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Bernard-Soulier syndrome (Hemorrhagiparous thrombocytic dystrophy)

Overview of attention for article published in Orphanet Journal of Rare Diseases, November 2006
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About this Attention Score

  • In the top 25% of all research outputs scored by Altmetric
  • Good Attention Score compared to outputs of the same age (79th percentile)
  • Good Attention Score compared to outputs of the same age and source (75th percentile)

Mentioned by

twitter
1 tweeter
facebook
2 Facebook pages
wikipedia
3 Wikipedia pages

Citations

dimensions_citation
100 Dimensions

Readers on

mendeley
94 Mendeley
citeulike
1 CiteULike
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Title
Bernard-Soulier syndrome (Hemorrhagiparous thrombocytic dystrophy)
Published in
Orphanet Journal of Rare Diseases, November 2006
DOI 10.1186/1750-1172-1-46
Pubmed ID
Authors

François Lanza

Abstract

Bernard-Soulier syndrome (BSS), also known as Hemorrhagiparous thrombocytic dystrophy, is a hereditary bleeding disorder affecting the megakaryocyte/platelet lineage and characterized by bleeding tendency, giant blood platelets and low platelet counts. This syndrome is extremely rare as only approximately 100 cases have been reported in the literature. Clinical manifestations usually include purpura, epistaxis, menorrhagia, gingival and gastrointestinal bleeding. The syndrome is transmitted as an autosomal recessive trait. The underlying defect is a deficiency or dysfunction of the glycoprotein GPIb-V-IX complex, a platelet-restricted multisubunit receptor required for normal primary hemostasis. The GPIb-V-IX complex binds von Willebrand factor, allowing platelet adhesion and platelet plug formation at sites of vascular injury. Genes coding for the four subunits of the receptor, GPIBA, GPIBB, GP5 and GP9, map to chromosomes 17p12, 22q11.2, 3q29, and 3q21, respectively. Defects have been identified in GPIBA, GPIBB, and GP9 but not in GP5. Diagnosis is based on a prolonged skin bleeding time, the presence of a small number of very large platelets (macrothrombocytopenia), defective ristocetin-induced platelet agglutination and low or absent expression of the GPIb-V-IX complex. Prothrombin consumption is markedly reduced. The prognosis is usually good with adequate supportive care but severe bleeding episodes can occur with menses, trauma and surgical procedures. Treatment of bleeding or prophylaxis during surgical procedures usually requires platelet transfusion.

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

Geographical breakdown

Country Count As %
United Kingdom 2 2%
India 1 1%
Morocco 1 1%
France 1 1%
Belgium 1 1%
Unknown 88 94%

Demographic breakdown

Readers by professional status Count As %
Student > Bachelor 14 15%
Student > Master 13 14%
Other 11 12%
Student > Ph. D. Student 10 11%
Student > Postgraduate 8 9%
Other 21 22%
Unknown 17 18%
Readers by discipline Count As %
Medicine and Dentistry 31 33%
Biochemistry, Genetics and Molecular Biology 17 18%
Agricultural and Biological Sciences 11 12%
Pharmacology, Toxicology and Pharmaceutical Science 5 5%
Engineering 2 2%
Other 10 11%
Unknown 18 19%

Attention Score in Context

This research output has an Altmetric Attention Score of 5. 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 26 July 2013.
All research outputs
#644,132
of 3,626,596 outputs
Outputs from Orphanet Journal of Rare Diseases
#143
of 665 outputs
Outputs of similar age
#55,617
of 275,719 outputs
Outputs of similar age from Orphanet Journal of Rare Diseases
#11
of 44 outputs
Altmetric has tracked 3,626,596 research outputs across all sources so far. Compared to these this one has done well and is in the 82nd percentile: it's in the top 25% of all research outputs ever tracked by Altmetric.
So far Altmetric has tracked 665 research outputs from this source. They receive a mean Attention Score of 3.7. This one has done well, scoring higher than 78% 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 275,719 tracked outputs that were published within six weeks on either side of this one in any source. This one has done well, scoring higher than 79% of its contemporaries.
We're also able to compare this research output to 44 others from the same source and published within six weeks on either side of this one. This one has done well, scoring higher than 75% of its contemporaries.