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Prevalence, incidence, and age at diagnosis in Marfan Syndrome

Overview of attention for article published in Orphanet Journal of Rare Diseases, December 2015
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  • Above-average Attention Score compared to outputs of the same age (53rd percentile)
  • Average Attention Score compared to outputs of the same age and source

Mentioned by

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3 tweeters

Citations

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103 Dimensions

Readers on

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156 Mendeley
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Title
Prevalence, incidence, and age at diagnosis in Marfan Syndrome
Published in
Orphanet Journal of Rare Diseases, December 2015
DOI 10.1186/s13023-015-0369-8
Pubmed ID
Authors

Kristian A. Groth, Hanne Hove, Kasper Kyhl, Lars Folkestad, Mette Gaustadnes, Niels Vejlstrup, Kirstine Stochholm, John R. Østergaard, Niels H. Andersen, Claus H. Gravholt

Abstract

Marfan syndrome is a genetic disorder with considerable morbidity and mortality. Presently, clinicians use the 2010 revised Ghent nosology, which includes optional genetic sequencing of the FBN1 gene, to diagnose patients. So far, only a few studies based on older diagnostic criteria have reported a wide range of prevalence and incidence. Our aim was to study prevalence, incidence, and age at diagnosis in patients with Marfan syndrome. Using unique Danish patient-registries, we identified all possible Marfan syndrome patients recorded by the Danish healthcare system (1977-2014). Following, we confirmed or rejected the diagnosis according to the 2010 revised Ghent nosology. We identified a total of 1628 persons with possible Marfan syndrome. We confirmed the diagnosis in 412, whereof 46 were deceased, yielding a maximum prevalence of 6.5/100,000 at the end of 2014. The annual median incidence was 0.19/100,000 (range: 0.0-0.7) which increased significantly with an incidence rate ratio of 1.03 (95 % CI: 1.02-1.04, p < 0.001). We found a median age at diagnose of 19.0 years (range: 0.0-74). The age at diagnosis increased during the study period, uninfluenced by the changes in diagnostic criteria. We found no gender differences. The increasing prevalence of Marfan syndrome during the study period is possibly due to build-up of a registry. Since early diagnosis is essential in preventing aortic events, diagnosing Marfan syndrome remains a task for both pediatricians and physicians caring for adults.

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 156 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Denmark 1 <1%
Unknown 155 99%

Demographic breakdown

Readers by professional status Count As %
Student > Bachelor 34 22%
Student > Master 19 12%
Researcher 14 9%
Student > Ph. D. Student 14 9%
Other 13 8%
Other 24 15%
Unknown 38 24%
Readers by discipline Count As %
Medicine and Dentistry 51 33%
Biochemistry, Genetics and Molecular Biology 23 15%
Nursing and Health Professions 7 4%
Agricultural and Biological Sciences 6 4%
Psychology 5 3%
Other 16 10%
Unknown 48 31%

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 31 December 2015.
All research outputs
#5,303,073
of 10,134,140 outputs
Outputs from Orphanet Journal of Rare Diseases
#719
of 1,222 outputs
Outputs of similar age
#133,467
of 309,918 outputs
Outputs of similar age from Orphanet Journal of Rare Diseases
#25
of 36 outputs
Altmetric has tracked 10,134,140 research outputs across all sources so far. This one is in the 45th percentile – i.e., 45% of other outputs scored the same or lower than it.
So far Altmetric has tracked 1,222 research outputs from this source. They typically receive a little more attention than average, with a mean Attention Score of 6.8. This one is in the 37th percentile – i.e., 37% 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 309,918 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 53% of its contemporaries.
We're also able to compare this research output to 36 others from the same source and published within six weeks on either side of this one. This one is in the 30th percentile – i.e., 30% of its contemporaries scored the same or lower than it.