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FAbry STabilization indEX (FASTEX): an innovative tool for the assessment of clinical stabilization in Fabry disease

Overview of attention for article published in Clinical Kidney Journal, September 2016
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Title
FAbry STabilization indEX (FASTEX): an innovative tool for the assessment of clinical stabilization in Fabry disease
Published in
Clinical Kidney Journal, September 2016
DOI 10.1093/ckj/sfw082
Pubmed ID
Authors

Renzo Mignani, Federico Pieruzzi, Francesco Berri, Alessandro Burlina, Benito Chinea, Maurizio Gallieni, Maurizio Pieroni, Alessandro Salviati, Marco Spada

Abstract

Two disease severity scoring systems, the Mainz Severity Score Index (MSSI) and Fabry Disease Severity Scoring System (DS3), have been validated for quantifying the disease burden of Fabry disease. We aimed to develop a dynamic mathematical model [the FASTEX (FAbry STabilization indEX)] to assess the clinical stability. A multidisciplinary panel of experts in Fabry disease first defined a novel score of severity [raw score (RS)] based on three domains with a small number items in each domain (nervous system domain: pain, cerebrovascular events; renal domain: proteinuria, glomerular filtration rate; cardiac domain: echocardiography parameters, electrocardiograph parameters and New York Heart Association class) and evaluated the clinical stability over time. The RS was tested in 28 patients (15 males, 13 females) with the classic form of Fabry disease. There was good statistical correlation between the newly established RS and a weighted score (WS), with DS3 and MSSI (R (2) = 0.914, 0.949, 0.910 and 0.938, respectively). In order to refine the RS further, a WS, which was expressed as a percentage value, was calculated. This was based on the relative clinical significance of each item within the domain with the panel agreeing on the attribution of a different weight of clinical damage to a specific organ system. To test the variation of the clinical burden over time, the RS was repeated after 1 year. The panel agreed on a cut-off of a 20% change from baseline as the clinical WS to define clinical stability. The FASTEX model showed good correlation with the clinical assessment and with clinical variation over time in all patients.

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Mendeley readers

Mendeley readers

The data shown below were compiled from readership statistics for 36 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 36 100%

Demographic breakdown

Readers by professional status Count As %
Other 9 25%
Professor > Associate Professor 6 17%
Student > Ph. D. Student 4 11%
Researcher 4 11%
Student > Bachelor 3 8%
Other 5 14%
Unknown 5 14%
Readers by discipline Count As %
Medicine and Dentistry 20 56%
Environmental Science 1 3%
Biochemistry, Genetics and Molecular Biology 1 3%
Nursing and Health Professions 1 3%
Business, Management and Accounting 1 3%
Other 2 6%
Unknown 10 28%
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 16 November 2016.
All research outputs
#13,988,427
of 22,886,568 outputs
Outputs from Clinical Kidney Journal
#1,316
of 1,729 outputs
Outputs of similar age
#184,038
of 330,061 outputs
Outputs of similar age from Clinical Kidney Journal
#25
of 33 outputs
Altmetric has tracked 22,886,568 research outputs across all sources so far. This one is in the 37th percentile – i.e., 37% of other outputs scored the same or lower than it.
So far Altmetric has tracked 1,729 research outputs from this source. They typically receive a lot more attention than average, with a mean Attention Score of 11.0. This one is in the 22nd percentile – i.e., 22% 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 330,061 tracked outputs that were published within six weeks on either side of this one in any source. This one is in the 42nd percentile – i.e., 42% of its contemporaries scored the same or lower than it.
We're also able to compare this research output to 33 others from the same source and published within six weeks on either side of this one. This one is in the 21st percentile – i.e., 21% of its contemporaries scored the same or lower than it.