits, we conducted an experiment with the Australian zebra finch (Teniopygia guttata) in which we independently varied the perceived opportunity for each member of a captive breeding pair to engage in extra-pair copulation (EPC) solicitation behavior; as an individual’s EPC opportunity increased, the partner’s EPC opportunity remained constant. Our results indicate that, for males, mate guarding intensity increases when their female’s EPC opportunity increases but decreases when their own (i.e., male) EPC opportunity increases. We did not find evidence of flexible female guarding behavior, but we found that females do not evade their partners more as female EPC opportunity increases.  相似文献   
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