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Cystinosis: a review

Overview of attention for article published in Orphanet Journal of Rare Diseases, April 2016
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About this Attention Score

  • Good Attention Score compared to outputs of the same age (68th percentile)
  • Above-average Attention Score compared to outputs of the same age and source (52nd percentile)

Mentioned by

twitter
6 tweeters

Citations

dimensions_citation
72 Dimensions

Readers on

mendeley
124 Mendeley
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Title
Cystinosis: a review
Published in
Orphanet Journal of Rare Diseases, April 2016
DOI 10.1186/s13023-016-0426-y
Pubmed ID
Authors

Mohamed A. Elmonem, Koenraad R. Veys, Neveen A. Soliman, Maria van Dyck, Lambertus P. van den Heuvel, Elena Levtchenko

Abstract

Cystinosis is the most common hereditary cause of renal Fanconi syndrome in children. It is an autosomal recessive lysosomal storage disorder caused by mutations in the CTNS gene encoding for the carrier protein cystinosin, transporting cystine out of the lysosomal compartment. Defective cystinosin function leads to intra-lysosomal cystine accumulation in all body cells and organs. The kidneys are initially affected during the first year of life through proximal tubular damage followed by progressive glomerular damage and end stage renal failure during mid-childhood if not treated. Other affected organs include eyes, thyroid, pancreas, gonads, muscles and CNS. Leucocyte cystine assay is the cornerstone for both diagnosis and therapeutic monitoring of the disease. Several lines of treatment are available for cystinosis including the cystine depleting agent cysteamine, renal replacement therapy, hormonal therapy and others; however, no curative treatment is yet available. In the current review we will discuss the most important clinical features of the disease, advantages and disadvantages of the current diagnostic and therapeutic options and the main topics of future research in cystinosis.

Twitter Demographics

The data shown below were collected from the profiles of 6 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 124 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
United States 1 <1%
Unknown 123 99%

Demographic breakdown

Readers by professional status Count As %
Student > Bachelor 19 15%
Researcher 19 15%
Student > Master 17 14%
Student > Ph. D. Student 15 12%
Other 11 9%
Other 21 17%
Unknown 22 18%
Readers by discipline Count As %
Medicine and Dentistry 48 39%
Biochemistry, Genetics and Molecular Biology 22 18%
Agricultural and Biological Sciences 14 11%
Pharmacology, Toxicology and Pharmaceutical Science 11 9%
Immunology and Microbiology 2 2%
Other 5 4%
Unknown 22 18%

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 16 July 2016.
All research outputs
#3,902,951
of 13,626,890 outputs
Outputs from Orphanet Journal of Rare Diseases
#553
of 1,497 outputs
Outputs of similar age
#81,275
of 260,529 outputs
Outputs of similar age from Orphanet Journal of Rare Diseases
#16
of 34 outputs
Altmetric has tracked 13,626,890 research outputs across all sources so far. This one has received more attention than most of these and is in the 71st percentile.
So far Altmetric has tracked 1,497 research outputs from this source. They typically receive a little more attention than average, with a mean Attention Score of 7.1. This one has gotten more attention than average, scoring higher than 62% 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 260,529 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 68% of its contemporaries.
We're also able to compare this research output to 34 others from the same source and published within six weeks on either side of this one. This one has gotten more attention than average, scoring higher than 52% of its contemporaries.