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The value of muscle biopsies in Pompe disease: identifying lipofuscin inclusions in juvenile- and adult-onset patients

Overview of attention for article published in Acta Neuropathologica Communications, January 2014
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Mentioned by

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1 tweeter

Citations

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48 Dimensions

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60 Mendeley
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Title
The value of muscle biopsies in Pompe disease: identifying lipofuscin inclusions in juvenile- and adult-onset patients
Published in
Acta Neuropathologica Communications, January 2014
DOI 10.1186/2051-5960-2-2
Pubmed ID
Authors

Erin J Feeney, Stephanie Austin, Yin-Hsiu Chien, Hanna Mandel, Benedikt Schoser, Sean Prater, Wuh-Liang Hwu, Evelyn Ralston, Priya S Kishnani, Nina Raben

Abstract

Pompe disease, an inherited deficiency of lysosomal acid alpha-glucosidase (GAA), is a metabolic myopathy with heterogeneous clinical presentations. Late-onset Pompe disease (LOPD) is a debilitating progressive muscle disorder that can occur anytime from early childhood to late adulthood. Enzyme replacement therapy (ERT) with recombinant human GAA is currently available for Pompe patients. Although ERT shows some benefits, the reversal of skeletal muscle pathology - lysosomal glycogen accumulation and autophagic buildup - remains a challenge. In this study, we examined the clinical status and muscle pathology of 22 LOPD patients and one atypical infantile patient on ERT to understand the reasons for muscle resistance to ERT.

Twitter Demographics

The data shown below were collected from the profile of 1 tweeter who shared this research output. Click here to find out more about how the information was compiled.

Mendeley readers

The data shown below were compiled from readership statistics for 60 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
United States 1 2%
Unknown 59 98%

Demographic breakdown

Readers by professional status Count As %
Student > Ph. D. Student 13 22%
Researcher 11 18%
Student > Bachelor 10 17%
Student > Master 6 10%
Professor 4 7%
Other 8 13%
Unknown 8 13%
Readers by discipline Count As %
Medicine and Dentistry 18 30%
Agricultural and Biological Sciences 12 20%
Biochemistry, Genetics and Molecular Biology 10 17%
Neuroscience 3 5%
Chemistry 3 5%
Other 5 8%
Unknown 9 15%

Attention Score in Context

This research output has an Altmetric Attention Score of 1. 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 03 January 2014.
All research outputs
#7,836,459
of 12,489,036 outputs
Outputs from Acta Neuropathologica Communications
#402
of 574 outputs
Outputs of similar age
#125,213
of 240,626 outputs
Outputs of similar age from Acta Neuropathologica Communications
#8
of 9 outputs
Altmetric has tracked 12,489,036 research outputs across all sources so far. This one is in the 23rd percentile – i.e., 23% of other outputs scored the same or lower than it.
So far Altmetric has tracked 574 research outputs from this source. They typically receive more attention than average, with a mean Attention Score of 8.0. This one is in the 23rd percentile – i.e., 23% of its peers scored the same or lower than it.
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 240,626 tracked outputs that were published within six weeks on either side of this one in any source. This one is in the 36th percentile – i.e., 36% of its contemporaries scored the same or lower than it.
We're also able to compare this research output to 9 others from the same source and published within six weeks on either side of this one.