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Fibrosis: a key feature of Fabry disease with potential therapeutic implications

Overview of attention for article published in Orphanet Journal of Rare Diseases, August 2013
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About this Attention Score

  • Good Attention Score compared to outputs of the same age (67th percentile)
  • High Attention Score compared to outputs of the same age and source (81st percentile)

Mentioned by

twitter
3 tweeters
facebook
3 Facebook pages

Citations

dimensions_citation
95 Dimensions

Readers on

mendeley
104 Mendeley
citeulike
1 CiteULike
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Title
Fibrosis: a key feature of Fabry disease with potential therapeutic implications
Published in
Orphanet Journal of Rare Diseases, August 2013
DOI 10.1186/1750-1172-8-116
Pubmed ID
Authors

Frank Weidemann, Maria D Sanchez-Niño, Juan Politei, João-Paulo Oliveira, Christoph Wanner, David G Warnock, Alberto Ortiz

Abstract

Fabry disease is a rare X-linked hereditary disease caused by mutations in the AGAL gene encoding the lysosomal enzyme alpha-galactosidase A. Enzyme replacement therapy (ERT) is the current cornerstone of Fabry disease management. Involvement of kidney, heart and the central nervous system shortens life span, and fibrosis of these organs is a hallmark of the disease. Fibrosis was initially thought to result from tissue ischemia secondary to endothelial accumulation of glycosphingolipids in the microvasculature. However, despite ready clearance of endothelial deposits, ERT is less effective in patients who have already developed fibrosis. Several potential explanations of this clinical observation may impact on the future management of Fabry disease. Alternative molecular pathways linking glycosphingolipids and fibrosis may be operative; tissue injury may recruit secondary molecular mediators of fibrosis that are unresponsive to ERT, or fibrosis may represent irreversible tissue injury that limits the therapeutic response to ERT. We provide an overview of Fabry disease, with a focus on the assessment of fibrosis, the clinical consequences of fibrosis, and recent advances in understanding the cellular and molecular mechanisms of fibrosis that may suggest novel therapeutic approaches to Fabry disease.

Twitter Demographics

The data shown below were collected from the profiles of 3 tweeters 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 104 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Spain 1 <1%
United States 1 <1%
Ukraine 1 <1%
Unknown 101 97%

Demographic breakdown

Readers by professional status Count As %
Researcher 17 16%
Student > Master 16 15%
Student > Bachelor 15 14%
Other 10 10%
Student > Ph. D. Student 7 7%
Other 19 18%
Unknown 20 19%
Readers by discipline Count As %
Medicine and Dentistry 36 35%
Biochemistry, Genetics and Molecular Biology 17 16%
Agricultural and Biological Sciences 11 11%
Nursing and Health Professions 3 3%
Computer Science 2 2%
Other 10 10%
Unknown 25 24%

Attention Score in Context

This research output has an Altmetric Attention Score of 4. 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 25 April 2017.
All research outputs
#6,698,077
of 21,347,688 outputs
Outputs from Orphanet Journal of Rare Diseases
#956
of 2,383 outputs
Outputs of similar age
#56,043
of 176,972 outputs
Outputs of similar age from Orphanet Journal of Rare Diseases
#3
of 11 outputs
Altmetric has tracked 21,347,688 research outputs across all sources so far. This one has received more attention than most of these and is in the 67th percentile.
So far Altmetric has tracked 2,383 research outputs from this source. They typically receive a little more attention than average, with a mean Attention Score of 7.2. This one has gotten more attention than average, scoring higher than 59% 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 176,972 tracked outputs that were published within six weeks on either side of this one in any source. This one has gotten more attention than average, scoring higher than 67% of its contemporaries.
We're also able to compare this research output to 11 others from the same source and published within six weeks on either side of this one. This one has done well, scoring higher than 81% of its contemporaries.