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Spinal muscular atrophy (SMA) is a devastating rare disease that affects individuals regardless of ethnicity, gender, and age. The first-approved disease-modifying therapy for SMA, nusinursen, was approved by Health Canada, as well as by American and European regulatory agencies following positive clinical trial outcomes. The trials were conducted in a narrow pediatric population defined by age, severity, and genotype. Broad approval of therapy necessitates close follow-up of potential rare adverse events and effectiveness in the larger real-world population.
The Canadian Neuromuscular Disease Registry (CNDR) undertook an iterative multi-stakeholder process to expand the existing SMA dataset to capture items relevant to patient outcomes in a post-marketing environment. The CNDR SMA expanded registry is a longitudinal, prospective, observational study of patients with SMA in Canada designed to evaluate the safety and effectiveness of novel therapies and provide practical information unattainable in trials.
The consensus expanded dataset includes items that address therapy effectiveness and safety and is collected in a multicenter, prospective, observational study, including SMA patients regardless of therapeutic status. The expanded dataset is aligned with global datasets to facilitate collaboration. Additionally, consensus dataset development aimed to standardize appropriate outcome measures across the network and broader Canadian community. Prospective outcome studies, data use, and analyses are independent of the funding partner.
Prospective outcome data collected will provide results on safety and effectiveness in a post-therapy approval era. These data are essential to inform improvements in care and access to therapy for all SMA patients.
We undertook a quality improvement project to address challenges with pulmonary artery catheter (PAC) line maintenance in a setting of low-baseline central-line infection rates. We observed a subsequent reduction in Staphylococcal PAC line infections and a trend toward a reduction in overall PAC infection rates over 1 year.
Background: Axial myopathy is a rare neuromuscular disorder of variable etiology characterised by preferential involvement of the paraspinal muscles. We reviewed clinical features of patients with axial myopathies and the diagnostic yield of myositis-associated antibodies and targeted next generation sequencing panels. Methods: We performed a retrospective review of patients presenting with axial myopathy at the Montreal Neurological Hospital from 2011-2018. Data collection included clinical presentation, disease course, results of electromyography, imaging, laboratory and genetic testing, and histopathology on muscle biopsy. Results: Twenty-five patients were identified. Initial manifestation of axial weakness was head drop (15), camptocormia (8), and rigid spine (2). Autoimmune myositis was diagnosed in 9 patients, seropositive in 7 out of 7 tested for myositis-associated antibodies. Genetic testing was consistent with oculopharyngeal muscular dystrophy in one patient and RYR-1 (ryanodine receptor 1) related core myopathy in another. Local radiotherapy or spine surgery preceded the onset of axial weakness in 1 and 6 patients, respectively. Muscle biopsies were available in 17 patients and revealed myopathic changes (16), inflammatory changes (6), and myopathy with vacuoles (3). Conclusions: Recent advancements in genetic and antibody testing, combined with paraspinal muscle biopsy, allow for more precise classification and identification of potentially treatable axial myopathies.
The importance of chronic low-grade inflammation in the pathology of numerous age-related chronic conditions is now clear. An unresolved inflammatory response is likely to be involved from the early stages of disease development. The present position paper is the most recent in a series produced by the International Life Sciences Institute's European Branch (ILSI Europe). It is co-authored by the speakers from a 2013 workshop led by the Obesity and Diabetes Task Force entitled ‘Low-grade inflammation, a high-grade challenge: biomarkers and modulation by dietary strategies’. The latest research in the areas of acute and chronic inflammation and cardiometabolic, gut and cognitive health is presented along with the cellular and molecular mechanisms underlying inflammation–health/disease associations. The evidence relating diet composition and early-life nutrition to inflammatory status is reviewed. Human epidemiological and intervention data are thus far heavily reliant on the measurement of inflammatory markers in the circulation, and in particular cytokines in the fasting state, which are recognised as an insensitive and highly variable index of tissue inflammation. Potential novel kinetic and integrated approaches to capture inflammatory status in humans are discussed. Such approaches are likely to provide a more discriminating means of quantifying inflammation–health/disease associations, and the ability of diet to positively modulate inflammation and provide the much needed evidence to develop research portfolios that will inform new product development and associated health claims.
As essay after essay in this series has reminded us, the term “neomedievalism” is too multivalent and maddeningly complex to define with any satisfaction: any attempt to create a definition invariably oversimplifies the concept or distorts it to fit current needs. In the case of neomedievalism, rather than attempt another iteration of an Ur-definition, Carol R. Robinson and Pamela Clements have done invaluable work in creating a field guide to understanding the characteristics of neomedievalism. In brief, we can call a text neomedieval when it does one or more of the following:
It is playful or ironic in nature.
It calls attention to its own construction, often as a work of bricolage.
It deliberately shatters any possibility for a “sealed world” of the text.
It refuses the nostalgic fantasy of being able to retrieve the medieval past.
Its task is to create a conscious vision of an alternative universe.
This last item holds the most promise as a way of reading George R. R. Martin's multi-volume A Song of Ice and Fire as a text concerned with particular ethical issues surrounding disability: the damage ableist discourses and narratives inflict on the disabled.
In the landmark case Arline vs. Nassau County, Justice William J. Brennan, Jr., summarizing the need for an inclusive definition of disability, noted the problems that narratives of disability posed for the disabled: “society's accumulated myths and fears about disability and disease are as handicapping as are the physical limitations that flow from actual impairment.”
Ethics in post-medieval responses to the Middle Ages form the main focus of this volume. The six opening essays tackle such issues as the legitimacy of reinventing medieval customs and ideas, at what point the production and enjoyment of caricaturizing the Middle Ages become inappropriate, how medievalists treat disadvantaged communities, and the tension between political action and ethics in medievalism. The eight subsequent articles then build on this foundation as they concentrate on capitalist motives for melding superficially incompatible narratives in medievalist video games, Dan Brown's use of Dante's Inferno to promote a positivist, transhumanist agenda, disjunctures from medieval literature to medievalist film in portrayals of human sacrifice, the influence of Beowulf on horror films and vice versa, portrayals of war in Beowulf films, socialism in William Morris's translation of Beowulf, bias in Charles Alfred Stothard's Monumental Effigies of Great Britain, and a medieval source for death in the Harry Potter novels. The volume as a whole invites and informs a much larger discussion on such vital issues as the ethical choices medievalists make, the implications of those choices for their makers, and the impact of those choices on the world around us. Karl Fugelso is Professor of Art History at Towson University in Baltimore, Maryland. Contributors: Mary R. Bowman, Harry Brown, Louise D'Arcens, Alison Gulley, Nickolas Haydock, Lisa Hicks, Lesley E. Jacobs, Michael R. Kightley, Phillip Lindley, Pascal J. Massie, Lauryn S. Mayer, Brent Moberley, Kevin Moberley, Daniel-Raymond Nadon, Jason Pitruzello, Nancy M. Resh, Carol L. Robinson, Christopher Roman, M.J. Toswell.
To describe a relatively unknown clinical entity – inflammatory cast of the tympanic membrane after acute otitis media – and its simple out-patient treatment.
Retrospective review of case series.
Subspecialty practice at a tertiary hospital.
Seven patients diagnosed previously with acute otitis media with perforation or otitis externa, and with persistent ear discomfort.
Retrospective chart review.
The patients presented with weeks to months of persistent hearing loss after acute otitis media with perforation or acute otitis externa. Visits to their primary care physicians had been uninformative. After comparison of the affected and unaffected tympanic membranes, a thin, hard cast was identified and removed from the affected tympanic membrane. Improvement in hearing was documented in the three patients who underwent audiometric testing; the remainder had subjective improvement without audiometric evaluation.
Otolaryngologists should be aware of the possibility of an inflammatory cast of the tympanic membrane following acute otitis media with perforation or otitis externa, and should carefully compare the unaffected and affected ears in such cases. Treatment – removal of the rigid cast – is both simple and effective.
Neutralization tests for poliovirus antibodies were carried out on 74 patients in an adult mental deficiency hospital: 37 patients with Down's syndrome and 37 non- Down's mental defectives. The distribution of antibody titres to poliovirus types 1, 2 and 3 did not differ significantly between the two groups. Most patients had antibody to at least one poliovirus typebut less than a third had antibodies at a titre of 1/8 or greater to all three types. The low level of poliovirus immunity in this population may be of epidemiological importance.
In this work, we present a study of epitaxial Aluminium Nitride (AlN) for thin film bulk acoustic wave (BAW) applications. Molecular beam epitaxy (MBE) was used to perform high crystalline quality AlN thin films growth on different silicon substrate preparations. A morphological study was performed by atomic force microscopy (AFM) and scanning electron microscopy (SEM), while structural properties and acoustic wave speed were respectively assessed by X-ray diffraction and acoustic picoseconds.
We present an overview of our work concerning the fabrication of GaN-based microcavities grown on silicon substrates dedicated to the observation of the strong light-matter coupling regime. In the view of recent promising results in the field, prospects regarding the improvement of heterostructures in order to observe room temperature polariton lasing from a GaN-based microcavity grown on a silicon substrate will be discussed.
During the last ten years, we have developed an efficient growth process of nitrides on silicon substrates by molecular beam epitaxy. In collaboration with partners AlGaN/GaN HEMTs on Si having promising performances have been fabricated. Focusing on the growth aspect and underlying some of the key issues, we present in this paper an overview of our contribution in the field of AlGaN/GaN HEMTs on Si substrates.