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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.
Background: SMA is a neurodegenerative disease caused by biallelic deletion/mutation of SMN1. Copies of a similar gene (SMN2) modify disease severity. In a phase 1 study, SMN GRT onasemnogene abeparvovec (AVXS-101) improved outcomes of symptomatic SMA patients with two SMN2 copies (2xSMN2) dosed ≤6 months. Because motor neuron loss can be insidious and disease progression is rapid, early intervention is critical. This study evaluates AVXS-101 in presymptomatic SMA newborns. Methods: SPR1NT is a multicenter, open-label, phase 3 study enrolling ≥27 SMA patients with 2–3xSMN2. Asymptomatic infants ≤6 weeks receive a one-time intravenous AVXS-101 infusion (1.1x1014 vg/kg). Safety and efficacy are assessed through study end (18 [2xSMN2] or 24 months [3xSMN2]). Primary outcomes: independent sitting for ≥30 seconds (18 months [2xSMN2]) or assisted standing (24 months [3xSMN2]). Results: From April–September 2018, 7 infants received AVXS-101 (4 female; 6 with 2xSMN2) at ages 8–37 days. Mean baseline CHOP-INTEND score was 41.7 (n=6), which increased by 6.8, 11.0, 18.0, and 22.5 points at day 14 (n=4), month 1 (n=3), 2 (n=3), and 3 (n=2). Updated data available at the time of the congress will be presented. Conclusions: Preliminary data from SPR1NT show rapid motor function improvements in presymptomatic 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.
Introduction: Competency-based skills development has driven the evolution of medical education. Simulation-based education is established as an essential tool to supplement clinical encounters and it provides the opportunity for low-stakes practice of common and high-acuity low-occurrence (HALO) procedures and scenarios. This is particularly important for emergency medicine trainees working to build confidence, knowledge, and skills in the field. Methods: In the procedural training sessions, learners rotate through 6 small-group stations over a 3-hour period. Skills topics are determined from faculty input, prior session feedback, and literature reviews. Topics included chest tubes, airway intervention, lumbar punctures and trauma interventions. Online content and brief written materials are used for pre-session learning. The small groups use hands-on faculty-guided training, with real-time feedback. Printed materials supplement key learning points at the stations. A combination of low-fidelity task trainers and simulated patients are used for practice and demonstration. R3 EM residents have the opportunity to mentor junior learners. Brief participant surveys are distributed at each session to gather qualitative and quantitative feedback. Results: Feedback forms were completed by 79/85 (92.9%) learners over a period of 4 years (2015-2018). Participants included medical students (11.8%), EM residents (52.9%), and non-EM residents (35.3%). 84.8% (67/79) gave positive qualitative feedback on the sessions, citing points such as the beneficial practice opportunities, quality of instruction, and utility of the models. Updated surveys (N = 26) used a 5-point Likert scale (1 = disagree strongly; 5 strongly agree) in addition to qualitative feedback. Participants indicated that sessions were valuable, and informative (M = 4.692, SD = 0.462; M = 4.270, SD = 0.710). They reported increased understanding of procedures discussed, and they were likely to recommend the session (M = 4.301, SD = 0.606; M = 4.808, SD = 0.394). Conclusion: The ongoing evaluation of our mentor guided hands-on low-fidelity and hybrid simulation-based procedural skills sessions facilitates meaningful programmatic changes to best meet the needs of EM learners. Sessions also provide a forum for EM resident mentorship of junior learners. Feedback indicates learners enjoyed the sessions and found this to be an engaging and effective instructional modality.
Introduction: Maintaining competence in high-acuity low-occurrence (HALO) procedures is often difficult due to their infrequent occurrence. While simulation is a valuable tool to hone skills, providing effective simulation-based education (SBE) to learners outside academic centers can be challenging. Utilizing a mobile tele-simulation unit (MTU) with expert instruction from a geographically separated mentor could prove a valuable approach to overcoming barriers in this setting. However, to maximize benefit and buy-in, the training modules developed for this unique delivery method must align with the needs of those practicing in rural settings. Objectives: - To evaluate the procedural skills training needs of emergency medicine (EM) physicians in rural Newfoundland and Labrador (NL) - To inform the development of simulation modules designed for use in a MTU Methods: A web-based needs assessment was distributed to physicians registered with the NL Medical Association, working in rural locations, and having EM listed as their primary specialty. Participants evaluated their comfort, performance frequency and desire to have further training for 12 HALO procedures. Two EM physicians selected these from a broader list of core procedural skill competencies for CCFP-EM residents at Memorial University. Participants were also able to suggest other procedures that might benefit from SBE. Results: The data collection occurred for 8 weeks with a 68% response rate (N = 22). No respondents had formal EM training outside of exposure in family medicine residency. 60% had 10+ years practicing EM. Chest tube insertion (100%), difficult intubation (92.3%) and surgical airway (92.3%) were the procedures that most respondents felt required more SBE. In practice, they most often performed bag-valve ventilation, splint application and procedural sedation (>10 per year). Additional procedures felt to require SBE were central venous line placement and trauma assessment. Opportunities to participate in SBE were limited (66.7%-less than annually). Despite this, most participants agreed SBE would be a significant benefit if accessible (93.3%). The greatest barriers to SBE included lack of equipment, rural location, and time necessary for travel to larger centres. Conclusion: The provision of medical care in rural settings can be particularly challenging when HALO procedures must be performed. Unique delivery methods of SBE targeted to the needs of rural practitioners may help bridge gaps in knowledge and technical skills.
The present review evaluated the effectiveness of environmental-based interventions aimed at improving the dietary and physical activity behaviours and body composition indices of adults in institutions.
A systematic review was conducted. Electronic databases (MEDLINE, Embase, PsycINFO, CINAHL, The Cochrane Library, Web of Science, ProQuest Dissertation and Theses, Scopus and Athena) were searched for relevant articles published between database inception and October 2017. Searching, selecting and reporting were undertaken according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement.
Military establishments and maritime workplaces.
Adults in institutions, aged 18–45 years.
A total of 27842 articles were screened for eligibility, nine studies (reported in eleven articles) were included in the review. Five studies used multilevel strategies and four used environmental strategies only. Duration of follow-up ranged from 3 weeks to 10 years. Eight of the studies reported significant positive effects on dietary behaviours, but effect sizes varied. The study that targeted physical activity had no effect on activity levels but did have a significant positive effect on physical fitness. No evidence was identified that the studies resulted in improvements in body composition indices.
The evidence base appears to be in favour of implementing environmental interventions in institutions to improve the dietary behaviours of adults. However, due to the small number of studies included in the review, and the variable methodological quality of the studies and intervention reporting, further well-designed evaluation studies are required.
Introduction: Interest groups have become increasingly popular as students explore potential career paths earlier in their undergraduate experience. Emergency medicine (EM) has grown as a specialty and the match has become quite competitive. Attractive features of EM cited by learners (diversity, procedural skills and flexible schedule) appeal broadly to the undergraduate population. Learners at Memorial University recognized this leadership opportunity and worked with faculty to reach this wide target audience through a streamlined iterative evaluation of their EM Interest Group (EMIG). Methods: The local EMIG was formed in 2010. Yearly, EMIG executive work with outgoing members using prior experiences, contacts and best practices to facilitate handover and progress. From 2015 to present, 305 surveys were collected, giving an 81.9% response rate. 59.7% of respondents were first year students, and 40.3% were second year. The survey consisted of Likert scale and open-response questions. The Likert scale questions yielded favorable responses. 304 students (99.6%) felt presenters were knowledgeable, 301 (98.6%) would recommend the sessions to others and 301 (98.6%) were satisfied they attended. Surprisingly, 133 students (43.6%) said they were not interested in Emergency Medicine, likely attending due to the appeal of session topics and transferrable of EM skills. 232 (76.0%) stated that attendance did increase their interest in EM. Top responses for aspects of EM most interesting to them included: ability to find a work/life balance, ability to work urban or rural, variety of cases seen, and the non-routine shifts. Results: Survey feedback is used to inform refinement of the content, delivery and format of EMIG activities, delivered by EM faculty. Hands-on sessions (eg. suturing & airway management) have been popular. Informational sessions, on specific medical topics (ECG, resuscitation cases) or broader topics (EM streams) have also been very well received. Inclusion of all interested students, particularly large numbers for hands-on sessions, has presented challenges. Beyond current survey results, it will be interesting to consider if EMIG participation translates to learning or behavioral changes relevant to later clinical encounters; a question that will be difficult to quantify. Conclusion: The EM interest group is one of the most active at Memorial University. Survey results indicate that participants enjoy the EMIG session content and the structured iterative approach used by the group has been successful in maintaining an effective student led organization.
Introduction: Safe and efficient provision of quality healthcare requires maintenance of knowledge and skills relevant to daily practice. This is particularly relevant in rural and remote locations where high-acuity low-occurrence procedures and clinical scenarios present even less frequently. Simulation based training is widely used to supplement clinical exposure and practice but effective delivery of this approach to the rural/ remote practitioner must address barriers of time, cost and geographical separation. Mobile tele-simulation is an innovative approach that may help in bridging the gap through delivery of effective mentoring using telemedicine technologies and tailored educational content. Methods: To help direct the iterative design cycle for the mobile tele-simulation unit, input from potential future users was felt to be essential. The Delphi method was employed to reach consensus among study participants on four key questions: 1) What applications would the MTU be best suited for?, 2) What technical requirements and teaching tools would be needed to make the MTU successful?, 3) Which fields, besides EM/medical education, may benefit from partnerships with the final MTU?, 4) What research studies could be developed using the MTU? It was decided in advance that two rounds would be the maximum due to time constraints of the larger MTU projects. The first questionnaire focused on demographics and the four questions above. Independent reviewers analyzed, compiled and compared responses. Participants were sent the updated list, asked to confirm their responses and then to rank the responses highest to lowest priority. Results: Fifteen of 17 first round participants completed the questions, giving an 88.2% response rate. All shared a simulation background. 66% were physicians, 13.3% medical students, and 20% staff at Memorials Simulation Center. 66% had been involved with simulation-based education less than 5 years, and the others greater than 5 year. 13 of 15 (86.7%) responded in round 2. Consensus was not reached statistically using Kendalls W test for each of the four questions. However, there were several responses that showed higher median ranks than the others under each question. Application use: rural healthcare training, and medical professional training Technical factors: reliable learner-mentor connection, and competent technical support Non-technical factors: knowledgeable mentor and content relevant to locations practice, Research studies: training needs assessment from rural sites, and learners experience compared to stationary simulation center Conclusion: Input from a heterogeneous group of simulation users was sought to help prioritize key features in the development of the Mobile Tele-simulation Unit. Although statistically the study did not reach consensus, valuable feedback was compiled and pragmatically applied in the iterative development cycle.
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.
As the number of gestating sows reared in group housing increases, a better understanding of behavioral traits needed to negotiate these more complex social interactions promises to increase animal welfare and productivity. However, little is known about different behavioral strategies or coping styles in sows, and even less is understood about their ontogeny. To study the development of coping styles in adult gestating sows, 36 sows from the same sire line and same commercial maternal genetics were followed from birth through their second parity. Each animal was observed in a battery of stress-related behavioral tests at 5 weeks, and 3 months of age as well as 24 h postpartum as a parity 1 sow, and during introduction to subsequent gestation period in group housing. The tests at different ages included response to handling, open field exploration, human interaction, litter handling and social interactions with conspecifics. Many of the observed behaviors were correlated during the same period of the animal’s life and provided the motivation for a principal component analysis by age. Using principal component analysis, multiple traits were determined at each age point; at 5 weeks old: active, non-exploratory and cautious explained 82.5% of the variance; at 3 months of age: active, non-exploratory and low fear of humans explained 87.7% of the variance; and as primiparous sows: active, aggressive/dominant and submissive explained 82.0% of the variance. Several individual juvenile behaviors were associated with adult behavioral traits. For instance, the response to handling at 5 weeks was significantly predictive (β=0.4; P<0.05) of the aggressive/dominant trait of parity 1 sows. Taken together results presented here suggest that early behavioral responses of prepuberal gilts during specific instances of elevated environmental or social stress can predict future behavioral response as gestating sows.