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Facioscapulohumeral dystrophy in children: design of a prospective, observational study on natural history, predictors and clinical impact (iFocus FSHD)

Overview of attention for article published in BMC Neurology, August 2016
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Title
Facioscapulohumeral dystrophy in children: design of a prospective, observational study on natural history, predictors and clinical impact (iFocus FSHD)
Published in
BMC Neurology, August 2016
DOI 10.1186/s12883-016-0664-6
Pubmed ID
Authors

Rianne J. M. Goselink, Tim H. A. Schreuder, Karlien Mul, Nicol C. Voermans, Maaike Pelsma, Imelda J. M. de Groot, Nens van Alfen, Bas Franck, Thomas Theelen, Richard J. Lemmers, Jean K. Mah, Silvère M. van der Maarel, Baziel G. van Engelen, Corrie E. Erasmus

Abstract

Facioscapulohumeral muscular dystrophy (FSHD; OMIM 158900 & 158901) is a progressive skeletal muscle dystrophy, characterized by an autosomal dominant inheritance pattern. One of the major unsolved questions in FSHD is the marked clinical heterogeneity, ranging from asymptomatic individuals to severely affected patients with an early onset. An estimated 10 % of FSHD patients have an early onset (onset before 10 years of age) and are traditionally classified as infantile FSHD. This subgroup is regarded as severely affected and extra-muscular symptoms, such as hearing loss and retinopathy, are frequently described. However, information on the prevalence, natural history and clinical management of early onset FSHD is currently lacking, thereby hampering adequate patient counselling and management. Therefore, a population-based prospective cohort study on FSHD in children is highly needed. This explorative study aims to recruit all children (aged 0-17 years) with a genetically confirmed diagnosis of FSHD in The Netherlands. The children will be assessed at baseline and at 2-year follow-up. The general aim of the study is the description of the clinical features and genetic characteristics of this paediatric cohort. The primary outcome is the motor function as measured by the Motor Function Measure. Secondary outcomes include quantitative and qualitative description of the clinical phenotype, muscle imaging, genotyping and prevalence estimations. The ultimate objective will be a thorough description of the natural history, predictors of disease severity and quality of life in children with FSHD. The results of this population-based study are vital for adequate patient management and clinical trial-readiness. Furthermore, this study is expected to provide additional insight in the epigenetic and environmental disease modifying factors. In addition to improve counselling, this could contribute to unravelling the aetiology of FSHD. clinicaltrials.gov NCT02625662 .

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Mendeley readers

Mendeley readers

The data shown below were compiled from readership statistics for 81 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 81 100%

Demographic breakdown

Readers by professional status Count As %
Researcher 12 15%
Student > Bachelor 10 12%
Student > Ph. D. Student 10 12%
Student > Master 10 12%
Student > Doctoral Student 7 9%
Other 5 6%
Unknown 27 33%
Readers by discipline Count As %
Medicine and Dentistry 22 27%
Nursing and Health Professions 11 14%
Biochemistry, Genetics and Molecular Biology 5 6%
Psychology 4 5%
Neuroscience 4 5%
Other 6 7%
Unknown 29 36%
Attention Score in Context

Attention Score in Context

This research output has an Altmetric Attention Score of 2. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 19 August 2016.
All research outputs
#13,476,553
of 22,883,326 outputs
Outputs from BMC Neurology
#1,069
of 2,440 outputs
Outputs of similar age
#182,711
of 342,741 outputs
Outputs of similar age from BMC Neurology
#40
of 72 outputs
Altmetric has tracked 22,883,326 research outputs across all sources so far. This one is in the 39th percentile – i.e., 39% of other outputs scored the same or lower than it.
So far Altmetric has tracked 2,440 research outputs from this source. They typically receive a little more attention than average, with a mean Attention Score of 6.7. This one has gotten more attention than average, scoring higher than 53% of its peers.
Older research outputs will score higher simply because they've had more time to accumulate mentions. To account for age we can compare this Altmetric Attention Score to the 342,741 tracked outputs that were published within six weeks on either side of this one in any source. This one is in the 45th percentile – i.e., 45% of its contemporaries scored the same or lower than it.
We're also able to compare this research output to 72 others from the same source and published within six weeks on either side of this one. This one is in the 38th percentile – i.e., 38% of its contemporaries scored the same or lower than it.