Dataset Information

The Canadian Neuromuscular Disease Registry 2010-2019: A Decade of Facilitating Clinical Research Througha Nationwide, Pan-NeuromuscularDisease Registry.

ABSTRACT: We report the recruitment activities and outcomes of a multi-disease neuromuscular patient registry in Canada. The Canadian Neuromuscular Disease Registry (CNDR) registers individuals across Canada with a confirmed diagnosis of a neuromuscular disease. Diagnosis and contact information are collected across all diseases and detailed prospective data is collected for 5 specific diseases: Amyotrophic Lateral Sclerosis (ALS), Duchenne Muscular Dystrophy (DMD), Myotonic Dystrophy (DM), Limb Girdle Muscular Dystrophy (LGMD), and Spinal Muscular Atrophy (SMA). Since 2010, the CNDR has registered 4306 patients (1154 pediatric and 3148 adult) with 91 different neuromuscular diagnoses and has facilitated 125 projects (73 academic, 3 not-for-profit, 3 government, and 46 commercial) using registry data. In conclusion, the CNDR is an effective and productive pan-neuromuscular registry that has successfully facilitated a substantial number of studies over the past 10 years.

SUBMITTER: Hodgkinson V

PROVIDER: S-EPMC7902956 | biostudies-literature | 2021

REPOSITORIES: biostudies-literature

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The Canadian Neuromuscular Disease Registry 2010-2019: A Decade of Facilitating Clinical Research Througha Nationwide, Pan-NeuromuscularDisease Registry.

Hodgkinson V V Lounsberry J J M'Dahoma S S Russell A A Jewett G G Benstead T T Brais B B Campbell C C Johnston W W Lochmüller H H McCormick A A Nguyen C T CT O'Ferrall E E Oskoui M M Abrahao A A Briemberg H H Bourque P R PR Botez S S Cashman N N Chapman K K Chrestian N N Crone M M Dobrowolski P P Dojeiji S S Dowling J J JJ Dupré N N Genge A A Gonorazky H H Grant I I Hasal S S Izenberg A A Kalra S S Katzberg H H Krieger C C Leung E E Linassi G G Mackenzie A A Mah J K JK Marrero A A Massie R R Matte G G McAdam L L McMillan H H Melanson M M Mezei M M MM O'Connell C C Pfeffer G G Phan C C Plamondon S S Poulin C C Rodrigue X X Schellenberg K K Selby K K Sheriko J J Shoesmith C C Smith R G RG Taillon M M Taylor S S Venance S S Warman-Chardon J J Worley S S Zinman L L Korngut L L

Journal of neuromuscular diseases 20210101 1

We report the recruitment activities and outcomes of a multi-disease neuromuscular patient registry in Canada. The Canadian Neuromuscular Disease Registry (CNDR) registers individuals across Canada with a confirmed diagnosis of a neuromuscular disease. Diagnosis and contact information are collected across all diseases and detailed prospective data is collected for 5 specific diseases: Amyotrophic Lateral Sclerosis (ALS), Duchenne Muscular Dystrophy (DMD), Myotonic Dystrophy (DM), Limb Girdle Mu ...[more]

PMID: 32925088

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Project description:BackgroundPaediatric outpatient prescription (POP) monitoring is pivotal to identify inadequate prescriptions and optimize drug use. We aimed at describing recent trends in POPs in France.MethodsAll reimbursed dispensations of outpatient prescribed drugs (excluding vaccines) were prospectively collected for the paediatric population (<18 years old) in the French national health database in 2010-2011 and 2018-2019 (mean 117,356,938/year). POP prevalence (proportion of children receiving ≥1 drug prescriptions/year) was calculated by age groups and compared by prevalence rate ratios (PRRs). Given the large sample size, 95% confidence intervals of POP prevalences and PRRs did not differ from estimates.FindingsAmong the 14,510,023 children resident in France in 2018-2019, mean POP prevalence was 857‰ children. Most prescribed therapeutic classes were analgesics (643‰), antibiotics (405‰), nasal corticosteroids (328‰), nonsteroidal anti-inflammatory drugs (NSAIDs) (244‰), antihistamines (246‰) and systemic corticosteroids (210‰). POPs decreased with age from 976‰ for infants to 782‰ for adolescents. Children <6 years old were notably more exposed to inhaled corticosteroids (PRR=3.06), non-penicillin beta-lactam antibacterial agents (PRR=3.05) and systemic corticosteroids (PRR=2.11) than older ones. The POP prevalence was slightly higher (PRR=1.04) during 2018-2019 than 2010-2011, with marked increases for anti-emetics (PRR=1.84), vitamin D (PRR=1.49), proton pump inhibitors (PRR=1.42), systemic contraceptives (PRR=1.24) and nasal corticosteroids (PRR=1.21) and decreases for propulsive/prokinetic agents (PRR=0.09), NSAIDs (PRR=0.73) and systemic antibiotics (PRR=0.88).InterpretationPOP remained highly prevalent in France throughout the 2010s, especially for children <6 years old, with only a few improvements for selected therapeutic classes. These findings should prompt clinical guidance campaigns and/or regulatory policies.FundingInternal funding.

Project description:PurposeIn this investigation, we aimed to describe trends in time to acceptance (TTA) and time to online publication (TTOP) of research published in leading radiation oncology journals from 2010 to 2019. We further sought to identify journal characteristics that might influence TTA and TTOP.Methods and materialsWe searched the publication history of 5 leading international radiation oncology journals. For all research articles accepted from January 1, 2010, to December 31, 2019, we tabulated the date of article receipt, the date of acceptance, and the date of online publication when available. The TTA was calculated as the number of elapsed days from article receipt to acceptance, and the TTOP was calculated as the number of elapsed days from article acceptance to online publication. Using the Mann-Kendall test, we assessed for monotonic trends over time and used the post hoc Theil-Sen method to estimate rates of change. We created a multiple regression model to identify journal characteristics associated with TTA and TTOP.ResultsIn total, 10,132 articles were included. Both the TTA and the TTOP decreased significantly from 2010 to 2019 (P = .005 and P < .001, respectively), with an estimated decrease of 1.5 days per year for the TTA and 7.0 days per year for the TTOP. Multiple regression modeling revealed that a higher journal impact factor was independently associated with an increased TTA (P < .001) and a decreased TTOP (P < .001). A higher number of accepted journal articles per year was associated with a decreased TTA (P < .001) and an increased TTOP (P < .001).ConclusionsRadiation oncology research has been accepted and published online at increasingly faster rates during the past decade. The TTA may be longer in higher-impact, more selective journals, possibly suggesting a need for comprehensive peer review and complex editorial decisions. However, these articles are also published online faster after article acceptance. Future work examining patterns of acceptance and publication speed is needed to encourage rapid dissemination of practice-guiding data.

Project description:IntroductionDetailed and up-to-date data on the epidemiology and healthcare costs of Influenza are fundamental for public health decision-making. We analyzed inpatient data on Influenza-associated hospitalizations (IAH), selected complications and risk factors, and their related direct costs for Germany during ten consecutive years.MethodsWe conducted a retrospective cost-of-illness study on patients with laboratory-confirmed IAH (ICD-10-GM code J09/J10 as primary diagnosis) by ICD-10-GM-based remote data query using the Hospital Statistics database of the German Federal Statistical Office. Clinical data and associated direct costs of hospital treatment are presented stratified by demographic and clinical variables.ResultsBetween January 2010 to December 2019, 156,097 persons were hospitalized due to laboratory-confirmed Influenza (J09/J10 primary diagnosis). The annual cumulative incidence was low in 2010, 2012 and 2014 (1.3 to 3.1 hospitalizations per 100,000 persons) and high in 2013 and 2015-2019 (12.6 to 60.3). Overall direct per patient hospitalization costs were mean (SD) 3521 EUR (± 8896) and median (IQR) 1805 EUR (1502; 2694), with the highest mean costs in 2010 (mean 8965 EUR ± 26,538) and the lowest costs in 2012 (mean 2588 EUR ± 6153). Mean costs were highest in 60-69 year olds, and in 50-59, 70-79 and 40-49 year olds; they were lowest in 10-19 year olds. Increased costs were associated with conditions such as diabetes (frequency 15.0%; 3.45-fold increase compared to those without diabetes), adiposity (3.3%; 2.09-fold increase) or immune disorders (5.6%; 1.88-fold increase) and with Influenza-associated complications such as Influenza pneumonia (24.3%; 1.95-fold), bacterial pneumonia (6.3%; 3.86-fold), ARDS (1.2%; 10.90-fold increase) or sepsis (2.3%; 8.30-fold). Estimated overall costs reported for the 10-year period were 549.6 Million euros (95% CI 542.7-556.4 million euros).ConclusionWe found that the economic burden of IAH in Germany is substantial, even when considering solely laboratory-confirmed IAH reported as primary diagnosis. The highest costs were found in the elderly, patients with certain underlying risk factors and patients who required advanced life support treatment, and median and mean costs showed considerable variations between single years. Furthermore, there was a relevant burden of disease in middle-aged adults, who are not covered by the current vaccination recommendations in Germany.

Project description:Abstract Background: Although many publications in diabetic retinopathy (DR) have been reported, there is no bibliometric analysis. Purpose: To perform a bibliometric analysis in the field of diabetic retinopathy (DR) research, to characterize the current international status of DR research, to identify the most effective factors involved in this field, and to explore research hotspots in DR research. Methods: Based on the Web of Science Core Collection (WoSCC), a bibliometric analysis was conducted to investigate the publication trends in research related to DR. Knowledge maps were constructed by VOSviewer v.1.6.10 to visualize the publications, the distribution of countries, international collaborations, author productivity, source journals, cited references and keywords, and research hotspots in this field. Results: In total, 11,839 peer-reviewed papers were retrieved on DR from 2010 to 2019, and the annual research output increased with time. The United States ranks highest among countries with the most publications. The most active institution is the University of Melbourne. Wong, TY contributed the largest number of publications in this field. Investigative Ophthalmology & Visual Science was the most prolific journal in DR research. The top-cited references mainly investigated the use of anti-vascular endothelial growth factor (VEGF) medications in the management of DR, and the keywords formed 6 clusters:1. pathogenesis of DR;2. epidemiology and risk factors for DR;3. treatments for DR;4. screening of DR;5. histopathology of DR; and6. diagnostic methods for DR. Discussion: With the improvement of living standard, DR has gradually become one of the important causes of blindness, and has become a hot spot of public health research in many countries. The application of deep learning and artificial intelligence in diabetes screening and anti-VEGF medications in the management of DR have been the research hotspots in recent 10 years. Conclusions: Based on data extracted from the WoSCC, this study provides a broad view of the current status and trends in DR research and may provide clinicians and researchers with insight into DR research and valuable information to identify potential collaborators and partner institutions and better predict their dynamic directions.

Dataset Information

The Canadian Neuromuscular Disease Registry 2010-2019: A Decade of Facilitating Clinical Research Througha Nationwide, Pan-NeuromuscularDisease Registry.

Publications

The Canadian Neuromuscular Disease Registry 2010-2019: A Decade of Facilitating Clinical Research Througha Nationwide, Pan-NeuromuscularDisease Registry.

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