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Life expectancy of parents with Hereditary Haemorrhagic Telangiectasia

Overview of attention for article published in Orphanet Journal of Rare Diseases, April 2016
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Title
Life expectancy of parents with Hereditary Haemorrhagic Telangiectasia
Published in
Orphanet Journal of Rare Diseases, April 2016
DOI 10.1186/s13023-016-0427-x
Pubmed ID
Authors

E. M. de Gussem, C. P. Edwards, A. E. Hosman, C. J. J. Westermann, R. J. Snijder, M. E. Faughnan, J. J. Mager

Abstract

Hereditary Haemorrhagic Telangiectasia (HHT) is an autosomal dominant disease associated with epistaxis, arteriovenous malformations and telangiectasias. Disease complications may result in premature death. We investigated life-expectancies of parents of HHT patients compared with their non-HHT partners using self- or telephone-administered questionnaires sent to their children. Patients were extracted from the databases of 2 participating HHT Centres: the Toronto HHT Database (Toronto, Canada) and the St. Antonius Hospital HHT Database (Nieuwegein, The Netherlands). Two hundred twenty five/407 (55 %) of respondents were included creating HHT- (n = 225) and control groups (n = 225) of equal size. Two hundred thirteen/225 (95 %) of the HHT group had not been screened for organ involvement of the disease prior to death. The life expectancy in parents with HHT was slightly lower compared to parents without (median age at death 73.3 years in patients versus 76.6 years in controls, p0.018). Parents with ACVRL 1 mutations had normal life expectancies, whereas parents with Endoglin mutations died 7.1 years earlier than controls (p = 0.024). Women with Endoglin mutations lived a median of 9.3 years shorter than those without (p = 0.04). Seven/123 (5 %) of deaths were HHT related with a median age at death of 61.5 years (IQ range 54.4-67.7 years). Our study showed that the life expectancy of largely unscreened HHT patients was lower than people without HHT. Female patients with Endoglin mutations were most strikingly at risk of premature death from complications. These results emphasize the importance of referring patients with HHT for screening of organ involvement and timely intervention to prevent complications.

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

Geographical breakdown

Country Count As %
Netherlands 1 3%
Unknown 29 97%

Demographic breakdown

Readers by professional status Count As %
Student > Master 7 23%
Student > Ph. D. Student 5 17%
Other 3 10%
Student > Doctoral Student 3 10%
Student > Bachelor 2 7%
Other 8 27%
Unknown 2 7%
Readers by discipline Count As %
Medicine and Dentistry 17 57%
Biochemistry, Genetics and Molecular Biology 6 20%
Agricultural and Biological Sciences 1 3%
Unspecified 1 3%
Computer Science 1 3%
Other 2 7%
Unknown 2 7%

Attention Score in Context

This research output has an Altmetric Attention Score of 1. 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 02 May 2016.
All research outputs
#3,952,024
of 7,639,268 outputs
Outputs from Orphanet Journal of Rare Diseases
#722
of 1,072 outputs
Outputs of similar age
#145,019
of 267,481 outputs
Outputs of similar age from Orphanet Journal of Rare Diseases
#45
of 55 outputs
Altmetric has tracked 7,639,268 research outputs across all sources so far. This one is in the 28th percentile – i.e., 28% of other outputs scored the same or lower than it.
So far Altmetric has tracked 1,072 research outputs from this source. They receive a mean Attention Score of 4.8. This one is in the 25th percentile – i.e., 25% of its peers scored the same or lower than it.
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We're also able to compare this research output to 55 others from the same source and published within six weeks on either side of this one. This one is in the 5th percentile – i.e., 5% of its contemporaries scored the same or lower than it.