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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.
In-line thickness monitoring of CoSi2 films is usually carried out by four-point probe resistivity measurements on blanket samples. This method, however, reveals no information about the thickness variation and stoichiometry of the CoSi2 films. Transmission Electron Microscopy (TEM) can provide this information but the technique is destructive, whereas Raman spectroscopy is non-invasive and offers a technique for patterned wafers compatible with current VLSI technology.
The CoSi and Co2Si phases exhibit very characteristic Raman signatures which, with glancing angle X-ray diffraction (GA-XRD), can be used to investigate the silicidation sequence. Although CoSi2 does not produce a Raman spectrum, the silicon local mode can be easily detected through the film and is very sensitive to variations in thickness. The intensity of the silicon local mode was measured on a series of ten samples of CoSi, on silicon and the precise CoSi, film thickness subsequently determined by cross sectional TEM. From these measurements, the absorption coefficient of CoSi2 at 500nm was determined.
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