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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.
Impairments of attention and memory are evident in early psychosis, and often lead to severe, longstanding functional disability. Effective non-pharmacological interventions are needed due to the unsuccessful results of pharmacological interventions.
To determine whether yoga is effective for cognitive impairments, and the neural mechanism underlying these effects.
It was a randomized controlled study of 12-week of yoga and aerobic exercise (walking and cycling) intervention vs wait-list control for female early psychotic patients. Memory was measured with Hong Kong List Learning Test and Digit Span test, and attention was measured with Letter Cancellation test. Cognitive data analysis was based on the Intention-to-Treat method using a mixed-model analysis. Seed based functional connectivity was applied using posterior cingulate cortex (PCC) as seed with AFNI. Cortical thickness analyses were performed using FreeSurfer. Results from yoga and control groups were presented.
A total of 140 women were recruited and randomized into three groups. 95 completed the study, and 115 were included for cognitive data analysis. For imaging data, 42 participants were used for cortical thickness analyses; and 60 were included for neural connectivity analyses. Yoga group demonstrated significant improvements in working memory, verbal acquisition and attention (P=.01). Cortical thickness increased in the postcentral gyrus (P<.01); connectivity between PCC and bilateral inferior parietal gyrus increased after yoga intervention (P<.005).
Yoga has been found to be effective for memory and attention in early psychotic patients. The increases of thickness and neural connectivity indicate the possible neural mechanisms underlying the improvements of cognition.
In this paper, a novel single-cavity triangular substrate-integrated waveguide (TSIW) dual-band filter loading a complementary triangular split ring resonator (CTSRR) is proposed, which has three transmission zeros (TZs) in the stopband in total. The dual-band response is achieved by the CTSRR and the degenerate modes of the TSIW cavity. In order to control the TZs, we propose two adjustment techniques, shift feeding technique and adding via perturbation. In addition, the CTSRR etched on the surface can produce a new TZ in the upper first-passband. Finally, a dual-band filter with three TZs is simulated, fabricated, and measured. There is a good agreement between the simulated results and measured ones.
The consumption of nitrate-rich vegetables can acutely lower blood pressure and improve mediators shown to optimise vascular health. However, we do not yet understand the impact of long-term habitual dietary nitrate intake and its association with CVD. Therefore, the aim of this investigation was to examine the relationship between habitual dietary nitrate intakes and risk of CHD in women from the Nurses’ Health Study. We prospectively followed 62 535 women who were free from diabetes, CVD and cancer at baseline in 1986. Information on diet was updated every 4 years with validated FFQ. The main outcome was CHD defined by the occurrence of non-fatal myocardial infarction or fatal CHD. Cox proportional hazard regression models were used to estimate the relative risks (RR) and 95 % CI. During 26 years of follow-up, 2257 cases of CHD were identified. When comparing the highest quintile of nitrate intake with the lowest quintile, in aged-adjusted analysis there was a protective association for CHD (RR=0·77, 95 % CI 0·68, 0·97; P=0·0002) which dissipated after further adjustment for smoking, physical activity, BMI and race (RR=0·91; 95 % CI 0·80, 1·04; P=0·27). This magnitude of association was further attenuated once we adjusted for the Alternative Healthy Eating Index excluding vegetable and fruit consumption (RR=1·04, 95 % CI 0·91, 1·20; P=0·34). Dietary nitrate intake was not related to the risk of CHD after adjustment for other lifestyle and non-vegetable dietary factors in a large group of US women.
Pheromones play an important role in mediating interspecific interactions in insects. In an insect community, pheromones can reveal information about the senders, which could be used by other members of the food web (competitor, natural enemies, etc.) to their own advantage. The aggregation pheromones of two closely related thrips species, Frankliniella occidentalis and Frankliniella intonsa, have been identified with the same major compounds, (R)-lavandulyl acetate and neryl (S)-2-methylbutanoate, but in different ratios. However, the roles of the aggregation pheromones in the interspecific interactions between these two closely related species are unknown. Here, we investigated the roles of major aggregation pheromone compounds in interspecific interactions between F. occidentalis and F. intonsa for both long and short ranges. The results showed that, at tested doses, neither aggregation pheromone-induced long range cross-attraction nor short range cross-mating was detected between F. occidentalis and F. intonsa. Field-trapping trials showed that the species-specificity in aggregation pheromones was regulated by the ratio of two major compounds. However, species-specific blends of the two major compounds had no effect on short-range interactions between these two species. Our data from the thrips species provide support for the ‘aggregation model of coexistence’, explaining the species-specific pheromone-mediated coexistence of closely related species. Thus, species-specific pheromones could be one of the factors affecting population dynamics and community structure in closely related insects with similar niches.
Diarrhea is a common cause of morbidity and mortality and the incidence of diarrhea in the world has changed little over the past four decades. To assess the prevalence of and healthcare practices for diarrhea, a cross-sectional study was conducted in Pudong, Shanghai, China. In October 2014, a total of 5324 community residents were interviewed. Respondents were asked if they had experienced diarrhea (defined as ⩾3 passages of watery, loose, bloody, or mucoid stools within a 24-h period) in the previous month prior to the interview. The monthly prevalence of diarrhea was 4·1% (95% CI: 3·3–4·8), corresponding to an incidence rate of 0·54 episodes per person-year. The proportion of individuals with diarrhea who sought healthcare was 21·2% (95% CI: 13·4–29·0). Diarrhea continues to impose a considerable burden on the community and healthcare system in Pudong. Young age and travel were identified as predictors of increased diarrhea occurrence.
An acousto-optical spectrograph (AOS) with a high spectral resolution of 28 kHz has been developed for spectral line observations of objects, such as dark clouds, where the line-widths are low. It has been mounted on the 4-m-diameter millimetre-wave telescope at CSIRO Division of Radiophysics at Epping, NSW and successfully tested using 115 GHz Co observations of a sample of southern dark clouds. At 115 GHz the velocity resolution is 0.07 km s-1.
Pompe disease is a lysosomal storage disorder caused by a deficiency of the
enzyme acid alpha-glucosidase. Patients have skeletal muscle and respiratory
weakness with or without cardiomyopathy. The objective of our review was to
systematically evaluate the quality of evidence from the literature to
formulate evidence-based guidelines for the diagnosis and management of
patients with Pompe disease. The literature review was conducted using
published literature, clinical trials, cohort studies and systematic
reviews. Cardinal treatment decisions produced seven management guidelines
and were assigned a GRADE classification based on the quality of evidence in
the published literature. In addition, six recommendations were made based
on best clinical practices but with insufficient data to form a guideline.
Studying outcomes in rare diseases is challenging due to the small number of
patients, but this is in particular the reason why we believe that informed
treatment decisions need to consider the quality of the evidence.
To study the interactive influence of implanted nano platinum black electrodes (as compared with pure platinum electrodes) on rabbit orbicularis oculi muscle morphology and function.
The influence of the two types of electrode on the orbicularis oculi muscle was monitored in a rabbit model of facial paralysis. Biological electric current and exciting current were administered to biological tissue, and morphological and functional changes were identified. Changes in orbicularis oculi muscle contraction, electrode configuration and performance associated with long-term electrical stimulation were observed over 28 days of implantation.
The nano platinum black electrode was superior to the pure platinum electrode in the following aspects: morphology and functionality, electrical excitation function of the orbicularis oculi muscle (as assessed by electromyography), muscle contraction function and biological tissue changes. Furthermore, the nano platinum black electrode features had good stability.
Microelectrode surface modification with nano platinum black can effectively increase the microelectrode surface area and improve electrode performance, and is associated with good tissue compatibility.
Patient registries represent an important method of organizing “real world” patient information for clinical and research purposes. Registries can facilitate clinical trial planning and recruitment and are particularly useful in this regard for uncommon and rare diseases. Neuromuscular diseases (NMDs) are individually rare but in aggregate have a significant prevalence. In Canada, information on NMDs is lacking. Barriers to performing Canadian multicentre NMD research exist which can be overcome by a comprehensive and collaborative NMD registry.
We describe the objectives, design, feasibility and initial recruitment results for the Canadian Neuromuscular Disease Registry (CNDR).
The CNDR is a clinic-based registry which launched nationally in June 2011, incorporates paediatric and adult neuromuscular clinics in British Columbia, Alberta, Ontario, Quebec, New Brunswick and Nova Scotia and, as of December 2012, has recruited 1161 patients from 12 provinces and territories. Complete medical datasets have been captured on 460 “index disease” patients. Another 618 “non-index” patients have been recruited with capture of physician-confirmed diagnosis and contact information. We have demonstrated the feasibility of blended clinic and central office-based recruitment. “Index disease” patients recruited at the time of writing include 253 with Duchenne and Becker muscular dystrophy, 161 with myotonic dystrophy, and 71 with ALS.
The CNDR is a new nationwide registry of patients with NMDs that represents an important advance in Canadian neuromuscular disease research capacity. It provides an innovative platform for organizing patient information to facilitate clinical research and to expedite translation of recent laboratory findings into human studies.
Little is known about the potential adherence to and the effectiveness of a low-carbohydrate (LC) diet on weight loss and cardiometabolic risk factors in Chinese adults with a habitually high carbohydrate intake. In the present controlled feeding trial, fifty overweight or obese women (age 47·9 (sem 0·9) years; BMI 26·7 (sem 0·3) kg/m2) were randomly assigned to a LC non-energy-restricted diet (initial carbohydrate intake 20 g/d, with a 10 g increase weekly) or an energy-restricted (ER) diet (carbohydrate intake 156–205 g/d, ER to 5021 or 6276 kJ/d, 35 % average energy reduction) for 12 weeks. Over the intervention period, the two diets had comparable compliance (96 %) and self-reported acceptability. At week 12, carbohydrate intake in the LC and ER groups contributed to 36·1 and 51·1 % of total energy, respectively (P< 0·001). Although both diets showed similarly decreased mean body weight (LC − 5·27 (95 % CI − 6·08, − 4·46) kg; ER − 5·09 (95 % CI − 5·50, − 4·67) kg, P= 0·67) and percentage of fat mass measured by dual-energy X-ray absorptiometry (LC − 1·19 (95 % CI − 1·88, − 0·50) %; ER − 1·56 (95 % CI − 2·20, − 0·92) %, P= 0·42), participants in the LC group had greater reductions in the ratio of total cholesterol:HDL-cholesterol (P= 0·03) and also in the ratio of TAG:HDL-cholesterol (P= 0·01) than those in the ER group. The present 12-week diet trial suggested that both a LC non-energy-restricted diet and an ER diet were acceptable to Chinese women and both diets were equally effective in reducing weight and fat mass. Moreover, the LC diet showed beneficial effects on blood lipid profiles.
Amyotrophic lateral sclerosis (ALS) is a devastating cause of progressive weakness, respiratory failure and death. To date there is no effective therapy to meaningfully extend survival but continuously emerging targets and putative treatments are studied in clinical trials. Canadian epidemiological data on ALS is scarce and the socioeconomic impact of ALS on Canadian society is unclear. The Canadian Neuromuscular Disease Registry (CNDR) is a national clinic-based registry of patients with neuromuscular diseases with the goal of facilitating the design and execution of clinical research.
We conducted a national stakeholder survey to assess interest for a Canadian ALS registry and an assessment of expected case ascertainment. A dataset derivation meeting was held to establish the registry medical dataset.
We report the results of the national stakeholder survey, case ascertainment assessment, and the derived dataset that have resulted in the current implementation of a Canadian registry of patients with ALS.
The development of this long sought-after resource is a significant step forward for the Canadian ALS patient and research communities that will result in more efficient clinical trial recruitment and advancements in our understanding of ALS in Canada.
We present electrogenerated chemiluminescence (ECL) and fluorescence lifetime mapping of MEH-PPV/PCBM thin films. The ECL results show that the oxidation peak of MEH-PPV near 0.7 V (vs. SCE) and ECL response of films shifted positively towards 1.2 V in the presence of PCBM. At the same time, the oxidation current density of MEH-PPV increases along with the decrease of ECL intensity in the presence of PCBM. The fluorescence lifetime images clearly show that the lifetime spatial heterogeneities are affected by different substrates and MEH-PPV/PCBM ratios. Meanwhile, the lifetime of MEH-PPV decreases with the increasing of film thickness. The lifetimes of MEH-PPV films on TiO2 substrate are lower than films on glass.
The Chinese Spectral Radioheliograph (CSRH) is under construction in Mingantu station of NAOC in China. Now, CSRH-I which includes antenna, receiver and correlator in decimetric wave range has been established. This paper introduced calibration on CSRH and present some results of data processing.
A fungal pathogen, Helminthosporium gramineum Rabehn f.sp. echinochloae (HGE), has been developed as a mycoherbicidal agent for the control of barnyardgrass in China. Under greenhouse conditions, the effect of the pathogen on disease incidence, mortality, and dry weight reduction of barnyardgrass was tested to determine the potential of this mycoherbicide. Field experiments during 2007 and 2008 showed that the conidia of HGE displayed excellent activity on barnyardgrass and good activity on a few other weed species. The HGE treatment increased the rice yield when compared with the untreated control and had no negative impact on the rice plant. In addition, the progression of HGE infection and the alteration of cellular ultrastructure in infected barnyardgrass were observed by using scanning and transmission electron microscopes. After infection, cell membranes of barnyardgrass leaves were found dramatically changed, and the ultrastructure of cells was severely deformed. This study clearly shows the scope of HGE as a potential mycoherbicide for control of barnyardgrass in agricultural cropping systems and has laid the groundwork for further studies on the mode of infection and the nosogenesis of HGE.
High rate deposition of hydrogenated microcrystalline silicon (μc-Si:H) films and solar cells were prepared by very high frequency plasma enhanced chemical vapor deposition (VHF-PECVD) process in a high power and high pressure regime. The experiment results demonstrate that in high-rate deposited μc-Si:H films, the structural evolution is much more dramatic than that in low-rate deposited μc-Si:H films. A novel VHF power profiling technique, which was designed by dynamically decreasing the VHF power step by step during the deposition of μc-Si:H intrinsic layers, has been developed to control the structural evolution along the growth direction. Another advantage of this VHF power profiling technique is the reduced ion bombardments on growth surface because of decreasing the VHF power. Using this method, a significant improvement in the solar cell performance has been achieved. A high conversion efficiency of 9.36% (Voc=542mV, Jsc=25.4mA/cm2, FF=68%) was obtained for a single junction μc-Si:H p-i-n solar cell with i-layer deposited at deposition rate over 10 �/s.
Somatostatin (SS) is a hormone that inhibits the secretion of growth hormone. Immunization against SS can promote the growth of animals. A novel SS-VP22 fused vaccine, pEGS2SS-V, was constructed from pEGS2SS plasmid with a VP22 gene fragment. Two times of immunization with pEGS2SS-V-induced anti-SS antibodies in mice. Compared with mice immunized with pEGS2SS and 0.85% saline, the growth performance of mice immunized with pEGS2SS-V was increased by 14.1% (P < 0.05) and 48.4% (P < 0.01) on the 2nd week after the first vaccination, respectively. The results indicated that the effects of the somatostatin DNA vaccine could be improved effectively by VP22 gene adjuvant.
In this paper, a new type PCF with three different air-hole diameters in cladding region is proposed and investigated by using a compact 2-D finite
difference frequency domain (FDFD) method with anisotropic perfectly matched
layers (PML) absorbing boundary conditions. Through numerical simulation we
found that by optimizing the geometrical parameters, our proposed structure
with fewer of air-hole rings greatly meets the performance criteria such as
ultraflattened dispersion and low confinement loss, which is highly
instructive for designing photonic crystal fibers.