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Background: Spinal muscular atrophy (SMA) is a children’s neuromuscular disorder. Although motor neuron loss is a major feature of the disease, we have identified fatty acid abnormalities in SMA patients and in preclinical animal models, suggesting metabolic perturbation is also an important component of SMA. Methods: Biochemical, histological, proteomic, and high resolution respirometry were used. Results: SMA patients are more susceptible to dyslipidemia than the average population as determined by a standard lipid profile in a cohort of 72 pediatric patients. As well, we observed a non-alcoholic liver disease phenotype in apreclinical mouse model. Denervation alone was not sufficient to induce liver steatosis, as a mouse model of ALS, did not develop fatty liver. Hyperglucagonemia in Smn2B/-mice could explain the hepatic steatosis by increasing plasma substrate availability via glycogen depletion and peripheral lipolysis. Proteomic analysis identified mitochondrion and lipid metabolism as major clusters. Alterations in mitochondrial function were revealed by high-resolution respirometry. Finally, low-fat diets led to increased survival in Smn2B/-mice. Conclusions: These results provide strong evidence for lipid metabolism defects in SMA. Further investigation will be required to establish the primary mechanism of these alterations and understand how they lead to additional co-morbidities in SMA patients.
Introduction: Efficient multidisciplinary team dynamics are crucial to the provision of optimal ED care. Physicians and nurses must use a collaborative approach to meet patient needs in this busy setting. This is especially important for high-acuity low-occurrence (HALO) procedures and clinical encounters. Simulation provides a safe environment where learning is enhanced through deliberate practice. Multidisciplinary participation in simulation-based education may augment team cohesiveness and performance. Methods: A web-based needs assessment survey was distributed to ED nurses, collecting information on demographics, opinions about simulation-based instruction and perceptions on the value of the proposed collaborative educational approach of the project. Experience and comfort with nursing roles in specific procedures (TV pacer, surgical airway, chest tube, central line, sedation) and clinical encounters (STEMI, CVA, sepsis, anaphylaxis, GI bleed) seen in the ED were also assessed. There were a number of suggestions for topics in addition to those listed. Responses will guide the collaborative development of simulation modules with nursing colleagues on desired topics. Results: 58/97 potential nurse participants from 2 urban ED's responded to the survey over an 8-week period, giving a response rate of 58.8%. 76% of respondents had less than 10 years of ED nursing experience, and 34.48% less than 5 years. Responses indicate limited familiarity with simulation-based education (SBE) on ED scenarios with 33.93% being not familiar; 55.36% somewhat familiar. Most prior simulation experience was with role-playing (82%) or low-fidelity setups (42%). Perceived benefit of SBE sessions was substantial (43.86%- very significant; 45.61%- significant). Most respondents had limited past exposure (22.81%- none; 64.91%- 1-5 sims). Similarly, there was little ongoing participation in SBE events with none in 43.64% and 40% just annually. For the 5 clinical scenarios, average responses were: Comfort with assisting 87.45%; Interest in further training 91.43%; Willingness to participate 94.13%. For the 5 procedures, averages were 36.35% (21.36% excluding sedation), 91.27%, 89.09%, respectively. Conclusion: Results indicate a low level of familiarity, experience and ongoing exposure with SBE relating to ED training and practice. Participants recognize the potential benefits of using simulation in a multidisciplinary educational setting and indicate a willingness to participate in collaborative teaching sessions.
In this study, we report developments towards the application of large zeolite particles, with diameter ca. 40 μm, as tracers in Positron Emission Particle Tracking (PEPT) imaging using 68Ga. The influence of intrapore Na+ and TEA+ (tetraethylammonium) cation concentrations and framework Si/Al ratio on the morphology of mordenite particles has been investigated, advancing understanding of the relationship between these factors. Moreover, the influence of ethanol concentration in the gel during aging on intrapore cation concentration, Si/Al ratio and particle morphology has also been investigated. Additionally, facile ion-exchange between aqueous Ga3+ and intrapore H+ in mordenite has been demonstrated. The influence of pH and gallium speciation on ion-exchange has been investigated to determine favourable conditions for 68Ga3+ uptake by the zeolite.
Children with CHD and acquired heart disease have unique, high-risk physiology. They may have a higher risk of adverse tracheal-intubation-associated events, as compared with children with non-cardiac disease.
Materials and methods
We sought to evaluate the occurrence of adverse tracheal-intubation-associated events in children with cardiac disease compared to children with non-cardiac disease. A retrospective analysis of tracheal intubations from 38 international paediatric ICUs was performed using the National Emergency Airway Registry for Children (NEAR4KIDS) quality improvement registry. The primary outcome was the occurrence of any tracheal-intubation-associated event. Secondary outcomes included the occurrence of severe tracheal-intubation-associated events, multiple intubation attempts, and oxygen desaturation.
A total of 8851 intubations were reported between July, 2012 and March, 2016. Cardiac patients were younger, more likely to have haemodynamic instability, and less likely to have respiratory failure as an indication. The overall frequency of tracheal-intubation-associated events was not different (cardiac: 17% versus non-cardiac: 16%, p=0.13), nor was the rate of severe tracheal-intubation-associated events (cardiac: 7% versus non-cardiac: 6%, p=0.11). Tracheal-intubation-associated cardiac arrest occurred more often in cardiac patients (2.80 versus 1.28%; p<0.001), even after adjusting for patient and provider differences (adjusted odds ratio 1.79; p=0.03). Multiple intubation attempts occurred less often in cardiac patients (p=0.04), and oxygen desaturations occurred more often, even after excluding patients with cyanotic heart disease.
The overall incidence of adverse tracheal-intubation-associated events in cardiac patients was not different from that in non-cardiac patients. However, the presence of a cardiac diagnosis was associated with a higher occurrence of both tracheal-intubation-associated cardiac arrest and oxygen desaturation.
Hemis National Park of the Trans-Himalayas is home to a large population of the snow leopard Panthera uncia and increasing numbers of agro-pastoralists. To persist in this harsh terrain, farmers have to either farm livestock or hunt free-ranging, native ungulates. The availability of more livestock and fewer natural prey created a dynamic whereby snow leopards depredated livestock, followed by retaliatory killing of snow leopards. In 1992, to assist farmers and wildlife, the government enacted a cost-compensation scheme. Following a decade with marginally fewer depredation events, in 2002, two additional strategies were implemented: predator-proof holding pens and the Himalayan Homestay Programme. We assessed 22 years (1992–2013) of depredation data, comparing the periods before and after the additional initiatives. Government records showed that during 1992–2013, 1,624 livestock were depredated from 339 sites, with c. USD 15,000 paid as compensation. There were significantly more kills annually before (a mean of 41) than after (3.5) the initiatives, and mass killings (≥ 5 animals killed per attack) were significantly reduced from 5.5 to 0.5 events per year. Goats and sheep (57%) and horses (13%) comprised the majority of losses. The marked reduction in depredation occurred whilst regulations against hunting were being enforced, probably resulting in an increase in the number of wild prey as alternative food. We conclude that together, cost-compensation, tighter hunting regulations, improved holding pens and the Homestay Programme helped support the well-being of the community while aiding conservation efforts.
To develop sustainable grazing systems, an understanding of the complex interactions between competing plant species and grazing herbivores is needed. An understanding of dietary preference is a prerequisite to predicting how much and from which plant species animals select. This work initially concentrated on dietary preferences of sheep grazing perennial ryegrass and white clover swards and was then extended to other animal species to test the generality of our findings.
HD 207739 = V1914 Cyg, a tidally distorted F8 supergiant plus an obscured hot non-degenerate star, is presently in a state of episodic mass dumping onto an accretion shell around the hot component. Unrelated to the 141 day orbital cycle, the obscuration has a 50 day periodicity during which most of the UV flux diminishes rapidly, then gradually brightens. The more evolved yet less massive F supergiant is thus presumably shedding mass due to its pulsation.
Introduction/Innovation Concept: Rural and remote practice of emergency medicine presents unique challenges, particularly when faced with infrequently encountered cases and procedures. Simulation-based training is a valuable tool in the acquisition and maintenance of knowledge and skills; however, simulators are often located in larger centers and they are not widely outside these centers due to geographic, cost and time constraints. Mobile tele-simulation has the potential to overcome barriers but challenges such as comfort, technical issues and ability to teach desired content via tele-simulation must be addressed. We are developing a mobile-tele-simulation unit (MTU) prototype that will enable emergency medicine practitioners and trainees to access simulation-based instruction in rural and remote settings. Methods: Through application of a mixed-methods approach with input of a multidisciplinary team we are iteratively developing an MTU prototype to assess key factors in design and function, including: technical issues, environmental features, and human factors. The Delphi method is being used to collect input from experts on key design components and feedback is also being collected from trainees after participating in trial deployments of the MTU in different educational and environmental settings. Curriculum, Tool, or Material: The effective application of the MTU in a variety of learning settings will be optimized through ongoing evaluation in the iterative design cycle. Feedback to ensure a quality learning experience in the MTU will direct features of physical design and technical performance that can be applied in deployment of the unit. In addition, challenges to the delivery of module content and instructional modality/ features of lessons to be executed will be important considerations as we move toward developing content that can effectively be taught using the MTU. Conclusion: To ensure effective use of tele-simulation in the delivery of a meaningful simulation experience to rural and remote trainees a number of important challenges must be overcome. We describe our evolving multidisciplinary mixed-methods approach to develop an effective mobile tele-simulation unit.
Toxigenic strains of Vibrio cholerae serogroups O1 and O139 have caused cholera epidemics, but other serogroups – such as O75 or O141 – can also produce cholera toxin and cause severe watery diarrhoea similar to cholera. We describe 31 years of surveillance for toxigenic non-O1, non-O139 infections in the United States and map these infections to the state where the exposure probably originated. While serogroups O75 and O141 are closely related pathogens, they differ in how and where they infect people. Oysters were the main vehicle for O75 infection. The vehicles for O141 infection include oysters, clams, and freshwater in lakes and rivers. The patients infected with serogroup O75 who had food traceback information available ate raw oysters from Florida. Patients infected with O141 ate oysters from Florida and clams from New Jersey, and those who only reported being exposed to freshwater were exposed in Arizona, Michigan, Missouri, and Texas. Improving the safety of oysters, specifically, should help prevent future illnesses from these toxigenic strains and similar pathogenic Vibrio species. Post-harvest processing of raw oysters, such as individual quick freezing, heat-cool pasteurization, and high hydrostatic pressurization, should be considered.
Introduction: In 2006, Alberta implemented an Electronic Health Record called the Alberta Netcare Portal (ANP). The ANP provides provincial read-only access to lab tests, diagnostic imaging, medication information and numerous text reports. There is no computerized order entry, and care is coordinated using a hybrid of paper charting and various electronic systems. Here, we quantify observed ANP use by physician participants providing care in four urban Emergency Departments (EDs) in Alberta. The results form part of a larger mixed methods research project aimed at detecting broader implications of ANP use for patient care. Methods: Between October 2014 and July 2015, ED physicians at four EDs (University of Alberta Hospital [UAH], Grey Nuns Community Hospital [GNCH], Foothills Medical Centre [FMC], Peter Lougheed Centre [PLC]) participated in structured clinical observations. Observations were purposively sampled during the first hours of shifts, when physicians orient themselves to the patients they will see during the rest of their shift, including reviewing available historic patient information. Observers used a tablet based tool to generate a time-stamped record of the information tools used alongside patient care. Information tools included permanent paper records, paper excluding permanent documentation, the ANP, clinical and other applications accessed via desktop computers, and mobile devices. Observers also recorded contextual data, including participant commentary, on paper field notes. Results: Across the 4 sites, 142 physicians participated in 376 sessions for a total of 566 observed physician-hours. Participants accessed information in different computerized applications and on paper (i.e., a ‘hybrid’ care environment). The highest proportion of observed physician time interacting with ANP was observed at the UAH (7.0%-8.1%, all values 95% Confidence Intervals). Physicians spent less time using ANP at GNCH (4.1%-4.8%), which was similar to the Calgary EDs (FMC: 4.4-5.3% and PLC: 5.2%-5.9%). Thematic analysis of field notes showed that ANP acceptance was very high. Patient safety concerns were recorded related to care provided alongside ‘hybrid’ patient records. Conclusion: We found high physician acceptance of ANP based on documented comments and observed usage. We posit a high potential for EHRs such as ANP to support improved care coordination which remains partly realized.
We report recent International Ultraviolet Explorer (IUE) observations of two interacting binaries, R Arae and HD 207739. The ultraviolet spectra indicate the presence of optically-thick, variable hot plasma in those binary systems. These two binaries may belong to a class of binaries that are currently undergoing a rarely observed and probably short-lived phase in their evolution. Their properties are compared with those of two other interacting binaries, U Cephei and ß Lyrae.
During the Skylab I, II and III missions, ultraviolet spectra were obtained in 188 fields with a. 15 cm aperture objective-prism spectrograph. The instrument has been described by Henize, Wray, Parsons, Benedict, Bruhweiler, Rybski and O’Callaghan (1975; hereafter referred to as Paper I).
The spectra cover the wavelength region from 1300 to 5000 Å and have a resolution of 2 Å at 1400 Å and 12 Å at 2000 Å. Absorption and/or emission lines of C II λ 1335, Si IV λ 1394, 1403 and C IV λ 1549 are visible in more than one hundred stars. The lines of Si IV and C IV are found to be particularly sensitive to stellar temperature and luminosity. Since these lines are visible in spectra of moderate to low resolution it is clear that they should be of special interest in any UV classification system for faint stars. This paper investigates the correlation of the intensities of the C IV and Si IV lines with MK spectral type, and presents a preliminary classification scheme for 04 to B2 stars based on these lines.
The intensities of the very strong lines of C IV λ 1549 Å and Si IV λλ 1394, 1403 Å observed in spectra obtained with Skylab experiment S019 provide a sensitive discrimination of spectral type between B0 and B2. Eye estimates of the Si IV/C IV ratio are tabulated for 33 B0-B2, class III-V stars of which 11 are emission-line stars. Seven of the emission-line stars show significantly smaller ratios than normal stars of the same MK class. The most outstanding examples are 60 Cyg, o Pup, η Cen, and Ara.
The S-019 experiment on Skylab (cf. Henize et al. 1975; “Paper I”) recorded far UV spectra in about 160 4° x 5° fields, covering 10% of the sky, on 101 film with a 15 cm aperture objective-prism telescope. Several hundred early-type stars were observed in the vicinity of 1550 Å with a resolution between 3 and 4 Å, as well as thousands of stars at longer wavelengths and correspondingly lower resolution. An atlas of spectra for types 04 to B5 is illustrated in Paper I. That figure shows that the P Cygni profile is a characteristic of all supergiants earlier than B3 and main sequence stars earlier than 08.
The incidence of recreational water-associated outbreaks in the United States has significantly increased, driven, at least in part, by outbreaks both caused by Cryptosporidium and associated with treated recreational water venues. Because of the parasite's extreme chlorine tolerance, transmission can occur even in well-maintained treated recreational water venues (e.g. pools) and a focal cryptosporidiosis outbreak can evolve into a community-wide outbreak associated with multiple recreational water venues and settings (e.g. childcare facilities). In August 2004 in Auglaize County, Ohio, multiple cryptosporidiosis cases were identified and anecdotally linked to pool A. Within 5 days of the first case being reported, pool A was hyperchlorinated to achieve 99·9% Cryptosporidium inactivition. A case-control study was launched to epidemiologically ascertain the outbreak source 11 days later. A total of 150 confirmed and probable cases were identified; the temporal distribution of illness onset was peaked, indicating a point-source exposure. Cryptosporidiosis was significantly associated with swimming in pool A (matched odds ratio 121·7, 95% confidence interval 27·4–∞) but not with another venue or setting. The findings of this investigation suggest that proactive implementation of control measures, when increased Cryptosporidium transmission is detected but before an outbreak source is epidemiologically ascertained, might prevent a focal cryptosporidiosis outbreak from evolving into a community-wide outbreak.
US cholera surveillance offers insight into global and domestic trends. Between 2001 and 2011, 111 cases were reported to the Centers for Disease Control and Prevention. Cholera was associated with international travel in 90 (81%) patients and was domestically acquired in 20 (18%) patients; for one patient, information was not available. From January 2001 to October 2010, the 42 (47%) travel-associated cases were associated with travel to Asia. In October 2010, a cholera epidemic started in Haiti, soon spreading to the Dominican Republic (Hispaniola). From then to December 2011, 40 (83%) of the 48 travel-associated cases were associated with travel to Hispaniola. Of 20 patients who acquired cholera domestically, 17 (85%) reported seafood consumption; 10 (59%) ate seafood from the US Gulf Coast. In summary, an increase in travel-associated US cholera cases was associated with epidemic cholera in Hispaniola in 2010–2011. Travel to Asia and consumption of Gulf Coast seafood remained important sources of US cholera cases.