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Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) represent a disease continuum with common genetic causes and molecular pathology. We recently identified mutations in the T-cell restricted intracellular antigen-1 (TIA1) protein as a cause of ALS +/− FTD. TIA1 is an RNA-binding protein containing a low complexity domain (LCD) that promotes the assembly of membrane-less organelles, such as stress granules (SG). Whole exome sequencing of two family members with fALS/FTD revealed a novel missense mutation in the TIA1 LCD (P362L). Subsequent screening identified five more TIA1 mutations in six additional ALS patients, but none in controls. All mutation carriers presented with weakness, behavioral abnormalities or language impairments and had a final diagnosis of ALS +/− FTD. Autopsy on five TIA1 mutation carriers showed widespread neurodegeneration with TDP-43 pathology. Round eosinophilic inclusions in lower motor neurons were a consistent feature. Cellular assays revealed abnormal SG dynamics in the presence of TIA1 mutations. In summary, missense mutations in the LCD of TIA1 are a newly recognized cause of ALS/FTD with TDP-43 pathology and strengthen the role of RNA metabolism in the pathogenesis in this disease.
Introduction: Situational awareness (SA) is essential for maintenance of scene safety and effective resource allocation in mass casualty incidents (MCI). Unmanned aerial vehicles (UAV) can potentially enhance SA with real-time visual feedback during chaotic and evolving or inaccessible events. The purpose of this study was to test the ability of paramedics to use UAV video from a simulated MCI to identify scene hazards, initiate patient triage, and designate key operational locations. Methods: A simulated MCI, including fifteen patients of varying acuity (blast type injuries), plus four hazards, was created on a college campus. The scene was surveyed by UAV capturing video of all patients, hazards, surrounding buildings and streets. Attendees of a provincial paramedic meeting were invited to participate. Participants received a lecture on SALT Triage and the principles of MCI scene management. Next, they watched the UAV video footage. Participants were directed to sort patients according to SALT Triage step one, identify injuries, and localize the patients within the campus. Additionally, they were asked to select a start point for SALT Triage step two, identify and locate hazards, and designate locations for an Incident Command Post, Treatment Area, Transport Area and Access/Egress routes. Summary statistics were performed and a linear regression model was used to assess relationships between demographic variables and both patient triage and localization. Results: Ninety-six individuals participated. Mean age was 35 years (SD 11), 46% (44) were female, and 49% (47) were Primary Care Paramedics. Most participants (80 (84%)) correctly sorted at least 12 of 15 patients. Increased age was associated with decreased triage accuracy [-0.04(-0.07,-0.01);p=0.031]. Fifty-two (54%) were able to localize 12 or more of the 15 patients to a 27x 20m grid area. Advanced paramedic certification, and local residency were associated with improved patient localization [2.47(0.23,4.72);p=0.031], [-3.36(-5.61,-1.1);p=0.004]. The majority of participants (78 (81%)) chose an acceptable location to start SALT triage step two and 84% (80) identified at least three of four hazards. Approximately half (53 (55%)) of participants designated four or more of five key operational areas in appropriate locations. Conclusion: This study demonstrates the potential of UAV technology to remotely provide emergency responders with SA in a MCI. Additional research is required to further investigate optimal strategies to deploy UAVs in this context.
Although Saturn's broad A, B, and C rings epitomize the concept of “planetary rings” in the minds of most people, much of our detailed knowledge of ring dynamics has come from the investigation of smaller-scale features such as density and bending waves, the numerous narrow gaps and their embedded ringlets, and the sharp edges which often demarcate various ring regions. In the case of Uranus, almost all of the ring mass is in the form of narrow rings. Narrow ringlets and gaps, and their associated sharp edges (including those of broad rings) form the subject of this chapter, along with the dynamical theories their study has spawned and the puzzles that continue to surround them.
Examples of several narrow gaps and ringlets in Saturn's rings, as well as the very prominent sharp outer edge of the B ring, are shown in Figure 11.1, from French et al. (2016b). Here one can see a total of eight narrow gaps in the region known as the Cassini Division, ranging in width from 5 km to 360 km, as well as four narrow ringlets. At least six more narrow gaps are found in the outer A ring and in the C ring, while three additional narrow ringlets occur in the C ring. The present chapter will cover all of these features, as well as the ten narrow Uranian rings. Recently a pair of narrow, dense rings has been discovered around the centaur object, Chariklo (Braga-Ribas et al., 2014). These are discussed separately in Chapter 7. We also do not discuss the more tenuous and dusty Jovian and Neptunian ring systems, nor the dusty ringlets found at Saturn and Uranus, all of which are covered in Chapter 12. The complex and unique F ring is described in Chapter 13.
We begin with a short overview of the relevant observations and their limitations in Section 11.2, before reviewing the kinematics, systematic width variations and internal structure of narrow ringlets in Section 11.3. In Section 11.4 we discuss the gaps in Saturn's rings, including searches for any embedded satellites. Section 11.5 deals with individual ringlet and gap edges, especially those that are controlled by resonances with external satellites and those that show evidence for local perturbations by unseen, embedded objects.
At the post-closure stage of a geological disposal facility for higher activity radioactive waste several species of gas are likely to be generated in the near-field environment. These could alter the sealing and chemical properties of the bentonite buffer and the local geochemical environment significantly. The authors' attempt to simulate multicomponent gas flow through variably saturated porous media is presented. Governing equations have been developed for a reactive gas-flow model to simulate the thermo-hydro-gas-chemical-mechanical behaviour, with specific reference to the performance of highly compacted bentonite buffer subjected to repository gas generation and migration. The developed equations have been included in the bespoke numerical model COMPASS and some generic simulations are also presented. The model presented extends current capability to assess buffer performance.
The U.S. Nuclear Regulatory Commission has the mission to protect people and the environment. To support this important mission, the U.S. NRC staff assesses techniques and methods to characterize, model, monitor, and remediate radionuclide releases and their migration through the subsurface. Insights from ongoing reviews of field investigations by NRC staff involving radionuclide transport in the subsurface illustrate the need to test and confirm conceptual site models (CSM). The assumptions and parameterization inherent to these CSMs which affect radionuclide release and transport should be tested. In particular, the unsaturated zone where many leaks and spills originate needs detailed characterization and confirmatory monitoring. A dose assessment to determine risk-informed compliance with regulatory criteria is used to evaluate the need for and selection of remediation methods. If remediation is warranted, the choice of remediation method(s) is based upon site- and source-characterization, modeling and monitoring data. These data should be used to the test the CSM. Remediation options range from highly-aggressive methods such as pump, treat, monitor, and recycle or release; to more passive methods such as monitored natural attenuation. All successful remediation strategies involve monitoring programs to determine their efficacy. This monitoring is coupled to performance assessment models using performance indicators (PIs). These PIs provide a measurable indication of remediation performance and are derived from analysis of the CSM and monitoring data.
Although pneumonia is a leading cause of death from infectious disease worldwide, comprehensive information about its causes and incidence in low- and middle-income countries is lacking. Active surveillance of hospitalized patients with pneumonia is ongoing in Thailand. Consenting patients are tested for seven bacterial and 14 viral respiratory pathogens by PCR and viral culture on nasopharyngeal swab specimens, serology on acute/convalescent sera, sputum smears and antigen detection tests on urine. Between September 2003 and December 2005, there were 1730 episodes of radiographically confirmed pneumonia (34·6% in children aged <5 years); 66 patients (3·8%) died. A recognized pathogen was identified in 42·5% of episodes. Respiratory syncytial virus (RSV) infection was associated with 16·7% of all pneumonias, 41·2% in children. The viral pathogen with the highest incidence in children aged <5 years was RSV (417·1/100 000 per year) and in persons aged ⩾50 years, influenza virus A (38·8/100 000 per year). These data can help guide health policy towards effective prevention strategies.
The antibody responses of 194 volunteers were studied for up to 3 years after primary immunization with one, two or three doses of human diploid cell rabies vaccine, administered either in 0·1 ml volumes intradermally (i.d.) or as 1·0 ml intramuscularly (i.m.). Sero-conversion occurred in 95% of subjects after the first injection and in 100% after the second. The highest titres and most durable antibody responses were induced by three injections of vaccine.
Booster doses were administered either by the subcutaneous (s.c.) or i.d. route, after 6, 12 or 24 months to randomly grouped volunteers; these induced responses ≥ 5·0 i.u. per ml in 95% of subjects. The responses were rapid and were neither influenced by the primary regimen nor by the timing and route of the booster dose.
Antibody titres after i.d. immunization were only two-fold lower than those induced by the larger volume of vaccine. The findings suggest that the i.d. route is both effective and economic.
Occupants of 482 long-stay and 33 short-stay beds in 11 Leicester City Council homes for the elderly were studied during a 30-week period from September 1988 to March 1989 to determine the incidence, aetiology, morbidity, and mortality of acute upper respiratory tract viral infections and the use of influenza vaccine.
Influenza immunization rates by home ranged from 15·4 to 90% (mean 45%). There were no differences in the distribution of medical conditions by home. The highest immunization rates were seen in people with chest disease (77%), heart disease (60%), diabetes (56%), and those with three medical conditions (75%). There was an average of 0·7 upper respiratory episodes per bed per annum with a mortality of 3·4% (6/179). Half of all episodes were seen by a general medical practitioner and 81 of 90 (90%) referrals were prescribed antibiotics costing approximately £7.50 per patient. Lower respiratory tract complications developed during 45 (25%) of 179 episodes including 3 of 12 coronavirus infections, 3 of 9 respiratory syncytial virus infections, 2 of 4 adenovirus infections, 1 of 11 rhinovirus infections, but none of 5 influenza infections. Respiratory infections were caused mostly by pathogens other than influenza virus during the influenza period documented nationally. This highlights the role of coronaviruses, respiratory syncytial virus, and unidentified agents in the elderly, and questions the assumptions made in American estimates on the impact of influenza and the value of influenza vaccines.
Groups of student volunteers were immunized with one of five different inactivated influenza virus vaccines. The concentration of virus in the various vaccines differed by both the international unitage test and by the concentration of haemagglutinin, as measured by the single radial diffusion test; the results of the two methods of standardization showed no correlation. The serum HI response to immunization was variable; volunteers given A/England/72 showed a 16·6-fold increase in homologous serum antibody titre whilst volunteers given A/Hong Kong/68 vaccine showed a 4·2-fold increase. The variable response of volunteers to immunization could not be explained by the varied concentration of virus in the vaccines, as measured by either test, the titres of serum HI antibody present before immunization, or a combination of these two factors.
The ability to infect volunteers with WRL 105 virus 4 weeks after immunization with heterologous, inactivated virus vaccine was directly related to the degree of cross-reactivity between the haemagglutinins of this vaccine virus and WRL 105 virus. Thus, the greatest number of infections by the challenge virus were seen in volunteers given A/Hong Kong/68 vaccine, less were observed in volunteers given A/England/72 vaccine, and least were found in groups given A/Port Chalmers/73 or A/Scotland/74 vaccine. However, compared with the incidence of infection in volunteers given B/Hong Kong/73 vaccine, all the heterologous influenza A vaccine gave some immunity to challenge infection.
In Canada, wetlands are defined as “… land that is saturated with water long enough to promote wetland or aquatic processes as indicated by poorly drained soils, hydrophytic vegetation, and various kinds of biological activity which are adapted to a wet environment.” (National Wetlands Working Group 1988). The environmental processes that control wetland development form hydrological, chemical, and biotic gradients and commonly have strong cross-correlations. These interrelated gradients have been divided into five nodes that define Canada's wetland classes, of which three classes are non-peat-forming wetlands generally having <40 cm of accumulated organics and two classes are peatlands with >40 cm of accumulated organics. Non-peat-forming wetlands are subdivided into: (1) shallow open waters, (2) marshes, or (3) swamps; whereas peatlands can be subdivided into: (1) fens or (2) bogs (Fig. 6.1).
Non-peat-forming wetlands have a poorly developed bryophyte layer, which results from strong seasonal water-level fluctuations and high vascular plant production (Campbell et al. 2000). Peat accumulation is limited however, as decomposition rates are high. This situation is in contrast to the swamps and marshes found in more-temperate regions of the globe where peat accumulation can occur.
Peatlands differ from non-peat-forming wetlands by a combination of interrelated hydrological, chemical, and biotic factors that results in a decrease in decomposition relative to plant production and therefore allows for the accumulation of peat. The stabilization of seasonal water levels and restriction of water flow through a wetland allows the establishment and development of a bryophyte layer.
The relationships between different levels of severity of ambulatory cerebral palsy, defined by the Gross Motor Function Classification System (GMFCS), and several pediatric outcome instruments were examined. Data from the Gross Motor Function Measure (GMFM), Pediatric Orthopaedic Data Collection Instrument (PODCI), temporal–spatial gait parameters, and oxygen cost were collected from six sites. The sample size for each assessment tool ranged from 226 to 1047 participants. There were significant differences among GMFCS levels I, II, and III for many of the outcome tools assessed in this study. Strong correlations were seen between GMFCS level and each of the GMFM sections D and E scores, the PODCI measures of Transfer and Mobility, and Sports and Physical Function, Gait Velocity, and Oxygen Cost. Correlations among tools demonstrated that the GMFM sections D and E scores correlated with the largest number of other tools. Logistic regression showed GMFM section E score to be a significant predictor of GMFCS level. GMFM section E score can be used to predict GMFCS level relatively accurately (76.6%). Study data indicate that the assessed outcome tools can distinguish between children with different GMFCS levels. This study establishes justification for using the GMFCS as a classification system in clinical studies.
Geopolymers are made by adding aluminosilicates to concentrated alkali solutions for dissolution and subsequent polymerization to form a solid. They are amorphous to semicrystalline three dimensional aluminosilicate networks. Although they have been used in several applications their widespread use is restricted due to lack of long term durability studies and detailed scientific understanding. Three important tools for the study of geopolymers are transmission electron microscopy (TEM), solid state magic angle spinning nuclear magnetic resonance (MAS NMR) and infra red (IR) spectroscopy.
Cs and Sr are two of the most difficult radionuclides to immobilize and are therefore suitable elements to study in assessing geopolymers as matrices for immobilization of radioactive wastes. In this study Cs or Sr was added to geopolymer samples prepared using fly ash precursors. A commercial metakaolinite geopolymer was studied for comparison.
The geopolymers were mainly amorphous as shown by TEM, whether they were made from fly ash or metakaolinite. In the fly ash geopolymer, Cs preferentially inhabited the amorphous phase over the minor crystalline phases, whereas Sr was shared in both. The MAS NMR showed that Cs is held mostly in the geopolymer structure for both fly ash and metakaolinite geopolymers. The IR spectra showed a slight shift in antisymmetric Si-O-Al stretch band to a lower wavenumber for the fly ash geopolymer, which implies that more Al is incorporated in this geopolymer structure than in the metakaolinite geopolymer.
Over a 33-year period, 260 patients (<17 years of age; 119 males, 141 females) from New South Wales, Australia who had peripheral neuropathies confirmed by nerve biopsy, were studied. Of these, 50 infants presented with symptoms or signs of neuropathy under 1 year of age: including 24 patients with demyelinating neuropathies and 21 axonal neuropathies; a further five patients had spinal muscular atrophy with associated secondary sensory axonopathy. Nineteen infants had hereditary motor sensory neuropathy, of whom 13 had myelin protein mutations confirmed by molecular genetic studies. Peripheral neuropathy is not an unusual diagnosis in infancy. Awareness of this association will aid early diagnosis and prognosis as well as facilitate interventional patient management.