↓ Skip to main content

Atypical hemolytic uremic syndrome

Overview of attention for article published in Orphanet Journal of Rare Diseases, January 2011
Altmetric Badge

About this Attention Score

  • In the top 25% of all research outputs scored by Altmetric
  • High Attention Score compared to outputs of the same age (82nd percentile)
  • High Attention Score compared to outputs of the same age and source (99th percentile)

Mentioned by

twitter
6 tweeters
facebook
2 Facebook pages
wikipedia
2 Wikipedia pages

Citations

dimensions_citation
478 Dimensions

Readers on

mendeley
335 Mendeley
You are seeing a free-to-access but limited selection of the activity Altmetric has collected about this research output. Click here to find out more.
Title
Atypical hemolytic uremic syndrome
Published in
Orphanet Journal of Rare Diseases, January 2011
DOI 10.1186/1750-1172-6-60
Pubmed ID
Authors

Chantal Loirat, Véronique Frémeaux-Bacchi

Abstract

Hemolytic uremic syndrome (HUS) is defined by the triad of mechanical hemolytic anemia, thrombocytopenia and renal impairment. Atypical HUS (aHUS) defines non Shiga-toxin-HUS and even if some authors include secondary aHUS due to Streptococcus pneumoniae or other causes, aHUS designates a primary disease due to a disorder in complement alternative pathway regulation. Atypical HUS represents 5 -10% of HUS in children, but the majority of HUS in adults. The incidence of complement-aHUS is not known precisely. However, more than 1000 aHUS patients investigated for complement abnormalities have been reported. Onset is from the neonatal period to the adult age. Most patients present with hemolytic anemia, thrombocytopenia and renal failure and 20% have extra renal manifestations. Two to 10% die and one third progress to end-stage renal failure at first episode. Half of patients have relapses. Mutations in the genes encoding complement regulatory proteins factor H, membrane cofactor protein (MCP), factor I or thrombomodulin have been demonstrated in 20-30%, 5-15%, 4-10% and 3-5% of patients respectively, and mutations in the genes of C3 convertase proteins, C3 and factor B, in 2-10% and 1-4%. In addition, 6-10% of patients have anti-factor H antibodies. Diagnosis of aHUS relies on 1) No associated disease 2) No criteria for Shigatoxin-HUS (stool culture and PCR for Shiga-toxins; serology for anti-lipopolysaccharides antibodies) 3) No criteria for thrombotic thrombocytopenic purpura (serum ADAMTS 13 activity > 10%). Investigation of the complement system is required (C3, C4, factor H and factor I plasma concentration, MCP expression on leukocytes and anti-factor H antibodies; genetic screening to identify risk factors). The disease is familial in approximately 20% of pedigrees, with an autosomal recessive or dominant mode of transmission. As penetrance of the disease is 50%, genetic counseling is difficult. Plasmatherapy has been first line treatment until presently, without unquestionable demonstration of efficiency. There is a high risk of post-transplant recurrence, except in MCP-HUS. Case reports and two phase II trials show an impressive efficacy of the complement C5 blocker eculizumab, suggesting it will be the next standard of care. Except for patients treated by intensive plasmatherapy or eculizumab, the worst prognosis is in factor H-HUS, as mortality can reach 20% and 50% of survivors do not recover renal function. Half of factor I-HUS progress to end-stage renal failure. Conversely, most patients with MCP-HUS have preserved renal function. Anti-factor H antibodies-HUS has favourable outcome if treated early.

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

Geographical breakdown

Country Count As %
Canada 3 <1%
Colombia 1 <1%
Malaysia 1 <1%
France 1 <1%
Hong Kong 1 <1%
Germany 1 <1%
India 1 <1%
Sweden 1 <1%
Spain 1 <1%
Other 1 <1%
Unknown 323 96%

Demographic breakdown

Readers by professional status Count As %
Student > Master 52 16%
Researcher 44 13%
Student > Postgraduate 38 11%
Student > Bachelor 36 11%
Student > Ph. D. Student 30 9%
Other 93 28%
Unknown 42 13%
Readers by discipline Count As %
Medicine and Dentistry 175 52%
Agricultural and Biological Sciences 40 12%
Pharmacology, Toxicology and Pharmaceutical Science 18 5%
Biochemistry, Genetics and Molecular Biology 15 4%
Nursing and Health Professions 11 3%
Other 29 9%
Unknown 47 14%

Attention Score in Context

This research output has an Altmetric Attention Score of 8. 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 23 May 2019.
All research outputs
#3,626,962
of 20,688,188 outputs
Outputs from Orphanet Journal of Rare Diseases
#466
of 2,317 outputs
Outputs of similar age
#23,511
of 138,169 outputs
Outputs of similar age from Orphanet Journal of Rare Diseases
#1
of 5 outputs
Altmetric has tracked 20,688,188 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 2,317 research outputs from this source. They typically receive a little more attention than average, with a mean Attention Score of 7.3. This one has done well, scoring higher than 79% 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 138,169 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 82% of its contemporaries.
We're also able to compare this research output to 5 others from the same source and published within six weeks on either side of this one. This one has scored higher than all of them