122,289 research outputs found
Response to Where in Maine? Letter to the editor describing Cundy\u27s Harbor, n
Response to Where in Maine? Letter to the editor describing Cundy\u27s Harbor, near Linekin Bay in Boothbay Harbor
Skeletal phenotype of mandibuloacral dysplasia associated with mutations in ZMPSTE24
Mandibuloacral dysplasia (MAD) is a rare recessively inherited premature aging disease characterized by skeletal and metabolic anomalies. It is part of the spectrum of diseases called laminopathies and results from mutations in genes regulating the synthesis of the nuclear laminar protein, lamin A. Homozygous or compound heterozygous mutations in the LMNA gene, which encodes both the precursor protein prelamin A and lamin C, are the commonest cause of MAD type A. In a few cases of MAD type B, mutations have been identified in the ZMPSTE24 gene encoding a zinc metalloproteinase important in the post-translational modification of lamin A. Here we describe a new case of MAD resulting from compound heterozygote mutations in ZMPSTE24 (p.N256S/p.Y70fs). The patient had typical skeletal changes of MAD, but in addition a number of unusual skeletal features including neonatal tooth eruption, amorphous calcific deposits, submetaphyseal erosions, vertebral beaking, severe cortical osteoporosis and delayed fracture healing. Treatment with conventional doses of pamidronate improved estimated volumetric bone density in the spine but did not arrest cortical bone loss. We reviewed the literature on cases of MAD associated with proven LMNA and ZMPSTE24 mutations and found that the unusual features described above were all substantially more prevalent in patients with mutations in ZMPSTE24 than in those with LMNA mutations. We conclude that MAD associated with ZMPSTE24 mutations has a more severe phenotype than that associated with LMNA mutations--probably reflecting the greater retention of unprocessed farnesylated prelamin A in the nucleus, which is toxic to cells
Electrokinetic iron pan generation in unconsolidated sediments: implications for contaminated land remediation and soil engineering
Electrokinetic remediation is an emerging technology that has generated considerable interest as a technique for the in situ remediation of clay-rich soils and sediments. Despite promising experimental results, however, at present there is no standardised universal electrokinetic soil/sediment remediation approach. Many of the current technologies are technically complex and energy intensive, and geared towards the removal of 90% or more of specific contaminants, under very specific field or laboratory-based conditions. However, in the real environment a low-tech, low-energy contaminant reduction/containment technique may be more appropriate and realistic. Such a technique, FIRS (Ferric Iron Remediation and Stabilisation), is discussed here. The FIRS technique involves the application of a low magnitude (typically less than 0.2 V/cm) direct electric potential between two or more sacrificial, Fe-rich, electrodes emplaced in, or either side of, a contaminated soil or sediment. The electric potential is used to generate a strong pH (and Eh) gradient within the soil column (pH 2–13), and force the precipitation of an Fe-rich barrier or “pan” in the soil between the electrodes. Geochemical and geotechnical data for FIRS-treated sediments from the Ravenglass estuary, Cumbria, UK indicate that the technique can significantly reduce contaminant concentration in treated soil, by remobilisation of contaminants and concentration on, or around, the Fe-rich barrier. Arsenic, in particular, seems highly amenable to the FIRS treatment, due to its solubility under the high pH conditions generated near to the cathode, and its marked geochemical affinity with the freshly precipitated Fe oxides and oxyhydroxides in the Fe barrier. Geotechnical tests indicate that the Fe barrier produced by the technique is practically impervious (permeability = 10?9 m/s or less), and has moderate mechanical strength (UCS ?11 N/mm2). Notably, a large increase in shear strength in the treated soil near to the anode electrode (due to Fe cementation and/or dewatering) is also observed, without significant loss of porosity. The data indicate that the FIRS technique shows considerable promise as an in situ method for contaminated land remediation and soil water containment, and for improving the mechanical properties of soils (contaminated or otherwise) for civil engineering purposes
Assessing the impact of historical coastal landfill sites on sensitive ecosystems: a case study from Dorset, Southern England
Uncontrolled landfill disposal can cause the release of significant contamination. In Southern England and in other parts of the UK, historical landfills are located along many coastal and estuarine marshes and mudflats. At these sites waste, often significantly contaminated with heavy metals and other contaminants, was dumped with little engineering control and without regard to the surrounding environment. The aim of this study is to investigate the degree to which heavy metals from these historical sites may have contaminated adjacent marshes and mudflats, using the Lodmoor marsh, Dorset, UK as a test site.Surface and sediment core samples were collected from brackish marsh and mudflat areas around the former landfill at Lodmoor, which was operational between 1949 and 1990. Sediment samples were investigated for metallic pollutants, grain size, and mineralogy, and core samples dated via 137Cs and 210Pb. To examine the transfer of heavy metals through the food chain, Phragmites australis leaves were analysed for metallic pollutants. Geochemical data revealed that sediments from the Lodmoor marsh are probably contaminated with Pb. 137Cs dating indicates that concentration maxima for heavy metals correlate to the 1950s and 1960s when landfill activities commenced in Lodmoor. Shallow electromagnetic surveys indicate potential continued leaching from the historic landfill complex.This study indicates the potential for possible landfill-derived contaminants to persist in coastal systems for decades after landfill closure. Over the longer term, it is possible that salinisation and enhanced coastal erosion may cause significant metal release from the landfills and their surrounding sedimentary systems into adjacent ecosystems
Helmets for snow sports: Prevalence, trends, predictors and attitudes to use
Background: Traumatic head injury is the leading cause of mortality and serious morbidity in snow sports and is increasing in incidence. Helmet use in snow sports can reduce the incidence of head injury by up to 60%. Mandatory helmet use is not legislated in most recreational settings for snow sports. This study aimed to quantify the prevalence and trends of helmet use by skiers and snowboarders as well as to investigate predictors of helmet use and reasons why they are worn. Methods: A cross-sectional study including observation of skiers and snowboarders allocated to adult and child groups (5,267 persons), retrospective review of ski patrol accident report forms recorded between 2003 and 2008 (3,984), and completion of specially designed questionnaires by randomly approached snow sport participants (1,029). Results: In 2008, 16% of adults and 67% of children wore helmets. Helmet use increased for adult and child participants between 2003 and 2008. Children, men, and snowboarders were significantly more likely to wear a helmet than their respective counterparts. Significant predictors of helmet use were level of experience, past major crash requiring medical assistance, snow sport lessons, and location of activity. Key reasons for helmet use and disuse were identified. Discussion: Child helmet use far exceeded adult helmet use. Rates of voluntary helmet use are increasing but many remain unprotected from the benefits of a helmet. Identification of the snow sport participants least likely to wear a helmet and their reasons for not doing so should allow targeted helmet promotion and injury prevention strategies to reduce serious head injuries in snow sports.Thomas P. Cundy, Brenton J. Systermans, William J. Cundy, Peter J. Cundy, Nancy E. Briggs, and Jeffrey B. Robinso
A Multi-Language Comparison of Influences on Author Verification using Character N-Grams
We create a new multi-language corpus for author verification based on Wikipedia talkpages, and evaluate the influence that differences in topic and time have on character n-gram author profiles. Topic alignment between two texts is found to increase author verification precision, and an authors writing style is found to change over time, but not more significantly after 3 years than after 1 year.Information ArchitectureWISElectrical Engineering, Mathematics and Computer Scienc
Fixation methods for acute injuries of the ankle syndesmosis: a systematic review protocol
Review question/objective: The objective of this review is to compare the effectiveness of three fixation methods for acute injuries of the ankle syndesmosis - metal screw fixation, bioabsorbable screw fixation and suture button fixation.The reviewers seek to critically evaluate the available evidence to provide an evidence-based appraisal of the comparative safety and efficacy of the treatment strategies.Tom Jan Gieroba, Zachary Munn, Peter J. Cundy, Nathan Eardley-Harri
Appropriate Similarity Measures for Author Cocitation Analysis
We provide a number of new insights into the methodological discussion about author cocitation analysis. We first argue that the use of the Pearson correlation for measuring the similarity between authors’ cocitation profiles is not very satisfactory. We then discuss what kind of similarity measures may be used as an alternative to the Pearson correlation. We consider three similarity measures in particular. One is the well-known cosine. The other two similarity measures have not been used before in the bibliometric literature. Finally, we show by means of an example that our findings have a high practical relevance.information science;Pearson correlation;cosine;similarity measure;author cocitation analysis
Electrokinetic generation of reactive iron-rich barriers in wet sediments: implications for contaminated land management
Here we describe preliminary research into the in situ electrokinetic generation of continuous iron-rich precipitates to act as sub-surface barriers for the containment of contaminated sites. This is achieved using sacrificial iron electrodes emplaced either side of a soil/sediment mass to introduce iron into the system, and their dissolution and re-precipitation under the influence of an applied (DC) electric field. Continuous vertical and horizontal iron-rich bands (up to 2 cm thick) have been generated over a timescale of 300–500 h, at voltages of <5 V with an electrode separation of between 15 and 30 cm. The thickness of the iron-rich band increases as the applied voltage is increased. Geotechnical tests in sand indicate that the iron-rich band produced is practically impervious (coefficient of permeability of 10?9 ms?1 or less), and has significant mechanical strength (unconfined compressive strength of 10.8 N mm?2). By monitoring the current, the integrity of the iron-rich band may be assessed, and by continued application of current, the barrier may ‘self heal’. The iron-rich barrier is composed of amorphous iron, goethite, lepidocrocite, maghemite and native iron
A cryogel-based bioreactor for water treatment applications
The aim of this study was to develop and test a non-diffusion limited, high cell density bioreactor for biodegradation of various phenol derivatives. The bioreactor was obtained using a straightforward one-step preparation method using cryostructuration and direct cross-linking of bacteria into a 3D structured (sponge-like) macroporous cryogel composite material consisting of 11.6% (by mass) cells and 1.2–1.7% polymer, with approximately 87% water (in the material pores). The macroporous cryogel composite material, composed of live bacteria, has pore sizes in the range of 20–150 μm (confirmed by SEM and Laser Scanning Confocal Microscopy). The enzymatic activity of bacteria within the cryogel structure and the effect of freezing on the viability of the cross-linked cells was estimated by MTT assay. Cryogels based on Pseudomonas mendocina, Rhodococcus koreensis and Acinetobacter radioresistens were exploited for the effective bioremediation of phenol and m-cresol, and to a lesser extent 2-chlorophenol and 4-chlorophenol, utilising these phenolic contaminants in water as their only source of carbon. For evaluation of treatment scalability the bioreactors were prepared in plastic “Kaldnes” carriers to improve their mechanical properties and allow application in batch or fluidised bed water treatment modes.</p
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